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Sample records for aortic root dilatation

  1. Valve-sparing aortic root replacement and aortic valve repair in a patient with acromegaly and aortic root dilatation

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    Karel Van Praet

    2015-07-01

    Full Text Available Aortic regurgitation and dilatation of the aortic root and ascending aorta are severe complications of acromegaly. The current trend for management of an aortic root aneurysm is valve-sparing root replacement as well as restoring the diameter of the aortic sinotubular junction (STJ and annulus. Our case report supports the recommendation that in patients with acromegaly, severe aortic root involvement may indicate the need for surgery.

  2. Dilatation and Dysfunction of the Neo-aortic Root and in 76 Patients After the Ross Procedure.

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    Zimmermann, Corina A; Weber, Roland; Greutmann, Matthias; Dave, Hitendu; Müller, Christoph; Prêtre, René; Seifert, Burkhardt; Buechel, Emanuela Valsangiacomo; Kretschmar, Oliver; Attenhofer Jost, Christine H

    2016-08-01

    Pulmonary autograft replacement (Ross procedure) is used as an alternative to prosthetic aortic valve replacement patients with aortic valve disease. There are limited data on incidence and risk factors for dilatation and dysfunction of the neo-aortic after the Ross procedure. Ross procedure was performed in 100 patients at our institution between 1993 and 2011. In 76 patients, complete follow-up data were available. Their median age at surgery was 16 (0.4-58) years (76 % males; 95 % with congenital aortic valve disease). Median follow-up duration was 5.2 years (0.3-16.0 years). We analyzed their clinical and echocardiographic follow-up to identify possible risk factors for neo-aortic root dilatation and dysfunction. Ross procedure included reduction plasty of the native ascending aorta in 25 % of patients. During follow-up, 21 patients (28 %) developed neo-aortic root dilatation, 38 patients (50 %) dilatation oft the native ascending aorta and 7 patients (9 %) at least moderate neo-aortic regurgitation. Univariate risk factors for neo-aortic root dilatation were preoperative aortic regurgitation (p = 0.04), concomitant reduction plasty of the ascending aorta (p = 0.009) and a longer duration of follow-up (p = 0.005). Younger age at surgery was associated with dilatation of the ascending aorta (p = 0.03). Reoperation on the neo-aortic root because of severe dilatation was necessary in 6 patients (8 %), where 2 patients had at least moderate neo-aortic root regurgitation. Neo-aortic root and aortic dilatation are common after the Ross procedure. This is often combined with neo-aortic valve dysfunction. Close follow-up of these patients is mandatory.

  3. Tetralogy of Fallot and aortic root dilation: a long-term outlook.

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    Nagy, Christian D; Alejo, Diane E; Corretti, Mary C; Ravekes, William J; Crosson, Jane E; Spevak, Philip J; Ringel, Richard; Carson, Kathryn A; Khalil, Sara; Dietz, Harry C; Cameron, Duke E; Vricella, Luca A; Traill, Thomas A; Holmes, Kathryn W

    2013-04-01

    Dilation of the sinus of Valsalva (SoV) has been increasingly observed after repaired tetralogy of Fallot (TOF). We estimate the prevalence of SoV dilation in adults with repaired TOF and analyze possible factors related to aortic disease. Adults with TOF [n = 109, median age 33.2 years (range 18.1 to 69.5)] evaluated at Johns Hopkins Hospital from 2001 to 2009 were reviewed in an observational retrospective cohort study. Median follow-up was 27.3 (range 0.1-48.8) years. SoV dilation was defined as >95 % confidence interval adjusted for age and body surface area (z-score > 2). The prevalence of SoV dilation was 51 % compared with that of a normal population with a mean z-score of 2.03. Maximal aortic diameters were ≥ 4 cm in 39 % (42 of 109), ≥ 4.5 cm in 21 % (23 of 109), ≥ 5 cm in 8 % (9 of 109), and ≥ 5.5 cm in 2 % (2 of 109). There was no aortic dissection or death due contributable to aortic disease. Aortic valve replacement was performed in 1.8 % and aortic root or ascending aorta (AA) replacement surgery in 2.8 % of patients. By multivariate logistic regression analysis, aortic regurgitation (AR) [odds ratio (OR) = 3.09, p = 0.005], residual ventricular septal defect (VSD) (OR = 4.14, p < 0.02), and TOF with pulmonary atresia (TOF/PA) (OR = 6.75, p = 0.03) were associated with increased odds of dilated aortic root. SoV dilation after TOF repair is common and persists with aging. AR, residual VSD, and TOF/PA are associated with increased odds of dilation. AA evaluation beyond the SoV is important. Indexed values are imperative to avoid bias on the basis of age and body surface area.

  4. Echocardiographic assessment of the aortic root dilatation in adult patients after tetralogy of Fallot repair.

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    Cruz, Cristina; Pinho, Teresa; Lebreiro, Ana; Silva Cardoso, José; Maciel, Maria Júlia

    2013-06-01

    Transthoracic echocardiography is an important tool after tetralogy of Fallot repair, of which aortic root dilatation is a recognized complication. In this study we aimed to assess its prevalence and potential predictors. We consecutively assessed adult patients by transthoracic echocardiography after tetralogy of Fallot repair, and divided them into two groups based on the maximum internal aortic diameter at the sinuses of Valsalva in parasternal long-axis view: group 1 with aortic root dilatation (≥38 mm) and group 2 without dilatation (de Cardiologia. Published by Elsevier España. All rights reserved.

  5. Segmental Aortic Stiffness in Children and Young Adults With Connective Tissue Disorders: Relationships With Age, Aortic Size, Rate of Dilation, and Surgical Root Replacement.

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    Prakash, Ashwin; Adlakha, Himanshu; Rabideau, Nicole; Hass, Cara J; Morris, Shaine A; Geva, Tal; Gauvreau, Kimberlee; Singh, Michael N; Lacro, Ronald V

    2015-08-18

    Aortic diameter is an imperfect predictor of aortic complications in connective tissue disorders (CTDs). Novel indicators of vascular phenotype severity such as aortic stiffness and vertebral tortuosity index have been proposed. We assessed the relation between aortic stiffness by cardiac MRI, surgical root replacement, and rates of aortic root dilation in children and young adults with CTDs. Retrospective analysis of cardiac MRI data on children and young adults with a CTD was performed to derive aortic stiffness measures (strain, distensibility, and β-stiffness index) at the aortic root, ascending aorta, and descending aorta. Vertebral tortuosity index was calculated as previously described. Rate of aortic root dilation before cardiac MRI was calculated as change in echocardiographic aortic root diameter z score per year. In 83 CTD patients (median age, 24 years; range, 1-55; 17% age; 60% male), ascending aorta distensibility was reduced in comparison with published normative values: median z score, -1.93 (range, -8.7 to 1.3; Pyoung adults with CTDs. © 2015 American Heart Association, Inc.

  6. [Aortic root dilatation rate in pediatric patients with Marfan syndrome treated with losartan].

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    Mariucci, Elisabetta; Guidarini, Marta; Donti, Andrea; Lovato, Luigi; Wischmeijer, Anita; Angeli, Emanuela; Gargiulo, Gaetano D; Picchio, Fernando M; Bonvicini, Marco

    2015-12-01

    Medical therapy with angiotensin II receptor blockers/angiotensin-converting enzyme inhibitors and/or beta-blockers was reported to reduce aortic root dilatation rates in pediatric patients with Marfan syndrome. No data are available in the literature on losartan effects after 3 years of therapy. The aim of our study was to establish whether losartan reduces aortic root dilatation rates in pediatric patients with Marfan syndrome in the mid and long term. This is a retrospective analysis of 38 pediatric patients with Marfan syndrome followed at the Marfan Clinic of S. Orsola-Malpighi Hospital of the University of Bologna (Italy). Aortic diameters were measured at sinuses of Valsalva and proximal ascending aorta with transthoracic echocardiography. After a mean follow-up of 4.5 ± 2.5 years (range 2-9 years), aortic root z score at sinuses of Valsalva and proximal ascending aorta remained stable. The average annual rate of change in aortic root z score was -0.1 ± 0.4 and 0 ± 0.3 at sinuses of Valsalva and proximal ascending aorta, respectively. The mean dose of losartan was 0.7 ± 0.3 mg/kg/day. Three patients were non-responders, probably because of late beginning or low dose of therapy. Eight patients underwent cardiac surgery (aortic root surgery in 5 and mitral valve repair in 3), all of them started losartan later in life. Despite the retrospective design of the study and the small sample size, a beneficial effect of losartan therapy was observed in pediatric patients with Marfan syndrome in the mid and long term. Late beginning or low doses of losartan can turn off the effects of therapy.

  7. Aortopathy in Congenital Heart Disease in Adults: Aortic Dilatation with Decreased Aortic Elasticity that Impacts Negatively on Left Ventricular Function.

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    Niwa, Koichiro

    2013-04-01

    Bicuspid aortic valve and/or coarctation of the aorta are consistently associated with ascending aortic and para-coarctation medial abnormalities. Medial abnormalities in the ascending aorta are prevalent in other types of patients with a variety of forms congenital heart disease (CHD), such as single ventricle, persistent truncus arteriosus, transposition of the great arteries, hypoplastic left heart syndrome, tetralogy of Fallot. These abnormalities encompass a wide age range, and may predispose to dilatation, aneurysm, and rupture that necessitates aortic valve and root surgery. This dilatation can develop in CHD patients without stenotic region. These CHDs exhibit ongoing dilatation of the aortic root and reduced aortic elasticity and increased aortic stiffness that may relate to intrinsic properties of the aortic root. The concept of aortic dilatation is shifting a paradigm of aortic dilatation, as so called post stenotic dilatation, to primary intrinsic aortopahy. These aortic dilatation and increased stiffness can induce aortic aneurysm, rupture of the aorta and aortic regurgitation, but also provoke left ventricular hypertrophy, reduced coronary artery flow and left ventricular failure. We can recognize this association of aortic pathophysiological abnormality, aortic dilation and aorto-left ventricular interaction as a new clinical entity: "aortopathy".

  8. The bicuspid aortic valve and its relation to aortic dilation

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    Shi-Min Yuan

    2010-01-01

    Full Text Available BACKGROUND: A bicuspid aortic valve (BAV is a common congenital heart disease, which affects 1-2% of the population. However, the relationship between BAVs and aortic dilation has not been sufficiently elucidated. METHODS: A total of 241 BAV patients who were referred to this hospital for cardiac surgey over a 4.75-year period were included in this study. In addition to the clinical characteristics of the included patients, the morphological features of the aortic valve and aorta, the length of the left main coronary artery, and the laboratory findings (the coagulation and hematological parameters as well as the total cholesterol concentration were determined and compared with those of the tricuspid aortic valve (TAV patients. RESULTS: The BAV patients were younger than the TAV patients for a valve surgery in the last 3 months of the study period. The BAV patients were predominantly male. Most of the BAVs that were surgically treated were stenotic, regurgitant, or combined, and only 19 (7.88% were normally functioning valves. According to echocardiography or operative records, 148 (78.31% were type A, 31 (16.40% were type B, and 10 (5.29% were type C. The left main coronary artery was much shorter in the BAV patients than it was in the TAV patients. There was no significant difference between BAV and TAV patients in the total cholesterol concentrations; whereas differences were noted between patients receiving lipid-lowering therapy and those not receiving lipid-lowering therapy. The dimensions of the aortic root, sinotubular junction, and ascending aorta were beyond normal limits, while they were significantly smaller in the BAV patients than in the TAV patients. They were also much smaller in patients receiving statin therapy than those not receiving statin therapy in both groups. Moreover, the aortic dilation in the BAV group was found to be significantly associated with patient age. CONCLUSIONS: The BAV patients developed aortic wall and

  9. Does altered aortic flow in marfan syndrome relate to aortic root dilatation?

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    Wang, Hung-Hsuan; Chiu, Hsin-Hui; Tseng, Wen-Yih Isaac; Peng, Hsu-Hsia

    2016-08-01

    To examine possible hemodynamic alterations in adolescent to adult Marfan syndrome (MFS) patients with aortic root dilatation. Four-dimensional flow MRI was performed in 20 MFS patients and 12 age-matched normal subjects with a 3T system. The cross-sectional areas of 10 planes along the aorta were segmented for calculating the axial and circumferential wall shear stress (WSSaxial , WSScirc ), oscillatory shear index (OSIaxial , OSIcirc ), and the nonroundness (NR), presenting the asymmetry of segmental WSS. Pearson's correlation analysis was performed to present the correlations between the quantified indices and the body surface area (BSA), aortic root diameter (ARD), and Z score of the ARD. P < 0.05 indicated statistical significance. Patients exhibited lower WSSaxial in the aortic root and the WSScirc in the arch (P < 0.05-0.001). MFS patients exhibited higher OSIaxial and OSIcirc in the sinotubular junction and arch, but lower OSIcirc in the descending aorta (all P < 0.05). The NR values were lower in patients (P < 0.05). The WSSaxial or WSScirc exhibited moderate to strong correlations with BSA, ARD, or Z score (R(2)  = 0.50-0.72) in MFS patients. The significant differences in the quantified indices, which were associated with BSA, ARD, or Z score, in MFS were opposite to previous reports for younger MFS patients, indicating that altered flows in MFS patients may depend on the disease progress. The possible time dependency of hemodynamic alterations in MFS patients strongly suggests that longitudinal follow-up of 4D Flow is needed to comprehend disease progress. J. Magn. Reson. Imaging 2016;44:500-508. © 2016 Wiley Periodicals, Inc.

  10. Impairment of flow-mediated dilation correlates with aortic dilation in patients with Marfan syndrome.

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    Takata, Munenori; Amiya, Eisuke; Watanabe, Masafumi; Omori, Kazuko; Imai, Yasushi; Fujita, Daishi; Nishimura, Hiroshi; Kato, Masayoshi; Morota, Tetsuro; Nawata, Kan; Ozeki, Atsuko; Watanabe, Aya; Kawarasaki, Shuichi; Hosoya, Yumiko; Nakao, Tomoko; Maemura, Koji; Nagai, Ryozo; Hirata, Yasunobu; Komuro, Issei

    2014-07-01

    Marfan syndrome is an inherited disorder characterized by genetic abnormality of microfibrillar connective tissue proteins. Endothelial dysfunction is thought to cause aortic dilation in subjects with a bicuspid aortic valve; however, the role of endothelial dysfunction and endothelial damaging factors has not been elucidated in Marfan syndrome. Flow-mediated dilation, a noninvasive measurement of endothelial function, was evaluated in 39 patients with Marfan syndrome. Aortic diameter was measured at the aortic annulus, aortic root at the sinus of Valsalva, sinotubular junction and ascending aorta by echocardiography, and adjusted for body surface area (BSA). The mean value of flow-mediated dilation was 6.5 ± 2.4 %. Flow-mediated dilation had a negative correlation with the diameter of the ascending thoracic aorta (AscAd)/BSA (R = -0.39, p = 0.020) and multivariate analysis revealed that flow-mediated dilation was an independent factor predicting AscAd/BSA, whereas other segments of the aorta had no association. Furthermore, Brinkman index had a somewhat greater influence on flow-mediated dilation (R = -0.42, p = 0.008). Although subjects who smoked tended to have a larger AscAd compared with non-smokers (AscA/BSA: 17.3 ± 1.8 versus 15.2 ± 3.0 mm/m(2), p = 0.013), there was no significant change in flow-mediated dilation, suggesting that smoking might affect aortic dilation via an independent pathway. Common atherogenic risks, such as impairment of flow-mediated dilation and smoking status, affected aortic dilation in subjects with Marfan syndrome.

  11. No beneficial effect of general and specific anti-inflammatory therapies on aortic dilatation in Marfan mice.

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    Romy Franken

    Full Text Available AIMS: Patients with Marfan syndrome have an increased risk of life-threatening aortic complications, mostly preceded by aortic dilatation. In the FBN1(C1039G/+ Marfan mouse model, losartan decreases aortic root dilatation. We recently confirmed this beneficial effect of losartan in adult patients with Marfan syndrome. The straightforward translation of this mouse model to man is reassuring to test novel treatment strategies. A number of studies have shown signs of inflammation in aortic tissue of Marfan patients. This study examined the efficacy of anti-inflammatory therapies in attenuating aortic root dilation in Marfan syndrome and compared effects to the main preventative agent, losartan. METHODS AND RESULTS: To inhibit inflammation in FBN1(C1039G/+ Marfan mice, we treated the mice with losartan (angiotensin II receptor type 1 inhibitor, methylprednisolone (corticosteroid or abatacept (T-cell-specific inhibitor. Treatment was initiated in adult Marfan mice with already existing aortic root dilatation, and applied for eight weeks. Methylprednisolone- or abatacept-treated mice did not reveal a reduction in aortic root dilatation. In this short time frame, losartan was the only treatment that significantly reduced aorta inflammation, transforming growth factor-beta (TGF-β signaling and aortic root dilatation rate in these adult Marfan mice. Moreover, the methylprednisolone-treated mice had significantly more aortic alcian blue staining as a marker for aortic damage. CONCLUSION: Anti-inflammatory agents do not reduce the aortic dilatation rate in Marfan mice, but possibly increase aortic damage. Currently, the most promising therapeutic drug in Marfan syndrome is losartan, by blocking the angiotensin II receptor type 1 and thereby inhibiting pSmad2 signaling.

  12. [Valve-sparing Replacement in Patients with Aortic Root Dilatation].

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    Yamazaki, Kazuhiro; Minatoya, Kenji; Ueda, Ryoma; Takehara, Masato; Sakamoto, Kazuhisa; Ide, Yujiro; Kanemitsu, Hideo; Ueyama, Koji; Ikeda, Tadashi

    2018-01-01

    Valve-sparing root replacement is increasingly used to overcome drawbacks associated with valvular prostheses. In our institution, 7 patients underwent valve-sparing root replacement from August 2016 to July 2017. The mean age was 45 years (range, 14~69 years). Three patients had Marfan syndrome and 1 had Loeys-Dietz syndrome with acute aortic dissection. All patients underwent surgery with reimplantation technique using a Valsalva graft. Two patients required repair of aortic valve leaflet prolapse. All patients had an excellent clinical course, with mild or no aortic regurgitation and a decrease in end-diastolic volume on echocardiography. These results support the continued use of valve-sparing root replacement in selected patients.

  13. Preventative Valve-Sparing Aortic Root Replacement and Pregnancy Outcome in Marfan Syndrome

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    Sokol, Vesna; Zlopaša, Gordan; Herman, Mislav; Planinić, Pavao; Micevska, Ana

    2012-01-01

    In Marfan syndrome, with dilatation of the aortic root secondary to an underlying connective tissue defect, pregnancy can cause hemodynamic stress leading to the development of an aortic aneurysm and even a fatal aortic dissection. In the presence of existing aortic root enlargement and a family history of aortic dissection, preventative elective surgery is suggested. Aortic root replacement with or without a valve-sparing procedure is superior to total aortic root replacement with ...

  14. Impact of pregnancy on autograft dilatation and aortic valve function following the Ross procedure.

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    Carvajal, Horacio G; Lindley, Kathryn J; Shah, Trupti; Brar, Anoop K; Barger, Philip M; Billadello, Joseph J; Eghtesady, Pirooz

    2018-03-01

    The effects of pregnancy on autograft dilatation and neoaortic valve function in patients with a Ross procedure have not been studied. We sought to evaluate the effect of pregnancy on autograft dilatation and valve function in these patients with the goal of determining whether pregnancy is safe after the Ross procedure. A retrospective chart review of female patients who underwent a Ross procedure was conducted. Medical records for 51 patients were reviewed. Among the 33 patients who met inclusion criteria, 11 became pregnant after surgery and 22 did not. Echocardiographic reports were used to record aortic root diameter and aortic insufficiency before, during, and after pregnancy. Patient's charts were reviewed for reinterventions and complications. Primary endpoints included reinterventions, aortic root dilation of ≥5 cm, aortic insufficiency degree ≥ moderate, and death. There were 18 pregnancies carried beyond 20 weeks in 11 patients. There was no significant difference in aortic root diameter between nulliparous patients and parous patients prior to their first pregnancy (3.53 ± 0.44 vs 3.57 ± 0.69 cm, P = .74). There was no significant change in aortic root diameter after first pregnancy (3.7 ± 0.4 cm, P = .056) although there was significant dilatation after the second (4.3 ± 0.7 cm, P = .009) and third (4.5 ± 0.7 cm, P = .009) pregnancies. Freedom from combined endpoints was significantly higher for patients in the pregnancy group than those in the nonpregnancy group (P = .002). Pregnancy was not associated with significantly increased adverse events in patients following the Ross procedure. Special care should be taken after the first pregnancy, as multiparity may lead to increased neoaortic dilatation. © 2017 Wiley Periodicals, Inc.

  15. Preventative valve-sparing aortic root replacement and pregnancy outcome in Marfan syndrome.

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    Sokol, Vesna; Zlopasa, Gordan; Herman, Mislav; Planinić, Pavao; Micevska, Ana

    2012-06-01

    In Marfan syndrome, with dilatation of the aortic root secondary to an underlying connective tissue defect, pregnancy can cause hemodynamic stress leading to the development of an aortic aneurysm and even a fatal aortic dissection. In the presence of existing aortic root enlargement and a family history of aortic dissection, preventative elective surgery is suggested. Aortic root replacement with or without a valve-sparing procedure is superior to total aortic root replacement with prosthetic valve/tube graft. It provides excellent survival with low rates of aortic - valve related complications.

  16. Can early aortic root surgery prevent further aortic dissection in Marfan syndrome?

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    Shimizu, Hideyuki; Kasahara, Hirofumi; Nemoto, Atsushi; Yamabe, Kentaro; Ueda, Toshihiko; Yozu, Ryohei

    2011-01-01

    We reviewed 50 patients with Marfan syndrome who underwent surgery for aortic root pathologies comprising a root aneurysm without (n = 25; group A) and with (n = 25; group B) dissection. Aortic root repair included Bentall (n = 37) and valve-sparing (n = 13) procedures. Hospital mortality was 4.0%. Twenty-two patients required 36 repeat surgeries on the distal aorta. The main indication for re-intervention was the dilation of the false lumen. In group A, the distal aorta was stable for up to ...

  17. Bovine aortic arch: A novel association with thoracic aortic dilation

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    Malone, C.D.; Urbania, T.H.; Crook, S.E.S.; Hope, M.D.

    2012-01-01

    Aim: To investigate whether there is a link between bovine arch and thoracic aortic aneurysm. Materials and methods: Computed tomography (CT) and magnetic resonance imaging (MRI) images of the thorax of 191 patients with dilated thoracic aortas and 391 consecutive, unselected patients as controls were retrospectively reviewed. Bovine arch was considered present if either a shared origin of the left common carotid and innominate arteries or an origin of the left common carotid from the innominate artery was identified. A chi-square test was used to evaluate the significance of differences between subgroups. Results: A trend towards increased prevalence of bovine arch was seen in patients with dilated aortas (26.2%) compared to controls (20.5%, p = 0.12). The association was statistically significant in patients over 70 years old (31.9%, p = 0.019) and when dilation involved the aortic arch (47.6%, p = 0.003). Conclusions: An association between bovine arch and aortic dilation is seen in older patients, and when dilation involves the aortic arch. Bovine arch should be considered a potential risk factor for thoracic aortic aneurysm.

  18. Natural history of aortic root dilation through young adulthood in a hypermobile Ehlers-Danlos syndrome cohort.

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    Ritter, Alyssa; Atzinger, Carrie; Hays, Brandon; James, Jeanne; Shikany, Amy; Neilson, Derek; Martin, Lisa; Weaver, Kathryn Nicole

    2017-06-01

    Hypermobile Ehlers-Danlos syndrome (hEDS) is a common inherited connective tissue disorder characterized by joint hypermobility. The natural history of aortic root dilation (AoD), a potential complication of EDS, has not been well characterized in this population. We describe the natural history of aortic root size in a large cohort of patients with hEDS. A cohort of 325 patients with HEDS was identified at Cincinnati Children's Hospital Medical Center (CCHMC), including 163 patients from a previous study. Medical records were reviewed and each participant's height, weight, and aortic dimensions from up to four echocardiograms were documented. Aortic root z-scores were calculated using two established formulas based on age (Boston or Devereux). Overall prevalence of AoD and prevalence by age were calculated and longitudinal regression was performed. The prevalence of AoD with a z-score ≥ 2.0 was 14.2% (46/325) and with a z-score of ≥3.0 was 5.5% (18/325). No significant increases in z-score were seen over time for patients with multiple echocardiograms. Participants under the age of 15 years had an average decline of 0.1 standard deviations (SDs)/year. No significant change was found after 15 of age. Between the ages of 15 and 21 years, Boston z-scores were 0.96 higher than Devereux z-scores. The nearly 1 z-score unit difference between formulas indicates caution prior to diagnosing AoD in patients with hEDS. In light of the low prevalence and lack of progression of AoD, routine echocardiograms may not be warranted for pediatric patients with hEDS. © 2017 Wiley Periodicals, Inc.

  19. Can early aortic root surgery prevent further aortic dissection in Marfan syndrome?

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    Shimizu, Hideyuki; Kasahara, Hirofumi; Nemoto, Atsushi; Yamabe, Kentaro; Ueda, Toshihiko; Yozu, Ryohei

    2012-02-01

    We reviewed 50 patients with Marfan syndrome who underwent surgery for aortic root pathologies comprising a root aneurysm without (n = 25; group A) and with (n = 25; group B) dissection. Aortic root repair included Bentall (n = 37) and valve-sparing (n = 13) procedures. Hospital mortality was 4.0%. Twenty-two patients required 36 repeat surgeries on the distal aorta. The main indication for re-intervention was the dilation of the false lumen. In group A, the distal aorta was stable for up to 7 years, but new dissection developed in 5 (33.3%) of the 15 patients who were followed up for >7 years after the root repair. Actuarial survival including operative mortality was 88.1 and 65.0% at 10 and 20 years, respectively; groups A and B did not significantly differ. Rates of freedom from all-cause death, new dissection or repeated aortic surgery were 60.1, 44.5 and 26.0% at 5, 10 and 15 years, respectively. Group A was significantly better than group B. Prophylactic aortic root repair apparently reduces the likelihood of overall adverse events, but it cannot guarantee the prevention of further aortic dissection. A multidisciplinary approach is needed for patients with Marfan syndrome.

  20. Losartan in combination with propranolol slows the aortic root dilatation in neonatal Marfan syndrome

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    Lu-Hang Liu

    2018-04-01

    Full Text Available Neonatal Marfan syndrome, in contrast to classical Marfan syndrome, is characterized by rapidly progressive multi-valvular cardiac disease and death from congestive heart failure, typically within the first year of life. Due to the rarity of this condition, treatment for neonatal Marfan syndrome has not been well studied. In this report, a combination of losartan and propranolol reduced the aortic root dilatation rate after three months of losartan therapy. Genetic analysis in this patient revealed a mutation in exon 25 of the FBN1 gene, which typically results in a shorter life expectancy. However, the patient's heart failure was controlled by losartan, propranolol and other anti-congestive medications, which may have prolonged his survival. Key Words: FBN1, losartan, neonatal Marfan syndrome

  1. Losartan in combination with propranolol slows the aortic root dilatation in neonatal Marfan syndrome.

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    Liu, Lu-Hang; Lin, Shan-Miao; Lin, Dar-Shong; Chen, Ming-Ren

    2018-04-01

    Neonatal Marfan syndrome, in contrast to classical Marfan syndrome, is characterized by rapidly progressive multi-valvular cardiac disease and death from congestive heart failure, typically within the first year of life. Due to the rarity of this condition, treatment for neonatal Marfan syndrome has not been well studied. In this report, a combination of losartan and propranolol reduced the aortic root dilatation rate after three months of losartan therapy. Genetic analysis in this patient revealed a mutation in exon 25 of the FBN1 gene, which typically results in a shorter life expectancy. However, the patient's heart failure was controlled by losartan, propranolol and other anti-congestive medications, which may have prolonged his survival. Copyright © 2017. Published by Elsevier B.V.

  2. Utility of Genetic Testing in Elite Volleyball Players with Aortic Root Dilation.

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    Herrick, Nicole; Davis, Christopher; Vargas, Lisa; Dietz, Hal; Grossfeld, Paul

    2017-07-01

    Basketball and volleyball attract individuals with a characteristic biophysical profile, mimicking features of Marfan syndrome. Consequently, identification of these abnormalities can be lifesaving. To determine how physical examination, echocardiography, and genetic screening can identify elite volleyball players with a previously undiagnosed aortopathy. We have performed cardiac screening on 90 US Volleyball National Team members and identified four individuals with dilated sinuses of Valsalva. This case series reports on three individuals who underwent a comprehensive genetics evaluation, including gene sequencing. Cardiac screening combined with genetic testing can identify previously undiagnosed tall athletes with an aortopathy, in the absence of noncardiac findings of a connective tissue disorder. Subject 1 had a revised Ghent systems (RGS) score of 2 and a normal aortopathy gene panel. Subject 2 had a RGS score of 1 and genetic testing revealed a de novo disease causing mutation in the gene encoding fibrillin-1 (FBN1). Subject 3 had an RGS score of 4.0 and had a normal aortopathy gene panel. Despite variable clinical features of Marfan syndrome, dilated sinuses of Valsalva were found in 4.9% of the athletes. A disease-causing mutation in the FBN1 gene was identified in subject 2, who had the lowest RGS but the largest aortic root measurement. Subjects 1 and 3, with the highest RGS, had a normal aortopathy gene panel. Our findings provide further evidence suggesting that a cardiac evaluation, including a screening echocardiogram, should be performed on all elite tall adult athletes independent of other physical findings. Genetic testing should be considered for athletes with dilated sinuses of Valsalva (male, >4.2 cm; female, >3.4 cm), regardless of other extracardiac findings.

  3. Shape of the dilated aorta in children with bicuspid aortic valve

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    Mart, Christopher R; McNerny, Bryn E

    2013-01-01

    The dilated aorta in adults with bicuspid aortic valve has been shown to have different shapes, but it is not known if this occurs in children. This observational study was performed to determine if there are different shapes of the dilated aorta in children with bicuspid aortic valve and their association with age, gender, hemodynamic alterations, and degree of aortic enlargement. One hundred and eighty-seven echocardiograms done on pediatric patients (0 – 18 years) for bicuspid aortic valve, during 2008, were reviewed. Aortic valve morphology, shape/size of the aorta, and pertinent hemodynamic alterations were documented. Aortic dilation was felt to be present when at least one aortic segment had a z-score > 2.0; global aortic enlargement was determined by summing the aortic segment z-scores. The aortic shape was assessed by age, gender, valve morphology, and hemodynamic alterations. Aortic dilation was present in 104/187 patients. The aorta had six different shapes designated from S1 through S6. There was no association between the aortic shape and gender, aortic valve morphology, or hemodynamic abnormalities. S3 was the most common after the age of six years and was associated with the most significant degree of global aortic enlargement. The shape of the dilated aorta in children with bicuspid aortic valve does not occur in a uniform manner and multiple shapes are seen. S2 and S3 are most commonly seen. As aortic dilation becomes more significant, a single shape (S3) becomes the dominant pattern

  4. Valve-Sparing Root Replacement Compared With Composite Valve Graft Procedures in Patients With Aortic Root Dilation.

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    Ouzounian, Maral; Rao, Vivek; Manlhiot, Cedric; Abraham, Nachum; David, Carolyn; Feindel, Christopher M; David, Tirone E

    2016-10-25

    Although aortic valve-sparing (AVS) operations are established alternatives to composite valve graft (CVG) procedures for patients with aortic root aneurysms, comparative long-term outcomes are lacking. This study sought to compare the results of patients undergoing AVS procedures with those undergoing CVG operations. From 1990 to 2010, a total of 616 patients age Marfan syndrome and lower rates of bicuspid aortic valve than those undergoing bio-CVG or m-CVG procedures. In-hospital mortality (0.3%) and stroke rate (1.3%) were similar among groups. After adjusting for clinical covariates, both bio-CVG and m-CVG procedures were associated with increased long-term major adverse valve-related events compared with patients undergoing AVS (hazard ratio [HR]: 3.4, p = 0.005; and HR: 5.2, p valve-related complications when compared with bio-CVG and m-CVG. AVS is the treatment of choice for young patients with aortic root aneurysm and normal or near-normal aortic cusps. Copyright © 2016 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

  5. Hybrid endovascular treatment of an aortic root and thoracoabdominal aneurysm in a high-risk patient with Marfan syndrome.

    Science.gov (United States)

    Gelpi, Guido; Mazzaccaro, Daniela; Romagnoni, Claudia; Contino, Monica; Antona, Carlo

    2013-05-01

    This report describes the hybrid endovascular treatment of an aortic root dilatation and a thoracoabdominal aneurysm in a high-risk patient with Marfan syndrome. A 50-year-old male, in hemodialysis for polycystic kidney and polycystic liver, was referred to our department for aortic root dilatation of 5 cm and a 6.3-cm thoracoabdominal aneurysm . He already underwent surgical repair of abdominal aortic aneurysm 10 years ago, complicated by pseudoaneurysm of the proximal anastomosis that had been treated in another center, with an endoprosthesis. The patient underwent aortic root replacement, aortic valve sparing operation, and rerouting of the superior mesenteric artery and celiac trunk to the ascending aorta. The thoracoabdominal aneurysm was excluded with an endoprosthesis few days after the surgical step. The 12-month computed tomography scan confirmed the complete exclusion of the thoracoabdominal aneurysm.

  6. Aortic root reconstruction by aortic valve-sparing operation (David type I reimplantation) in Marfan syndrome accompanied by annuloaortic ectasia and acute type-A aortic dissection.

    Science.gov (United States)

    Inamura, Shunichi; Furuya, Hidekazu; Yagi, Kentarou; Ikeya, Eriko; Yamaguchi, Masaomi; Fujimura, Takabumi; Kanabuchi, Kazuo

    2006-09-20

    To reconstruct the aortic root for aneurysm of the ascending aorta accompanied by aortic regurgitation, annuloaortic ectasia (AAE) and acute type-A dissection with root destruction, the Bentall operation using a prosthetic valve still is the standard procedure today. Valve-sparing procedures have actively been used for aortic root lesions, and have also been attempted in aortic root reconstruction for Marfan syndrome which may have abnormalities in the valve leaflets. We conducted a valve-sparing procedure in a female patient with Marfan syndrome who had AAE accompanied by type-A acute aortic dissection. The patient was a 37-year-old woman complaining of severe pain from the chest to the back. The limbs were long, and funnel breast was observed. Diastolic murmurs were heard. On chest computed tomography, a dissection cavity was present from the ascending aorta to the left common iliac artery, and the root dilated to 55 mm. Grade II aortic regurgitation was observed on ultrasound cardiography. Regarding her family history, her father had died suddenly at 54 years of age. She was diagnosed with type-A acute dissection concurrent with Marfan syndrome and AAE. The structure of the aortic valve was normal, and root reconstruction by a valve-sparing operation and total replacement of the aortic arch was conducted. On postoperative ultrasound cardiography, the aortic regurgitation was within the allowable range, and the shortterm postoperative results were good.

  7. Impact of Aortic Insufficiency on Ascending Aortic Dilatation and Adverse Aortic Events After Isolated Aortic Valve Replacement in Patients With a Bicuspid Aortic Valve.

    Science.gov (United States)

    Wang, Yongshi; Wu, Boting; Li, Jun; Dong, Lili; Wang, Chunsheng; Shu, Xianhong

    2016-05-01

    Aberrant flow pattern and congenital fragility bestows bicuspid aortic valve (BAV) with a propensity toward ascending aorta dilatation, aneurysm, and dissection. Whether isolated aortic valve replacement (AVR) can prevent further dilatation in BAV ascending aorta and what indicates concurrent aortic intervention in the case of valve operation remain controversial. From June 2006 to January 2009, patients with a BAV who underwent isolated AVR were consecutively included and categorized into aortic insufficiency (BAV-AI, n = 84) and aortic stenosis (n = 112) groups, and another population of patients with a tricuspid aortic valve with aortic insufficiency (n = 149) was also recruited during the same period for comparison of annual aortic dilatation rate and adverse aortic events after isolated AVR. With a median follow-up period of 72 months (interquartile range, 66 to 78 months), ascending aorta dilatation rates were faster in the BAV-AI group than the BAV plus aortic stenosis and tricuspid aortic valve with aortic insufficiency groups (both p regression analysis identified aortic insufficiency (hazard ratio, 3.7; 95% confidence interval, 1.2 to 11.1; p = 0.019) as an independent risk factor for adverse aortic events among patients with BAV in general, whereas preoperative ascending aortic diameter larger than 45 mm (hazard ratio, 13.8; 95% confidence interval, 3.0 to 63.3; p = 0.001) served as a prognostic indicator in the BAV-AI group. An aggressive policy of preventive aortic interventions seemed appropriate in patients with BAV-AI during AVR, and BAV phenotype presenting as either insufficiency or stenosis should be taken into consideration when contemplating optimal surgical strategies for BAV aortopathy. Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  8. Reinforced aortic root reconstruction for acute type A aortic dissection involving the aortic root

    Directory of Open Access Journals (Sweden)

    Han Qing-qi

    2013-06-01

    Full Text Available OBJECTIVE: There are debates regarding the optimal approach for AAAD involving the aortic root. We described a modified reinforced aortic root reconstruction approach for treating AAAD involving the aortic root. METHODS: A total of 161 patients with AAAD involving the aortic root were treated by our modified reinforced aortic root reconstruction approach from January 1998 to December 2008. Key features of our modified approach were placement of an autologous pericardial patch in the false lumen, lining of the sinotubular junction lumen with a polyester vascular ring, and wrapping of the vessel with Teflon strips. Outcome measures included post-operative mortality, survival, complications, and level of aortic regurgitation. RESULTS: A total of 161 patients were included in the study (mean age: 43.3 1 15.5 years. The mean duration of follow-up was 5.1 1 2.96 years (2-12 years. A total of 10 (6.2% and 11 (6.8% patients died during hospitalization and during follow-up, respectively. Thirty-one (19.3% patients experienced postoperative complications. The 1-, 3-, 5-, and 10-year survival rates were 99.3%, 98%, 93.8%, and 75.5%, respectively. There were no instances of recurrent aortic dissection, aortic aneurysm, or pseudoaneurysm during the entire study period. The severity of aortic regurgitation dramatically decreased immediately after surgery (from 28.6% to 0% grade 3-4 and thereafter slightly increased (from 0% to 7.2% at 5 years and 9.1% at 10 years. CONCLUSION: This modified reinforced aortic root reconstruction was feasible, safe and durable/effective, as indicated by its low mortality, low postoperative complications and high survival rate.

  9. Impact of aortic root size on left ventricular afterload and stroke volume.

    Science.gov (United States)

    Sahlén, Anders; Hamid, Nadira; Amanullah, Mohammed Rizwan; Fam, Jiang Ming; Yeo, Khung Keong; Lau, Yee How; Lam, Carolyn S P; Ding, Zee Pin

    2016-07-01

    The left ventricle (LV) ejects blood into the proximal aorta. Age and hypertension are associated with stiffening and dilation of the aortic root, typically viewed as indicative of adverse remodeling. Based on analytical considerations, we hypothesized that a larger aortic root should be associated with lower global afterload (effective arterial elastance, EA) and larger stroke volume (SV). Moreover, as antihypertensive drugs differ in their effect on central blood pressure, we examined the role of antihypertensive drugs for the relation between aortic root size and afterload. We studied a large group of patients (n = 1250; 61 ± 12 years; 78 % males; 64 % hypertensives) from a single-center registry with known or suspected coronary artery disease. Aortic root size was measured by echocardiography as the diameter of the tubular portion of the ascending aorta. LV outflow tract Doppler was used to record SV. In the population as a whole, after adjusting for key covariates in separate regression models, aortic root size was an independent determinant of both SV and EA. This association was found to be heterogeneous and stronger in patients taking a calcium channel blocker (CCB; 10.6 % of entire population; aortic root size accounted for 8 % of the explained variance of EA). Larger aortic root size is an independent determinant of EA and SV. This association was heterogeneous and stronger in patients on CCB therapy.

  10. Reoperative Aortic Root Replacement in Patients with Previous Aortic Root or Aortic Valve Procedures

    Directory of Open Access Journals (Sweden)

    Byung Kwon Chong

    2016-08-01

    Full Text Available Background: Generalization of standardized surgical techniques to treat aortic valve (AV and aortic root diseases has benefited large numbers of patients. As a consequence of the proliferation of patients receiving aortic root surgeries, surgeons are more frequently challenged by reoperative aortic root procedures. The aim of this study was to evaluate the outcomes of redo-aortic root replacement (ARR. Methods: We retrospectively reviewed 66 patients (36 male; mean age, 44.5±9.5 years who underwent redo-ARR following AV or aortic root procedures between April 1995 and June 2015. Results: Emergency surgeries comprised 43.9% (n=29. Indications for the redo-ARR were aneurysm (n=12, pseudoaneurysm (n=1, or dissection (n=6 of the residual native aortic sinus in 19 patients (28.8%, native AV dysfunction in 8 patients (12.1%, structural dysfunction of an implanted bioprosthetic AV in 19 patients (28.8%, and infection of previously replaced AV or proximal aortic grafts in 30 patients (45.5%. There were 3 early deaths (4.5%. During follow- up (median, 54.65 months; quartile 1–3, 17.93 to 95.71 months, there were 14 late deaths (21.2%, and 9 valve-related complications including reoperation of the aortic root in 1 patient, infective endocarditis in 3 patients, and hemorrhagic events in 5 patients. Overall survival and event-free survival rates at 5 years were 81.5%±5.1% and 76.4%±5.4%, respectively. Conclusion: Despite technical challenges and a high rate of emergency conditions in patients requiring redo-ARR, early and late outcomes were acceptable in these patients.

  11. Aortic root replacement after previous surgical intervention on the aortic valve, aortic root, or ascending aorta.

    Science.gov (United States)

    Kirsch, E W Matthias; Radu, N Costin; Mekontso-Dessap, Armand; Hillion, Marie-Line; Loisance, Daniel

    2006-03-01

    Aortic root replacement after a previous operation on the aortic valve, aortic root, or ascending aorta remains a major challenge. Records of 56 consecutive patients (44 men; mean age, 56.4 +/- 13.6 years) undergoing reoperative aortic root replacement between June 1994 and June 2005 were reviewed retrospectively. Reoperation was performed 9.4 +/- 6.7 years after the last cardiac operation. Indications for reoperation were true aneurysm (n = 14 [25%]), false aneurysm (n = 10 [18%]), dissection or redissection (n = 9 [16%]), structural or nonstructural valve dysfunction (n = 10 [18%]), prosthetic valve-graft infection (n = 12 [21%]), and miscellaneous (n = 1 [2%]). Procedures performed were aortic root replacement (n = 47 [84%]), aortic root replacement plus mitral valve procedure (n = 5 [9%]), and aortic root replacement plus arch replacement (n = 4 [7%]). In 14 (25%) patients coronary artery bypass grafting had to be performed unexpectedly during the same procedure or immediately after the procedure to re-establish coronary perfusion. Hospital mortality reached 17.9% (n = 10). Multivariate logistic regression analysis revealed the need for unplanned perioperative coronary artery bypass grafting as the sole independent risk factor for hospital death (P = .005). Actuarial survival was 83.8% +/- 4.9% at 1 month, 73.0% +/- 6.3% at 1 year, and 65.7% +/- 9.0% at 5 years after the operation. One patient had recurrence of endocarditis 6.7 months after the operation and required repeated homograft aortic root replacement. Reoperative aortic root replacement remains associated with a high postoperative mortality. The need to perform unplanned coronary artery bypass grafting during reoperative aortic root replacement is a major risk factor for hospital death. The optimal technique for coronary reconstruction in this setting remains to be debated.

  12. Aortic dilatation in Turner syndrome: the role of MRI in early recognition

    International Nuclear Information System (INIS)

    Chalard, Francois; Ferey, Solene; Kalifa, Gabriel; Teinturier, Cecile

    2005-01-01

    Aortic dilatation and dissection are rare but important complications of Turner syndrome that increase the risk of sudden death in young patients. To assess the value of aortic MRI in patients with Turner syndrome; in particular to demonstrate early aortic dilatation. A total of 21 patients with Turner syndrome underwent MRI of the thoracic aorta with measurement of vessel diameter at four levels. Measurements were normal for age in 15 cases, two patients presented with values at the upper limit of normal and four had obvious dilatation of the ascending aorta. All were symptom free. MRI allows the non-invasive demonstration of early aortic dilatation, which may lead to earlier surgery in asymptomatic individuals. (orig.)

  13. Aortic dilatation in Turner syndrome: the role of MRI in early recognition

    Energy Technology Data Exchange (ETDEWEB)

    Chalard, Francois; Ferey, Solene; Kalifa, Gabriel [Saint Vincent de Paul Hospital, Department of Paediatric Radiology, Paris Cedex 14 (France); Teinturier, Cecile [Saint Vincent de Paul Hospital, Department of Paediatric Endocrinology, Paris (France)

    2005-03-01

    Aortic dilatation and dissection are rare but important complications of Turner syndrome that increase the risk of sudden death in young patients. To assess the value of aortic MRI in patients with Turner syndrome; in particular to demonstrate early aortic dilatation. A total of 21 patients with Turner syndrome underwent MRI of the thoracic aorta with measurement of vessel diameter at four levels. Measurements were normal for age in 15 cases, two patients presented with values at the upper limit of normal and four had obvious dilatation of the ascending aorta. All were symptom free. MRI allows the non-invasive demonstration of early aortic dilatation, which may lead to earlier surgery in asymptomatic individuals. (orig.)

  14. Aortic dilatation in Turner syndrome: the role of MRI in early recognition.

    Science.gov (United States)

    Chalard, François; Ferey, Solène; Teinturier, Cécile; Kalifa, Gabriel

    2005-03-01

    Aortic dilatation and dissection are rare but important complications of Turner syndrome that increase the risk of sudden death in young patients. To assess the value of aortic MRI in patients with Turner syndrome; in particular to demonstrate early aortic dilatation. A total of 21 patients with Turner syndrome underwent MRI of the thoracic aorta with measurement of vessel diameter at four levels. Measurements were normal for age in 15 cases, two patients presented with values at the upper limit of normal and four had obvious dilatation of the ascending aorta. All were symptom free. MRI allows the non-invasive demonstration of early aortic dilatation, which may lead to earlier surgery in asymptomatic individuals.

  15. Aortic valve sparing root surgery for Marfan syndrome.

    Science.gov (United States)

    Matalanis, George; Perera, Nisal K

    2017-11-01

    Aortic valve sparing root surgery (AVSRS) is a safe and durable alternative for patients with dilated roots or pure aortic regurgitation (AR), which avoids the risks of anticoagulation or valvular degeneration with prosthetic valves. Notwithstanding the theoretical challenges of greater tissue fragility in Marfan syndrome (MFS), AVSRS has been demonstrated to have equal outcomes in this condition as it does in those without MFS. The benefits of retaining the native aortic valve in this generally younger age group extend beyond those of avoiding the inconvenience and complications of prolonged exposure to anticoagulants and include ease of management for future aortic, cardiac and non-cardiac procedures which are the norm for these patients. The essential principles of AVSRS in MFS do not differ from those for the rest of the population. Successful repair and durable valve function depend on a sound understanding of the close interaction between the structure and function of this exquisitely designed piece of engineering. We are fortunate to have numerous tools in our surgical armamentarium to preserve these valves. It is the purpose of this paper to demystify the complex structure-function interactions of the aortic valve, thereby gaining an intuition for AVSRS. We will also elaborate on specific technical details of established techniques that we have found successful in preserving the normal function of these valves in the long term.

  16. Aortic stiffness and diameter predict progressive aortic dilatation in patients with Marfan syndrome

    NARCIS (Netherlands)

    Nollen, Gijs J.; Groenink, Maarten; Tijssen, Jan G. P.; van der Wall, Ernst E.; Mulder, Barbara J. M.

    2004-01-01

    Aim Patients with Marfan syndrome may develop dissection due to progressive dilatation in the entire aorta, which is not always predictable by mere anatomic assessment of the aortic diameter, especially of the descending aorta. The aim of this study was to identify the predictive value of aortic

  17. Aortic dilatation in patients with Turner's syndrome without structural cardiac anomaly.

    Science.gov (United States)

    Alami Laroussi, Nassiba; Dahdah, Nagib; Dallaire, Frédéric; Thérien, Johanne; Fournier, Anne

    2016-03-01

    Dilatation of the ascending aorta is described in Turner's syndrome with variable prevalence (6.8-32%). Reported series typically include patients with associated cardiac anomalies. To characterise the prevalence, age of onset, and the progress of dilatation of the ascending aorta in Turner's syndrome patients free of structural cardiac anomalies. Potential risk factors such as karyotype and growth hormone therapy were analysed for correlation with aortic dilatation. We carried out a retrospective study with data collected from medical records and echocardiography studies. Patients with Tuner's syndrome followed-up between 1992 and 2010 with at least two echocardiography studies were eligible. Patients with previous cardiac surgery or under anti-hypertensive medication were excluded. Ascending aorta diameter measurements were adjusted for body surface area, and dilatation was defined as Z-score>2. The study population consisted of 44 patients, aged 11.9±7.4 years at the first echocardiogram and 17.9±7.3 years at the last follow-up, with a follow-up duration of 6.0±3.7 years. A total of 13 (29.5%) patients exhibited aortic dilatation during follow-up, suggesting an actuarial estimate of the freedom from aortic dilatation dropping from 86 to 70% and then to 37% at 10, 20, and 30 years of age, respectively. There was no statistically significant impact of karyotype or growth hormone therapy on aortic Z-score progression. The prevalence of dilatation of the ascending aorta in Turner's syndrome patients free of structural aortic anomalies is comparable with published data with associated lesions. Growth hormone therapy and karyotype had no significant impact; however, longitudinal follow-up is warranted.

  18. Correction of aortic insufficiency with an external adjustable prosthetic aortic ring.

    Science.gov (United States)

    Gogbashian, Andrew; Ghanta, Ravi K; Umakanthan, Ramanan; Rangaraj, Aravind T; Laurence, Rita G; Fox, John A; Cohn, Lawrence H; Chen, Frederick Y

    2007-09-01

    Less invasive, valve-sparing options are needed for patients with aortic insufficiency (AI). We sought to evaluate the feasibility of reducing AI with an external adjustable aortic ring in an ovine model. To create AI, five sheep underwent patch plasty enlargement of the aortic annulus and root by placement of a 10 x 15 mm pericardial patch between the right and noncoronary cusps. An adjustable external ring composed of a nylon band was fabricated and placed around the aortic root. Aortic flow, aortic pressure, and left ventricular pressures were measured with the ring loose (off) and tightened (on). Mean regurgitant orifice area decreased by 86%, from 0.07 +/- 0.03 cm2 (ring loose, off) to 0.01 +/- 0.00 cm2 (ring tightened, on) [p < 0.01]. The regurgitant fraction decreased from 18 +/- 4% to 2 +/- 1% [p < 0.01]. The ring did not significantly affect stroke volume and aortic pressure. An ovine model of aortic root dilatation resulting in acute AI has been developed. In this model, application of an external, adjustable constricting aortic ring eliminated AI. An aortic ring may be a useful adjunct in reducing AI secondary to annular dilatation.

  19. Aortic valve-sparing operations in aortic root aneurysms: remodeling or reimplantation?

    Science.gov (United States)

    Rahnavardi, Mohammad; Yan, Tristan D; Bannon, Paul G; Wilson, Michael K

    2011-08-01

    A best evidence topic was written according to a structured protocol. The question addressed was whether the reimplantation (David) technique or the remodeling (Yacoub) technique provides the optimum event free survival in patients with an aortic root aneurysm suitable for an aortic valve-sparing operation. In total, 392 papers were found using the reported search criteria, of which 14 papers provided the best evidence to answer the clinical question. A total of 1338 patients (Yacoub technique in 606 and David technique in 732) from 13 centres were included. In most series, cardiopulmonary bypass time and aortic cross-clamp time were longer for the David technique compared to the Yacoub technique. Early mortality was comparable between the two techniques (0-6.9% for the Yacoub technique and 0-6% for the David technique). There is a tendency for a higher freedom from significant long-term aortic insufficiency in the David group than the Yacoub group, which does not necessarily result in a higher reoperation rate in the Yacoub group. In the largest series reported, freedom from a moderate-to-severe aortic insufficiency at 12 years was 82.6 ± 6.2% in the Yacoub and 91.0 ± 3.8% in the David group (P=0.035). Freedom from reoperation at the same time point was 90.4 ± 4.7% in the Yacoub group and 97.4 ± 2.2% in the David group (P=0.09). In another series, freedom from reoperation at a follow-up time of about four years was 89 ± 4% in the Yacoub group and 98 ± 2% in the David group. Although some authors merely preferred the Yacoub technique for a bicuspid aortic valve, the accumulated evidence in the current review indicates comparable results for both techniques in a bicuspid aortic valve. Current evidence is in favour of the David rather than the Yacoub technique in pathologies such as Marfan syndrome, acute type A aortic dissection, and excessive annular dilatation that may impair aortic root integrity. Careful selection of patients for each technique and

  20. Aortic root surgery in Marfan syndrome: Bentall procedure with the composite mechanical valved conduit versus aortic valve reimplantation with Valsalva graft.

    Science.gov (United States)

    Nardi, Paolo; Pellegrino, Antonio; Versaci, Francesco; Mantione, Ludmilla; Polisca, Patrizio; Iorio, Fiore S; Chiariello, Luigi

    2010-09-01

    The aim of the study is to compare mid-term results of Bentall aortic root replacement with composite mechanical valved conduit and aortic valve reimplantation procedure using the Valsalva graft for the treatment of aortic root aneurysm in patients with Marfan syndrome. We retrospectively compared data of 23 patients (mean age 38 + or - 14 years) who had undergone the Bentall procedure (group B) to those of 24 patients (mean age 36 + or - 12 years) who had undergone aortic valve reimplantation (group R) during a 14-year period. Follow-up (mean duration 65 + or - 44 months) was 100% complete. There were no operative deaths in either group. In group B, as compared with group R, preoperative aortic insufficiency (3.2 + or - 1.1/4 vs. 1.7 + or - 1.4/4, P Marfan patients, the Bentall procedure is associated with excellent mid-term outcome. The reimplantation technique, adopted for less dilated aortas, provides similarly satisfactory results. The Valsalva graft seems, with time, to allow a stable aortic valve function.

  1. A correlative study of aortic valve rotation angle and thoracic aortic sizes using ECG gated CT angiography

    Energy Technology Data Exchange (ETDEWEB)

    Saremi, Farhood, E-mail: fsaremi@usc.edu; Cen, Steven; Tayari, Nazila; Alizadeh, Houman; Emami, Amir; Lin, Leah; Fleischman, Fernando

    2017-04-15

    Objective: Various degrees of aortic valve rotation may be seen in individuals with no history of congenital cardiovascular malformations, but its association with aortic sizes has not been studied. Methods: Gated computed tomographic (CT angiograms in 217 patients were studied (66.7 ± 15; 22–97 years old)). Aortic diameters were determined at 5 anatomic locations. The length of the aorta from sinus to left subclavian artery was measured. The angle of valve rotation was recorded by measuring the angle between a line connecting the midpoint of the non-coronary sinus to the anterior commissure and another line along the interatrial septum. Rotation angles were correlated with aortic measurements. Patients were separated into two groups based on aortic sizes and into three groups based on age. The threshold for aortic dilatation was set at maximum ascending aorta diameter ≥40 mm (≥21 mm body surface area [BSA] indexed). Results: No significant difference in rotation angles was seen between the three age groups or between genders. Rotation angles were significantly correlated with maximal, average, and BSA adjustment of the aortic root and ascending aortic measurements. The aortic root angles were significantly different between the dilated versus nondilated aortas. There was no significant association between the rotation angles and age, length of ascending aorta, or diameters of descending aorta. Multivariate adaptive regression splines showed 25° of aortic root rotation as the diagnostic cut off for ascending aorta dilation. Above the 25° rotation, every 10° of increasing rotation was associated with a 3.78 ± 0.87 mm increase in aortic diameter (p < 0.01) and a 1.73 ± 0.25 times increased risk for having a dilated aorta (p < 0.01). Conclusion: Rotation angles of the aortic valve may be an independent non-invasive imaging marker for dilatation of the ascending aorta. Patients with increased rotation angle of the aortic valve may have higher risk for

  2. Determinants of Aortic Root Dilatation and Reference Values Among Young Adults Over a 20-Year Period: Coronary Artery Risk Development in Young Adults Study.

    Science.gov (United States)

    Teixido-Tura, Gisela; Almeida, Andre L C; Choi, Eui-Young; Gjesdal, Ola; Jacobs, David R; Dietz, Harry C; Liu, Kiang; Sidney, Stephen; Lewis, Cora E; Garcia-Dorado, David; Evangelista, Artur; Gidding, Samuel; Lima, João A C

    2015-07-01

    Aortic size increases with age, but factors related to such dilatation in healthy young adult population have not been studied. We aim to evaluate changes in aortic dimensions and its principal correlates among young adults over a 20-year time period. Reference values for aortic dimensions in young adults by echocardiography are also provided. Healthy Coronary Artery Risk Development in Young Adults (CARDIA) study participants aged 23 to 35 years in 1990-1991 (n=3051) were included after excluding 18 individuals with significant valvular dysfunction. Aortic root diameter (ARD) by M-mode echocardiography at year-5 (43.7% men; age, 30.2 ± 3.6 years) and year-25 CARDIA exams was obtained. Univariable and multivariable analyses were performed to assess associations of ARD with clinical data at years-5 and -25. ARD from year-5 was used to establish reference values of ARD in healthy young adults. ARD at year-25 was greater in men (33.3 ± 3.7 versus 28.7 ± 3.4 mm; Pyoung adulthood. Our study also provides reference values for ARD in young adults. © 2015 American Heart Association, Inc.

  3. Effect of personalized external aortic root support on aortic root motion and distension in Marfan syndrome patients.

    Science.gov (United States)

    Izgi, Cemil; Nyktari, Evangelia; Alpendurada, Francisco; Bruengger, Annina Studer; Pepper, John; Treasure, Tom; Mohiaddin, Raad

    2015-10-15

    Personalized external aortic root support (PEARS) is a novel surgical approach with the aim of stabilizing the aortic root size and decreasing risk of dissection in Marfan syndrome patients. A bespoke polymer mesh tailored to each patient's individual aorta shape is produced by modeling and then surgically implanted. The aim of this study is to assess the mechanical effects of PEARS on the aortic root systolic downward motion (an important determinant of aortic wall stress), aortic root distension and on the left ventricle (LV). A cohort of 27 Marfan patients had a prophylactic PEARS surgery between 2004 and 2012 with 24 having preoperative and follow-up cardiovascular magnetic resonance imaging studies. Systolic downward aortic root motion, aortic root distension, LV volumes/mass and mitral annular systolic excursion before the operation and in the latest follow-up were measured randomly and blinded. After a median follow-up of 50.5 (IQR 25.5-72) months following implantation of PEARS, systolic downward motion of aortic root was significantly decreased (12.6±3.6mm pre-operation vs 7.9±2.9mm latest follow-up, p<0.00001). There was a tendency for a decrease in systolic aortic root distension but this was not significant (median 4.5% vs 2%, p=0.35). There was no significant change in LV volumes, ejection fraction, mass and mitral annular systolic excursion in follow-up. PEARS surgery decreases systolic downward aortic root motion which is an important determinant of longitudinal aortic wall stress. Aortic wall distension and Windkessel function are not significantly impaired in the follow-up after implantation of the mesh which is also supported by the lack of deterioration of LV volumes or mass. Crown Copyright © 2015. Published by Elsevier Ireland Ltd. All rights reserved.

  4. Mitral valve disease in patients with Marfan syndrome undergoing aortic root replacement.

    Science.gov (United States)

    Kunkala, Meghana R; Schaff, Hartzell V; Li, Zhuo; Volguina, Irina; Dietz, Harry C; LeMaire, Scott A; Coselli, Joseph S; Connolly, Heidi

    2013-09-10

    Cardiac manifestations of Marfan syndrome include aortic root dilation and mitral valve prolapse (MVP). Only scant data exist describing MVP in patients with Marfan syndrome undergoing aortic root replacement. We retrospectively analyzed data from 166 MFS patients with MVP who were enrolled in a prospective multicenter registry of patients who underwent aortic root aneurysm repair. Of these 166 patients, 9% had mitral regurgitation (MR) grade >2, and 10% had MR grade 2. The severity of MVP and MR was evaluated by echocardiography preoperatively and ≤ 3 years postoperatively. Forty-one patients (25%) underwent composite graft aortic valve replacement, and 125 patients (75%) underwent aortic valve-sparing procedures; both groups had similar prevalences of MR grade >2 (P=0.7). Thirty-three patients (20%) underwent concomitant mitral valve (MV) intervention (repair, n=29; replacement, n=4), including all 15 patients with MR grade >2. Only 1 patient required MV reintervention during follow-up (mean clinical follow-up, 31 ± 10 months). Echocardiography performed 21 ± 13 months postoperatively revealed MR >2 in only 3 patients (2%). One early death and 2 late deaths occurred. Although the majority of patients with Marfan syndrome who undergo elective aortic root replacement have MVP, only 20% have concomitant MV procedures. These concomitant procedures do not seem to increase operative risk. In patients with MR grade ≤ 2 who do not undergo a concomitant MV procedure, the short-term incidence of progressive MR is low; however, more follow-up is needed to determine whether patients with MVP and MR grade ≤ 2 would benefit from prophylactic MV intervention.

  5. A multicentre observational study of the outcomes of screening detected sub-aneurysmal aortic dilatation

    DEFF Research Database (Denmark)

    Wild, J B; Stather, P W; Biancari, F

    2013-01-01

    of patients with screening detected sub aneurysmal aortic dilatation. DESIGN AND METHODS: Individual patient data was obtained from 8 screening programmes that had performed long term follow up of patients with sub aneurysmal aortic dilatation. Outcome measures recorded were the progression to true aneurysmal...... dilatation (aortic diameter 30 mm or greater), progression to size threshold for surgical intervention (55 mm) and aneurysm rupture. RESULTS: Aortic measurements for 1696 men and women (median age 66 years at initial scan) with sub-aneurysmal aortae were obtained, median period of follow up was 4.0 years...... (range 0.1-19.0 years). Following Kaplan Meier and life table analysis 67.7% of patients with 5 complete years of surveillance reached an aortic diameter of 30 mm or greater however 0.9% had an aortic diameter of 54 mm. A total of 26.2% of patients with 10 complete years of follow up had an AAA...

  6. Valve-sparing aortic root replacement†

    NARCIS (Netherlands)

    Koolbergen, David R.; Manshanden, Johan S. J.; Bouma, Berto J.; Blom, Nico A.; Mulder, Barbara J. M.; de Mol, Bas A. J. M.; Hazekamp, Mark G.

    2015-01-01

    To evaluate our results of valve-sparing aortic root replacement and associated (multiple) valve repair. From September 2003 to September 2013, 97 patients had valve-sparing aortic root replacement procedures. Patient records and preoperative, postoperative and recent echocardiograms were reviewed.

  7. Valve-sparing aortic root replacement in children: intermediate-term results.

    Science.gov (United States)

    Patel, Nishant D; Arnaoutakis, George J; George, Timothy J; Allen, Jeremiah G; Alejo, Diane E; Dietz, Harry C; Cameron, Duke E; Vricella, Luca A

    2011-03-01

    Valve-sparing root replacement (VSRR) is an attractive option for aortic aneurysm in children with low-operative risk, but mid- and late-term results are not yet known. Between 1997 and 2009, 56 children (mean age 11.5 years) underwent VSRR at our institution. Twenty-six (46.4%) had Marfan syndrome and 24 (42.8%) had Loeys-Dietz syndrome. Mean preoperative max sinus diameter was 4.2±0.8 cm (Z-score 7.7±2.9). Five (8.9%) had >2+ aortic insufficiency (AI). Two (3.6%) underwent David I reimplantation with a straight-tube, 12 (21.4%) had a Yacoub remodeling procedure, and 42 (75.0%) had reimplantation using a Valsalva-graft. There were one (1.8%) operative and three (5.4%) late deaths. One patient required reoperation for bleeding and one required late repair of a distal pseudoaneurysm. Mean follow-up was 5.2 years (range 0-12 years). No patients suffered thromboembolic events or had endocarditis. Of the 12 remodeling patients, four (33.3%) developed >2+ AI and required aortic valve repair or replacement. No patient developed >2+ AI after reimplantation. VSRR in children is a safe alternative to aortic root replacement with mechanical or biological prostheses. In this particular group of patients with connective tissue disorders and proclivity toward annular dilation and late AI, reimplantation is superior to remodeling.

  8. Aortic dilatation in Marfan syndrome: role of arterial stiffness and fibrillin-1 variants.

    Science.gov (United States)

    Salvi, Paolo; Grillo, Andrea; Marelli, Susan; Gao, Lan; Salvi, Lucia; Viecca, Maurizio; Di Blasio, Anna Maria; Carretta, Renzo; Pini, Alessandro; Parati, Gianfranco

    2018-01-01

    Marfan syndrome (MFS) is an autosomal dominant genetic disorder characterized by aortic root dilation and dissection and an abnormal fibrillin-1 synthesis. In this observational study, we evaluated aortic stiffness in MFS and its association with ascending aorta diameters and fibrillin-1 genotype. A total of 116 Marfan adult patients without history of cardiovascular surgery, and 144 age, sex, blood pressure and heart rate matched controls were enrolled. All patients underwent arterial stiffness evaluation through carotid-femoral pulse wave velocity (PWV) and central blood pressure waveform analysis (PulsePen tonometer). Fibrillin-1 mutations were classified based on the effect on the protein, into 'dominant negative' and 'haploinsufficient' mutations. PWV and central pulse pressure were significantly higher in MFS patients than in controls [respectively 7.31 (6.81-7.44) vs. 6.69 (6.52-6.86) m/s, P = 0.0008; 41.3 (39.1-43.5) vs. 34.0 (32.7-35.3) mmHg, P < 0.0001], with a higher age-related increase of PWV in MFS (β 0.062 vs. 0.036). Pressure amplification was significantly reduced in MFS [18.2 (15.9-20.5) vs. 33.4 (31.6-35.2)%, P < 0.0001]. Central pressure profile was altered even in MFS patients without aortic dilatation. Multiple linear regression models showed that PWV independently predicted aortic diameters at the sinuses of Valsalva (ß = 0.243, P = 0.002) and at the sinotubular junction (ß = 0.186, P = 0.048). PWV was higher in 'dominant negative' than 'haploinsufficient' fibrillin-1 mutations [7.37 (7.04-7.70) vs. 6.60 (5.97-7.23) m/s, P = 0.035], although this difference was not significant after adjustment. Aortic stiffness is increased in MFS, independently from fibrillin-1 genotype and is associated with diameters of ascending aorta. Alterations in central hemodynamics are present even when aortic diameter is within normal limits. Our findings suggest an accelerated arterial aging in MFS.

  9. Aortic events in a nationwide Marfan syndrome cohort

    DEFF Research Database (Denmark)

    Groth, Kristian A; Krag, Kirstine Stochholm; Hove, Hanne

    2017-01-01

    BACKGROUND: Marfan syndrome is associated with morbidity and mortality due to aortic dilatation and dissection. Preventive aortic root replacement has been the standard treatment in Marfan syndrome patients with aortic dilatation. In this study, we present aortic event data from a nationwide Marfan...... syndrome cohort. METHOD: The nationwide cohort of Danish Marfan syndrome patients was established from the Danish National Patient Registry and the Cause of Death Register, where we retrieved information about aortic surgery and dissections. We associated aortic events with age, sex, and Marfan syndrome...

  10. Valve-sparing aortic root reconstruction in children, teenagers, and young adults.

    Science.gov (United States)

    Tweddell, James S; Earing, Michael G; Bartz, Peter J; Dunham-Ingles, Jennifer L; Woods, Ronald K; Mitchell, Michael E

    2012-08-01

    We reviewed our experience with valve-sparing aortic root reconstruction (VSARR) using the sinus of Valsalva graft in children, teenagers, and young adults with connective tissue disorders. Results of a single-center experience with VSARR in children, teenagers, and young adults were retrospectively analyzed. End points were death, freedom from reintervention, and freedom from valve dysfunction. Between 2003 and 2010, 16 patients (Marfan, 9; Loeys Dietz syndrome, 6; conotruncal, 1) underwent VSARR. The mean age was 20±7.4 (range, 9 to 36 years). Indications for VSAAR were aortic root enlargement in 14 (sinus of Valsalva Z-score, 6.2±2) and aortic dissection in 2. Additional procedures included replacement of the ascending aorta in 7, with additional replacement of the aortic arch in 2. No early or late deaths occurred. One patient required a pacemaker. One patient with Loeys-Dietz syndrome required reoperation for aneurysmal dilatation of the coronary buttons. Two patients underwent replacement of the thoracoabdominal aorta for chronic dissection. Follow-up by echocardiography or magnetic resonance imaging at a mean of 33±29 months showed more than mild aortic regurgitation in 2 patients. Both patients with moderate aortic insufficiency also had a bicuspid aortic valve. VSARR using the sinus of Valsalva graft is a reproducible technique that achieves acceptable early and intermediate results. It is suitable for children, teenagers, and young adults. Anticoagulation is avoided. The procedure is appropriate for emergency operations but should be used with caution in patients with a bicuspid aortic valve. Copyright © 2012 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  11. Dilatation of aortic grafts over time: what to expect and when to be concerned

    DEFF Research Database (Denmark)

    Schroeder, Torben V; Eldrup, Nikolaj; Just, Sven

    2009-01-01

    Dilatation of aortic prosthetic grafts is commonly reported, but most reports are anecdotal, with little objective data in the literature. We performed a prospective trial of 303 patients who underwent prosthetic graft repair for aortic aneurysm or occlusive disease, randomizing patients between...... insertion of a woven polyester or expanded polytetrafluoroethylene (ePTFE) graft. Patients were followed with computed tomography and ultrasonography for up to 5 years in order to assess the frequency and magnitude of postoperative dilatation. Graft dilatation was documented in patients with polyester...... grafts at 12 months. Thereafter and up to 60 months, polyester grafts did not dilate further. After 5 years, polyester prostheses had dilated by 25% and ePTFE by 12.5%, as determined by computed tomography imaging. These observations suggest that dilatation of prosthetic grafts is more frequent...

  12. [Aortic valve-sparing root reconstruction in Marfan syndrome].

    Science.gov (United States)

    Ogino, H; Sasaki, H; Hanafusa, Y; Hirata, M; Numata, S; Ando, M; Yagihara, T; Kitamura, S

    2002-07-01

    The outcome of aortic valve-sparing root reconstruction in Marfan syndrome was reviewed. Thirteen patients with Marfan syndrome underwent aortic valve-sparing root reconstruction for annuloaortic ectasia or aortic root dissection between 1994 and 1999. The grade of preoperative aortic regurgitation was I in 4, II in 2, III in 5, IV in 2 patients. The procedures of aortic valve-sparing were reimplantation in 7 and remodeling in 5 patients. There was no hospital and late death. Recurrence of aortic regurgitation greater than moderate grade developed in 1 patient immediately after the surgery and in the other 4 patients in the late stage. One patient of them required aortic valve replacement for it. Aortic valve-sparing root reconstruction is applicable in Marfan patients, although the indication should be cautious. Close observation is needed for recurrence of aortic regurgitation.

  13. Expanding indications for valve-sparing aortic root reconstruction: early and midterm results.

    Science.gov (United States)

    Valo, Johanna; Jokinen, Janne J; Kaarne, Markku; Ihlberg, Leo

    2013-02-01

    Valve-sparing aortic root reconstruction (VSRR) is an accepted method to treat patients with aortic root dilation. The role of the VSRR is less well defined for patients with bicuspid aortic valve, severe aortic valve insufficiency, congenital heart defects, and type A aortic dissection. We studied the clinical outcome of patients who underwent VSRR for expanded indications. Seventy-eight patients underwent VSRR between the 2005 and 2012. Seventy-two patients (92%) underwent reimplantation and 6 patients (8%) were operated on with the remodeling technique. The mean age was 51 ± 12 years (range 24 to 73). For 71 patients (91%), the operation was elective, and for 7 (9%; all with type A aortic dissection), on an emergency basis. Preoperatively, the degree of aortic insufficiency was graded as 2+ or greater for 27 patients (35%). Connective tissue disorder (Marfan or Loeys-Dietz), bicuspid aortic valve, or congenital heart disease was present in 15 (19%), 15 (19%), and 7 patients (9%), respectively. Concomitant aortic valve leaflet repair was performed for 39 patients (50%). The mean follow-up time was 2.4 ± 1.7 years (range, 0.1 to 7.0). Thirty-day mortality was zero. The rate of postoperative complications was low: stroke 3%, renal failure 3%, prosthesis infection 1%, and low cardiac output syndrome 1%. Survival was 100% at 1 year and 97% at 5 years. Freedom from recurrent aortic valve insufficiency (≥2+) during the follow-up was 94%. The midterm results of VSRR in terms of survival, freedom from recurrent aortic valve insufficiency, and the need for reoperation are excellent, even for high-risk patients. Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  14. Valve-sparing aortic root replacement†.

    Science.gov (United States)

    Koolbergen, David R; Manshanden, Johan S J; Bouma, Berto J; Blom, Nico A; Mulder, Barbara J M; de Mol, Bas A J M; Hazekamp, Mark G

    2015-02-01

    To evaluate our results of valve-sparing aortic root replacement and associated (multiple) valve repair. From September 2003 to September 2013, 97 patients had valve-sparing aortic root replacement procedures. Patient records and preoperative, postoperative and recent echocardiograms were reviewed. Median age was 40.3 (range: 13.4-68.6) years and 67 (69.1%) were male. Seven (7.2%) patients were younger than 18 years, the youngest being 13.4 years. Fifty-four (55.7%) had Marfan syndrome, 2 (2.1%) other fibrous tissue diseases, 15 (15.5%) bicuspid aortic valve and 3 (3.1%) had earlier Fallot repair. The reimplantation technique was used in all, with a straight vascular prosthesis in 11 (26-34 mm) and the Valsalva prosthesis in 86 (26-32 mm). Concomitant aortic valve repair was performed in 43 (44.3%), mitral valve repair in 10 (10.3%), tricuspid valve repair in 5 (5.2%) and aortic arch replacement in 3 (3.1%). Mean follow-up was 4.2 ± 2.4 years. Follow-up was complete in all. One 14-year old patient died 1.3 years post-surgery presumably of ventricular arrhythmia. One patient underwent reoperation for aneurysm of the proximal right coronary artery after 4.9 years and 4 patients required aortic valve replacement, 3 of which because of endocarditis after 0.1, 0.8 and 1.3 years and 1 because of cusp prolapse after 3.8 years. No thrombo-embolic complications occurred. Mortality, root reoperation and aortic regurgitation were absent in 88.0 ± 0.5% at 5-year follow-up. Results of valve-sparing root replacement are good, even in association with a high incidence of concomitant valve repair. Valve-sparing aortic root replacement can be performed at a very young age as long as an adult size prosthesis can be implanted. © The Author 2014. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

  15. Aortic regurgitation after valve-sparing aortic root replacement: modes of failure.

    Science.gov (United States)

    Oka, Takanori; Okita, Yutaka; Matsumori, Masamichi; Okada, Kenji; Minami, Hitoshi; Munakata, Hiroshi; Inoue, Takeshi; Tanaka, Akiko; Sakamoto, Toshihito; Omura, Atsushi; Nomura, Takuo

    2011-11-01

    Despite the positive clinical results of valve-sparing aortic root replacement, little is known about the causes of reoperations and the modes of failure. From October 1999 to June 2010, 101 patients underwent valve-sparing aortic root replacement using the David reimplantation technique. The definition of aortic root repair failure included the following: (1) intraoperative conversion to the Bentall procedure; (2) reoperation performed because of aortic regurgitation; and (3) aortic regurgitation equal to or greater than a moderate degree at the follow-up. Sixteen patients were considered to have repair failure. Three patients required intraoperative conversion to valve replacement, 3 required reoperation within 3 months, and another 8 required reoperation during postoperative follow-up. At initial surgery 5 patients had moderate to severe aortic regurgitation, 6 patients had acute aortic dissections, 3 had Marfan syndrome, 2 had status post Ross operations, 3 had bicuspid aortic valves, and 1 had aortitis. Five patients had undergone cusp repair, including Arantius plication in 3 and plication at the commissure in 2. The causes of early failure in 6 patients included cusp perforation (3), cusp prolapse (3), and severe hemolysis (1). The causes of late failure in 10 patients included cusp prolapse (4), commissure dehiscence (3), torn cusp (2), and cusp retraction (1). Patients had valve replacements at a mean of 23 ± 20.9 months after reimplantation and survived. Causes of early failure after valve-sparing root replacement included technical failure, cusp lesions, and steep learning curve. Late failure was caused by aortic root wall degeneration due to gelatin-resorcin-formalin glue, cusp degeneration, or progression of cusp prolapse. Copyright © 2011 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  16. Pre- and Postoperative Imaging of the Aortic Root

    Science.gov (United States)

    Chan, Frandics P.; Mitchell, R. Scott; Miller, D. Craig; Fleischmann, Dominik

    2016-01-01

    Three-dimensional datasets acquired using computed tomography and magnetic resonance imaging are ideally suited for characterization of the aortic root. These modalities offer different advantages and limitations, which must be weighed according to the clinical context. This article provides an overview of current aortic root imaging, highlighting normal anatomy, pathologic conditions, imaging techniques, measurement thresholds, relevant surgical procedures, postoperative complications and potential imaging pitfalls. Patients with a range of clinical conditions are predisposed to aortic root disease, including Marfan syndrome, bicuspid aortic valve, vascular Ehlers-Danlos syndrome, and Loeys-Dietz syndrome. Various surgical techniques may be used to repair the aortic root, including placement of a composite valve graft, such as the Bentall and Cabrol procedures; placement of an aortic root graft with preservation of the native valve, such as the Yacoub and David techniques; and implantation of a biologic graft, such as a homograft, autograft, or xenograft. Potential imaging pitfalls in the postoperative period include mimickers of pathologic processes such as felt pledgets, graft folds, and nonabsorbable hemostatic agents. Postoperative complications that may be encountered include pseudoaneurysms, infection, and dehiscence. Radiologists should be familiar with normal aortic root anatomy, surgical procedures, and postoperative complications, to accurately interpret pre- and postoperative imaging performed for evaluation of the aortic root. Online supplemental material is available for this article. ©RSNA, 2015 PMID:26761529

  17. Aortic Root Enlargement or Sutureless Valve Implantation?

    Directory of Open Access Journals (Sweden)

    Nikolaos G. Baikoussis

    2016-11-01

    Full Text Available Aortic valve replacement (AVR in patients with a small aortic annulus is a challenging issue. The importance of prosthesis–patient mismatch (PPM post aortic valve replacement (AVR is controversial but has to be avoided. Many studies support the fact that PPM has a negative impact on short and long term survival. In order to avoid PPM, aortic root enlargement may be performed. Alternatively and keeping in mind that often some comorbidities are present in old patients with small aortic root, the Perceval S suturelles valve implantation could be a perfect solution. The Perceval sutureless bioprosthesis provides reasonable hemodynamic performance avoiding the PPM and providing the maximum of aortic orifice area. We would like to see in the near future the role of the aortic root enlargement techniques in the era of surgical implantation of the sutureless valve (SAVR and the transcatheter valve implantation (TAVI.

  18. Hemodynamic Correlates of Abnormal Aortic Root Dimension in an Adult Population: The Strong Heart Study.

    Science.gov (United States)

    de Simone, Giovanni; Roman, Mary J; De Marco, Marina; Bella, Jonathan N; Izzo, Raffaele; Lee, Elisa T; Devereux, Richard B

    2015-09-28

    We evaluated the relationship of aortic root dimension (ARD) with flow output and both peripheral and central blood pressure, using multivariable equations predicting ideal sex-specific ARD at a given age and body height. We measured echocardiographic diastolic ARD at the sinuses of Valsalva in 3160 adults (aged 42±16 years, 61% women) from the fourth examination of the Strong Heart Study who were free of prevalent coronary heart disease, and we compared measured data with the theoretical predicted value to calculate a z score. Central blood pressure was estimated by applanation tonometry of the radial artery in 2319 participants. ARD z scores were divided into tertiles representing small, normal, and large ARD. Participants with large ARD exhibited greater prevalence of central obesity and higher levels of inflammatory markers and lipids (0.05Aortic root dilatation is associated with high diastolic blood pressure, high stroke volume, central fat distribution, and inflammatory status. In contrast, at a given diastolic blood pressure and stroke volume, aortic root dilatation is associated with lower pulse pressure and systolic blood pressure. © 2015 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley Blackwell.

  19. Strategy to avoid patient-prosthesis mismatch: aortic root enlargement.

    Science.gov (United States)

    Srivastava, Dharmendra Kumar; Sanki, Prokash; Bhattacharya, Subhankar; Siddique, Javed Veqar

    2014-02-01

    The choice of a valve with an effective orifice area matching the body surface area and providing efficient hemodynamics is an important factor affecting mortality and morbidity in patients undergoing aortic valve replacement. Our preventative strategy was to implant a larger prosthetic valve by aortic root enlargement using the Nunez procedure in 17 patients between February 2010 and January 2011. The decision to enlarge the aortic root was taken when the 19-mm sizer could not be negotiated easily through the aortic root, or on the basis of body surface area of the patient or type of prosthesis available. Postoperative reductions in peak and mean pressure gradients across aortic valve of 12.8-16.5 and 10.2-12.6 mm Hg, respectively, were observed. Postoperative effective orifice areas of the aortic valves were 1.1-1.5 cm(2). By upsizing the aortic valve, we were able to eliminate patient-prosthesis mismatch in 5 patients, and reduce severe patient-prosthesis mismatch to moderate in 11. Aortic root enlargement is a safe procedure. Therefore, cardiac surgeons should not be reluctant to enlarge the aortic root with an autologous pericardial patch to permit implantation of an adequate size of aortic valve prosthesis, with minimal additional aortic crossclamp time and no added cost.

  20. Aortic distensibility after aortic root replacement assessed with cardiovascular magnetic resonance.

    Science.gov (United States)

    Melina, Giovanni; Rajappan, Kim; Amrani, Mohamed; Khaghani, Asghar; Pennell, Dudley J; Yacoub, Magdi H

    2002-01-01

    The changes in geometry of the aortic root during the cardiac cycle are thought to be essential for optimal valve function, both in terms of leaflet stress and dynamic behavior. Using cardiac magnetic resonance (CMR), the study aim was to determine aortic root distensibility of the homograft (group H) and the Medtronic Freestyle xenograft (group F) after aortic root replacement, from a prospective randomized trial. CMR was performed in 15 patients (six homografts, nine Freestyle) at six months and one year after surgery. Percentage change in aortic radius (PCR) and pressure strain elastic modulus (PSEM) were measured as indices of distensibility, and results related to left ventricular mass (LVM). At six months after surgery, mean PCR was 12+/-2.5 in group H and 12.9+/-6.1 in group F (p = NS), and PSEM was 428.5+/-69.8 and 493.5+/-72.7 g/cm2, respectively (p = NS). PCR was reduced to 10+/-1.7% in group H, and by 8.5+/-2.8% in group F (p = NS), while PSEM was increased to 520.5+/-87.3 and 825+/-420.4, respectively (p = NS) at the one-year follow up. Regression analysis showed a correlation between PCR and LVM (r = 0.52, p = 0.08) and LVM index (r = 0.46, p = 0.14), respectively. In addition, there was a relationship between PSEM, LVM and LVM index, suggesting that the stiffer the root wall, the higher the postoperative LVM. Up to one year after aortic root replacement, the wall of both the allogenic and xenogenic valves retained near-normal distensibility. For the first time, a correlation was demonstrated between the elastic properties of the aortic root and LVM. The longer-term behavior and clinical implications of these findings require further investigation.

  1. A patient with ascending aortic dilatation, similar to phenotypes of connective tissue disorders.

    Science.gov (United States)

    Onrat, S T; Emmiler, M; Sivaci, Y; Söylemez, Z; Ozgöz, A; Imirzalioğlu, N

    2009-04-14

    We report on the clinical and molecular findings of a patient who presented alopecia, epicanthus, micrognathia, retrognathia, high arched palate, hypertelorism, Chiari type I malformation, mixed-type hearing loss but with normal heartbeat Q-T interval, malformed earlobes, down-slanted palpebral fissures, downturned corners of the mouth, syndactyly, atopic eczema, and seizures. The patient was a male adult, 23 years old, with short stature (153 cm) and low weight (50.5 kg), due to severe aortic insufficiency and dilatation of the ascending aorta. Conventional cytogenetic screening did not show any chromosomal gains or losses. Molecular genetic screening was conducted for gene mutations involved in various syndromes; the mutations found included [beta-fibrinogen -455 G>A wt/wt (wt/mut), PAI-1 4G/5G (4G/4G), HPA1 a/b (a/a), MTHFR C677T wt/wt (wt/mut), ACE I/D (I/I), and Apo E E3/E4]. Many clinical and molecular genetics findings overlapped with other conditions associated with arterial tortuosity and arterial aneurysms, including the Marfan, Ehler-Danlos, Shprintzen-Goldberg, and Loeys-Dietz syndromes. Although a diagnosis of Shprintzen-Goldberg syndrome was based on clinical findings and radiographic findings indicate other syndromes, aortic root dilatation seems to be a new symptom, similar to phenotypes of connective tissue disorders. The unique grouping of clinical manifestations in this patient and the molecular genetics findings lead us to suggest that this case could be an example of a previously unrecognized syndrome.

  2. Surgical management of aortic root disease in Marfan syndrome and other congenital disorders associated with aortic root aneurysms.

    Science.gov (United States)

    Treasure, Tom; Takkenberg, J J M; Pepper, John

    2014-10-01

    Elective root replacement in Marfan syndrome has improved life expectancy in affected patients. Three forms of surgery are now available: total root replacement (TRR) with a valved conduit, valve sparing root replacement (VSRR) and personalised external aortic root support (PEARS) with a macroporous mesh sleeve. TRR can be performed irrespective of aortic dimensions and a mechanical replacement valve is a secure and near certain means of correcting aortic valve regurgitation but has thromboembolic and bleeding risks. VSRR offers freedom from anticoagulation and attendant risks of bleeding but reoperation for aortic regurgitation runs at 1.3% per annum. A prospective multi-institutional study has found this to be an underestimate of the true rate of valve-related adverse events. PEARS conserves the aortic root anatomy and optimises the chance of maintaining valve function but average follow-up is under 5 years and so the long-term results are yet to be determined. Patients are on average in their 30s and so the cumulative lifetime need for reoperation, and of any valve-related complications, are consequently substantial. With lowering surgical risk of prophylactic root replacement, the threshold for intervention has reduced progressively over 30 years to 4.5 cm and so an increasing number of patients who are not destined to have a dissection are now having root replacement. In evaluation of these three forms of surgery, the number needed to treat to prevent dissection and the balance of net benefit and harm in future patients must be considered.

  3. Valve-sparing root replacement in children with aortic root aneurysm: mid-term results.

    Science.gov (United States)

    Lange, Rüdiger; Badiu, Catalin C; Vogt, Manfred; Voss, Bernhard; Hörer, Jürgen; Prodan, Zsolt; Schreiber, Christian; Mazzitelli, Domenico

    2013-05-01

    We aimed at evaluating the results of aortic valve-sparing root replacement (AVSRR) in children with aortic root aneurysm (ARA) due to genetic disorders in terms of mortality, reoperation and recurrent aortic valve regurgitation (AVR). Thirteen patients (mean age 9.7 ± 6.5 years, 10 months-18 years) underwent AVSRR for ARA between 2002 and 2011. Six of the 13 patients had Marfan syndrome, 3 Loeys-Dietz syndrome (LDS), 2 bicuspid aortic valve syndrome and 2 an unspecified connective tissue disorder. AVR was graded as none/trace, mild and severe in 5, 7 and 1 patient, respectively. The mean pre-operative root diameter was 45 ± 10 mm (mean Z-score 10.3 ± 2.0). Remodelling of the aortic root was performed in 4 patients, reimplantation of the aortic valve in 9 and a concomitant cusp repair in 4. The diameter of the prostheses used for root replacement varied from 22 to 30 mm (mean Z-score = 2.3 ± 3). The follow-up was 100% complete with a mean follow-up time of 3.7 years. There was no operative mortality. One patient with LDS died 2.5 years after the operation due to spontaneous rupture of the descending aorta. Root re-replacement with mechanical conduit was necessary in 1 patient for severe recurrent AVR 8 days after remodelling of the aortic root. At final follow-up, AVR was graded as none/trace and mild in all patients. Eleven patients presented in New York Heart Association functional Class I and 1 in Class II. In paediatric patients with ARA, valve-sparing root replacement can be performed with low operative risk and excellent mid-term valve durability. Hence, prosthetic valve-related morbidity may be avoided. Due to the large diameters of the aortic root and the ascending aorta, the size of the implanted root prostheses will not limit later growth of the native aorta.

  4. Consecutive operative procedures in patients with Marfan syndrome up to 28 years after initial aortic root surgery.

    Science.gov (United States)

    Puluca, Nazan; Burri, Melchior; Cleuziou, Julie; Krane, Markus; Lange, Rüdiger

    2018-02-28

    Most patients (75%) with Marfan syndrome present with aortic root dilatation that may require surgical intervention. However, associated cardiovascular disorders are not limited to the aortic root. These patients frequently require consecutive operations on the remaining thoracic aorta or the heart valves. Our intent was to characterize the spectrum of such procedures. Data from all patients with Marfan syndrome undergoing aortic root surgery at our centre between 1988 and 2016 were analysed retrospectively. Overall, 73 patients (26 women) were selected for the study. The median age at 1st operation was 30 years (3-68 years). Indications for aortic root surgery were aneurysm (78%) and dissection (22%). Initially, 33 Bentall procedures and 40 valve-sparing root replacement procedures were performed, with a 97% rate of follow-up completion. The median follow up was 8 years (0-28 years). Survival at 1, 10 and 15 years was 100%, 85% and 82%, respectively. During follow-up monitoring, 48 subsequent procedures were performed in 33 patients (aorta, 23; aortic valve, 11; mitral valve, 7 and combined procedures, 7). The 30-day mortality rate after subsequent procedures was 4.2%. Freedom from subsequent operation and death (combined end-point) after 5, 10 and 15 years was 70%, 53% and 34%, respectively. After the initial surgery, subsequent procedures required in the setting of Marfan syndrome most often involve the remaining native aorta, followed by the aortic and mitral valves. The continued need for additional operative procedures remains high, even decades after the initial surgeries are undertaken. Therefore, long-term patient monitoring at specialized centres is imperative.

  5. Aortic root reimplantation procedure: a new milestone in aortic valve-sparing operations

    Directory of Open Access Journals (Sweden)

    А. М. Чернявский

    2016-08-01

    Full Text Available Aim: Emphasis in this study was placed on clinical and functional assessment of a modified "Florida Sleeve" procedure during surgical correction of ascending aorta aneurysms with concomitant aortic insufficiency.Methods: 32 patients with an aneurysm of the ascending aorta and aortic insufficiency underwent a modified "Florida Sleeve" procedure. The average follow-up was 17 (0-60 months. The average age of patients was 57±13 (23-73 years 56±13 years.Results: The expected 4-year cumulative survival rate was 84.3%. Overall freedom from aortic insufficiency in the late period was 88.9%. Median aortic regurgitation was 1+ (1; 2. Long-term follow-up revealed no valve-associated complications.Conclusion: The aortic root reimplantation procedure enables optimal correction of the existing lesions of the aortic root without performing aortic valve replacement and demonstrates stable clinical and functional outcomes in the long-term period.Key words: aortic aneurysm; aortic valve; valve-sparing operations.FundingThe study had no sponsorship.Conflict of interestThe authors declare no conflict of interest.

  6. Unexpected death caused by rupture of a dilated aorta in an adult male with aortic coarctation

    DEFF Research Database (Denmark)

    Leth, Peter Mygind; Knudsen, Peter Juel Thiis

    2015-01-01

    Aortic coarctation (AC) is a congenital aortic narrowing. We describe for the first time the findings obtained by unenhanced post mortem computed tomography (PMCT) in a case where the death was caused by cardiac tamponade from a ruptured aneurysmal dilatation of the ascending aorta and the aortic...

  7. A geometric approach to aortic root surgical anatomy.

    Science.gov (United States)

    Contino, Monica; Mangini, Andrea; Lemma, Massimo Giovanni; Romagnoni, Claudia; Zerbi, Pietro; Gelpi, Guido; Antona, Carlo

    2016-01-01

    The aim of this study was the analysis of the geometrical relationships between the different structures constituting the aortic root, with particular attention to interleaflet triangles, haemodynamic ventriculo-arterial junction and functional aortic annulus in normal subjects. Sixteen formol-fixed human hearts with normal aortic roots were studied. The aortic root was isolated, sectioned at the midpoint of the non-coronary sinus, spread apart and photographed by a high-resolution digital camera. After calibration and picture resizing, the software AutoCAD 2004 was used to identify and measure all the elements of the interleaflets triangles and of the aortic root that were objects of our analysis. Multiple comparisons were performed with one-way analysis of variance for continuous data and with Kruskal-Wallis analysis for non-continuous data. Linear regression and Pearson's product correlation were used to correlate root element dimensions when appropriate. Student's t-test was used to compare means for unpaired data. Heron's formula was applied to estimate the functional aortic annular diameters. The non coronary-left coronary interleaflets triangles were larger, followed by inter-coronary and right-non-coronary ones. The apical angle is <60° and its standard deviation can be considered an asymmetry index. The sinu-tubular junction was shown to be 10% larger than the virtual basal ring (VBR). The mathematical relationship between the haemodynamic ventriculo-arterial junction and the VBR calculated by linear regression and expressed in terms of the diameter was: haemodynamic ventriculo-arterial junction = 2.29 VBR (diameter) + 47. Conservative aortic surgery is based on a better understanding of aortic root anatomy and physiology. The relationships among its elements are of paramount importance during aortic valve repair/sparing procedures and they can be useful also in echocardiographic analysis and in computed tomography reconstruction. © The Author 2015

  8. Results of aortic root reimplantation in patients with ascending aortic aneurysm and concomitant aortic insufficiency

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    А. М. Чернявский

    2016-01-01

    Full Text Available Objective. The research was designed to evaluate the results of valve-sparing operations: aortic root reimplantation versus aortic valve reimplantation when repairing an ascending aortic aneurysm with concomitant aortic insufficiency.Methods. Within a blind prospective randomized study conducted over a period from 2011 to 2015, 64 patients underwent aortic valve-sparing surgery. The inclusion criteria were the presence of an aortic aneurysm of the ascending aorta exceeding 4.5 cm and concomitant aortic insufficiency. All patients were divided into two groups: FS-group, aortic root reimplantation (modified Florida Sleeve technique (n = 32 and D-group, aortic valve reimplantation (David procedure (n = 32. The average age of patients was 57±13 (23–73 years in the FS-group and 55±11 (15–72 years in the D-group (p = 0.54. Both groups had 78% of males (p>0.99. A Marfan syndrome was identified in 6% and 9% in the FS-group and D-group respectively (p>0.99. Mean diameter of the sinuses of Valsalva was 51±7 mm and 56±10 mm (p = 0.09, aortic insufficiency 2.6±0.7 and 2.8±0.8 (p = 0.15 in the FS-group and D-group respectively. In the FS-group and D-group LVEDD amounted to 5.5±0.7 mm and 5.9±1.0 mm (p = 0.09 respectively. All patients took echocardiography in the preoperative, postoperative and follow-up periods.Results. In the long-term period, the degree of aortic regurgitation was 1.2±0.1 in the FS-group and 1.3±0.6 in the D-group (p = 0.72. LVEDD was 123±23 mm in the FS-group and 139.6±80 mm in the D-group at follow-up (p = 0.77. There were no statistically significant differences in the analysis of complications. Overall 30-day in-hospital mortality was 7.8%. There were 2 (6.3% deaths in the FS-group and 3 (9.4% in the D-group (p = 0.5.Late mortality was 6.3% in the FS-group and 3.1% in the D-group (p>0.99. Cumulative survival at 4 years was 84.3% and 84.8% in the FS-group and the D-group respectively (p = 0.94. Cumulative freedom from

  9. Imaging techniques in aortic valve and root surgery

    NARCIS (Netherlands)

    Regeer, M.V.

    2017-01-01

    Aortic valve sparing surgery for aortic regurgitation and/or aortopathy serves as an alternative to aortic valve and root replacement. One of the advantages of aortic valve sparing surgery over conventional replacement is that there is no need for life-long anticoagulation, which is particularly

  10. Surgical management of aortic root disease in Marfan syndrome and other congenital disorders associated with aortic root aneurysms

    NARCIS (Netherlands)

    T. Treasure (Tom); J.J.M. Takkenberg (Hanneke); J. Pepper (John)

    2014-01-01

    textabstractElective root replacement in Marfan syndrome has improved life expectancy in affected patients. Three forms of surgery are now available: total root replacement (TRR) with a valved conduit, valve sparing root replacement (VSRR) and personalised external aortic root support (PEARS) with a

  11. Surgical management of aortic root disease in Marfan syndrome and other congenital disorders associated with aortic root aneurysms

    NARCIS (Netherlands)

    T. Treasure (Tom); J.J.M. Takkenberg (Hanneke); J. Pepper (John)

    2016-01-01

    textabstractElective root replacement in Marfan syndrome has improved life expectancy in affected patients. Three forms of surgery are now available: total root replacement (TRR) with a valved conduit, valve sparing root replacement (VSRR) and personalised external aortic root support (PEARS) with a

  12. Incidence and progression of mild aortic regurgitation after Tirone David reimplantation valve-sparing aortic root replacement.

    Science.gov (United States)

    Stephens, Elizabeth H; Liang, David H; Kvitting, John-Peder Escobar; Kari, Fabian A; Fischbein, Michael P; Mitchell, R Scott; Miller, D Craig

    2014-01-01

    The study objective was to determine whether recurrent or residual mild aortic regurgitation, which occurs after valve-sparing aortic root replacement, progresses over time. Between 2003 and 2008, 154 patients underwent Tirone David-V valve-sparing aortic root replacement; 96 patients (62%) had both 1-year (median, 12 ± 4 months) and mid-term (62 ± 22 months) transthoracic echocardiograms available for analysis. Age of patients averaged 38 ± 13 years, 71% were male, 31% had a bicuspid aortic valve, 41% had Marfan syndrome, and 51% underwent aortic valve repair, predominantly cusp free margin shortening. Forty-one patients (43%) had mild aortic regurgitation on 1-year echocardiogram. In 85% of patients (n = 35), mild aortic regurgitation remained stable on the most recent echocardiogram (median, 57 ± 20 months); progression to moderate aortic regurgitation occurred in 5 patients (12%) at a median of 28 ± 18 months and remained stable thereafter; severe aortic regurgitation developed in 1 patient, eventually requiring reoperation. Five patients (5%) had moderate aortic regurgitation at 1 year, which did not progress subsequently. Two patients (2%) had more than moderate aortic regurgitation at 1 year, and both ultimately required reoperation. Although mild aortic regurgitation occurs frequently after valve-sparing aortic root replacement, it is unlikely to progress over the next 5 years and should not be interpreted as failure of the valve-preservation concept. Further, we suggest that mild aortic regurgitation should not be considered nonstructural valve dysfunction, as the 2008 valve reporting guidelines would indicate. We need 10- to 15-year follow-up to learn the long-term clinical consequences of mild aortic regurgitation early after valve-sparing aortic root replacement. Copyright © 2014 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.

  13. Republished review: Surgical management of aortic root disease in Marfan syndrome and other congenital disorders associated with aortic root aneurysms.

    Science.gov (United States)

    Treasure, Tom; Takkenberg, J J M; Pepper, John

    2016-02-01

    Elective root replacement in Marfan syndrome has improved life expectancy in affected patients. Three forms of surgery are now available: total root replacement (TRR) with a valved conduit, valve sparing root replacement (VSRR) and personalised external aortic root support (PEARS) with a macroporous mesh sleeve. TRR can be performed irrespective of aortic dimensions and a mechanical replacement valve is a secure and near certain means of correcting aortic valve regurgitation but has thromboembolic and bleeding risks. VSRR offers freedom from anticoagulation and attendant risks of bleeding but reoperation for aortic regurgitation runs at 1.3% per annum. A prospective multi-institutional study has found this to be an underestimate of the true rate of valve-related adverse events. PEARS conserves the aortic root anatomy and optimises the chance of maintaining valve function but average follow-up is under 5 years and so the long-term results are yet to be determined. Patients are on average in their 30s and so the cumulative lifetime need for reoperation, and of any valve-related complications, are consequently substantial. With lowering surgical risk of prophylactic root replacement, the threshold for intervention has reduced progressively over 30 years to 4.5 cm and so an increasing number of patients who are not destined to have a dissection are now having root replacement. In evaluation of these three forms of surgery, the number needed to treat to prevent dissection and the balance of net benefit and harm in future patients must be considered. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/

  14. Valve-sparing aortic root replacement in Loeys-Dietz syndrome.

    Science.gov (United States)

    Patel, Nishant D; Arnaoutakis, George J; George, Timothy J; Allen, Jeremiah G; Alejo, Diane E; Dietz, Harry C; Cameron, Duke E; Vricella, Luca A

    2011-08-01

    Loeys-Dietz syndrome (LDS) is a recently recognized aggressive aortic disorder characterized by root aneurysm, arterial tortuosity, hypertelorism, and bifid uvula or cleft palate. The results of prophylactic root replacement using valve-sparing procedures (valve-sparing root replacement [VSRR]) in patients with LDS is not known. We reviewed all patients with clinical and genetic (transforming growth factor-β receptor mutations) evidence of LDS who underwent VSRR at our institution. Echocardiographic and clinical data were obtained from hospital and follow-up clinic records. From 2002 to 2009, 31 patients with a firm diagnosis of LDS underwent VSRR for aortic root aneurysm. Mean age was 15 years, and 24 (77%) were children. One (3%) patient had a bicuspid aortic valve. Preoperative sinus diameter was 3.9±0.8 cm (z score 7.0±2.9) and 2 (6%) had greater than 2+ aortic insufficiency. Thirty patients (97%) underwent reimplantation procedures using a Valsalva graft. There were no operative deaths. Mean follow-up was 3.6 years (range, 0 to 7 years). One patient required late repair of a pseudoaneurysm at the distal aortic anastomosis, and 1 had a conversion to a David reimplantation procedure after a Florida sleeve operation. No patient suffered thromboembolism or endocarditis, and 1 (3%) patient experienced greater than 2+ late aortic insufficiency. No patient required late aortic valve repair or replacement. Loeys-Dietz syndrome is an aggressive aortic aneurysm syndrome that can be addressed by prophylactic aortic root replacement with low operative risk. Valve-sparing procedures have encouraging early and midterm results, similar to those in Marfan syndrome, and are an attractive option for young patients. Copyright © 2011 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  15. MMP-2 Isoforms in Aortic Tissue and Serum of Patients with Ascending Aortic Aneurysms and Aortic Root Aneurysms

    Science.gov (United States)

    Tscheuschler, Anke; Meffert, Philipp; Beyersdorf, Friedhelm; Heilmann, Claudia; Kocher, Nadja; Uffelmann, Xenia; Discher, Philipp; Siepe, Matthias; Kari, Fabian A.

    2016-01-01

    Objective The need for biological markers of aortic wall stress and risk of rupture or dissection of ascending aortic aneurysms is obvious. To date, wall stress cannot be related to a certain biological marker. We analyzed aortic tissue and serum for the presence of different MMP-2 isoforms to find a connection between serum and tissue MMP-2 and to evaluate the potential of different MMP-2 isoforms as markers of high wall stress. Methods Serum and aortic tissue from n = 24 patients and serum from n = 19 healthy controls was analyzed by ELISA and gelatin zymography. 24 patients had ascending aortic aneurysms, 10 of them also had aortic root aneurysms. Three patients had normally functioning valves, 12 had regurgitation alone, eight had regurgitation and stenosis and one had only stenosis. Patients had bicuspid and tricuspid aortic valves (9/15). Serum samples were taken preoperatively, and the aortic wall specimen collected during surgical aortic repair. Results Pro-MMP-2 was identified in all serum and tissue samples. Pro-MMP-2 was detected in all tissue and serum samples from patients with ascending aortic/aortic root aneurysms, irrespective of valve morphology or other clinical parameters and in serum from healthy controls. We also identified active MMP-2 in all tissue samples from patients with ascending aortic/aortic root aneurysms. None of the analyzed serum samples revealed signals relatable to active MMP-2. No correlation between aortic tissue total MMP-2 or tissue pro-MMP-2 or tissue active MMP-2 and serum MMP-2 was found and tissue MMP-2/pro-MMP-2/active MMP-2 did not correlate with aortic diameter. This evidence shows that pro-MMP-2 is the predominant MMP-2 species in serum of patients and healthy individuals and in aneurysmatic aortic tissue, irrespective of aortic valve configuration. Active MMP-2 species are either not released into systemic circulation or not detectable in serum. There is no reliable connection between aortic tissue—and serum MMP-2

  16. Sex Differences in Phenotypes of Bicuspid Aortic Valve and Aortopathy: Insights From a Large Multicenter, International Registry.

    Science.gov (United States)

    Kong, William K F; Regeer, Madelien V; Ng, Arnold C T; McCormack, Louise; Poh, Kian Keong; Yeo, Tiong Cheng; Shanks, Miriam; Parent, Sarah; Enache, Roxana; Popescu, Bogdan A; Yip, James W; Ma, Lawrence; Kamperidis, Vasileios; van der Velde, Enno T; Mertens, Bart; Ajmone Marsan, Nina; Delgado, Victoria; Bax, Jeroen J

    2017-03-01

    This large multicenter, international bicuspid aortic valve (BAV) registry aimed to define the sex differences in prevalence, valve morphology, dysfunction (aortic stenosis/regurgitation), aortopathy, and complications (endocarditis and aortic dissection). Demographic, clinical, and echocardiographic data at first presentation of 1992 patients with BAV (71.5% men) were retrospectively analyzed. BAV morphology and valve function were assessed; aortopathy configuration was defined as isolated dilatation of the sinus of Valsalva or sinotubular junction, isolated dilatation of the ascending aorta distal to the sinotubular junction, or diffuse dilatation of the aortic root and ascending aorta. New cases of endocarditis and aortic dissection were recorded. There were no significant sex differences regarding BAV morphology and frequency of normal valve function. When presenting with moderate/severe aortic valve dysfunction, men had more frequent aortic regurgitation than women (33.8% versus 22.2%, P <0.001), whereas women were more likely to have aortic stenosis (34.5% versus 44.1%, P <0.001). Men had more frequently isolated dilatation of the sinus of Valsalva or sinotubular junction (14.2% versus 6.7%, P <0.001) and diffuse dilatation of the aortic root and ascending aorta (16.2% versus 7.3%, P <0.001) than women. Endocarditis (4.5% versus 2.5%, P =0.037) and aortic dissections (0.5% versus 0%, P <0.001) occurred more frequently in men. Although there is a male predominance among patients with BAV, men with BAV had more frequently moderate/severe aortic regurgitation at first presentation compared with women, whereas women presented more often with moderate/severe aortic stenosis compared with men. Furthermore, men had more frequent aortopathy than women. © 2017 American Heart Association, Inc.

  17. Successful Aortic Banding for Type IA Endoleak Due to Neck Dilatation after Endovascular Abdominal Aortic Aneurysm Repair: Case Report.

    Science.gov (United States)

    Tashima, Yasushi; Tamai, Koichi; Shirasugi, Takehiro; Sato, Kenichiro; Yamamoto, Takahiro; Imamura, Yusuke; Yamaguchi, Atsushi; Adachi, Hideo; Kobinata, Toshiyuki

    2017-09-25

    A 69-year-old man with a type IA endoleak that developed approximately 21 months after endovascular abdominal aortic aneurysm repair (EVAR) of a 46 mm diameter aneurysm was referred to our department. He had impaired renal function, Parkinson's disease, and previous cerebral infarction. Computed tomography angiography showed a type IA endoleak with neck dilatation and that the aneurysm had grown to 60 mm in diameter. We decided to perform aortic banding. The type IA endoleak disappeared after banding and the patient was discharged on postoperative day 10. Aortic banding may be effective for type IA endoleak after EVAR and less invasive for high-risk patients in particular.

  18. Aortic root surgery in Marfan syndrome.

    Science.gov (United States)

    Sheick-Yousif, Basheer; Sheinfield, Ami; Tager, Salis; Ghosh, Probal; Priesman, Sergey; Smolinsky, Aram K; Raanani, Ehud

    2008-03-01

    As the shortcomings of the Bentall operation and its variants in the Marfan syndrome have become apparent, the recent cusp-sparing techniques (remodeling or reimplantation) bear promise of better mid-term and long-term outcomes. To examine the results of aortic root surgery in patients with Marfan syndrome. During the period March 1994 to September 2007, 220 patients underwent aortic valve-sparing surgery; 20 were Marfan patients (group 1) who were compared with another 20 Marfan patients undergoing composite aortic root replacement (group 2). Fourteen patients had aortic dissection and 26 had thoracic aortic aneurysm. There were 31 males and 9 females with a mean age of 37.9 +/- 13.8 years. In group 1, reimplantation was used in 13 patients, remodeling in 4, and aortic valve repair with sinotubular junction replacement in 3. In group 2, a mechanical valve conduit was used. Mean logistic Euroscore was 12.27 +/- 14.6% for the whole group, five of whom were emergent cases Group 2 had more previous cardiac procedures compared to group 1 (9 vs. 2, P = 0.03) and shorter cross-clamp time (122 +/- 27.1 vs. 153.9 +/- 23.7 minutes, P = 0.0004). Overall mortality was 10%. Early mortality was 10% in group 2 and 5% in group 1 (NS). Mean follow-up time was 25 months for group 2 and 53 months for group 1. Three patients were reoperated; all had undergone the remodeling. Five year freedom from reoperation and death was 86% and 90% in group 2 and 70% and 95% in group 1 (P = 0.6, P = 0.6), respectively. Late survival of patients with Marfan syndrome was similar in both groups. Root reconstruction tends towards a higher incidence of late reoperations if the remodeling technique is used. We now prefer to use the reimplantation technique.

  19. Relation of Bicuspid Aortic Valve Morphology to the Dilatation Pattern of the Proximal Aorta: Focus on the Transvalvular Flow

    Directory of Open Access Journals (Sweden)

    Evaldas Girdauskas

    2012-01-01

    Full Text Available Whether the dilatation of proximal aorta in patients with bicuspid aortic valve is secondary to hemodynamic effects related to the abnormal aortic valve or a primary manifestation of the genetic disorder remains controversial. We discuss in this paper the recent data on the BAV function and transvalvular flow patterns in relation with the dilatation type of the proximal aorta. Different morphological forms of bicuspid aortic valve in relation with the specific transvalvular blood flow patterns are focus of the first paragraph of this paper. In the second part of this paper we present the pathogenetic insight into the different clinically observed phenotypes of bicuspid aortic valve disease (i.e., association of proximal aortic shapes with the specific cusp fusion patterns, based on the data from recent rheological studies.

  20. Transcatheter aortic valve implantation for a failed bio-bentall in patients with Marfan syndrome.

    Science.gov (United States)

    Beigel, Roy; Siegel, Robert J; Kahlon, Ravi S; Jilaihawi, Hasan; Cheng, Wen; Makkar, Raj R

    2014-01-01

    Patients with Marfan syndrome are at risk for ascending aortic dilation and dissection at the level of the aortic sinuses, making aortic root and valve replacement common. Patients undergoing an aortic root replacement with concomitant replacement of the aortic valve with a bioprosthesis (Bio-Bentall) are predisposed to bioprosthesis failure. Transcatheter aortic valve implantation (TAVI) has become an option for aortic valve replacement, avoiding cardiopulmonary bypass and/or median sternotomy. We present the first 2 reported patients with Marfan syndrome who underwent a valve-in-valve TAVI in the setting of a prior Bio-Bentall. © 2014 S. Karger AG, Basel.

  1. Angiotensin, transforming growth factor β and aortic dilatation in Marfan syndrome: Of mice and humans

    Directory of Open Access Journals (Sweden)

    Christopher Yu

    2018-03-01

    Full Text Available Marfan syndrome is consequent upon mutations in FBN1, which encodes the extracellular matrix microfibrillar protein fibrillin-1. The phenotype is characterised by development of thoracic aortic aneurysm. Current understanding of the pathogenesis of aneurysms in Marfan syndrome focuses upon abnormal vascular smooth muscle cell signalling through the transforming growth factor beta (TGFβ pathway. Angiotensin II (Ang II can directly induce aortic dilatation and also influence TGFβ synthesis and signalling. It has been hypothesised that antagonism of Ang II signalling may protect against aortic dilatation in Marfan syndrome. Experimental studies have been supportive of this hypothesis, however results from multiple clinical trials are conflicting. This paper examines current knowledge about the interactions of Ang II and TGFβ signalling in the vasculature, and critically interprets the experimental and clinical findings against these signalling interactions. Keywords: Aneurysm, Angiotensin blocker, Cell Signalling, Clinical trial

  2. Reoperative aortic root replacement: Outcome in a contemporary series.

    Science.gov (United States)

    Esaki, Jiro; Leshnower, Bradley G; Binongo, Jose N; Lasanajak, Yi; McPherson, LaRonica; Thourani, Vinod H; Chen, Edward P

    2017-09-01

    Reoperative aortic root replacement is a challenging procedure associated with significant mortality and morbidity. The purpose of this study was to investigate the outcomes of reoperative aortic root replacement when performed in a number of complex clinical settings and to identify risk factors for operative mortality and long-term survival. From 2006 to 2015, 280 consecutive patients at an academic center underwent reoperative aortic root replacement after a variety of previous aortic or cardiac operations. Logistic regression and extended Cox proportional hazards regression analyses were used to determine risk factors for operative mortality and long-term survival, respectively. The mean age of patients was 52.5 ± 14.1 years. Prior operations included proximal aortic replacement in 113 patients, valve surgery in 162 patients, and coronary artery bypass grafting in 46 patients. Concomitant procedures included arch replacement in 135 patients, coronary artery bypass grafting in 68 patients, and mitral valve repair/replacement in 18 patients. Operative mortality was 14.3%. Five-year survival was 74.0%. Univariable analysis did not find previous root replacement, prior proximal aortic surgery, and concomitant arch replacement to be risk factors for operative mortality. In the multivariable analysis, chronic lung disease, prior myocardial infarction, and concomitant mitral valve surgery were risk factors for operative mortality. Age, peripheral artery disease, emergency, and concomitant mitral valve surgery were risk factors for mortality in the late phase. Reoperative aortic root replacement represents complex procedures carrying significant morbidity and mortality. Chronic lung disease, prior myocardial infarction, and concomitant mitral valve surgery were risk factors for operative mortality. Age, peripheral artery disease, emergency, and concomitant mitral valve surgery were risk factors for long-term mortality. Copyright © 2017 The American Association for

  3. Analysis of aortic root surgery with composite mechanical aortic valve conduit and valve-sparing reconstruction.

    Science.gov (United States)

    Dias, Ricardo Ribeiro; Mejia, Omar Asdrubal Vilca; Fiorelli, Alfredo Inácio; Pomerantzeff, Pablo Maria Alberto; Dias, Altamiro Ribeiro; Mady, Charles; Stolf, Noedir Antonio Groppo

    2010-01-01

    Comparative analysis of early and late results of aortic root reconstruction with aortic valve sparing operations and the composite mechanical valve conduit replacement. From November 2002 to September 2009, 164 consecutive patients with mean age 54 ± 15 years, 115 male, underwent the aortic root reconstruction (125 mechanical valve conduit replacements and 39 valve sparing operations). Sixteen percent of patients had Marfan syndrome and 4.3% had bicuspid aortic valve. One hundred and forty-four patients (88%) were followed for a mean period of 41.1 ± 20.8 months. The hospital mortality was 4.9%, 5.6% in operations with valved conduits and 2.6% in the valve sparing procedures (P valve sparing operations, respectively (95% CI = 70% - 95%, P = 0.001), (95% CI = 82% - 95% P = 0.03) and (95% CI = 81% - 95%, P = 0.03). Multivariate analysis showed that creatinine greater than 1.4 mg/dl, Cabrol operation and renal dialysis were predictors of mortality, respectively, with occurrence chance of 6 (95% CI = 1.8 - 19.5, P = 0.003), 12 (95% CI = 3 - 49.7, P = 0.0004) and 16 (95% CI = 3.6 - 71.3, P = 0.0002). The aortic root reconstruction has a low early and late mortality, high survival free of complications and low need for reoperation. During the late follow-up, valve sparing aortic root reconstructions presented fewer incidences of bleeding, thromboembolic events and endocarditis.

  4. Prosthetic valve sparing aortic root replacement: an improved technique.

    Science.gov (United States)

    Leacche, Marzia; Balaguer, Jorge M; Umakanthan, Ramanan; Byrne, John G

    2008-10-01

    We describe a modified surgical technique to treat patients with a previous history of isolated aortic valve replacement who now require aortic root replacement for an aneurysmal or dissected aorta. This technique consists of replacing the aortic root with a Dacron conduit, leaving intact the previously implanted prosthesis, and re-implanting the coronary arteries in the Dacron graft. Our technique differs from other techniques in that we do not leave behind any aortic tissue remnant and also in that we use a felt strip to obliterate any gap between the old sewing ring and the newly implanted graft. In our opinion, this promotes better hemostasis. We demonstrate that this technique is safe, feasible, and results in acceptable outcomes.

  5. Automatic aortic root segmentation in CTA whole-body dataset

    Science.gov (United States)

    Gao, Xinpei; Kitslaar, Pieter H.; Scholte, Arthur J. H. A.; Lelieveldt, Boudewijn P. F.; Dijkstra, Jouke; Reiber, Johan H. C.

    2016-03-01

    Trans-catheter aortic valve replacement (TAVR) is an evolving technique for patients with serious aortic stenosis disease. Typically, in this application a CTA data set is obtained of the patient's arterial system from the subclavian artery to the femoral arteries, to evaluate the quality of the vascular access route and analyze the aortic root to determine if and which prosthesis should be used. In this paper, we concentrate on the automated segmentation of the aortic root. The purpose of this study was to automatically segment the aortic root in computed tomography angiography (CTA) datasets to support TAVR procedures. The method in this study includes 4 major steps. First, the patient's cardiac CTA image was resampled to reduce the computation time. Next, the cardiac CTA image was segmented using an atlas-based approach. The most similar atlas was selected from a total of 8 atlases based on its image similarity to the input CTA image. Third, the aortic root segmentation from the previous step was transferred to the patient's whole-body CTA image by affine registration and refined in the fourth step using a deformable subdivision surface model fitting procedure based on image intensity. The pipeline was applied to 20 patients. The ground truth was created by an analyst who semi-automatically corrected the contours of the automatic method, where necessary. The average Dice similarity index between the segmentations of the automatic method and the ground truth was found to be 0.965±0.024. In conclusion, the current results are very promising.

  6. Full-root aortic valve replacement with stentless xenograft achieves superior regression of left ventricular hypertrophy compared to pericardial stented aortic valves.

    Science.gov (United States)

    Tavakoli, Reza; Auf der Maur, Christoph; Mueller, Xavier; Schläpfer, Reinhard; Jamshidi, Peiman; Daubeuf, François; Frossard, Nelly

    2015-02-03

    Full-root aortic valve replacement with stentless xenografts has potentially superior hemodynamic performance compared to stented valves. However, a number of cardiac surgeons are reluctant to transform a classical stented aortic valve replacement into a technically more demanding full-root stentless aortic valve replacement. Here we describe our technique of full-root stentless aortic xenograft implantation and compare the early clinical and midterm hemodynamic outcomes to those after aortic valve replacement with stented valves. We retrospectively compared the pre-operative characteristics of 180 consecutive patients who underwent full-root replacement with stentless aortic xenografts with those of 80 patients undergoing aortic valve replacement with stented valves. In subgroups presenting with aortic stenosis, we further analyzed the intra-operative data, early postoperative outcomes and mid-term regression of left ventricular mass index. Patients in the stentless group were younger (62.6 ± 13 vs. 70.3 ± 11.8 years, p regression of the left ventricular mass index in the stentless (p replacement can be performed without adversely affecting the early morbidity or mortality in patients operated on for aortic valve stenosis provided that the coronary ostia are not heavily calcified. The additional time necessary for the full-root stentless compared to the classical stented aortic valve replacement is therefore not detrimental to the early clinical outcomes and is largely rewarded in patients with aortic stenosis by lower transvalvular gradients at mid-term and a better regression of their left ventricular mass index.

  7. Aortic root surgery in Marfan syndrome: Comparison of aortic valve-sparing reimplantation versus composite grafting.

    Science.gov (United States)

    Karck, Matthias; Kallenbach, Klaus; Hagl, Christian; Rhein, Christine; Leyh, Rainer; Haverich, Axel

    2004-02-01

    The objective of this study was to compare the results of aortic valve-sparing reimplantation and aortic root replacement with mechanical valve conduits in patients with Marfan syndrome undergoing operation for aortic root aneurysms. Patients and methods Between March 1979 and April 2002, 119 patients with clinical evidence of Marfan syndrome underwent composite graft replacement with mechanical valve conduits (n = 74) or aortic valve-sparing reimplantation according to David (n = 45). The underlying causes were aortic dissection type A (43 patients) and aneurysms (76 patients). Patients undergoing aortic valve reimplantation were younger compared with patients undergoing composite grafting (28 vs 35 years, P =.002) and had longer intraoperative aortic crossclamp times (125 vs 78 minutes, P valve reimplantation (P =.15). Mean follow-up was 30 months for patients undergoing aortic valve reimplantation and 114 months for patients undergoing composite grafting. Freedom from reoperation and death after 5 years postoperatively was 92% and 89% in patients undergoing composite grafting and 84% and 96% in patients undergoing aortic valve reimplantation (P =.31; P =.54), respectively. Thromboembolic complications or late postoperative bleeding occurred in 17 patients undergoing composite grafting, and an early postoperative event occurred in 1 patient undergoing aortic valve reimplantation. The results of aortic valve reimplantation and composite grafting of the aortic valve and ascending aorta with mechanical valve conduits are similar with regard to early and mid-term postoperative mortality and to the incidence of late reoperations in patients with Marfan syndrome. The low risk of thromboembolic or bleeding complications favors aortic valve reimplantation in these patients.

  8. High-pressure balloon dilation in a dog with supravalvular aortic stenosis.

    Science.gov (United States)

    Pinkos, A; Stauthammer, C; Rittenberg, R; Barncord, K

    2017-02-01

    A 6-month-old female intact Goldendoodle was presented for diagnostic work up of a grade IV/VI left basilar systolic heart murmur. An echocardiogram was performed and revealed a ridge of tissue distal to the aortic valve leaflets at the sinotubular junction causing an instantaneous pressure gradient of 62 mmHg across the supravalvular aortic stenosis and moderate concentric hypertrophy of the left ventricle. Intervention with a high-pressure balloon dilation catheter was pursued and significantly decreased the pressure gradient to 34 mmHg. No complications were encountered. The patient returned in 5 months for re-evaluation and static long-term reduction in the pressure gradient was noted. Copyright © 2016 Elsevier B.V. All rights reserved.

  9. Evaluation by MRA of aortic dilation late after repair of tetralogy of Fallot.

    Science.gov (United States)

    Kay, W Aaron; Cook, Stephen C; Daniels, Curt J

    2013-09-10

    This study evaluated predictors for aortic dilation (AD) in patients with repaired tetralogy of Fallot (rTOF) using magnetic resonance angiography (MRA). AD is common in patients with rTOF and may result in increased morbidity and mortality. There are no guidelines for evaluation of AD for rTOF patients. All adults with rTOF who previously underwent MRA had retrospective aortic measurements at the sinuses of Valsalva (SoV) and ascending aorta (AsAo). Rate of change in diameter was determined in patients with multiple MRAs. Chart review identified risk factors for AD. Univariate and multivariate analyses tested predictors of AD. Of the 87 patients who met the inclusion criteria, 12 (14%) had AD. At baseline, mean diameter was 3.6 ± 0.6 cm and 3.1 ± 0.6 cm at the SoV and AsAo, respectively. The AsAo was larger than the SoV in 17%. Predictors of AD included male gender, age, right aortic arch, pregnancy, older age at complete repair, smoking, and systemic hypertension. Serial studies were available in 55 patients; the rate of growth was slow: 0.4 ± 0.9 mm/year (SoV) and 0.1 ± 0.8mm/year (AsAo). AD is common in rTOF at the SoV and AsAo. Transthoracic echocardiography, which does not always image the AsAo as well as MRA, may not image AD in rTOF in cases in which the AsAo is dilated. Although several risk factors correlate with AD in rTOF, the rate of aortic growth is slow, suggesting that rTOF patients may not require frequent aortic imaging. Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.

  10. Long-Term Results of Aortic Root Surgery in Marfan Syndrome Patients: A Single-Center Experience.

    Science.gov (United States)

    Nicolo, Francesco; Romeo, Francesco; Lio, Antonio; Bovio, Emanuele; Scafuri, Antonio; Bassano, Carlo; Polisca, Patrizio; Pellegrino, Antonio; Nardi, Paolo; Chiariello, Luigi; Ruvolo, Giovanni

    2017-07-01

    The study aim was to compare long-term results of Marfan syndrome (MFS) patients affected by aortic root disease undergoing aortic root replacement with the Bentall or David operation. Since 1994, a total of 59 patients has been followed at the authors' Marfan Center, having undergone either a Bentall operation (Bentall group, n = 30) or a David operation (David group, n = 29). No operative mortality was recorded. After 20 years (mean follow up 97 ± 82 months; range 1 to 369 months) no prosthesis-related major bleeding or thromboembolic events had been observed; the 20-year survival was 94 ± 6% in the Bentall group, and 100% in the David group (p = 0.32). Freedom from reintervention for aortic valve dysfunction was 100% in the Bentall group, and 75 ± 13% in the David group (p = 0.04). This inter-group difference became relevant after the first eight-year period of follow-up, and was mainly associated with a particular familiar genetic phenotype involving three out of four reoperated patients. Freedom from all-cause death, myocardial infarction, stroke, prosthetic valve-related complications, and reintervention on any aortic segment was 69 ± 12% in the Bentall group, and 67 ± 14% in the David group (p = 0.33). The Bentall and David operations are both associated with satisfactory long-term results in MFS patients. The low rate of valve prosthesis-related complications suggested that the Bentall operation would continue to be a standard surgical treatment. The reimplantation technique, adopted for less-dilated aortas, provides satisfactory freedom from reoperation. Careful attention should be paid to the reimplantation technique in patients affected by a serious familiar genetic phenotype.

  11. The thoracic aortography by Gd-DTPA enhanced ultrafast cine MR imaging. Assessment of thoracic aortic dilatation in aging and in patients with hypertension and aortic valve disease

    International Nuclear Information System (INIS)

    Matsumura, Kentaro; Nakase, Emiko; Kawai, Ichiyoshi; Saito, Takayuki; Kikkawa, Nobutada; Haiyama, Toru

    1995-01-01

    To assess the morphology of thoracic aorta, we had a trial of Gd-DTPA enhanced ultrafast cine MR imaging on the thoracic aorta. This method was provided with high quality thoracic aortogram during 15-20 seconds. In patients without hypertension and aortic valve disease, dimensions of ascending aorta and aortic arch were significantly correlated with aging. In patients with hypertension, dimensions of ascending aorta and aortic arch were significantly dilated. In patients with aortic valve disease, thoracic aorta was diffusely enlarged, especially in ascending aorta. Gd-DTPA enhanced ultrafact cine MR imaging was useful to assess the thoracic aortic anatomy and diseases. (author)

  12. Monozygotic twins with Marfan's syndrome and ascending aortic aneurysm.

    Science.gov (United States)

    Redruello, Héctor Jorge; Cianciulli, Tomas Francisco; Rostello, Eduardo Fernandez; Recalde, Barbara; Lax, Jorge Alberto; Picone, Victorio Próspero; Belforte, Sandro Mario; Prezioso, Horacio Alberto

    2007-08-01

    Marfan's syndrome is a hereditary connective tissue disease, in which cardiovascular abnormalities (especially aortic root dilatation) are the most important cause of morbidity and mortality. In this report, we describe two 24-year-old twins, with a history of surgery for lens subluxation and severe cardiovascular manifestations secondary to Marfan's syndrome. One of the twins suffered a type A aortic dissection, which required replacement of the ascending aorta, and the other twin had an aneurysmal dilatation of the ascending aorta (46mm) and was prescribed medical treatment with atenolol and periodic controls to detect the presence of a critical diameter (50mm) that would indicate the need for prophylactic surgery.

  13. Long-term results of aortic root repair using the reimplantation technique.

    Science.gov (United States)

    David, Tirone E; Armstrong, Susan; Manlhiot, Cedric; McCrindle, Brian W; Feindel, Christopher M

    2013-03-01

    Aortic valve sparing is frequently performed to treat patients with aortic root aneurysm, but there is an inadequate amount of information regarding its long-term durability. This study examines the long-term results of reimplantation of the aortic valve in patients with aortic root aneurysms. From August 1989 to December 2010, 296 consecutive patients had reimplantation of the aortic valve into a tubular Dacron graft. Their mean age was 45 years (range, 11-79 years), and 78% were men. Of the patients, 36% had Marfan syndrome and 11% had bicuspid aortic valve. Patients were followed prospectively with periodic images of the aortic root and remaining aorta. The mean follow-up was 6.9 ± 4.5 years. There were 21 patients at risk at 15 years. There were 4 operative and 18 late deaths. The survival at 5, 10, and 15 years was 95.1% ± 3.5%, 93.1% ± 4.4%, and 76.5% ± 18%, respectively. Only 3 patients required reoperation on the aortic valve; all 3 patients had the Bentall procedure. Freedom from reoperation at 5, 10, and 15 years was 99.7% ± 2.0%, 97.8% ± 5.3%, and 97.8% ± 5.3%, respectively. During follow-up, moderate aortic insufficiency developed in 9 patients, and severe aortic insufficiency developed in 2 patients. Freedom from moderate or severe aortic insufficiency at 5, 10, and 15 years was 98.3% ± 3.5%, 92.9% ± 6.5%, and 89.4% ± 12%, respectively. The function of the aortic valve implanted inside a tubular Dacron graft remains normal at 15 years in most patients after this type of aortic valve-sparing operation. Copyright © 2013 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.

  14. Aortic Root Surgery in Marfan Syndrome: Medium-Term Outcome in a Single-Center Experience.

    Science.gov (United States)

    Attenhofer Jost, Christine H; Connolly, Heidi M; Scott, Christopher G; Ammash, Naser M; Bowen, Juan M; Schaff, Hartzell V

    2017-01-01

    The study aim was to analyze the authors' experience with aortic root surgery in Marfan syndrome (MFS), and to expand the surgical outcome data of patients meeting the Ghent criteria (Marfan registry). Analyses were performed of data acquired from MFS patients (who met the Ghent criteria), including an aortic root surgery and Kaplan-Meier survival. Between April 2004 and February 2012, a total of 59 MFS patients (mean age at surgery 36 ± 13 years) underwent 67 operations for aortic root aneurysm (n = 52), aortic valve (AV) regurgitation (n = 15), acute aortic dissection (n = 2), and/or mitral valve (MV) regurgitation resulting from MV prolapse (n = 7). Of 59 initial operations, 21 (36%) involved AV-replacing root surgery, 38 (64%) AV-sparing root surgery, seven (12%) aortic arch or hemi-arch repair, and five (8%) simultaneous MV surgery. There were no early mortalities. The mean follow up was 6.8 ± 1.2 years, with five deaths (8%) and a relatively low reoperation rate (10 reoperations in nine patients; 14%). Seven reoperations involved AV or aortic root surgery (including four for AV regurgitation following failed AV-sparing surgery), two MV repair/replacements, and one coronary artery bypass graft. Eight patients (21%) with AV-sparing surgery had moderate/severe AV regurgitation at the last follow up before re-intervention. The mean five-year freedom from postoperative death was 91.2 ± 8.8%, from cardiac reoperation 86.3 ± 4.5%, and more-than-moderate AV regurgitation 90.3 ± 4.8%. Prophylactic aortic surgery in MFS patients with AV-replacing root or AV-sparing root surgery carries a low risk of operative morbidity and death when performed at an experienced center. AV-sparing root surgery increases the risk of AV regurgitation and, possibly, of re-intervention. Regular clinical follow up is important after any aortic root surgery in MFS patients, with a delineation of risk factors for AV regurgitation after AV rootsparing surgery.

  15. Characteristics of children and young adults with Marfan syndrome and aortic root dilation in a randomized trial comparing atenolol and losartan therapy

    NARCIS (Netherlands)

    Lacro, R.V.; Guey, L.T.; Dietz, H.C.; Pearson, G.D.; Yetman, A.T.; Gelb, B.D.; Loeys, B.L.; Benson, D.W.; Bradley, T.J.; Backer, J. de; Forbus, G.A.; Klein, G.L.; Lai, W.W.; Levine, J.C.; Lewin, M.B.; Markham, L.W.; Paridon, S.M.; Pierpont, M.E.; Radojewski, E.; Selamet Tierney, E.S.; Sharkey, A.M.; Wechsler, S.B.; Mahony, L.; et al.,

    2013-01-01

    BACKGROUND: The Pediatric Heart Network designed a clinical trial to compare aortic root growth and other short-term cardiovascular outcomes in children and young adults with Marfan syndrome randomized to receive atenolol or losartan. We report here the characteristics of the screened population and

  16. Long-term outcomes of aortic root operations for Marfan syndrome: A comparison of Bentall versus aortic valve-sparing procedures.

    Science.gov (United States)

    Price, Joel; Magruder, J Trent; Young, Allen; Grimm, Joshua C; Patel, Nishant D; Alejo, Diane; Dietz, Harry C; Vricella, Luca A; Cameron, Duke E

    2016-02-01

    Prophylactic aortic root replacement improves survival in patients with Marfan syndrome with aortic root aneurysms, but the optimal procedure remains undefined. Adult patients with Marfan syndrome who had Bentall or aortic valve-sparing root replacement (VSRR) procedures between 1997 and 2013 were identified. Comprehensive follow-up information was obtained from hospital charts and telephone contact. One hundred sixty-five adult patients with Marfan syndrome (aged > 20 years) had either VSRR (n = 98; 69 reimplantation, 29 remodeling) or Bentall (n = 67) procedures. Patients undergoing Bentall procedure were older (median, 37 vs 36 years; P = .03), had larger median preoperative sinus diameter (5.5 cm vs 5.0 cm; P = .003), more aortic dissections (25.4% vs 4.1%; P Marfan syndrome, patients undergoing Bentall and valve-sparing procedures have similar late survival, freedom from root reoperation, and freedom from endocarditis. However, valve-sparing procedures result in significantly fewer thromboembolic and hemorrhagic events. Copyright © 2016 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.

  17. [Modern aortic surgery in Marfan syndrome--2011].

    Science.gov (United States)

    Kallenbach, K; Schwill, S; Karck, M

    2011-09-01

    Marfan syndrome is a hereditary disease with a prevalence of 2-3 in 10,000 births, leading to a fibrillin connective tissue disorder with manifestations in the skeleton, eye, skin, dura mater and in particular the cardiovascular system. Since other syndromes demonstrate similar vascular manifestations, but therapy may differ significantly, diagnosis should be established using the revised Ghent nosology in combination with genotypic analysis in specialized Marfan centres. The formation of aortic root aneurysms with the subsequent risk of acute aortic dissection type A (AADA) or aortic rupture limits life expectancy in patients with Marfan syndrome. Therefore, prophylactic replacement of the aortic root needs to be performed before the catastrophic event of AADA can occur. The goal of surgery is the complete resection of pathological aortic tissue. This can be achieved with excellent results by using a (mechanically) valved conduit that replaces both the aortic valve and the aortic root (Bentall operation). However, the need for lifelong anticoagulation with Coumadin can be avoided using the aortic valve sparing reimplantation technique according to David. The long-term durability of the reconstructed valve is favourable, and further technical improvements may improve longevity. Although results of prospective randomised long-term studies comparing surgical techniques are lacking, the David operation has become the surgical method of choice for aortic root aneurysms, not only at the Heidelberg Marfan Centre. Replacement of the aneurysmal dilated aortic arch is performed under moderate hypothermic circulatory arrest combined with antegrade cerebral perfusion using a heart-lung machine, which we also use in thoracic or thoracoabdominal aneurysms. Close post-operative follow-up in a Marfan centre is pivotal for the early detection of pathological changes on the diseased aorta.

  18. Flow measurement at the aortic root

    DEFF Research Database (Denmark)

    Bertelsen, Litten; Svendsen, Jesper Hastrup; Køber, Lars

    2016-01-01

    during CMR and aortic stenosis were excluded from the analyses. Stroke volumes were measured volumetrically (SVref) from steady-state free precision short axis images covering the entire left ventricle, excluding the papillary muscles and including the left ventricular outflow tract. Flow sequences......BACKGROUND: Cardiovascular magnetic resonance (CMR) is considered the gold standard of cardiac volumetric measurements. Flow in the aortic root is often measured at the sinotubular junction, even though placing the slice just above valve level may be more precise. It is unknown how much flow...... theoretically be equal to flow measurements, SVV and SVST were compared to SVref. RESULTS: Initially, 152 patients were included. 22 were excluded because of arrhythmias during scans and 9 were excluded for aortic stenosis. Accordingly, data from 121 patients were analysed and of these 63 had visually evident...

  19. MDCT evaluation of aortic root and aortic valve prior to TAVI. What is the optimal imaging time point in the cardiac cycle?

    Energy Technology Data Exchange (ETDEWEB)

    Jurencak, Tomas; Turek, Jakub; Nijssen, Estelle C. [Maastricht University Medical Center, Department of Radiology, P. Debyelaan 25, P.O. Box 5800, AZ, Maastricht (Netherlands); Kietselaer, Bastiaan L.J.H. [Maastricht University Medical Center, Department of Radiology, P. Debyelaan 25, P.O. Box 5800, AZ, Maastricht (Netherlands); Maastricht University Medical Center, CARIM School for Cardiovascular Diseases, Maastricht (Netherlands); Maastricht University Medical Center, Department of Cardiology, Maastricht (Netherlands); Mihl, Casper; Kok, Madeleine; Wildberger, Joachim E.; Das, Marco [Maastricht University Medical Center, Department of Radiology, P. Debyelaan 25, P.O. Box 5800, AZ, Maastricht (Netherlands); Maastricht University Medical Center, CARIM School for Cardiovascular Diseases, Maastricht (Netherlands); Ommen, Vincent G.V.A. van [Maastricht University Medical Center, Department of Cardiology, Maastricht (Netherlands); Garsse, Leen A.F.M. van [Maastricht University Medical Center, Department of Cardiothoracic Surgery, Maastricht (Netherlands)

    2015-07-15

    To determine the optimal imaging time point for transcatheter aortic valve implantation (TAVI) therapy planning by comprehensive evaluation of the aortic root. Multidetector-row CT (MDCT) examination with retrospective ECG gating was retrospectively performed in 64 consecutive patients referred for pre-TAVI assessment. Eighteen different parameters of the aortic root were evaluated at 11 different time points in the cardiac cycle. Time points at which maximal (or minimal) sizes were determined, and dimension differences to other time points were evaluated. Theoretical prosthesis sizing based on different measurements was compared. Largest dimensions were found between 10 and 20 % of the cardiac cycle for annular short diameter (10 %); mean diameter (10 %); effective diameter and circumference-derived diameter (20 %); distance from the annulus to right coronary artery ostium (10 %); aortic root at the left coronary artery level (20 %); aortic root at the widest portion of coronary sinuses (20 %); and right leaflet length (20 %). Prosthesis size selection differed depending on the chosen measurements in 25-75 % of cases. Significant changes in anatomical structures of the aortic root during the cardiac cycle are crucial for TAVI planning. Imaging in systole is mandatory to obtain maximal dimensions. (orig.)

  20. Valve-sparing aortic root replacement in patients with Marfan syndrome enrolled in the National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions.

    Science.gov (United States)

    Song, Howard K; Preiss, Liliana R; Maslen, Cheryl L; Kroner, Barbara; Devereux, Richard B; Roman, Mary J; Holmes, Kathryn W; Tolunay, H Eser; Desvigne-Nickens, Patrice; Asch, Federico M; Milewski, Rita K; Bavaria, Joseph; LeMaire, Scott A

    2014-05-01

    The long-term outcomes of aortic valve-sparing (AVS) root replacement in Marfan syndrome (MFS) patients remain uncertain. The study aim was to determine the utilization and outcomes of AVS root replacement in MFS patients enrolled in the Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC). At the time of this analysis, 788 patients with MFS were enrolled in the GenTAC Registry, of whom 288 had undergone aortic root replacement. Patients who had undergone AVS procedures were compared to those who had undergone aortic valve replacement (AVR). AVS root replacement was performed in 43.5% of MFS patients, and the frequency of AVS was increased over the past five years. AVS patients were younger at the time of surgery (31.0 versus 36.3 years, p = 0.006) and more likely to have had elective rather than emergency surgery compared to AVR patients, in whom aortic valve dysfunction and aortic dissection was the more likely primary indication for surgery. After a mean follow up of 6.2 +/- 3.6 years, none of the 87 AVS patients had required reoperation; in contrast, after a mean follow up of 10.5 +/- 7.6 years, 11.5% of AVR patients required aortic root reoperation. Aortic valve function has been durable, with 95.8% of AVS patients having aortic insufficiency that was graded as mild or less. AVS root replacement is performed commonly among the MFS population, and the durability of the aortic repair and aortic valve function have been excellent to date. These results justify a continued use of the procedure in an elective setting. The GenTAC Registry will be a useful resource to assess the long-term durability of AVS root replacement in the future.

  1. Valve Sparing Aortic Root Replacement in Patients with Marfan Syndrome Enrolled in the National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions

    Science.gov (United States)

    Song, Howard K.; Preiss, Liliana R.; Maslen, Cheryl L.; Kroner, Barbara; Devereux, Richard B.; Roman, Mary J.; Holmes, Kathryn W.; Tolunay, H. Eser; Desvigne-Nickens, Patrice; Asch, Federico M.; Milewski, Rita K.; Bavaria, Joseph; LeMaire, Scott A.

    2016-01-01

    Background The long-term outcomes of aortic valve sparing (AVS) root replacement in Marfan syndrome (MFS) patients remain uncertain. We sought to determine the utilization and outcomes of AVS root replacement in MFS patients enrolled in the Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC). Methods At the time of this analysis, 788 patients with MFS were enrolled in the GenTAC Registry, of whom 288 have undergone aortic root replacement. Patients who have undergone AVS procedures were compared to those who have undergone aortic valve replacing (AVR) procedures. Results AVS root replacement was performed in 43.5% of MFS patients and the frequency of AVS increased over the past 5 years. AVS patients were younger at the time of surgery (31.0 vs. 36.3 years, p=0.006) and more likely to have had elective rather than emergency surgery compared to AVR patients. AVR patients were more likely to have had aortic valve dysfunction and aortic dissection as a primary indication for surgery. After mean follow-up of 6.2 (SD=3.6) years, none of the 87 AVS patients have required reoperation; in contrast, after mean follow up of 10.5 (SD=7.6) years, 11.5% of AVR patients have required aortic root reoperation. Aortic valve function has been durable with 95.8% of AVS patients with aortic insufficiency graded as mild or less. Conclusions AVS root replacement is performed commonly in the MFS population. The durability of the aortic repair and aortic valve function have been excellent to date. These results justify the continued use of the procedure in the elective setting. The GenTAC Registry will be a useful resource to assess the long-term durability of AVS root replacement in the future. PMID:25296451

  2. Smooth muscle LDL receptor-related protein-1 deletion induces aortic insufficiency and promotes vascular cardiomyopathy in mice.

    Directory of Open Access Journals (Sweden)

    Joshua E Basford

    Full Text Available Valvular disease is common in patients with Marfan syndrome and can lead to cardiomyopathy. However, some patients develop cardiomyopathy in the absence of hemodynamically significant valve dysfunction, suggesting alternative mechanisms of disease progression. Disruption of LDL receptor-related protein-1 (Lrp1 in smooth muscle cells has been shown to cause vascular pathologies similar to Marfan syndrome, with activation of smooth muscle cells, vascular dysfunction and aortic aneurysms. This study used echocardiography and blood pressure monitoring in mouse models to determine whether inactivation of Lrp1 in vascular smooth muscle leads to cardiomyopathy, and if so, whether the mechanism is a consequence of valvular disease. Hemodynamic changes during treatment with captopril were also assessed. Dilation of aortic roots was observed in young Lrp1-knockout mice and progressed as they aged, whereas no significant aortic dilation was detected in wild type littermates. Diastolic blood pressure was lower and pulse pressure higher in Lrp1-knockout mice, which was normalized by treatment with captopril. Aortic dilation was followed by development of aortic insufficiency and subsequent dilated cardiomyopathy due to valvular disease. Thus, smooth muscle cell Lrp1 deficiency results in aortic dilation and insufficiency that causes secondary cardiomyopathy that can be improved by captopril. These findings provide novel insights into mechanisms of cardiomyopathy associated with vascular activation and offer a new model of valvular cardiomyopathy.

  3. Aortic root reoperations after pulmonary autograft implantation

    NARCIS (Netherlands)

    J.A. Bekkers (Jos); L.M.A. Klieverik (Loes Maria Anne); G. Bol-Raap (Goris); J.J.M. Takkenberg (Hanneke); A.J.J.C. Bogers (Ad)

    2010-01-01

    textabstractObjective: To report the results of aortic root reoperations after pulmonary autograft implantation. Methods: All consecutive patients in our prospective Ross research database were selected for analysis, and additional information for patients requiring reoperation was obtained from the

  4. [Surgical treatment of the aortic root aneurysm related to Marfan syndrome].

    Science.gov (United States)

    Zheng, Si-hong; Sun, Yan-qing; Meng, Xu; Zhang, Hong; Hou, Xiao-tong; Wang, Jian-gang; Gao, Feng

    2005-08-24

    To review the experience of surgical treatment of aortic root aneurysm of Marfan syndrome. We The clinical data of 84 Marfan syndrome patients, 61 males and 23 females, aged 35 +/- 12 (5 - 62), 41 cases presenting with aortic dissection (Debakey type I in 32 cases and type II in 9), 52 cases with moderate to severe aortic regurgitation, and 9 cases with moderate to severe mitral regurgitation, 43 cases with cardiac function of class I - II, 30 with class III and 11 with class IV according the New York Heart Association (NYHA) standard, who underwent surgical treatment for aortic root aneurysm with a mean diameter of 68 mm +/- 14 mm, were analyzed. Bentall procedure was performed in 68 cases, Wheat procedure in 6, Cabrol procedure in 5, and aortic valve replacement and aortoplasty in 5. Concomitant procedures included mitral value replacement and mitral valvuoplasty in 3 cases respectively. Urgent surgery was conducted in 28 cases, and elective operation in 56 cases. There were 3 in-hospital deaths (3.57%). 76 cases were followed up for a mean duration of 55 +/- 31 months. Three patients underwent reoperation. The cardiac function returned to class I - II except for 2 cases that remained at the class III. Bentall procedure should be the first choice of the surgery for aortic root aneurysm of Marfan syndrome with a low mortality and a good late outcome.

  5. Selective Aortic Arch and Root Replacement in Repair of Acute Type A Aortic Dissection.

    Science.gov (United States)

    Fleischman, Fernando; Elsayed, Ramsey S; Cohen, Robbin G; Tatum, James M; Kumar, S Ram; Kazerouni, Kayvan; Mack, Wendy J; Barr, Mark L; Cunningham, Mark J; Hackmann, Amy E; Baker, Craig J; Starnes, Vaughn A; Bowdish, Michael E

    2018-02-01

    Controversy exists regarding the optimal extent of repair for type A aortic dissection. Our approach is to replace the ascending aorta, and only replace the aortic root or arch when intimal tears are present in those areas. We examined intermediate outcomes with this approach to acute type A aortic dissection repair. Between March 2005 and October 2016, 195 patients underwent repair of acute type A aortic dissection. Repair was categorized by site of proximal and distal anastomosis and extent of repair. Mean follow-up was 31.0 ± 30.9 months. Kaplan-Meier analysis was used to assess survival. Multiple variable Cox proportional hazards modeling was utilized to identify factors associated with overall mortality. Overall survival was 85.1%, 83.9%, 79.1%, and 74.4% at 6, 12, 36, and 60 months, respectively. Eight patients required reintervention. The cumulative incidence of aortic reintervention at 1 year with death as a competing outcome was 3.95%. Multiple variable regression analysis identified factors such as age, preoperative renal failure, concomitant thoracic endograft, postoperative myocardial infarction and sepsis, and need for extracorporeal membrane oxygenation as predictive of overall mortality. Neither proximal or distal extent of repair, nor need for reintervention affected overall survival (proximal: hazard ratio 1.63, 95% confidence interval: 0.75 to 3.51, p = 0.22; distal: hazard ratio 1.12, 95% confidence interval: 0.43 to 2.97, p = 0.81; reintervention: hazard ratio 0.03, 95% confidence interval: 0.002 to 0.490, p < 0.01). A selective approach to root and arch repair in acute type A aortic dissection is safe. If aortic reintervention is needed, survival does not appear to be affected. Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  6. Dilatation of the Great Arteries in an Infant with Marfan Syndrome and Ventricular Septal Defect

    Directory of Open Access Journals (Sweden)

    L. Rozendaal

    2011-01-01

    Full Text Available We describe an infant presenting with contractures of the fingers, a large ventricular septal defect (VSD, and severe pulmonary artery dilatation. He had clinical and echocardiographic features of both neonatal or infantile Marfan syndrome (MFS and congenital contractural arachnodactyly. After surgical VSD closure, the aortic root developed progressive dilatation while the size of pulmonary artery returned to normal limits. Eventually the diagnosis of MFS was confirmed by DNA analysis.

  7. Aortic events in a nationwide Marfan syndrome cohort.

    Science.gov (United States)

    Groth, Kristian A; Stochholm, Kirstine; Hove, Hanne; Kyhl, Kasper; Gregersen, Pernille A; Vejlstrup, Niels; Østergaard, John R; Gravholt, Claus H; Andersen, Niels H

    2017-02-01

    Marfan syndrome is associated with morbidity and mortality due to aortic dilatation and dissection. Preventive aortic root replacement has been the standard treatment in Marfan syndrome patients with aortic dilatation. In this study, we present aortic event data from a nationwide Marfan syndrome cohort. The nationwide cohort of Danish Marfan syndrome patients was established from the Danish National Patient Registry and the Cause of Death Register, where we retrieved information about aortic surgery and dissections. We associated aortic events with age, sex, and Marfan syndrome diagnosis prior or after the first aortic event. From the total cohort of 412 patients, 150 (36.4 %) had an aortic event. Fifty percent were event free at age 49.6. Eighty patients (53.3 %) had prophylactic surgery and seventy patients (46.7 %) a dissection. The yearly event rate was 0.02 events/year/patient in the period 1994-2014. Male patients had a significant higher risk of an aortic event at a younger age with a hazard ratio of 1.75 (CI 1.26-2.42, p = 0.001) compared with women. Fifty-three patients (12.9 %) were diagnosed with MFS after their first aortic event which primarily was aortic dissection [n = 44 (83.0 %)]. More than a third of MFS patients experienced an aortic event and male patients had significantly more aortic events than females. More than half of the total number of dissections was in patients undiagnosed with MFS at the time of their event. This emphasizes that diagnosing MFS is lifesaving and improves mortality risk by reducing the risk of aorta dissection.

  8. [Valve-sparing aortic root replacement for young female patients with Marfan syndrome].

    Science.gov (United States)

    Nawata, Kan; Morota, T

    2009-10-01

    Annuloaortic ectasia is frequently related with Marfan syndrome, and Bentall procedure or aortic root replacement with valved conduit has been the conventional standard operation for this disease. Recently, some institutes have adopted valve-sparing aortic root replacement (VSARR) instead of Bentall procedure. Young female patients with Marfan syndrome who wishes for childbearing seem to be a group of good candidates of this type of operation, because it let them free from morbidities after artificial valve implantation. Valve-sparing operation should be taken into consideration when the size of Valsalva sinus reaches 45 mm for patients with Marfan syndrome and when it reaches 40 mm for patients with past histories or family histories of aortic dissection or aortic rupture. Since pregnancy is one of the most serious risk factors for aortic events, Valsalva sinus of 40 mm or larger could be the new standard for surgical indication if VSARR is applicable.

  9. Decision-making in aortic root surgery in Marfan syndrome: bleeding, thromboembolism and risk of reintervention after valve-sparing or mechanical aortic root replacement.

    Science.gov (United States)

    Schoenhoff, Florian S; Langhammer, Bettina; Wustmann, Kerstin; Reineke, David; Kadner, Alexander; Carrel, Thierry

    2015-12-01

    Valve-sparing root replacement (VSRR) is thought to reduce the rate of thromboembolic and bleeding events compared with aortic root replacement using a mechanical aortic root replacement (MRR) with a composite graft by avoiding oral anticoagulation. But as VSRR carries a certain risk for subsequent reinterventions, decision-making in the individual patient can be challenging. Of 100 Marfan syndrome (MFS) patients who underwent 169 aortic surgeries and were followed at our institution since 1995, 59 consecutive patients without a history of dissection or prior aortic surgery underwent elective VSRR or MRR and were retrospectively analysed. VSRR was performed in 29 (David n = 24, Yacoub n = 5) and MRR in 30 patients. The mean age was 33 ± 15 years. The mean follow-up after VSRR was 6.5 ± 4 years (180 patient-years) compared with 8.8 ± 9 years (274 patient-years) after MRR. Reoperation rates after root remodelling (Yacoub) were significantly higher than after the reimplantation (David) procedure (60 vs 4.2%, P = 0.01). The need for reinterventions after the reimplantation procedure (0.8% per patient-year) was not significantly higher than after MRR (P = 0.44) but follow-up after VSRR was significantly shorter (P = 0.03). There was neither significant morbidity nor mortality associated with root reoperations. There were no neurological events after VSRR compared with four stroke/intracranial bleeding events in the MRR group (log-rank, P = 0.11), translating into an event rate of 1.46% per patient-year following MRR. The calculated annual failure rate after VSRR using the reimplantation technique was lower than the annual risk for thromboembolic or bleeding events. Since the perioperative risk of reinterventions following VSRR is low, patients might benefit from VSRR even if redo surgery may become necessary during follow-up. © The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

  10. Factors associated with the development of aortic valve regurgitation over time after two different techniques of valve-sparing aortic root surgery.

    Science.gov (United States)

    Hanke, Thorsten; Charitos, Efstratios I; Stierle, Ulrich; Robinson, Derek; Gorski, Armin; Sievers, Hans-H; Misfeld, Martin

    2009-02-01

    Early results after aortic valve-sparing root reconstruction are excellent. Longer-term follow-up, especially with regard to aortic valve function, is required for further judgment of these techniques. Between July of 1993 and September of 2006, 108 consecutive patients (mean age 53.0 +/- 15.8 years) underwent the Yacoub operation (group Y) and 83 patients underwent the David operation (group D). Innovative multilevel hierarchic modeling methods were used to analyze aortic regurgitation over time. In general, aortic regurgitation increased with time in both groups. Factors associated with the development of a significant increase in aortic regurgitation were Marfan syndrome, concomitant cusp intervention, and preoperative aortic anulus dimension. In Marfan syndrome, the initial aortic regurgitation was higher in group Y versus group D (0.56 aortic regurgitation vs 0.29 aortic regurgitation, P = .049), whereas the mean annual progression rate of aortic regurgitation was marginally higher in group Y (0.132 aortic regurgitation vs 0.075 aortic regurgitation, P = .1). Concomitant cusp intervention was associated with a significant aortic regurgitation increase in both groups (P Marfan syndrome and a large preoperative aortic annulus diameter were better treated with the reimplantation technique, whereas those with a smaller diameter were better treated with the remodeling technique. Concomitant free-edge plication of prolapsing cusps was disadvantageous in both groups. Considering these factors may serve to improve the aortic valve longevity after valve-sparing aortic root surgery.

  11. Aortic root pathology in Marfan syndrome increases the risk of migraine with aura

    DEFF Research Database (Denmark)

    Koppen, H; Vis, J C; Gooiker, D J

    2012-01-01

    To assess the lifetime prevalence of migraine in patients with Marfan syndrome (MFS) and to investigate a history of aortic root replacement (AR) as a possible risk factor.......To assess the lifetime prevalence of migraine in patients with Marfan syndrome (MFS) and to investigate a history of aortic root replacement (AR) as a possible risk factor....

  12. Initial Surgical Experience with Aortic Valve Repair: Clinical and Echocardiographic Results

    Directory of Open Access Journals (Sweden)

    Francisco Diniz Affonso da Costa

    Full Text Available Abstract Introduction: Due to late complications associated with the use of conventional prosthetic heart valves, several centers have advocated aortic valve repair and/or valve sparing aortic root replacement for patients with aortic valve insufficiency, in order to enhance late survival and minimize adverse postoperative events. Methods: From March/2012 thru March 2015, 37 patients consecutively underwent conservative operations of the aortic valve and/or aortic root. Mean age was 48±16 years and 81% were males. The aortic valve was bicuspid in 54% and tricuspid in the remaining. All were operated with the aid of intraoperative transesophageal echocardiography. Surgical techniques consisted of replacing the aortic root with a Dacron graft whenever it was dilated or aneurysmatic, using either the remodeling or the reimplantation technique, besides correcting leaflet prolapse when present. Patients were sequentially evaluated with clinical and echocardiographic studies and mean follow-up time was 16±5 months. Results: Thirty-day mortality was 2.7%. In addition there were two late deaths, with late survival being 85% (CI 95% - 68%-95% at two years. Two patients were reoperated due to primary structural valve failure. Freedom from reoperation or from primary structural valve failure was 90% (CI 95% - 66%-97% and 91% (CI 95% - 69%-97% at 2 years, respectively. During clinical follow-up up to 3 years, there were no cases of thromboembolism, hemorrhage or endocarditis. Conclusions: Although this represents an initial series, these data demonstrates that aortic valve repair and/or valve sparing aortic root surgery can be performed with satisfactory immediate and short-term results.

  13. Experiencia a largo plazo con la operación de Ross en pacientes pediátricos

    Directory of Open Access Journals (Sweden)

    José M. Caffarena Calvar

    2008-07-01

    Conclusions: The Ross and Ross-Konno operations have changed the prognosis of pediatric patients with complex disease of the valve and aortic root. There is growth of the neo-aortic root in young patients. Sinotubular dilatation of the autograft with neoaortic valve insufficiency has been detected in patients with previously dilated aortic root when root replacement technique was performed. The best results are obtained in the younger andlower weight patients with complex left ventricular outflow obstruction without root dilatation. A close follow-up of patients with aortic root dilatation is mandatory to definitely validate the Ross procedure over aortic prosthesis.

  14. Midterm outcome of valve-sparing aortic root replacement in inherited connective tissue disorders.

    Science.gov (United States)

    Tanaka, Hiroshi; Ogino, Hitoshi; Matsuda, Hitoshi; Minatoya, Kenji; Sasaki, Hiroaki; Iba, Yutaka

    2011-11-01

    This study determined the midterm outcome of valve-sparing aortic root replacement for patients with inherited connective tissue disorders. From 1993 to 2008, 94 patients underwent valve-sparing aortic root replacement. Sixty patients (64%), average age 33 years (range, 15 to 61 years), had inherited connective tissue disorders: Marfan syndrome, 54 (92%); Loeys-Dietz syndrome, 5 (8%); and smooth muscle α-actin (ACTA2) mutation in 1. Median preoperative sinus diameter was 52 mm (range, 42 to 76 mm), and moderate/severe aortic regurgitation was present in 14 (23%). Seven (12%, 1993 to 1999) underwent remodeling procedures, and 53 had reimplantation procedures. Cusp repair was performed in 4. Median follow-up was 55 months (range, 1 to 149 months). There were 15 patients in the early term (1993 to 2000) and 45 in the late term (2001 to 2008). Four late deaths occurred (cardiac, 3; aortic, 1), with 10-year survival of 86%. Rates of freedom from aortic valve replacement at 5 and 10 years were 85% and 58% in remodeling and 96% and 58% in reimplantation. Risk factors for reoperations were postprocedure intraoperative aortic insufficiency greater than mild (p = 0.046), remodeling procedure (p = 0.016), and early term (p = 0.0002). One patient (2%) with none/trivial postprocedure aortic insufficiency required aortic valve replacement. Freedom from reoperation in patients with none/trivial postprocedure aortic insufficiency at 5 and 10 years was 100% and 67%. Meticulous control of aortic insufficiency during operation would bring favorable midterm durability in valve-sparing aortic root replacement using a reimplantation technique, even in patients with inherited connective tissue disorders. Copyright © 2011 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  15. Aortic root abscess resulting from endocarditis: spectrum of angiographic findings

    International Nuclear Information System (INIS)

    Miller, S.W.; Dinsmore, R.E.

    1984-01-01

    Abscesses in the aortic root are a serious complication of infective endocarditis and require accurate diagnosis for antibiotic and surgical management. Nineteen cases of endocarditis of a native valve or prosthetic valve and adjacent abscess cavities were identified with angiography. Of 6 patients with endocarditis of a native valve, 5 had bicuspid aortic valves and all had severe aortic regurgitation. Of 13 patients with endocarditis of a prosthetic aortic valve, all had paravalvular regurgitation. Fistulas were detected into the mitral anulus in 8 patients, and into the right ventricle in 3 patients. No complications from the catheterization were recorded during the 48-hour follow-up

  16. Early and medium term results of the sleeve valve-sparing procedure for aortic root ectasia.

    Science.gov (United States)

    Gamba, Amando; Tasca, Giordano; Giannico, Floriana; Lobiati, Elisabetta; Skouse, Douglas; Galanti, Andrea; Martino, Antonello Stefano; Triggiani, Michele

    2015-04-01

    The aim of this retrospective study was to evaluate our experience of using a simplified aortic valve sleeve procedure to treat aortic root ectasia and aneurysms with or without aortic regurgitation. In experienced hands, 2 aortic valve-sparing procedures, ie, Yacoub and David, have yielded excellent long-term results in the treatment of aortic root aneurysms, with or without aortic regurgitation. However, these techniques are demanding and not widely used. Recently, a new and simplified valve-sparing technique, named "sleeve procedure," has been proposed, and has yielded encouraging early results. Ninety consecutive patients with aortic root aneurysms underwent sleeve procedures from October 2006 to October 2012. Follow-up data (clinical 100% complete and echocardiographic 93% complete) were acquired from our outpatient clinic or from the referring cardiologist. The mean age of the patients was 61.5 ± 12.5 years, 79% were male, 16 (18%) had a bicuspid valve, 3 had Marfan syndrome, and 2 had aortic dissection. Over a mean clinical follow-up of 34 ± 19 months, 2 patients died from noncardiac causes and 1 was reoperated on for the recurrence of aortic regurgitation. On follow-up echocardiography after a mean of 18 ± 9 months, aortic regurgitation was absent/negligible, mild or moderate in 62%, 37%, and 1% of patients, respectively, and the diameters of the annulus, Valsalva sinuses, and sinotubular junction were 27.3 + 2.2, 37.0 + 3.4, and 30.6 + 3.1 mm, respectively. Our encouraging early and medium term results suggest that the sleeve procedure is a safe and effective aortic valve-sparing technique for the treatment of aortic root ectasia and aneurysm. However, longer follow-up is needed in order to draw definitive conclusions. Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  17. 5B.05: MARFAN SYNDROME: ASSESSMENT OF AORTIC DISSECTION RISK BY ANALYSIS OF AORTIC VISCOELASTIC PROPERTIES.

    Science.gov (United States)

    Grillo, A; Pini, A; Marelli, S; Gan, L; Giuliano, A; Trifirò, G; Santini, F; Salvi, L; Salvi, P; Viecca, F; Carretta, R; Parati, G

    2015-06-01

    Marfan syndrome is an autosomal dominant genetic disorder characterized by an abnormal fibrillin-1 synthesis. Aortic root dilation and dissection are the main problems affecting patients prognosis in these patients. Their pharmacological prophylaxis with losartan or with a beta-blocker counteracts the aortic root dilation, but a close follow-up is required to assess therapeutic response rate and to identify non-responders. Unfortunately genotype-phenotype studies do not allow to determine the exact risk profile in these patients and there is no reliable method to accurately predict their risk of aortic dissection. Aim of this study was to evaluate non-invasive markers for identification of Marfan patients at higher risk of aortic complications. We studied 187 Marfan patients (identified according to 2010 Revised Ghent Criteria and positive genetic analysis), age 32.3 ± 16.5 yrs (mean ± SD). 52 patients (27.8%) had undergone surgical ascending aorta replacement (David or Bentall procedure). Central pressure curves were recorded by PulsePen tonometer, and the aortic viscoelastic aortic properties were studied by determination of carotid-femoral pulse wave velocity (PWV). With reference to the age related distribution of PWV values in a normal population, defined according to Arterial-Stiffness-Collaboration, PWV mean values in Marfan patients corresponded to 60th percentile in non- operated patients and to the 67th percentile in those operated. Adult Marfan patients (n = 146) generally displayed a low blood pressure, because of the pharmacological prophylaxis, and were compared with a population of 189 adult healthy subjects (81 males), matched by age (38 ± 13 vs 38 ± 16 yrs.), heart rate (64 ± 9 bpm vs 64 ± 11 bpm) and blood pressure (mean BP = 78 ± 9 mmHg vs 79 ± 4 mmHg) values. Average PWV value was higher than in healthy controls (PWV = 7.0 ± 1.7) both in not operated (PWV = 7.6 ± 1.6; p = 0

  18. Resveratrol Inhibits Aortic Root Dilatation in the Fbn1C1039G/+ Marfan Mouse Model

    NARCIS (Netherlands)

    Hibender, Stijntje; Franken, Romy; van Roomen, Cindy; ter Braake, Anique; van der Made, Ingeborg; Schermer, Edith E.; Gunst, Quinn; van den Hoff, Maurice J.; Lutgens, Esther; Pinto, Yigal M.; Groenink, Maarten; Zwinderman, Aeilko H.; Mulder, Barbara J. M.; de Vries, Carlie J. M.; de Waard, Vivian

    2016-01-01

    Marfan syndrome (MFS) is a connective tissue disorder caused by mutations in the fibrillin-1 gene. Patients with MFS are at risk of aortic aneurysm formation and dissection. Usually, blood pressure-lowering drugs are used to reduce aortic events; however, this is not sufficient for most patients. In

  19. Mid-term follow-up of aortic root remodelling compared to Bentall operation.

    Science.gov (United States)

    Bassano, C; De Matteis, G M; Nardi, P; Buratta, M M; Zeitani, J; De Paulis, R; Chiariello, L

    2001-05-01

    Aortic valve sparing with root remodelling has proven useful in cases of aortic regurgitation secondary to ascending aorta disease. An excessive rate of re-operation for recurrent aortic regurgitation after this conservative approach might compensate the prosthesis-related risk of the Bentall operation. From January 1995 to September 2000, 69 consecutive patients with aortic expansive aneurysm and concomitant aortic valve disease, were submitted to the Bentall operation (group A, n=37) in the presence of an abnormal valve, or to root remodelling (group B, n=32) in cases of secondary aortic incompetence. One patient in group A and four in group B had Marfan syndrome. The follow-up was 1021 patient-months (range, 1-68 months) in group A and 926 in group B (1-64 months). The event-free survival was calculated using the Kaplan-Meier method, and the difference between curves was evaluated using the Mantel-Cox log-rank test. The operative mortality was 5% in group A and 0% in group B. One patient died at follow-up in group A and none in group B. Four patients (three Marfan) in group B were re-operated on because of recurrent aortic regurgitation. The 5-year event-free survival was 88+/-7% in group A and 82+/-8% in group B (P=0.58). Early residual aortic regurgitation remained stable over time only in patients with good early results. Mid-term follow-up failed to reveal statistically significant differences in the clinical outcome between remodelling and the Bentall operation. Our results support the widespread use of root remodelling, provided that an indication to this conservative approach is achieved after careful, case-by-case evaluation. A good early operative result is likely to remain stable over time.

  20. Coronary artery narrowing after aortic root reconstruction with resorcin-formalin glue.

    Science.gov (United States)

    Martinelli, L; Graffigna, A; Guarnerio, M; Bonmassari, R; Disertori, M

    2000-11-01

    Severe stenosis of right and left main coronary artery ostia developed after aortic root reconstruction with gelatin-resorcin-formol glue for correction of acute type A aortic dissection. Surgical treatment of this condition required grafting of the right and left anterior descending arteries with bilateral mammary arteries on the beating heart.

  1. Current role of endovascular therapy in Marfan patients with previous aortic surgery

    Directory of Open Access Journals (Sweden)

    Ibrahim Akin

    2008-02-01

    Full Text Available Ibrahim Akin, Stephan Kische, Tim C Rehders, Tushar Chatterjee, Henrik Schneider, Thomas Körber, Christoph A Nienaber, Hüseyin InceDepartment of Medicine, Division of Cardiology at the University Hospital Rostock, Rostock School of Medicine, Ernst-Heydemann-Str. 6, 18057 Rostock, GermanyAbstract: The Marfan syndrome is a heritable disorder of the connective tissue which affects the cardiovascular, ocular, and skeletal system. The cardiovascular manifestation with aortic root dilatation, aortic valve regurgitation, and aortic dissection has a prevalence of 60% to 90% and determines the premature death of these patients. Thirty-four percent of the patients with Marfan syndrome will have serious cardiovascular complications requiring surgery in the first 10 years after diagnosis. Before aortic surgery became available, the majority of the patients died by the age of 32 years. Introduction in the aortic surgery techniques caused an increase of the 10 year survival rate up to 97%. The purpose of this article is to give an overview about the feasibility and outcome of stent-graft placement in the descending thoracic aorta in Marfan patients with previous aortic surgery.Keywords: Marfan syndrome, aortic dissection, root replacement, stent-graft, previous aortic surgery

  2. Feature-based US to CT registration of the aortic root

    Science.gov (United States)

    Lang, Pencilla; Chen, Elvis C. S.; Guiraudon, Gerard M.; Jones, Doug L.; Bainbridge, Daniel; Chu, Michael W.; Drangova, Maria; Hata, Noby; Jain, Ameet; Peters, Terry M.

    2011-03-01

    A feature-based registration was developed to align biplane and tracked ultrasound images of the aortic root with a preoperative CT volume. In transcatheter aortic valve replacement, a prosthetic valve is inserted into the aortic annulus via a catheter. Poor anatomical visualization of the aortic root region can result in incorrect positioning, leading to significant morbidity and mortality. Registration of pre-operative CT to transesophageal ultrasound and fluoroscopy images is a major step towards providing augmented image guidance for this procedure. The proposed registration approach uses an iterative closest point algorithm to register a surface mesh generated from CT to 3D US points reconstructed from a single biplane US acquisition, or multiple tracked US images. The use of a single simultaneous acquisition biplane image eliminates reconstruction error introduced by cardiac gating and TEE probe tracking, creating potential for real-time intra-operative registration. A simple initialization procedure is used to minimize changes to operating room workflow. The algorithm is tested on images acquired from excised porcine hearts. Results demonstrate a clinically acceptable accuracy of 2.6mm and 5mm for tracked US to CT and biplane US to CT registration respectively.

  3. Valve-sparing operation for aortic root aneurysm in patients with Marfan syndrome.

    Science.gov (United States)

    Wang, R; Ma, W G; Tian, L X; Sun, L Z; Chang, Q

    2010-03-01

    We report our experience with aortic valve-sparing procedures in patients with Marfan syndrome and aortic root aneurysm. Between August 2003 and July 2007, we performed aortic valve-sparing procedures in 20 patients with aortic root aneurysm resulting from Marfan syndrome. Mean age was 28 +/- 10 years (range, 10 to 57 years), and there were 9 females and 11 males. A reimplantation technique was used in 9 cases, a remodeling technique in 8 and a patch technique in 3 cases. Additional procedures included total aorta replacement in 1 patient, and aortic arch replacement plus stented elephant trunk in 2 patients. The mean follow-up time was 46 +/- 16 months (range, 17 to 64 months). No in-hospital or late death occurred. Reexploration for bleeding was required in one case on postoperative day 1. No valve-related complications occurred during the follow-up period. At the end of follow-up, trivial or no aortic regurgitation was demonstrated in 14 patients, mild in 4 patients, moderate in 1 and severe in 1. Two patients with moderate and severe aortic regurgitation required reoperation. The early and mid-term results of the valve-sparing operations were favorable, and the durability of the preserved valve should encourage use of this technique in patients with Marfan syndrome.

  4. Aortic root geometry in aortic stenosis patients (a SEAS substudy)

    DEFF Research Database (Denmark)

    Bahlmann, Edda; Nienaber, Christoph A; Cramariuc, Dana

    2011-01-01

    -specified requirements for the aortic root geometry for current available prostheses, CoreValve and Edwards-Sapien. The ratio of sinus of Valsalva height to sinus width was 1:2. In multivariate linear regression analysis, larger sinus of Valsalva height was associated with older age, larger sinus of Valsalva diameter......, lower ejection fraction and smaller supracoronary diameter (multiple R(2) = 0.19, PSapien prosthesis in 66.9%. Overall, annular dimension feasible for TAVI using any available prosthesis was found...

  5. Valve-sparing aortic root replacement in Marfan syndrome.

    Science.gov (United States)

    Cameron, Duke E; Vricella, Luca A

    2005-01-01

    Marfan syndrome is the most common inherited connective tissue disorder, affecting approximately 1 in 10,000 live births. The cardinal features of Marfan syndrome are the abnormalities of the skeleton (tall stature, arachnodactyly, and joint hyperelasticity), eye (lens subluxation), and aorta (root aneurysm with proclivity toward rupture and dissection). Aortic catastrophe accounts for most of the premature mortality among Marfan patients, a risk that climbs steeply during adolescence and results in death of half of Marfan patients by the age of 40 years. Most of the improvement in life expectancy that has been achieved in Marfan syndrome is attributable to early recognition of aortic root aneurysms and prophylactic replacement with composite grafts (mechanical valve prostheses within Dacron conduits) before rupture or dissection occurs. Despite the excellent early and late results with composite grafts, there has been growing interest in operative procedures that replace the sinuses but preserve the aortic valve leaflets, to avoid anticoagulation and minimize the risk of prosthesis-related endocarditis. These procedures are still in evolution and late results are not yet known, but as with mitral repair in the setting of myxomatous disease, valve-sparing procedures in Marfan syndrome have weathered a storm of initial criticism and skepticism and are steadily gaining acceptance.

  6. Comparison of aortic root replacement in patients with Marfan syndrome.

    Science.gov (United States)

    Bernhardt, Alexander M J; Treede, Hendrik; Rybczynski, Meike; Sheikzadeh, Sara; Kersten, Jan F; Meinertz, Thomas; von Kodolitsch, Yskert; Reichenspurner, Hermann

    2011-11-01

    Although the aortic-valve-sparing (AVS) reimplantation technique according to David has shown favorable durability results in mid-term and long-term studies, composite valve grafting (CVG) according to Bentall is still considered the standard procedure. Retrospectively, we evaluated the results of aortic root replacement of patients with Marfan syndrome (MFS) who underwent surgery between January 1995 and January 2010. MFS was diagnosed using the Ghent criteria. AVS was used in 58 patients and CVG in 30 patients with MFS. AVS was done for aortic-root aneurysm (n=48) or aortic dissection type A (n=10). CVG was used for aortic-root aneurysm in 14 patients or aortic dissection type A in 16 patients. The mean follow-up was 3.2 (95% CI: 2.4-4.2) years. In both groups, 30-day mortality was 0%. Three patients (10.0%) in the CVG group required resternotomy for postoperative bleeding versus two patients (3.4%) in the AVS group (p=0.3). At follow-up, mortality was 10% in the CVG group versus 3.4% in the AVS group (p=0.3). Re-operation was required in two patients (3.4%) after AVS and in three patients after CVG (10%) (p=0.3). Three patients (10.0%) who underwent CVG had endocarditis and two patients (6.7%) had a stroke during follow-up, whereas no endocarditis and stroke occurred after AVS. After 14 years, stratified event-free survival was better in the AVS group (event-free survival was 82.3% vs 58.6%, log-rank test p=0.086), especially after aneurysm (p=0.057). After 10 years, freedom from aortic regurgitation ≥II° in the AVS group was 80% for aneurysm and 50% after dissection (p=0.524). The reimplantation technique according to David was associated with excellent survival, good valve function and a low rate of re-operation, endocarditis, and stroke. There was a trend to better event-free survival for AVS patients making it the procedure of choice in MFS patients. Copyright © 2011 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights

  7. Computed tomography of cystic nerve root sleeve dilatation.

    Science.gov (United States)

    Neave, V C; Wycoff, R R

    1983-10-01

    A case of cystic nerve root sleeve dilatation in the lumbar area associated with a chronic back pain syndrome is presented. Prominent computed tomography (CT) findings include: (a) rounded masses in the region of the foramina isodense with cerebrospinal fluid in the subarachnoid space; (b) associated asymmetry of epidural fat distribution; (c) enlargement of the neural foramina in axial sections with scalloped erosion of the adjacent posteriolateral vertebral body, pedicle, and pedicular-laminar junction with preservation of cortex and without bony sclerosis or infiltrative appearance; (d) prominent or ectatic dural sac with lack of usual epidural landmarks between the sac and vertebral body; and (e) multilevel abnormalities throughout the entire lumbar region. Myelographic and CT correlations are demonstrated with a review of the literature. A discussion of the various cystic abnormalities involving nerve root sheaths is undertaken in an attempt to clarify the confusing nomenclature applied to nerve root sleeve pathology.

  8. Aortic Root Replacement for Children With Loeys-Dietz Syndrome.

    Science.gov (United States)

    Patel, Nishant D; Alejo, Diane; Crawford, Todd; Hibino, Narutoshi; Dietz, Harry C; Cameron, Duke E; Vricella, Luca A

    2017-05-01

    Loeys-Dietz syndrome (LDS) is an aggressive aortopathy with a proclivity for aortic aneurysm rupture and dissection at smaller diameters than other connective tissue disorders. We reviewed our surgical experience of children with LDS to validate our guidelines for prophylactic aortic root replacement (ARR). We reviewed all children (younger than 18 years) with a diagnosis of LDS who underwent ARR at our institution. The primary endpoint was mortality, and secondary endpoints included complications and the need for further interventions. Thirty-four children with LDS underwent ARR. Mean age at operation was 10 years, and 15 (44%) were female. Mean preoperative root diameter was 4 cm. Three children (9%) had composite ARR with a mechanical prosthesis, and 31 (91%) underwent valve-sparing ARR. Concomitant procedures included arch replacement in 2 (6%), aortic valve repair in 1 (3%), and patent foramen ovale closure in 16 (47%). There was no operative mortality. Two children (6%) required late replacement of the ascending aorta, 5 (15%) required arch replacement, 1 (3%) required mitral valve replacement, and 2 (6%) had coronary button aneurysms/pseudoaneurysms requiring repair. Three children required redo valve-sparing ARR after a Florida sleeve procedure, and 2 had progressive aortic insufficiency requiring aortic valve replacement after a valve-sparing procedure. There were 2 late deaths (6%). These data confirm the aggressive aortopathy of LDS. Valve-sparing ARR should be performed when feasible to avoid the risks of prostheses. Serial imaging of the arterial tree is critical, given the rate of subsequent intervention. Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  9. Risk Factors for Late Aortic Valve Dysfunction After the David V Valve-Sparing Root Replacement.

    Science.gov (United States)

    Esaki, Jiro; Leshnower, Bradley G; Binongo, Jose N; Lasanajak, Yi; McPherson, LaRonica; Guyton, Robert A; Chen, Edward P

    2017-11-01

    Valve-sparing root replacement (VSRR) is an established therapy for aortic root pathology. However, late aortic valve dysfunction requiring reoperation remains a primary concern of this procedure. This study examines risk factors for late aortic insufficiency (AI) and aortic stenosis (AS) after David V VSRR. A retrospective review from 2005 to 2015 at a US academic center identified 282 patients who underwent VSRR. Cox proportional hazards regression analysis was used to identify risk factors for late AI and AS after VSRR. The mean age was 46.4 years. Sixty-four patients (22.7%) had bicuspid valves, and 41 patients (14.5%) had Marfan syndrome. The incidence of reoperations was 27 (9.6%), and 42 cases (14.9%) presented with acute type A dissection. Operative mortality was 8 (2.8%). Seven-year survival was 90.9%. Seven-year cumulative incidence of reoperation, greater than 2+ AI and greater than moderate AS were 3.1%, 2.2%, and 0.8%, respectively. Multivariable analysis showed aortic root size 55 mm or larger (hazard ratio 3.44, 95% confidence interval: 1.27 to 9.29, p = 0.01) to be a risk factor for late AI whereas bicuspid valve (hazard ratio 16.07, 95% confidence interval: 3.12 to 82.68, p = 0.001) and cusp repair were found to be risk factors (hazard ratio 5.91, 95% confidence interval: 1.17 to 29.86, p = 0.03) for late AS. Valve-sparing root replacement can be performed with low operative risk and good overall long-term survival even in complex clinical settings. Durable valve function can be expected; however, aortic root size 55 cm or more, bicuspid valve anatomy, and cusp repair represent independent risk factors for late aortic valve dysfunction after these procedures. Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  10. Numerical investigation on effect of aortic root geometry on flow induced structural stresses developed in a bileaflet mechanical heart valve

    Science.gov (United States)

    Abbas, S. S.; Nasif, M. S.; Said, M. A. M.; Kadhim, S. K.

    2017-10-01

    Structural stresses developed in an artificial bileaflet mechanical heart valve (BMHV) due to pulsed blood flow may cause valve failure due to yielding. In this paper, von-Mises stresses are computed and compared for BMHV placed in two types of aortic root geometries that are aortic root with axisymmetric sinuses and with axisymmetric bulb, at different physiological blood flow rates. With BMHV placed in an aortic root with axisymmetric sinuses, the von-Mises stresses developed in the valve were found to be up to 47% higher than BMHV placed in aortic root with axisymmetric bulb under similar physiological conditions. High velocity vectors and therefore high von-Mises stresses have been observed for BMHV placed in aortic root with axisymmetric sinuses, that can lead to valve failure.

  11. Survival and freedom from aortic valve-related reoperation after valve-sparing aortic root replacement in 1015 patients.

    Science.gov (United States)

    Kari, Fabian A; Doll, Kai-Nicolas; Hemmer, Wolfgang; Liebrich, Markus; Sievers, Hans-Hinrich; Richardt, Doreen; Reichenspurner, Hermann; Detter, Christian; Siepe, Matthias; Czerny, Martin; Beyersdorf, Friedhelm

    2016-04-01

    The aim of this study was to characterize mortality and aortic valve replacement after valve-sparing aortic root replacement (V-SARR) in a multicentre cohort. Between 1994 and 2014, 1015 patients had V-SARR with (n = 288, 28%) or without cusp/commissure repair (n = 727, 72%) at the centres of Lübeck (n = 343, 34%), Stuttgart (n = 346, 34%), Hamburg (n = 109, 11%) and Freiburg (n = 217, 21%), Germany. Comparative survival of an age- and gender-matched general population was calculated. Log-rank tests and multiple logistic regression were used to identify risk factors. The mean follow-up was 5.2 ± 3.9 years. Cumulative follow-up comprised 2933 patient-years. Early survival was 98%. NYHA status and aneurysm size were predictive of death during mid-term follow-up (P = 0.025). Freedom from aortic valve replacement was 90% at 8 years, with the type of V-SARR (root remodelling, David II) being a risk factor (P = 0.015). Bicuspid aortic valve (P = 0.26) and initial valve function (P = 0.4) did not impact reoperation. The need of additional valve repair (cusps/commissures) was not linked to reoperation: freedom from aortic valve replacement at 8 years was 84% if cusp repair was performed versus 90% if V-SARR alone was performed (P = 0.218). Marfan syndrome had no impact on survival or on aortic valve replacement. Mid-term survival of patients after V-SARR is comparable with that of a matched general population. The regurgitant bicuspid aortic valve is a favourable substrate for V-SARR. Prophylactic surgery should be performed before symptoms or large aneurysms are present to achieve optimal mid-term outcomes. © The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

  12. Dietary fatty acids on aortic root calcification in mice with metabolic syndrome.

    Science.gov (United States)

    Naranjo, Maria C; Bermudez, Beatriz; Garcia, Indara; Lopez, Sergio; Abia, Rocio; Muriana, Francisco J G; Montserrat-de la Paz, Sergio

    2017-04-19

    Metabolic syndrome (MetS) is associated with obesity, dyslipidemia, type 2 diabetes, and chronic low-grade inflammation. The aim of this study was to determine the role of high-fat low-cholesterol diets (HFLCDs) rich in SFAs (HFLCD-SFAs), MUFAs (HFLCD-MUFAs) or MUFAs plus omega-3 long-chain PUFAs (HFLCD-PUFAs) on vascular calcification by the modulation of the RANKL/RANK/OPG system in the aortic roots of Lep ob/ob LDLR -/- mice. Animals fed with HFLCD-SFAs had increased weight and a greater atheroma plaque size, calcification, and RANKL/CATHK expression in the aortic root than mice on MUFA-enriched diets, with an increasing OPG expression in the aortic roots of the latter. Our study demonstrates that compared to dietary SFAs, MUFAs from olive oil protect against atherosclerosis by interfering with vascular calcification via the RANKL/RANK/OPG system in the setting of MetS. These findings open opportunities for developing novel nutritional strategies with olive oil as the most important dietary source of MUFAs (notably oleic acid) to prevent cardiovascular complications in MetS.

  13. Sex, pregnancy and aortic disease in Marfan syndrome.

    Science.gov (United States)

    Renard, Marjolijn; Muiño-Mosquera, Laura; Manalo, Elise C; Tufa, Sara; Carlson, Eric J; Keene, Douglas R; De Backer, Julie; Sakai, Lynn Y

    2017-01-01

    Sex-related differences as well as the adverse effect of pregnancy on aortic disease outcome are well-established phenomena in humans with Marfan syndrome (MFS). The underlying mechanisms of these observations are largely unknown. In an initial (pilot) step we aimed to confirm the differences between male and female MFS patients as well as between females with and without previous pregnancy. We then sought to evaluate whether these findings are recapitulated in a pre-clinical model and performed in-depth cardiovascular phenotyping of mutant male and both nulliparous and multiparous female Marfan mice. The effect of 17β-estradiol on fibrillin-1 protein synthesis was compared in vitro using human aortic smooth muscle cells and fibroblasts. Our small retrospective study of aortic dimensions in a cohort of 10 men and 20 women with MFS (10 pregnant and 10 non-pregnant) confirmed that aortic root growth was significantly increased in the pregnant group compared to the non-pregnant group (0.64mm/year vs. 0.12mm/year, p = 0.018). Male MFS patients had significantly larger aortic root diameters compared to the non-pregnant and pregnant females at baseline and follow-up (p = 0.002 and p = 0.007, respectively), but no significant increase in aortic root growth was observed compared to the females after follow-up (p = 0.559 and p = 0.352). In the GT-8/+ MFS mouse model, multiparous female Marfan mice showed increased aortic diameters when compared to nulliparous females. Aortic dilatation in multiparous females was comparable to Marfan male mice. Moreover, increased aortic diameters were associated with more severe fragmentation of the elastic lamellae. In addition, 17β-estradiol was found to promote fibrillin-1 production by human aortic smooth muscle cells. Pregnancy-related changes influence aortic disease severity in otherwise protected female MFS mice and patients. There may be a role for estrogen in the female sex protective effect.

  14. Prognostic Implications of Raphe in Bicuspid Aortic Valve Anatomy.

    Science.gov (United States)

    Kong, William K F; Delgado, Victoria; Poh, Kian Keong; Regeer, Madelien V; Ng, Arnold C T; McCormack, Louise; Yeo, Tiong Cheng; Shanks, Miriam; Parent, Sarah; Enache, Roxana; Popescu, Bogdan A; Liang, Michael; Yip, James W; Ma, Lawrence C W; Kamperidis, Vasileios; van Rosendael, Philippe J; van der Velde, Enno T; Ajmone Marsan, Nina; Bax, Jeroen J

    2017-03-01

    Little is known about the association between bicuspid aortic valve (BAV) morphologic findings and the degree of valvular dysfunction, presence of aortopathy, and complications, including aortic valve surgery, aortic dissection, and all-cause mortality. To investigate the association between BAV morphologic findings (raphe vs nonraphe) and the degree of valve dysfunction, presence of aortopathy, and prognosis (including need for aortic valve surgery, aortic dissection, and all-cause mortality). In this large international multicenter registry of patients with BAV treated at tertiary referral centers, 2118 patients with BAV were evaluated. Patients referred for echocardiography from June 1, 1991, through November 31, 2015, were included in the study. Clinical and echocardiographic data were analyzed retrospectively. The morphologic BAV findings were categorized according to the Sievers and Schmidtke classification. Aortic valve function was divided into normal, regurgitation, or stenosis. Patterns of BAV aortopathy included the following: type 1, dilation of the ascending aorta and aortic root; type 2, isolated dilation of the ascending aorta; and type 3, isolated dilation of the sinus of Valsalva and/or sinotubular junction. Association between the presence and location of raphe and the risk of significant (moderate and severe) aortic valve dysfunction and aortic dilation and/or dissection. Of the 2118 patients (mean [SD] age, 47 [18] years; 1525 [72.0%] male), 1881 (88.8%) had BAV with fusion raphe, whereas 237 (11.2%) had BAV without raphe. Bicuspid aortic valves with raphe had a significantly higher prevalence of valve dysfunction, with a significantly higher frequency of aortic regurgitation (622 [33.1%] vs 57 [24.1%], P < .001) and aortic stenosis (728 [38.7%] vs 51 [21.5%], P < .001). Furthermore, aortic valve replacement event rates were significantly higher among patients with BAV with raphe (364 [19.9%] at 1 year, 393 [21.4%] at 2 years, and 447

  15. Type A aortic dissection associated with Dietzia maris.

    Science.gov (United States)

    Reyes, Guillermo; Navarro, José-Luis; Gamallo, Carlos; delas Cuevas, María-Carmen

    2006-10-01

    Aortitis is a rare cause of aortic dissection. We report the unusual presentation of a 77-year-old male patient who underwent emergency surgery for an aortic dissection type A. A purulent pericardial fluid and inflammatory aorta were found after chest opening. Several samples were sent for analysis. The ascending aorta presented a mild dilatation with a large haematoma infiltrating the aortic root. The distal part of the ascending aorta seemed unaffected. The aortic rupture was found one centimetre above the non-coronary cusp. Aortic wall tissues were extremely fragile and with an inflammatory aspect. The patient died in the theatre room. In the histological study one out of three fragments of ascending aorta displayed longitudinal splitting of the outer media, with blood extravasation in the adventitial layer. In this level, the presence of a detritus material that reminded of bacterial colonies was noteworthy, together with abundant fibrinous exudates. In the laboratory a new specimen, Dietzia maris, was found in the pericardial liquid and in the aortic wall. We believe that this is the first reported finding of Dietzia maris in a patient with aortic disease.

  16. Prediction of optimal deployment projection for transcatheter aortic valve replacement: angiographic 3-dimensional reconstruction of the aortic root versus multidetector computed tomography.

    OpenAIRE

    Binder Ronald K; Leipsic Jonathon; Wood David; Moore Teri; Toggweiler Stefan; Willson Alex; Gurvitch Ronen; Freeman Melanie; Webb John G

    2012-01-01

    BACKGROUND Identifying the optimal fluoroscopic projection of the aortic valve is important for successful transcatheter aortic valve replacement (TAVR). Various imaging modalities including multidetector computed tomography (MDCT) have been proposed for prediction of the optimal deployment projection. We evaluated a method that provides 3 dimensional angiographic reconstructions (3DA) of the aortic root for prediction of the optimal deployment angle and compared it with MDCT. METHODS AND RES...

  17. Reference Values of Aortic Root in Male and Female White Elite Athletes According to Sport.

    Science.gov (United States)

    Boraita, Araceli; Heras, Maria-Eugenia; Morales, Francisco; Marina-Breysse, Manuel; Canda, Alicia; Rabadan, Manuel; Barriopedro, Maria-Isabel; Varela, Amai; de la Rosa, Alejandro; Tuñón, José

    2016-10-01

    There is limited information regarding the aortic root upper physiological limits in all planes in elite athletes according to static and dynamic cardiovascular demands and sex. A cross-sectional study was performed in 3281 healthy elite athletes (2039 men and 1242 women) aged 23.1±5.7 years, with body surface area of 1.9±0.2 m 2 and 8.9±4.9 years and 19.2±9.6 hours/week of training. Maximum end-diastolic aortic root diameters were measured in the parasternal long axis by 2-dimensional echocardiography. Age, left ventricular mass, and body surface area were the main predictors of aortic dimensions. Raw values were greater in males than in females (Pvalues >40 mm and 34 mm, respectively. Raw and corrected aortic measures at all levels were significantly greater in sports, with a high dynamic component in both sexes, except for corrected values of the sinotubular junction in women. Aortic root dimensions in healthy elite athletes are within the established limits for the general population. This study describes the normal dimensions for healthy elite athletes classified according to sex and dynamic and static components of their sports. © 2016 American Heart Association, Inc.

  18. Personalised External Aortic Root Support (PEARS Compared with Alternatives for People with Life-Threatening Genetically Determined Aneurysms of the Aortic Root

    Directory of Open Access Journals (Sweden)

    Tom Treasure

    2015-01-01

    Full Text Available Personalised external aortic support was first proposed in 2000 by Tal Golesworthy, an engineer with familial Marfan syndrome and an aortic root aneurysm. After putting together a research and development team, and finding a surgeon to take on the challenge to join him in this innovative approach, he was central to the manufacture of the device, custom made for his own aorta. He was the patient for the ‘first in man’ operation in 2004. Ten years later he is well and 45 other people have had their own personalised device implanted. In this account, the stepwise record of proof of principle, comparative quantification of the surgical and perioperative requirements, 10 years of results, and development and research plans for the future are presented.

  19. Aortic valve replacement with 17-mm St. Jude Medical prostheses for a small aortic root in elderly patients.

    Science.gov (United States)

    Takaseya, Tohru; Kawara, Takemi; Tokunaga, Shigehiko; Kohno, Michitaka; Oishi, Yasuhisa; Morita, Shigeki

    2007-06-01

    There are few reports about the optimal prosthesis for elderly patients who have small aortic roots with an aortic annular size of less than 19 mm. From October 2004 to October 2006, 11 women aged 70 years or older (mean age, 74.9 +/- 3.5 years) underwent aortic valve replacement with a 17-mm Regent prosthesis (St. Jude Medical, St. Paul, MN), with the size determined using the manufacturer's sizer. Clinical status and the results of preoperative and postoperative echocardiography were evaluated. The patients had a mean body surface area of 1.33 +/- 0.13 m2. Preoperative average New York Heart Association (NYHA) functional class was 2.5 +/- 0.7. Preoperative echocardiography showed a mean indexed effective orifice area of 0.33 +/- 0.14 cm2/m2 and a left ventricular mass index of 175 +/- 63 g/m2. Seven patients underwent associated procedures. Postoperative ventilation time was 14 +/- 5.6 hours, and the intensive care unit stay was 1.0 +/- 0.4 days. No patients died perioperatively. The NYHA functional class improved to class I in 9 patients. No obstruction of valve opening was observed. A significant increase in the mean indexed effective orifice area (0.87 +/- 0.10 cm2/m2) and regression of left ventricular mass index were found (114 +/- 46 cm2/m2) on postoperative echocardiography. Aortic valve replacement in elderly patients with small aortic roots (less than 19 mm) using a 17-mm Regent prosthesis showed satisfactory clinical and hemodynamic results.

  20. Acute Type II Aortic Dissection with Severe Aortic Regurgitation and Chronic Descending Aortic Dissection in Pregnant Patient with Marfan Syndrome

    OpenAIRE

    Lee, Seok-Soo; Jung, Tae-Eun; Lee, Dong Hyup

    2012-01-01

    Aortic dilatation and dissection are severe complications during pregnancy that can be fatal to both the mother and the fetus. The risks of these complications are especially high in pregnant patients with Marfan syndrome; however, incidents of descending aortic dissection are very rare. This case report involves a successful Bentall procedure for and recovery from a rare aortic dissection in a pregnant Marfan patient who developed acute type II aortic dissection with severe aortic regurgitat...

  1. Landmark lecture: Perloff lecture: Tribute to Professor Joseph Kayle Perloff and lessons learned from him: aortopathy in adults with CHD.

    Science.gov (United States)

    Niwa, Koichiro

    2017-12-01

    Marfan syndrome, bicuspid aortic valve, and/or coarctation of the aorta are associated with medial abnormalities of the ascending aortic or para-coarctation aorta. Medial abnormalities in the ascending aorta are prevalent in other type of patients with a variety of CHDs such as single ventricle, persistent truncus arteriosus, transposition of the great arteries, hypoplastic left heart syndrome, and tetralogy of Fallot, encompassing a wide age range and may predispose to dilatation, aneurysm, and rapture necessitating aortic valve and root surgery. These CHDs exhibit ongoing dilatation of the aortic root and reduced aortic elasticity and increased aortic stiffness that may relate to intrinsic properties of the aortic root. These aortic dilatation and increased stiffness can induce aortic aneurysm, rapture of the aorta, and aortic regurgitation, but also provoke left ventricular hypertrophy, reduced coronary artery flow, and left ventricular failure. Therefore, a new clinical entity can be used to call this association of aortic pathophysiological abnormality, aortic dilation, and aorto-left ventricular interaction - "aortopathy".

  2. Left Ventricular Assist Device Implantation with Concomitant Aortic Valve and Ascending Aortic Replacement.

    Science.gov (United States)

    Huenges, Katharina; Panholzer, Bernd; Cremer, Jochen; Haneya, Assad

    2018-01-01

    Left ventricular assist device (LVAD) is nowadays a routine therapy for patients with advanced heart failure. We present the case of a 74-year-old male patient who was admitted to our center with terminal heart failure in dilated cardiomyopathy and ascending aortic aneurysm with aortic valve regurgitation. The LVAD implantation with simultaneous aortic valve and supracoronary ascending aortic replacement was successfully performed.

  3. Surgery of the aortic root: should we go for the valve-sparing root reconstruction or the composite graft-valve replacement is still the first choice of treatment for these patients?

    Science.gov (United States)

    Lamana, Fernando de Azevedo; Dias, Ricardo Ribeiro; Duncan, Jose Augusto; Faria, Leandro Batisti de; Malbouisson, Luiz Marcelo Sa; Borges, Luciano de Figueiredo; Mady, Charles; Jatene, Fábio Biscegli

    2015-01-01

    To compare the results of the root reconstruction with the aortic valve-sparing operation versus composite graft-valve replacement. From January 2002 to October 2013, 324 patients underwent aortic root reconstruction. They were 263 composite graft-valve replacement and 61 aortic valve-sparing operation (43 reimplantation and 18 remodeling). Twenty-six percent of the patients were NYHA functional class III and IV; 9.6% had Marfan syndrome, and 12% had bicuspid aortic valve. There was a predominance of aneurysms over dissections (81% vs. 19%), with 7% being acute dissections. The complete follow-up of 100% of the patients was performed with median follow-up time of 902 days for patients undergoing composite graft-valve replacement and 1492 for those undergoing aortic valve-sparing operation. In-hospital mortality was 6.7% and 4.9%, respectively for composite graft-valve replacement and aortic valve-sparing operation (ns). During the late follow-up period, there was 0% moderate and 15.4% severe aortic regurgitation, and NYHA functional class I and II were 89.4% and 94%, respectively for composite graft-valve replacement and aortic valve-sparing operation (ns). Root reconstruction with aortic valve-sparing operation showed lower late mortality (P=0.001) and lower bleeding complications (P=0.006). There was no difference for thromboembolism, endocarditis, and need of reoperation. The aortic root reconstruction with preservation of the valve should be the operation being performed for presenting lower late mortality and survival free of bleeding events.

  4. Valve-sparing aortic root repair in acute type A dissection: how many sinuses have to be repaired for curative surgery?

    Science.gov (United States)

    Urbanski, Paul P; Hijazi, Husam; Dinstak, Witold; Diegeler, Anno

    2013-09-01

    The aim of the study was to evaluate operative and long-term results of valve-sparing aortic root surgery in acute type A dissection. The repair consisted of selective replacement of all dissected and pathological sinuses. Forty-six patients (mean age 62 ± 14; range 29-88 years, 3 with Marfan syndrome), operated on between August 2001 and July 2011 due to acute type A aortic dissection, underwent valve-sparing root repair, resulting in a valve preservation rate of 56% in acute aortic dissection surgery involving the aortic root. Insufficiency grades of 0/1+, 2+, 3+ and 4+ were presented in 16, 17, 12 and 1 patients, respectively. Root repair with resection of the whole of the pathological aortic wall without the use of any glue was performed in all patients. Replacement of 1, 2 or 3 sinuses of Valsalva was performed in 29, 12 and 5 patients, respectively. Concomitant cusp repair was necessary in 7 patients. All perioperative data were collected prospectively and an intention-to-treat analysis was performed. A total of 6 patients (median age 76, range 63-81 years) died, on average 10 months (range 0.9-44) after surgery resulting in an overall survival of 87% at the mean follow-up of 54 ± 37, range 0.9-132 months. The linearized death rate was 2.9%/year, and the actuarial survival rate at 8 years was 85.5 ± 5.6%. No death was related to the aortic valve or aortic root. There were no valve-related events and no patient required reoperation on the proximal aorta/aortic valve during the follow-up. At the last echocardiography (47.8 ± 35.6 months after surgery), 33 patients showed no and 13 patients slight (1+) aortic insufficiency. Curative repair with replacement of all pathological sinuses of Valsalva leads to an excellent long-term outcome. Selected sinus repair is a simple and effective method of curative, valve-sparing root repair in acute aortic dissection because replacement of all sinuses is seldom necessary.

  5. Surgery of the aortic root: should we go for the valve-sparing root reconstruction or the composite graft-valve replacement is still the first choice of treatment for these patients?

    Directory of Open Access Journals (Sweden)

    Fernando de Azevedo Lamana

    2015-09-01

    Full Text Available AbstractObjective:To compare the results of the root reconstruction with the aortic valve-sparing operation versus composite graftvalve replacement.Methods:From January 2002 to October 2013, 324 patients underwent aortic root reconstruction. They were 263 composite graft-valve replacement and 61 aortic valve-sparing operation (43 reimplantation and 18 remodeling. Twenty-six percent of the patients were NYHA functional class III and IV; 9.6% had Marfan syndrome, and 12% had bicuspid aortic valve. There was a predominance of aneurysms over dissections (81% vs. 19%, with 7% being acute dissections. The complete follow-up of 100% of the patients was performed with median follow-up time of 902 days for patients undergoing composite graft-valve replacement and 1492 for those undergoing aortic valve-sparing operation.Results:In-hospital mortality was 6.7% and 4.9%, respectively for composite graft-valve replacement and aortic valve-sparing operation (ns. During the late follow-up period, there was 0% moderate and 15.4% severe aortic regurgitation, and NYHA functional class I and II were 89.4% and 94%, respectively for composite graft-valve replacement and aortic valve-sparing operation (ns. Root reconstruction with aortic valve-sparing operation showed lower late mortality (P=0.001 and lower bleeding complications (P=0.006. There was no difference for thromboembolism, endocarditis, and need of reoperation.Conclusion:The aortic root reconstruction with preservation of the valve should be the operation being performed for presenting lower late mortality and survival free of bleeding events.

  6. Acute Type II Aortic Dissection with Severe Aortic Regurgitation and Chronic Descending Aortic Dissection in Pregnant Patient with Marfan Syndrome.

    Science.gov (United States)

    Lee, Seok-Soo; Jung, Tae-Eun; Lee, Dong Hyup

    2012-12-01

    Aortic dilatation and dissection are severe complications during pregnancy that can be fatal to both the mother and the fetus. The risks of these complications are especially high in pregnant patients with Marfan syndrome; however, incidents of descending aortic dissection are very rare. This case report involves a successful Bentall procedure for and recovery from a rare aortic dissection in a pregnant Marfan patient who developed acute type II aortic dissection with severe aortic regurgitation and chronic descending aortic dissection immediately after Cesarean section. Regular follow-up will be needed to monitor the descending aortic dissection.

  7. David valve-sparing aortic root replacement: equivalent mid-term outcome for different valve types with or without connective tissue disorder.

    Science.gov (United States)

    Kvitting, John-Peder Escobar; Kari, Fabian A; Fischbein, Michael P; Liang, David H; Beraud, Anne-Sophie; Stephens, Elizabeth H; Mitchell, R Scott; Miller, D Craig

    2013-01-01

    Although implicitly accepted by many that the durability of valve-sparing aortic root replacement in patients with bicuspid aortic valve disease and connective tissue disorders will be inferior, this hypothesis has not been rigorously investigated. From 1993 to 2009, 233 patients (27% bicuspid aortic valve, 40% Marfan syndrome) underwent Tirone David valve-sparing aortic root replacement. Follow-up averaged 4.7 ± 3.3 years (1102 patient-years). Freedom from adverse outcomes was determined using log-rank calculations. Survival at 5 and 10 years was 98.7% ± 0.7% and 93.5% ± 5.1%, respectively. Freedom from reoperation (all causes) on the aortic root was 92.2% ± 3.6% at 10 years; 3 reoperations were aortic valve replacement owing to structural valve deterioration. Freedom from structural valve deterioration at 10 years was 96.1% ± 2.1%. No significant differences were found in survival (P = .805, P = .793, respectively), reoperation (P = .179, P = .973, respectively), structural valve deterioration (P = .639, P = .982, respectively), or any other functional or clinical endpoints when patients were stratified by valve type (tricuspid aortic valve vs bicuspid aortic valve) or associated connective tissue disorder. At the latest echocardiographic follow-up (95% complete), 202 patients (94.8%) had none or trace aortic regurgitation, 10 (4.7%) mild, 0 had moderate to severe, and 1 (0.5%) had severe aortic regurgitation. Freedom from greater than 2+ aortic regurgitation at 10 years was 95.3% ± 2.5%. Six patients sustained acute type B aortic dissection (freedom at 10 years, 90.4% ± 5.0%). Tirone David reimplantation valve-sparing aortic root replacement in carefully selected young patients was associated with excellent clinical and echocardiographic outcome in patients with either a tricuspid aortic valve or bicuspid aortic valve. No demonstrable adverse influence was found for Marfan syndrome or connective tissue disorder on durability, clinical outcome

  8. Aortic Root Aneurism Found in a 42-Year-Old Epitomizes the Importance of Auscultation in Routine Exams.

    Science.gov (United States)

    Fredricks, Todd R; Benseler, Jeffrey S

    2016-05-01

    Bicuspid aortic valve disease (BAV) is the most common cardiac valve pathology. BAV is associated with aortic root disorders. The literature has few case reports identifying this condition during routine physical exam. A 42-yr-old military reservist flight medic presented for his annual military flight physical. He was found to have a faint cardiac murmur. His past family and medical history were remarkable for familial essential hypertension and being told at age 9 that he had a "murmur." He was referred for cardiology consultation, echocardiography, stress testing, and a computerized tomography angiogram (CTa), which identified BAV with a 4.3-cm aortic root aneurysm. A follow-up at 1 yr was recommended. In the interim he developed severe aortic valve insufficiency, a 4.6-cm aortic root aneurysm. The valve and aortic root were repaired and a single left anterior descending coronary artery lesion was bypassed during surgery. The flight medic made a full recovery but was not returned to flight status. This case emphasizes the importance of periodic reassessment by thorough history and careful cardiac auscultation during flight physicals. BAV aortopathy is an uncommon condition seen in the military aviation community. Most flight surgeons will not have the opportunity to identify it specifically via auscultation. This patient had over nine annual flight physicals prior to the one reported and no pathology was ever identified through routine auscultation. The potential role of point-of-care ultrasound (POCUS) for survey of vascular and valve status of aviation personnel merits further research.

  9. Serum Levels of Matrix Metalloproteinases 2 and 9 and TGFBR2 Gene Screening in Patients with Ascending Aortic Dilatation

    Czech Academy of Sciences Publication Activity Database

    Šímová, J.; Škvor, J.; Reissigová, Jindra; Dudra, J.; Lindner, J.; Čapek, P.; Zvárová, Jana

    2013-01-01

    Roč. 59, č. 4 (2013), s. 154-161 ISSN 0015-5500 R&D Projects: GA MŠk LN00B107; GA MŠk(CZ) 1M06014 Institutional support: RVO:67985807 Keywords : aortic dilatation * MMP-2 * MMP-9 * TGFBR2 Subject RIV: FA - Cardiovascular Diseases incl. Cardiotharic Surgery Impact factor: 0.778, year: 2013 http://fb.cuni.cz/file/5695/FB2013A0021.pdf

  10. Surgical management of aortic root disease in Marfan syndrome: a systematic review and meta-analysis.

    Science.gov (United States)

    Benedetto, Umberto; Melina, Giovanni; Takkenberg, Johanna J M; Roscitano, Antonino; Angeloni, Emiliano; Sinatra, Riccardo

    2011-06-01

    Surgical treatment of aortic root aneurysm in Marfan syndrome (MFS) patients. To compare results of total root replacement versus valve-sparing aortic root replacement in MFS patients. PubMed, Embase and Cochrane library were searched from January 1966 until February 2010 looking for papers reporting on aortic root operations in MFS patients. 530 studies were retrieved. Finally, 11 publications were enrolled. Inclusion criteria were observational studies reporting valve-related morbidity and mortality after total root replacement (TTR) and/or valve-sparing root replacement (VSRR) in patients with MFS and study size n≥30, reflecting the centre's experience. Data obtained from papers reporting both TRR and VSRR cohorts were analysed separately. In case of multiple publications, the most recent and complete report was selected. If the total number of patient-years was not provided, we calculated it by multiplying the number of hospital survivors with the mean follow-up duration of that study. Overall, 1,385 patients were analysed (972 patients had TTR and 413 patients had VSRR). Reintervention rate was 0.3%/year (95% CI 0.1 to 0.5) versus 1.3%/year (95% CI 0.3 to 2.2) (p=0.02) and thromboembolic events rate was 0.7%/year (95% CI 0.5 to 0.9) versus 0.3%/year (95% CI 0.1 to 0.6) (p=0.01) after TRR and VSRR, respectively. When composite valve-related events were compared, no difference existed between the two surgical strategies (p=0.41). Among patients undergoing VSRR, reimplantation was associated with a reduced rate of reintervention compared with remodelling (0.7%/year vs 2.4%/year, p=0.02). VSRR may represent a valuable option for patients with MFS with aortic aneurysm. However, this technique should be used with caution in patients with valve characteristics at risk for decreased durability.

  11. Evolving Practice Trends of Aortic Root Surgery in North America.

    Science.gov (United States)

    Caceres, Manuel; Ma, Yicheng; Rankin, J Scott; Saha-Chaudhuri, Paramita; Gammie, James S; Suri, Rakesh M; Thourani, Vinod H; Englum, Brian R; Esmailian, Fardad; Czer, Lawrence S; Puskas, John D; Svensson, Lars G

    2014-08-19

    Aortic-valve sparing (AVS) techniques have emerged as alternatives to composite graft-valve replacement (CVR) for treatment of aortic root aneurysm. This study analyzed recent practice trends of aortic root surgery using the Society of Thoracic Surgeons database. From January 2000 through June 2011, 31,747, Overall patients received AVS (n=3,585/31,747; 11.3%) or CVR (n=28,162/31,747; 88.7%). A High-Risk Subgroup was defined as: age >75 years, endocarditis, aortic stenosis, dialysis, multi-valve surgery, valve reoperation, or emergency/salvage status, and high-risk patients were less likely to receive AVS (n=20,356/31,747 [64.1%]; 6% AVS; unadjusted operative mortality 10.5% AVS and 11.7% CVR). The remaining patients comprised a Low-Risk Subgroup, in which AVS was more common (n=11,388/31,747 [35.9%]; 21% AVS; unadjusted operative mortality 1.4% AVS and 3.1% CVR). Procedural changes over 3 equal time periods (P1-P2-P3) were evaluated by Cochran-Armitage trends analysis. Compared to AVS, Overall CVR patients had worse baseline risk profiles and higher unadjusted operative mortality. In High-Risk patients, AVS mortality was comparable to CVR (10.5% vs 11.7%, p=0.19), but AVS mortality was lower in the Low-Risk group (1.4% vs 3%, pAVS percentages and trend p-values were: High-Risk (6%/6%/7%, p=0.26) and Low-Risk (12%/21%/25%, pAVS increased over time in Low-Risk patients while bioprostheses increased in CVR. Favorable outcomes support the trend toward further expansion of AVS. Copyright © 2014 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  12. Reimplantation valve-sparing aortic root replacement in Marfan syndrome using the Valsalva conduit: an intercontinental multicenter study.

    Science.gov (United States)

    Settepani, Fabrizio; Szeto, Wilson Y; Pacini, Davide; De Paulis, Ruggero; Chiariello, Luigi; Di Bartolomeo, Roberto; Gallotti, Roberto; Bavaria, Joseph E

    2007-02-01

    Introduced by DePaulis in 2000, the Gelweave Valsalva graft (Sulzer Vascutek, Refrewshire, Scotland) is a modified Dacron conduit (DuPont, Wilmington, DE), with prefashioned sinuses of Valsalva. The aim of this study was to evaluate the mid-term results of the reimplantation valve-sparing aortic root replacement using the Gelweave Valsalva prosthesis in Marfan syndrome patients. A retrospective review was performed of 35 patients with Marfan syndrome in four centers who underwent the reimplantation valve-sparing aortic root replacement using the Gelweave Valsalva prosthesis. The patients were predominantly men, with a mean age of 36.5 +/- 12.6 years (range, 14 to 62 years). Two patients presented with acute type A dissections and underwent emergent operations. Elective hemiarch reconstruction using hypothermic circulatory arrest was required in 11 patients. Aortic valve cusp repair was performed in 2 patients. There were no operative or hospital deaths, and no patients died during follow-up. The mean follow-up was 19 months (range, 1 to 60 months). Significant (>2+) aortic insufficiency (AI), requiring aortic valve replacement, developed in 3 patients during follow-up that requiring aortic valve replacement. The 5-year freedom from reoperation owing to structural valve deterioration was 88.9% +/- 8.1%. There were no episodes of clinically significant thromboembolism. Reimplantation valve-sparing aortic root replacement with the Gelweave Valsalva prosthesis in Marfan patients provides satisfactory mid-term results, thus encouraging further use of this type of repair. However, long-term results are needed in order to define the durability of this technique.

  13. Aortic root repair for thoracic aorta false aneurysm following Bentall procedure.

    Science.gov (United States)

    Kumar, Sanjay; Jones, Steve; Sivananthan, U M; McGoldrick, J P

    2008-08-01

    The Bentall procedure for aortic root replacement in Marfan's syndrome is safe and durable. We describe successful repair of periprosthetic valvular leak, 12 years following Bentall repair with composite graft. The aim of this report is to analyse and evaluate technical factors leading to this unusual occurrence.

  14. Three-dimensional flow structures past a bio-prosthetic valve in an in-vitro model of the aortic root.

    Science.gov (United States)

    Hasler, David; Obrist, Dominik

    2018-01-01

    The flow field past a prosthetic aortic valve comprises many details that indicate whether the prosthesis is functioning well or not. It is, however, not yet fully understood how an optimal flow scenario would look, i.e. which subtleties of the fluid dynamics in place are essential regarding the durability and compatibility of a prosthetic valve. In this study, we measured and analyzed the 3D flow field in the vicinity of a bio-prosthetic heart valve in function of the aortic root size. The measurements were conducted within aortic root phantoms of different size, mounted in a custom-built hydraulic setup, which mimicked physiological flow conditions in the aorta. Tomographic particle image velocimetry was used to measure the 3D instantaneous velocity field at various instances. Several 3D fields (e.g. instantaneous and mean velocity, 3D shear rate) were analyzed and compared focusing on the impact of the aortic root size, but also in order to gain general insight in the 3D flow structure past the bio-prosthetic valve. We found that the diameter of the aortic jet relative to the diameter of the ascending aorta is the most important parameter in determining the characteristics of the flow. A large aortic cross-section, relative to the cross-section of the aortic jet, was associated with higher levels of turbulence intensity and higher retrograde flow in the ascending aorta.

  15. Early and 1-year outcomes of aortic root surgery in patients with Marfan syndrome: a prospective, multicenter, comparative study.

    Science.gov (United States)

    Coselli, Joseph S; Volguina, Irina V; LeMaire, Scott A; Sundt, Thoralf M; Connolly, Heidi M; Stephens, Elizabeth H; Schaff, Hartzell V; Milewicz, Dianna M; Vricella, Luca A; Dietz, Harry C; Minard, Charles G; Miller, D Craig

    2014-06-01

    To compare the 1-year results after aortic valve-sparing (AVS) or valve-replacing (AVR) aortic root replacement from a prospective, international registry of 316 patients with Marfan syndrome (MFS). Patients underwent AVS (n = 239, 76%) or AVR (n = 77, 24%) aortic root replacement at 19 participating centers from 2005 to 2010. One-year follow-up data were complete for 312 patients (99%), with imaging findings available for 293 (94%). The time-to-events were compared between groups using Kaplan-Meier curves and Cox proportional hazards models. Two patients (0.6%)--1 in each group--died within 30 days. No significant differences were found in early major adverse valve-related events (MAVRE; P = .6). Two AVS patients required early reoperation for coronary artery complications. The 1-year survival rates were similar in the AVR (97%) and AVS (98%) groups; the procedure type was not significantly associated with any valve-related events. At 1 year and beyond, aortic regurgitation of at least moderate severity (≥2+) was present in 16 patients in the AVS group (7%) but in no patients in the AVR group (P = .02). One AVS patient required late AVR. AVS aortic root replacement was not associated with greater 30-day mortality or morbidity rates than AVR root replacement. At 1 year, no differences were found in survival, valve-related morbidity, or MAVRE between the AVS and AVR groups. Of concern, 7% of AVS patients developed grade ≥2+ aortic regurgitation, emphasizing the importance of 5 to 10 years of follow-up to learn the long-term durability of AVS versus AVR root replacement in patients with MFS. Copyright © 2014 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.

  16. Personalised external aortic root support (PEARS) in Marfan syndrome: analysis of 1–9 year outcomes by intention-to-treat in a cohort of the first 30 consecutive patients to receive a novel tissue and valve-conserving procedure, compared with the published results of aortic root replacement

    Science.gov (United States)

    Treasure, Tom; Takkenberg, Johanna J M; Golesworthy, Tal; Rega, Filip; Petrou, Mario; Rosendahl, Ulrich; Mohiaddin, Raad; Rubens, Michael; Thornton, Warren; Lees, Belinda; Pepper, John

    2014-01-01

    Objective Among people with Marfan syndrome who have a typical aortic root aneurysm, dissection is a characteristic cause of premature death. To pre-empt Type A dissection, composite root replacement with a mechanical valve became the standard of care in the 1980s and 1990s. This is being superseded by valve-sparing aortic root replacement to avoid lifelong anticoagulation. In 2004, a total root and valve-sparing procedure, personalised external aortic support, was introduced. We report here results among the first 30 recipients. Methods From cross-sectional digital images, the patient's own aorta is modelled by computer aided design and a replica is made in thermoplastic by rapid prototyping. On this, a personalised support of a macroporous polymer mesh is manufactured. The mesh is positioned around the aorta, closely applied from the aortoventricular junction to beyond the brachiocephalic artery. The operation is performed with a beating heart and usually without cardiopulmonary bypass. Results Between 2004 and 2011, 30 patients, median age 28 years (IQR 20–44) had this operation and have been prospectively followed for 1.4–8.8 years by February 2013. During a total of 133 patient-years there were no deaths or cerebrovascular, aortic or valve-related events. These early outcomes are better than published results for the more radical extirpative root replacement operations. Conclusions The aortic valve, the root architecture, and the blood/endothelia interface are conserved. The perioperative burden is less and there has been freedom from aortic and valvular events. A prospective comparative study is planned. PMID:24395977

  17. Reimplantation valve-sparing aortic root replacement with the Valsalva graft: what have we learnt after 100 cases?

    Science.gov (United States)

    Settepani, Fabrizio; Bergonzini, Marcello; Barbone, Alessandro; Citterio, Enrico; Basciu, Alessio; Ornaghi, Diego; Gallotti, Roberto; Tarelli, Giuseppe

    2009-07-01

    Reimplantation valve-sparing aortic root replacement has been increasingly performed with improving perioperative and mid-term results. The success of this operation primarily depends on preserving the highly sophisticated dynamic function of the aortic valve by recreating an anatomical three-dimensional configuration similar to the normal aortic root, thus minimizing the mechanical stress and strain on the cusps. Over the years several techniques have been proposed to reproduce the sinuses of Valsalva. We reviewed our experience with aortic valve reimplantation by means of a modified Dacron graft that incorporates sinuses of Valsalva, in a series of 100 consecutive patients. During a 60-month period, 100 patients with aortic root aneurysm underwent aortic valve reimplantation using the Gelweave Valsalva prosthesis. There were 74 males and the mean age was 60+/-12 years (range 28-83 years). Five patients had the Marfan's syndrome, 15 had a bicuspid aortic valve. Cusp repair was performed in five patients. The mean follow-up time was 28.6 months (range 1-60). Transesophageal echocardiogram was performed at the end of each procedure to assess the aortic valve in terms of competence, dynamic motion and level of coaptation within the graft. There was one hospital death and two late deaths. Overall survival at 60 months was 91.7+/-5.1%. Five patients developed severe aortic incompetence (AI) during follow-up requiring aortic valve replacement (AVR). The 60 months freedom from re-operation due to AI was 90.9+/-4.4%. One patient had moderate AI at latest echocardiographic study. The 60 months freedom from AI>2+ was 91.6+/-7.9%. Cox regression identified cusp's repair as independent risk factor (P=0.001) for late reimplantation failure (AVR or AI>2+). There were no episodes of endocarditis and the majority of the patients (88%) were in New York Heart Association functional class I. The aortic valve reimplantation with the Gelweave Valsalva prosthesis provided satisfactory

  18. Personalised external aortic root support (PEARS) in Marfan syndrome: analysis of 1-9 year outcomes by intention-to-treat in a cohort of the first 30 consecutive patients to receive a novel tissue and valve-conserving procedure, compared with the published results of aortic root replacement.

    Science.gov (United States)

    Treasure, Tom; Takkenberg, Johanna J M; Golesworthy, Tal; Rega, Filip; Petrou, Mario; Rosendahl, Ulrich; Mohiaddin, Raad; Rubens, Michael; Thornton, Warren; Lees, Belinda; Pepper, John

    2014-06-01

    Among people with Marfan syndrome who have a typical aortic root aneurysm, dissection is a characteristic cause of premature death. To pre-empt Type A dissection, composite root replacement with a mechanical valve became the standard of care in the 1980s and 1990s. This is being superseded by valve-sparing aortic root replacement to avoid lifelong anticoagulation. In 2004, a total root and valve-sparing procedure, personalised external aortic support, was introduced. We report here results among the first 30 recipients. From cross-sectional digital images, the patient's own aorta is modelled by computer aided design and a replica is made in thermoplastic by rapid prototyping. On this, a personalised support of a macroporous polymer mesh is manufactured. The mesh is positioned around the aorta, closely applied from the aortoventricular junction to beyond the brachiocephalic artery. The operation is performed with a beating heart and usually without cardiopulmonary bypass. Between 2004 and 2011, 30 patients, median age 28 years (IQR 20-44) had this operation and have been prospectively followed for 1.4-8.8 years by February 2013. During a total of 133 patient-years there were no deaths or cerebrovascular, aortic or valve-related events. These early outcomes are better than published results for the more radical extirpative root replacement operations. The aortic valve, the root architecture, and the blood/endothelia interface are conserved. The perioperative burden is less and there has been freedom from aortic and valvular events. A prospective comparative study is planned. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

  19. Myocardial ischemia in severe aortic regurgitation despite angiographically normal coronary arteries

    International Nuclear Information System (INIS)

    Aksoy, S.; Cam, N.; Guney, M.R.; Gurkan, U.; Oz, D.; Poyraz, E.; Eksik, A.; Agirbasli, M.

    2012-01-01

    Patients with severe aortic regurgitation frequently present with angina pectoris. The exact pathophysiology for angina in aortic regurgitation is not clear. Left ventricular hypertrophy and myocardial blood supply-demand mismatch have been the suggested mechanisms to explain ischemia. However, no conclusive clinical study exists to define the incidence of ischemia in patients with severe aortic regurgitation and normal coronary arteries. We, therefore, investigated the frequency of myocardial ischemia in relation to left ventricular hypertrophy or dilatation in patients with severe aortic regurgitation and normal coronary arteries. We reviewed the medical records of all patients (n=311) with aortic valve replacement due to aortic regurgitation between 2007 and 2010. We selected subjects with normal coronary arteries (n=182) for the study purpose, and we identified 35 patients who underwent myocardial perfusion scintigraphy prior to the coronary angiography (19 female and 16 male subjects; age 45.0±8.9 years). Left ventricular hypertrophy and dilatation were detected in 9 (26%) and 5 (14%) patients, respectively. Myocardial perfusion scintigraphy showed evidence of ischemia in 10 (29%) patients with normal coronary arteries. The presence of ischemia did not relate to the presence of left ventricular hypertrophy and/or dilatation. As a potential mechanism, aortic regurgitation causes backflow of blood from the aorta into the left ventricle, hence disturbs coronary flow dynamics. In conclusion, myocardial ischemia is common (nearly one-third) among patients with severe aortic regurgitation even in the absence of coronary obstruction, left ventricular hypertrophy and/or dilatation. (author)

  20. [Effect of native aortic valve sparing aortic root reconstruction surgery on short- and long-term prognosis in Marfan syndrome patients:a meta-analysis].

    Science.gov (United States)

    Hu, Rui; Wang, Zhiwei; Hu, Xiaoping; Wu, Hongbing; Zhou, Zhen

    2014-05-01

    This meta-analysis was performed to analyze the effect of preserving the native aortic valve on short- and long-term prognosis post aortic root reconstruction surgery for patients with Marfan syndrome. Database including Pubmed,Embase, Cochrane library, CNKI, Wanfang,VIP and CBM were searched to collect studies comparing clinical results of valve sparing surgery with composite valve graft surgery for patients with Marfan syndrome. Study quality was assessed by Newcastle-Ottawa Scale and publication bias was assessed by visual inspection of the funnel plot together with Egger test. Clinical outcomes data was extracted from the manuscripts and analyzed with Revman 5.0 supplied by Cochrane collaboration. Seven clinical trials with 690 patients were included. Meta- analysis demonstrated that valve sparing surgery was associated with a lower incidence of re-exploration (RR = 0.51, 95%CI:0.29- 0.90, P 0.05). Valve sparing aortic root reconstruction surgery is a superior procedure to composite valve graft surgery in term of improving the short- and long-term prognosis for patients with Marfan syndrome.

  1. Surgical reconstruction of aortic root in Marfan syndrome patients: a systematic review.

    Science.gov (United States)

    Hu, Rui; Wang, Zhiwei; Hu, Xiaoping; Wu, Hongbing; Wu, Zhiyong; Zhou, Zhen

    2014-07-01

    Several recent studies have compared the clinical results of valve-sparing (VS) surgery and composite valve graft (CVG) surgery in the aortic root reconstruction of Marfan syndrome (MS) patients. The study aim was to investigate whether it is appropriate to preserve the native aortic valve in root surgery of MFS patients when taking the short-term and long-term prognoses into consideration. A thorough literature search of PubMed, Embase and Cochrane library was conducted to identify studies comparing the outcomes of VS and CVG surgery in MFS patients. The Newcastle-Ottawa Scale evaluation scheme was used to assess the methodological quality of the included articles. Data were extracted from reports and analyzed using Revman 5.0, supplied by Cochrane collaboration. Six clinical trials incorporating 539 patients were included. Compared to CVG, VS surgery was associated with a lower risk for re-exploration (RR 0.48, 95% CI 0.24-0.97; p = 0.04), thromboembolic events (RR 0.17, 95% CI 0.05-0.57; p = 0.004) and endocarditis (RR 0.31, 95% CI 0.11-0.94; p = 0.04). Despite an inherent incidence of aortic regurgitation, VS surgery resulted in an elevation of long-term survival rate; however, no statistical differences were found between groups with regards to reoperation (RR 1.07, 95% CI 0.35-3.27; p = 0.91). Root reconstruction with VS surgery can effectively improve the prognosis of MFS patients and provide a promising alternative for surgical treatment. However, the results must be interpreted with caution due to the retrospective nature of the included studies; large-scale prospective control trials are needed to confirm these findings.

  2. Systematic review and meta-analysis of surgical outcomes in Marfan patients undergoing aortic root surgery by composite-valve graft or valve sparing root replacement.

    Science.gov (United States)

    Flynn, Campbell D; Tian, David H; Wilson-Smith, Ashley; David, Tirone; Matalanis, George; Misfeld, Martin; Mastrobuoni, Stefano; El Khoury, Gebrine; Yan, Tristan D

    2017-11-01

    A major, life-limiting feature of Marfan syndrome (MFS) is the presence of aneurysmal disease. Cardiovascular intervention has dramatically improved the life expectancy of Marfan patients. Traditionally, the management of aortic root disease has been undertaken with composite-valve graft replacing the aortic valve and proximal aorta; more recently, valve sparing procedures have been developed to avoid the need for anticoagulation. This meta-analysis assesses the important surgical outcomes of the two surgical techniques. A systematic review and meta-analysis of 23 studies reporting the outcomes of aortic root surgery in Marfan patients with data extracted for outcomes of early and late mortality, thromboembolic events, late bleeding complications and surgical reintervention rates. The outcomes of 2,976 Marfan patients undergoing aortic root surgery were analysed, 1,624 patients were treated with composite valve graft (CVG) and 1,352 patients were treated with valve sparing root replacement (VSRR). When compared against CVG, VSRR was associated with reduced risk of thromboembolism (OR =0.32; 95% CI, 0.16-0.62, P=0.0008), late hemorrhagic complications (OR =0.18; 95% CI, 0.07-0.45; P=0.0003) and endocarditis (OR =0.27; 95% CI, 0.10-0.68; P=0.006). Importantly there was no significant difference in reintervention rates between VSRR and CVG (OR =0.89; 95% CI, 0.35-2.24; P=0.80). There is an increasing body of evidence that VSRR can be reliably performed in Marfan patients, resulting in a durable repair with no increased risk of re-operation compared to CVG, thus avoiding the need for systemic anticoagulation in selected patients.

  3. Aortic root dimensions are predominantly determined by genetic factors: a classical twin study

    International Nuclear Information System (INIS)

    Celeng, Csilla; Kolossvary, Marton; Kovacs, Attila; Molnar, Andrea Agnes; Szilveszter, Balint; Karolyi, Mihaly; Jermendy, Adam L.; Karady, Julia; Merkely, Bela; Maurovich-Horvat, Pal; Horvath, Tamas; Tarnoki, Adam D.; Tarnoki, David L.; Voros, Szilard; Jermendy, Gyoergy

    2017-01-01

    Previous studies using transthoracic echocardiography (TTE) observed moderate heritability of aortic root dimensions. Computed tomography angiography (CTA) might provide more accurate heritability estimates. Our primary aim was to assess the heritability of the aortic root with CTA. Our secondary aim was to derive TTE-based heritability and compare this with the CTA-based results. In the BUDAPEST-GLOBAL study 198 twin subjects (118 monozygotic, 80 dizygotic; age 56.1 ± 9.4 years; 126 female) underwent CTA and TTE. We assessed the diameter of the left ventricular outflow tract (LVOT), annulus, sinus of Valsalva, sinotubular junction and ascending aorta. Heritability was assessed using ACDE model (A additive genetic, C common environmental, D dominant genetic, E unique environmental factors). Based on CTA, additive genetic effects were dominant (LVOT: A = 0.67, E = 0.33; annulus: A = 0.76, E = 0.24; sinus of Valsalva: A = 0.83, E = 0.17; sinotubular junction: A = 0.82, E = 0.18; ascending aorta: A = 0.75, E = 0.25). TTE-derived measurements showed moderate to no genetic influence (LVOT: A = 0.38, E = 0.62; annulus: C = 0.47, E = 0.53; sinus of Valsalva: C = 0.63, E = 0.37; sinotubular junction: C = 0.45, E = 0.55; ascending aorta: A = 0.67, E = 0.33). CTA-based assessment suggests that aortic root dimensions are predominantly determined by genetic factors. TTE-based measurements showed moderate to no genetic influence. The choice of measurement method has substantial impact on heritability estimates. (orig.)

  4. Aortic root dimensions are predominantly determined by genetic factors: a classical twin study

    Energy Technology Data Exchange (ETDEWEB)

    Celeng, Csilla; Kolossvary, Marton; Kovacs, Attila; Molnar, Andrea Agnes; Szilveszter, Balint; Karolyi, Mihaly; Jermendy, Adam L.; Karady, Julia; Merkely, Bela; Maurovich-Horvat, Pal [Semmelweis University, MTA-SE Cardiovascular Imaging Research Group, Heart and Vascular Center, Budapest (Hungary); Horvath, Tamas [Budapest University of Technology and Economics, Department of Hydrodynamic Systems, Budapest (Hungary); Tarnoki, Adam D.; Tarnoki, David L. [Semmelweis University, Department of Radiology and Oncotherapy, Budapest (Hungary); Voros, Szilard [Global Genomics Group, Atlanta, GA (United States); Jermendy, Gyoergy [Bajcsy-Zsilinszky Hospital, Medical Department, Budapest (Hungary)

    2017-06-15

    Previous studies using transthoracic echocardiography (TTE) observed moderate heritability of aortic root dimensions. Computed tomography angiography (CTA) might provide more accurate heritability estimates. Our primary aim was to assess the heritability of the aortic root with CTA. Our secondary aim was to derive TTE-based heritability and compare this with the CTA-based results. In the BUDAPEST-GLOBAL study 198 twin subjects (118 monozygotic, 80 dizygotic; age 56.1 ± 9.4 years; 126 female) underwent CTA and TTE. We assessed the diameter of the left ventricular outflow tract (LVOT), annulus, sinus of Valsalva, sinotubular junction and ascending aorta. Heritability was assessed using ACDE model (A additive genetic, C common environmental, D dominant genetic, E unique environmental factors). Based on CTA, additive genetic effects were dominant (LVOT: A = 0.67, E = 0.33; annulus: A = 0.76, E = 0.24; sinus of Valsalva: A = 0.83, E = 0.17; sinotubular junction: A = 0.82, E = 0.18; ascending aorta: A = 0.75, E = 0.25). TTE-derived measurements showed moderate to no genetic influence (LVOT: A = 0.38, E = 0.62; annulus: C = 0.47, E = 0.53; sinus of Valsalva: C = 0.63, E = 0.37; sinotubular junction: C = 0.45, E = 0.55; ascending aorta: A = 0.67, E = 0.33). CTA-based assessment suggests that aortic root dimensions are predominantly determined by genetic factors. TTE-based measurements showed moderate to no genetic influence. The choice of measurement method has substantial impact on heritability estimates. (orig.)

  5. Laser Fenestration of Aortic Stent-Grafts Followed by Noncompliant vs Cutting Balloon Dilation: A Scanning Electron Microscopy Study.

    Science.gov (United States)

    Lin, Jing; Parikh, Niraj; Udgiri, Naval; Wang, Shaoxia; Miller, Daniel F; Li, Chaojing; Panneton, Jean; Nutley, Mark; Zhang, Ze; Huang, Yunfan; Lu, Jun; Zhang, Jingyi; Wang, Lu; Guidoin, Robert

    2018-06-01

    To examine the effects of in situ laser fenestration and subsequent balloon dilation (noncompliant vs cutting) on the graft fabric of 4 aortic stent-graft models. In an in vitro setup, the Zenith TX2, Talent, Endurant, and Anaconda aortic stent-grafts (all made of polyester graft material) were subjected to laser fenestration with a 2.3-mm-diameter probe at low and high energy in a physiologic saline solution followed by balloon dilation of the hole. For the first series of tests, 6-mm-diameter noncompliant balloons were used and replaced for the second series by 6-mm-diameter cutting balloons. Each procedure was performed 5 times (5 fenestrations per balloon type). The fenestrations were examined visually and with light and scanning electron microscopy. Each fenestration demonstrated various degrees of fraying and/or tearing regardless of the device. The monofilament twill weave of the Talent endograft tore in the warp direction up to 7.09±0.46 mm at high energy compared with 2.41±0.26 mm for the Endurant multifilament device. The fenestrations of the 3 endografts with multifilament weave (Zenith, Anaconda, and Endurant) showed more fraying; fenestration areas in the multifilament Endurant were >10 mm 2 at low and high energy. The fenestrations were free of melted fibers, but minor blackening of the filaments was observed in all devices. Overall, the cutting balloons resulted in worse tearing and damage. Of note, the edges of the dilated laser-formed fenestrations of the Talent and the Endurant grafts demonstrated evidence of additional shredded yarns. In situ fenestration does not cause any melting of the polyester; however, the observed structural damage to the fabric construction must be carefully considered. Cutting balloons caused various levels of tearing compared to the noncompliant balloons and cannot be recommended for use in this application. Rather, noncompliant balloons should be employed, but only with endografts constructed from multifilament yarns

  6. [Long-term outcome of aortic valve sparing procedures in connective tissue disorders].

    Science.gov (United States)

    Tanaka, Hiroshi; Ogino, H; Matsuda, H; Minatoya, K; Sasaki, N

    2009-10-01

    The aim of this study is to determine the long-term outcome of aortic valve sparing procedures for patients having connective tissue disorder. Between 1993 and 2008, the aortic valve sparing surgery was performed in 94 patients having aortic root dilatation. Eighty patients of them (37.2 +/- 13.4 years, 50 male) had cystic medial necrosis in the aortic wall, which was confirmed the pathological examination. We reviewed these patients. Sixty percent (48/80) had Marfan syndrome, 5% (4/80) had Loeyz-Dietz syndrome, 2% (2/80) had bicuspid aortic valve, and 11% (9/80) had aortic dissection. Our reimplantation procedure has been refined as followed: with a tube graft in 41, a tube graft with creation of neo-sinuses in 11, and a Valsalva graft in 14. Fourteen patients underwent the remodeling procedure. The follow-up rate was 100% with the duration of 3.7+/- 3.4 years. There were no operative death but six late deaths. Seventeen (21.3%) patients required aortic valve replacement, for recurrent aortic insufficiency in 13 and infection in 4. Freedom from reoperation was 80%, 43%, and freedom from moderate or severe aortic insufficiency was 80%, 54%, at 5 and 10 years, respectively. Pathological findings of the aortic valve obtained in the reoperations showed elongation and prolapse of the aortic valve due to myxomatous degeneration and fibrous thickening caused by aortic insufficiency. Even in connective tissue disorders, aortic valve sparing operation is associated with acceptable long-term durability, although cusp degeneration resulting in recurrent aortic insufficiency might be progressive.

  7. Fate of remnant sinuses of Valsalva in patients with bicuspid and trileaflet valves undergoing aortic valve, ascending aorta, and aortic arch replacement.

    Science.gov (United States)

    Milewski, Rita Karianna; Habertheuer, Andreas; Bavaria, Joseph E; Siki, Mary; Szeto, Wilson Y; Krause, Eric; Korutla, Varun; Desai, Nimesh D; Vallabhajosyula, Prashanth

    2017-08-01

    In patients presenting with aortic valvulopathy with concomitant ascending aortic aneurysm, surgical management of the sinus of Valsalva segment remains undefined, especially for moderately dilated aortic roots. In patients with this pathology undergoing aortic valve replacement with supracoronary ascending aorta replacement, we assessed the fate of the remnant preserved sinus of Valsalva segment stratified by aortic valve morphology and pathology. From 2002 to 2015, 428 patients underwent elective aortic valve replacement with supracoronary ascending aorta replacement. Patients were stratified on the basis of valvular morphology (bicuspid aortic valve [n = 254] and tricuspid aortic valve [n = 174]), valvular pathology (bicuspid aortic valve with aortic stenosis [n = 178], bicuspid aortic valve with aortic insufficiency [n = 76], tricuspid aortic valve with aortic stenosis [n = 61], tricuspid aortic valve with aortic insufficiency [n = 113]), and preoperative sinus of Valsalva dimensions (45 mm). Kaplan-Meier analysis revealed no significant difference in freedom from reoperation in tricuspid aortic valve versus bicuspid aortic valve (P = .576). Multivariable Cox regression model performed with sinus of Valsalva dimensions at baseline and follow-up as time-varying covariates did not adversely affect survival. A repeated-measure, mixed-effects model constructed to assess longitudinal sinus of Valsalva trends revealed that the retained sinus of Valsalva dimensions remain stable over long-term follow-up (discharge to ≥10 years), irrespective of valvular morphology/pathology (bicuspid aortic valve with aortic insufficiency, tricuspid aortic valve with aortic insufficiency, tricuspid aortic valve with aortic stenosis) and preoperative sinus of Valsalva groups (45 mm). In patients with nonaneurysmal sinuses of Valsalva undergoing aortic valve replacement with supracoronary ascending aorta replacement, the sinus segment can be preserved irrespective of

  8. Gothic aortic arch and cardiac mechanics in young patients after arterial switch operation for d-transposition of the great arteries.

    Science.gov (United States)

    Di Salvo, Giovanni; Bulbul, Ziad; Pergola, Valeria; Issa, Ziad; Siblini, Ghassan; Muhanna, Nisreen; Galzerano, Domenico; Fadel, Bahaa; Al Joufan, Mansour; Al Fayyadh, Majid; Al Halees, Zohair

    2017-08-15

    In patients who have undergone arterial switch operation (ASO) for d-transposition of the great arteries a gothic aortic arch (GA) morphology has been found and it has been associated with abnormal aortic bio-elastic properties. GA is frequent in ASO patients and may have an impact on cardiac mechanics. Our study aims were to assess 1- the occurrence of GA in a large sample of patients after ASO; 2- the association between GA and aortic bio-elastic properties; and 3- the impact of GA on left ventricular (LV) function using speckle tracking echocardiography (STE). We studied one hundred and five asymptomatic patients, who have undergone first stage ASO for d-TGA, with normal left ventricular ejection fraction (LVEF ≥53%). Forty-six (44%) patients showed a GA (mean age 11.5±7.2years, 26 males) while fifty-nine (56%) patients (mean age 9.6±6.7years, 37 males) did not present GA. The two groups were comparable for age, sex, BSA, and office blood pressure values. In group GA aortic root was significantly dilated (27.4±7.5mm vs. 21.2±6.9mm, p<0.0001), aortic stiffness index (Group GA=1.8±1.2 vs. 1.4±0.7, p=0.025) was significantly increased, left atrial volume was larger (p=0.0145), global longitudinal strain (Group GA=-18.4±2.5% vs. -20.1±3.3%, p=0.012) and basal LV longitudinal strains (Group GA=-16.9±4.8% vs. -20.4±7.0%, p=0.013) were significantly reduced. After ASO the presence of a GA is associated with a significantly dilated aortic root, stiffer aortic wall, larger left atrial volume, and worse LV longitudinal systolic deformations, well known predictors of cardiovascular morbidity and mortality. Copyright © 2017 Elsevier B.V. All rights reserved.

  9. Recent Clinical Drug Trials Evidence in Marfan Syndrome and Clinical Implications.

    Science.gov (United States)

    Singh, Michael N; Lacro, Ronald V

    2016-01-01

    Marfan syndrome is a genetic disorder of connective tissue with principal manifestations in the cardiovascular, ocular, and skeletal systems. Cardiovascular disease, mainly progressive aortic root dilation and aortic dissection, is the leading cause of morbidity and mortality. The primary aims of this report were to examine the evidence related to medical therapy for Marfan syndrome, including recently completed randomized clinical trials on the efficacy of β-blockers and angiotensin II receptor blockers for the prophylactic treatment of aortic enlargement in Marfan syndrome, and to provide recommendations for medical therapy on the basis of available evidence. Medical therapy for Marfan syndrome should be individualized according to patient tolerance and risk factors such as age, aortic size, and family history of aortic dissection. The Pediatric Heart Network trial showed that atenolol and losartan each reduced the rate of aortic dilation. All patients with known or suspected Marfan syndrome and aortic root dilation should receive medical therapy with adequate doses of either β-blocker or angiotensin receptor blocker. The Pediatric Heart Network trial also showed that atenolol and losartan are more effective at reduction of aortic root z score in younger subjects, which suggests that medical therapy should be prescribed even in the youngest children with aortic dilation. For patients with Marfan syndrome without aortic dilation, the available evidence is less clear. If aortic dilation is severe and/or progressive, therapy with a combination of β-blocker and angiotensin receptor blocker should be considered, although trial results are mixed with respect to the efficacy of combination therapy vs monotherapy. Copyright © 2016 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.

  10. Evaluation of Marfan patients status post valve-sparing aortic root replacement with 4D flow.

    Science.gov (United States)

    Hope, Thomas A; Kvitting, John-Peder Escobar; Hope, Michael D; Miller, D Craig; Markl, Michael; Herfkens, Robert J

    2013-11-01

    Over the past two decades elective valve-sparing aortic root replacement (V-SARR) has become more common in the treatment of patients with aortic root and ascending aortic aneurysms. Currently there are little data available to predict complications in the post-operative population. The study goal was to determine if altered flow patterns in the thoracic aorta, as measured by MRI, are associated with complications after V-SARR. Time-resolved three-dimensional phase-contrast MRI (4D flow) was used to image 12 patients with Marfan syndrome after V-SARR. The patients were followed up for an average of 5.8 years after imaging and 8.2 years after surgery. Additionally 5 volunteers were imaged for comparison. Flow profiles were visualized during peak systole using streamlines. Wall shear stress estimates and normalized flow displacement were evaluated at multiple planes in the thoracic aorta. During the follow-up period, a single patient developed a Stanford Type B aortic dissection. At initial imaging, prior to the development of the dissection, the patient had altered flow patterns, wall shear stress estimates, and increased normalized flow displacement in the thoracic aorta in comparison to the remaining V-SARR patients and volunteers. This is the first follow-up study of patients after 4D flow imaging. An aortic dissection developed in one patient with altered flow patterns and hemodynamic stresses in the thoracic aorta. These results suggest that flow and altered hemodynamics may play a role in the development of post-operative intramural hematomas and dissections. Copyright © 2013 Elsevier Inc. All rights reserved.

  11. Long-term results of aortic valve replacement with Edwards Prima Plus stentless bioprosthesis: eleven years' follow up.

    Science.gov (United States)

    Auriemma, Stefano; D'Onofrio, Augusto; Brunelli, Massimo; Magagna, Paolo; Paccanaro, Mariemma; Rulfo, Fanny; Fabbri, Alessandro

    2006-09-01

    The Edwards Lifesciences Prima Plus stentless valve (ELSV) is a bioprosthesis manufactured from a porcine aortic root. The study aim was to evaluate late clinical outcomes after aortic valve replacement (AVR) with ELSV implanted as a miniroot in patients with aortic valve disease. Between 1993 and 2004, 318 patients (232 males, 86 females; mean age 69 +/- 9 years; range: 37-83 years) underwent AVR with the ELSV. Preoperatively, 102 patients (32%), 162 (51%) and 54 (17%) were in NYHA classes I/II, III and IV, respectively. Aortic stenosis, aortic regurgitation and combined lesions were present in 124 patients (39%), 114 (36%) and 41 (13%), respectively. Twenty patients (6%) were referred for an acute aortic dissection, 20 (6%) for an aortic root aneurysm, and 139 (44%) had an associated aneurysmal dilatation of the ascending aorta. The ascending aorta was replaced in 159 patients (50%); aortic arch replacement was required in 10 (3%). Coronary artery bypass graft was performed in 86 patients (27%). The follow up was based on clinical data. Operative mortality was 5% (n = 17). There were 49 late deaths (5.2%/pt-yr). Valve-related mortality occurred in 10 patients (1%/pt-yr). Actuarial survival at five and 10 years was 78% and 33%, respectively. Actuarial freedom from valve reoperation and structural valve deterioration at 10 years were 100% and 64%. Actuarial freedom from embolic events and endocarditis at 10 years were 84% and 81%, respectively. The ELSV, when implanted as a miniroot, provided good early and long-term results in terms of survival and freedom from major complications.

  12. Comparison of the Ross/Ross-Konno aortic root in children before and after the age of 18 months.

    Science.gov (United States)

    Lo Rito, Mauro; Davies, Ben; Brawn, William J; Jones, Timothy J; Khan, Natasha; Stickley, John; Barron, David J

    2014-09-01

    Evaluation of long-term results after the Ross/Ross-Konno operation in patients Ross/Ross-Konno operation (1991-2011). Data were obtained from hospital records and follow-up was 100% complete. Between January 1991 and December 2011, 140 patients underwent the Ross/Ross-Konno operation and 22 patients were Ross operation and 8 patients a Ross-Konno operation. Presentation at surgery was aortic valve stenosis in 13, regurgitation in 7 and mixed disease in 2. Only 4 patients (18%) had no surgery prior to Ross/Ross-Konno, and among the others the previous most frequent operation was aortic valvotomy (55%). There were 3 early deaths, all in high-risk cases with poor preoperative left ventricular function. At discharge there was no neoaortic regurgitation in 10, trivial in 4 and mild in 5. The median follow-up is 10.8 years (range 0.96-21). There was 1 late death due to progressive ventricular dysfunction 2.4 years after Ross-Konno and mitral valve replacement. Survival for patients 18-months patients (P Ross/Ross-Konno can be invaluable in the younger age group but not without risk in the setting of ventricular dysfunction. Long-term performance of the neoaortic valve is significantly better than in older children, which is related to maintenance of normal root dimensions compared with progressive dilatation in patients undergoing Ross/Ross-Konno at older ages. © The Author 2014. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

  13. Proximal thoracic aorta dimensions after continuous-flow left ventricular assist device implantation: Longitudinal changes and relation to aortic valve insufficiency.

    Science.gov (United States)

    Fine, Nowell M; Park, Soon J; Stulak, John M; Topilsky, Yan; Daly, Richard C; Joyce, Lyle D; Pereira, Naveen L; Schirger, John A; Edwards, Brooks S; Lin, Grace; Kushwaha, Sudhir S

    2016-04-01

    In this study we examined the impact of continuous-flow left ventricular assist device (CF-LVAD) support on proximal thoracic aorta dimensions. Aortic root and ascending aorta diameter were measured from serial echocardiograms before and after CF-LVAD implantation in patients with ≥6 months of support, and correlated with the development of >mild aortic valve insufficiency (AI). Of 162 patients included, mean age was 58 ± 11 years and 128 (79%) were male. Seventy-nine (63%) were destination therapy patients. Mean aortic root and ascending aorta diameters at baseline, 1 month, 6 months, 12 months and long-term follow-up (mean 2.0 ± 1.4 years) were 3.5 ± 0.4, 3.5 ± 0.3, 3.9 ± 0.3, 3.9 ± 0.2 and 4.0 ± 0.3, and 3.3 ± 0.2, 3.3 ± 0.3, 3.6 ± 0.2, 3.6 ± 0.3 and 3.6 ± 0.3 cm, respectively. Only change in aortic root diameter from 1-month to 6-month follow-up reached statistical significance (p = 0.03). Nine (6%) patients had accelerated proximal thoracic aorta expansion (>0.5 cm/year), occurring predominantly in the first 6 months after implantation. These patients were older and more likely to have hypertension and baseline proximal thoracic aorta dilation. Forty-five (28%) patients developed >mild AI at long-term follow-up, including 7 of 9 (78%) of those with accelerated proximal thoracic aorta expansion. All 7 had aortic valves that remained closed throughout the cardiac cycle, and this, along with duration of CF-LVAD support and increase in aortic root diameter, were significantly associated with developing >mild AI. CF-LVAD patients have small increases in proximal thoracic aorta dimensions that predominantly occur within the first 6 months after implantation and then stabilize. Increasing aortic root diameter was associated with AI development. Copyright © 2016 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.

  14. Aortic stenosis with abnormal eccentric left ventricular remodeling secondary to hypothyroidism in a Bourdeaux Mastiff

    Directory of Open Access Journals (Sweden)

    Guilherme Augusto Minozzo

    Full Text Available ABSTRACT: This paper describes a case of congenital aortic stenosis with eccentric left ventricular hypertrophy associated with hypothyroidism in a 1-year-old Bourdeaux Mastiff dog. The dog had ascites, apathy, alopecic and erythematous skin lesions in different parts of the body. A two-dimensional echocardiogram revealed aortic valve stenosis, with poststenotic dilation in the ascending aorta. The same exam showed eccentric hypertrophy and dilation of the left ventricle during systole and diastole. Aortic stenosis usually results in concentric left ventricular hypertrophy instead of eccentric hypertrophy; and therefore, this finding was very unusual. Hypothyroidism, which is uncommon in young dogs, may be incriminated as the cause of ventricular dilation, making this report even more interesting. Because hypothyroidism would only result in dilatation, the eccentric hypertrophy was attributed to pressure overload caused by aortic stenosis. Thus, cardiac alterations of this case represent a paradoxical association of both diseases.

  15. David I reimplantation procedure for aortic root replacement in Marfan patients: medium-term outcome.

    Science.gov (United States)

    Kari, Fabian A; Beyersdorf, Friedhelm; Rylski, Bartosz; Stephens, Elizabeth H; Russe, Maximilian; Siepe, Matthias

    2014-11-01

    Technical variations of the David reimplantation valve-sparing aortic root replacement (V-SARR) procedure have been proposed to be advantageous in patients with connective tissue disease, such as the Marfan syndrome (MFS). We report results of a Marfan cohort treated exclusively with the non-modified David I procedure. Forty-eight Marfan patients (25 males, mean age 33 ± 12 years, range 15-62 years) underwent the original variant of the David V-SARR (David I) between 1997 and 2013. Forty-two operations (88%) were performed as elective procedures for aortic root aneurysms and six for acute dissections (12%). Seventeen had aortic regurgitation (AR) grades ≥2+ preoperatively, and 3 had AR >2+. No patients with severe AR (4+) were selected for V-SARR. Three full or hemi-arch replacements were performed. Patients who were operated on using a variation of the David I or David II procedure were excluded. Mean prosthesis size was 28 ± 3 mm (18-30 mm). Mean clinical and echocardiographic follow-up (98% complete) was 3.8 ± 3.7 years with a cumulative follow-up of 178 patient-years. The early mortality rate was 2% (one hospital death). The survival rate was 98% (95% confidence 84-99%) at 4 years and 90% (57-98%) at 8 years with 5 patients at risk at 10 years. The rate of freedom from root or valve reoperation was 97% (79-99%) and 97% (79-99%) at 4 and 8 years, respectively. Only one patient required mechanical aortic valve replacement for progression of AR. Despite potential theoretical drawbacks of the David I V-SARR technique without neo-sinuses or a neo-sinotubular junction, it results in a favourable mid-term outcome in Marfan patients and compares well with reported results of different modifications of David V-SARR. © The Author 2014. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

  16. Direct transcatheter aortic valve implantation with self-expandable bioprosthesis: Feasibility and safety

    Energy Technology Data Exchange (ETDEWEB)

    Fiorina, Claudia, E-mail: clafiorina@yahoo.it [Cardiac Catheterization Laboratory, Cardiothoracic Department, Spedali Civili, Brescia (Italy); Maffeo, Diego; Curello, Salvatore [Cardiac Catheterization Laboratory, Cardiothoracic Department, Spedali Civili, Brescia (Italy); Lipartiti, Felicia [Division of Cardiology, Cardiothoracic Department, Spedali Civili, Brescia (Italy); Chizzola, Giuliano [Cardiac Catheterization Laboratory, Cardiothoracic Department, Spedali Civili, Brescia (Italy); D' Aloia, Antonio [Division of Cardiology, Cardiothoracic Department, Spedali Civili, Brescia (Italy); Adamo, Marianna [Cardiac Catheterization Laboratory, Cardiothoracic Department, Spedali Civili, Brescia (Italy); Mastropierro, Rosy [Division of Cardiothoracic Anestesiology, Cardiothoracic Department, Spedali Civili, Brescia (Italy); Gavazzi, Emanuele [Department of Radiology, University of Brescia, Spedali Civili, Brescia (Italy); Ciccarese, Camilla; Chiari, Ermanna [Division of Cardiology, Cardiothoracic Department, Spedali Civili, Brescia (Italy); Ettori, Federica [Cardiac Catheterization Laboratory, Cardiothoracic Department, Spedali Civili, Brescia (Italy)

    2014-06-15

    Background: Balloon valvuloplasty has been considered a mandatory step of the transcatheter aortic valve implantation (TAVI), although it is not without risk. The aim of this work was to evaluate the feasibility and safety of TAVI performed without pre-dilation (direct TAVI) of the stenosed aortic valve. Material and Methods: Between June 2012 and June 2013, 55 consecutive TAVI performed without pre-dilation at our institution using the self-expandable CoreValve prosthesis (Medtronic, Minneapolis, MN) were analyzed and compared with 45 pre-dilated TAVI performed the previous year. Inclusion criteria were a symptomatic and severe aortic stenosis. Exclusion criteria were defined as presence of pure aortic regurgitation, degenerated surgical bioprosthesis or bicuspid aortic valve and prior procedure of balloon aortic valvuloplasty performed as a bridge to TAVI. Results: High-burden calcification in the device landing zone, assessed by CT scan, was found in most of the patients. The valve size implanted was similar in both groups. Device success was higher in direct TAVI (85% vs. 64%, p = 0.014), mostly driven by a significant lower incidence of paravalvular leak (PVL ≥2; 9% vs. 33%, p = 0.02). Safety combined end point at 30 days was similar in both groups. Conclusion: Compared to TAVI with pre-dilation, direct TAVI is feasible regardless of the presence of bulky calcified aortic valve and the valve size implanted. Device success was higher in direct TAVI, mostly driven by a lower incidence of paravalvular leak. Safety at 30 days was similar in two groups.

  17. Prediction of optimal deployment projection for transcatheter aortic valve replacement: angiographic 3-dimensional reconstruction of the aortic root versus multidetector computed tomography.

    Science.gov (United States)

    Binder, Ronald K; Leipsic, Jonathon; Wood, David; Moore, Teri; Toggweiler, Stefan; Willson, Alex; Gurvitch, Ronen; Freeman, Melanie; Webb, John G

    2012-04-01

    Identifying the optimal fluoroscopic projection of the aortic valve is important for successful transcatheter aortic valve replacement (TAVR). Various imaging modalities, including multidetector computed tomography (MDCT), have been proposed for prediction of the optimal deployment projection. We evaluated a method that provides 3-dimensional angiographic reconstructions (3DA) of the aortic root for prediction of the optimal deployment angle and compared it with MDCT. Forty patients undergoing transfemoral TAVR at St Paul's Hospital, Vancouver, Canada, were evaluated. All underwent preimplant 3DA and 68% underwent preimplant MDCT. Three-dimensional angiographic reconstructions were generated from images of a C-arm rotational aortic root angiogram during breath-hold, rapid ventricular pacing, and injection of 32 mL contrast medium at 8 mL/s. Two independent operators prospectively predicted perpendicular valve projections. The implant angle was chosen at the discretion of the physician performing TAVR. The angles from 3DA, from MDCT, the implant angle, and the postdeployment perpendicular prosthesis view were compared. The shortest distance from the postdeployment perpendicular prosthesis projection to the regression line of predicted perpendicular projections was calculated. All but 1 patient had adequate image quality for reproducible angle predictions. There was a significant correlation between 3DA and MDCT for prediction of perpendicular valve projections (r=0.682, Pregression line of predicted angles to the postdeployment prosthesis view was 5.1±4.6° for 3DA and 7.9±4.9° for MDCT (P=0.01). Three-dimensional angiographic reconstructions and MDCT are safe, practical, and accurate imaging modalities for identifying the optimal perpendicular valve deployment projection during TAVR.

  18. Clinical Outcomes of the David V Valve-Sparing Root Replacement Compared With Bioprosthetic Valve-Conduits for Aortic Root Aneurysms.

    Science.gov (United States)

    Esaki, Jiro; Leshnower, Bradley G; Binongo, Jose N; Lasanajak, Yi; McPherson, LaRonica; Halkos, Michael E; Guyton, Robert A; Chen, Edward P

    2017-06-01

    Valve-sparing root replacement (VSRR) is an established therapy for aortic root pathology. Limited insight exists when the results of VSRR are compared with those of conventional root replacement with use of a bioprosthetic composite conduit (BIO). This study compares the operative and midterm results of VSRR and BIO. A retrospective review from 2002 to 2015 at a United States academic center identified 282 patients who underwent VSRR and 425 patients who underwent BIO. Propensity-score matching was performed based on 20 preoperative characteristics, and 123 matched pairs were identified. The mean age (VSRR 53.5 ± 11.1, BIO 53.0 ± 13.0; p = 0.74) and left ventricular ejection fraction (VSRR 54.5 ± 9.2%, BIO 54.4 ± 9.4%; p = 0.99) were equivalent in both groups. The incidence of bicuspid valves (VSRR 26.0%, BIO 27.6%; p = 0.77), Marfan syndrome (VSRR 6.5%, BIO 4.9%; p = 0.58), type A dissection (VSRR 13.0%, BIO 13.0%; p = 0.99), reoperation (VSRR 15.4%, BIO 20.3%; p = 0.32) and arch replacement (VSRR 60.2%, BIO 63.4%; p = 0.60) were similar between the groups. Operative mortality was 5.7% in VSRR and 0.8% in BIO (p = 0.07). There were no significant differences in postoperative renal failure (VSRR 0.8%, BIO 0.0%; p = 0.99) or stroke (VSRR 3.3%, BIO 0.8%; p = 0.37) between the groups. The 7-year survival (VSSR 82.4%, BIO 83.0%; p = 0.53), 7-year freedom from reoperation (VSRR 97.4%, BIO 95.8%; p = 0.48), and 7-year freedom from greater than moderate aortic insufficiency (AI) (VSRR 98.1%, BIO 100.0%; p = 0.47) were similar between groups. VSRR and BIO result in equivalent operative mortality and morbidity with similar midterm survival and valve durability. VSRR is an effective alternative to BIO for aortic root pathologic conditions; however, careful patient selection is paramount. Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  19. Comparison of cine-MRI and transthoracic echocardiography for the assessment of aortic root diameters in patients with suspected Marfan syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Bannas, P.; Derlin, T.; Yamamura, J.; Lund, G.; Adam, G. [University Medical Center Hamburg-Eppendorf, Hamburg (Germany). Dept. of Diagnostic and Interventional Radiology; Rybczynski, M.; Sheikhzadeh, S.; Kodolitsch, Y. von [University Medical Center Hamburg-Eppendorf, Hamburg (Germany). Dept. of General and Interventional Cardiology; Groth, M. [University Medical Center Hamburg-Eppendorf, Hamburg (Germany). Section for Pediatric Radiology

    2015-11-15

    Patients with Marfan syndrome require repeated imaging for monitoring of aortic root aneurysms. Therefore, we evaluated the agreement and reproducibility of cine-MRI and echocardiography measurements of the sinuses of Valsalva in patients with suspected Marfan syndrome. 51 consecutive patients with suspected Marfan syndrome were prospectively examined using cine-MRI and echocardiography. Two readers independently measured aortic root diameters at the level of the sinuses of Valsalva in both cine-MRI and echocardiography. Statistics included intraclass correlation coefficient, Pearson correlation coefficient, Bland-Altman analysis, and two-sided t-test. In 38 of the 51 individuals (74.5 %), the diagnosis of Marfan syndrome was established according to the criteria of the Ghent-2 nosology. Cine-MRI measurements of the sinuses of Valsalva revealed a strong correlation with echocardiography (r=0.929), but a statistically significant bias of -1.0 mm (p<0.001). The mean absolute diameter for sinuses of Valsalva obtained by cine-MRI was 32.3 ± 5.8 mm as compared to 33.4 ± 5.4 mm obtained by echocardiography. Interobserver agreement of measurements of the sinuses of Valsalva was higher for cine-MRI than for echocardiography (p=0.029). Despite small, but statistically significant differences in terms of agreement and reproducibility, cine-MRI and echocardiographic measurements of aortic root diameters provide comparable results without a significant clinical difference. Therefore both techniques may be used for monitoring of the aortic root in patients with Marfan syndrome.

  20. Comparison of cine-MRI and transthoracic echocardiography for the assessment of aortic root diameters in patients with suspected Marfan syndrome

    International Nuclear Information System (INIS)

    Bannas, P.; Derlin, T.; Yamamura, J.; Lund, G.; Adam, G.; Rybczynski, M.; Sheikhzadeh, S.; Kodolitsch, Y. von; Groth, M.

    2015-01-01

    Patients with Marfan syndrome require repeated imaging for monitoring of aortic root aneurysms. Therefore, we evaluated the agreement and reproducibility of cine-MRI and echocardiography measurements of the sinuses of Valsalva in patients with suspected Marfan syndrome. 51 consecutive patients with suspected Marfan syndrome were prospectively examined using cine-MRI and echocardiography. Two readers independently measured aortic root diameters at the level of the sinuses of Valsalva in both cine-MRI and echocardiography. Statistics included intraclass correlation coefficient, Pearson correlation coefficient, Bland-Altman analysis, and two-sided t-test. In 38 of the 51 individuals (74.5 %), the diagnosis of Marfan syndrome was established according to the criteria of the Ghent-2 nosology. Cine-MRI measurements of the sinuses of Valsalva revealed a strong correlation with echocardiography (r=0.929), but a statistically significant bias of -1.0 mm (p<0.001). The mean absolute diameter for sinuses of Valsalva obtained by cine-MRI was 32.3 ± 5.8 mm as compared to 33.4 ± 5.4 mm obtained by echocardiography. Interobserver agreement of measurements of the sinuses of Valsalva was higher for cine-MRI than for echocardiography (p=0.029). Despite small, but statistically significant differences in terms of agreement and reproducibility, cine-MRI and echocardiographic measurements of aortic root diameters provide comparable results without a significant clinical difference. Therefore both techniques may be used for monitoring of the aortic root in patients with Marfan syndrome.

  1. Thoracic aorta 3D hemodynamics in pediatric and young adult patients with bicuspid aortic valve.

    Science.gov (United States)

    Allen, Bradley D; van Ooij, Pim; Barker, Alex J; Carr, Maria; Gabbour, Maya; Schnell, Susanne; Jarvis, Kelly B; Carr, James C; Markl, Michael; Rigsby, Cynthia; Robinson, Joshua D

    2015-10-01

    To evaluate the 3D hemodynamics in the thoracic aorta of pediatric and young adult bicuspid aortic valve (BAV) patients. 4D flow MRI was performed in 30 pediatric and young adult BAV patients (age: 13.9 ± 4.4 (range: [3.4, 20.7]) years old, M:F = 17:13) as part of this Institutional Review Board-approved study. Nomogram-based aortic root Z-scores were calculated to assess aortic dilatation and degree of aortic stenosis (AS) severity was assessed on MRI. Data analysis included calculation of time-averaged systolic 3D wall shear stress (WSSsys ) along the entire aorta wall, and regional quantification of maximum and mean WSSsys and peak systolic velocity (velsys ) in the ascending aorta (AAo), arch, and descending aorta (DAo). The 4D flow MRI AAo velsys was also compared with echocardiography peak velocity measurements. There was a positive correlation with both mean and max AAo WSSsys and peak AAo velsys (mean: r = 0.84, P max: r = 0.94, P max: rS  = 0.70, P < 0.001). AAo peak velocity was significantly higher when measured with echo compared with 4D flow MRI (2.1 ± 0.98 m/s versus 1.27 ± 0.49 m/s, P < 0.001). In pediatric and young adult patients with BAV, AS and peak ascending aorta velocity are associated with increased AAo WSS, while aortic dilation, age, and body surface area do not significantly impact AAo hemodynamics. Prospective studies are required to establish the role of WSS as a risk-stratification tool in these patients. © 2015 Wiley Periodicals, Inc.

  2. Nicotinamide Phosphoribosyltransferase in Smooth Muscle Cells Maintains Genome Integrity, Resists Aortic Medial Degeneration, and Is Suppressed in Human Thoracic Aortic Aneurysm Disease.

    Science.gov (United States)

    Watson, Alanna; Nong, Zengxuan; Yin, Hao; O'Neil, Caroline; Fox, Stephanie; Balint, Brittany; Guo, Linrui; Leo, Oberdan; Chu, Michael W A; Gros, Robert; Pickering, J Geoffrey

    2017-06-09

    The thoracic aortic wall can degenerate over time with catastrophic consequences. Vascular smooth muscle cells (SMCs) can resist and repair artery damage, but their capacities decline with age and stress. Recently, cellular production of nicotinamide adenine dinucleotide (NAD + ) via nicotinamide phosphoribosyltransferase (Nampt) has emerged as a mediator of cell vitality. However, a role for Nampt in aortic SMCs in vivo is unknown. To determine whether a Nampt-NAD + control system exists within the aortic media and is required for aortic health. Ascending aortas from patients with dilated aortopathy were immunostained for NAMPT, revealing an inverse relationship between SMC NAMPT content and aortic diameter. To determine whether a Nampt-NAD + control system in SMCs impacts aortic integrity, mice with Nampt -deficient SMCs were generated. SMC- Nampt knockout mice were viable but with mildly dilated aortas that had a 43% reduction in NAD + in the media. Infusion of angiotensin II led to aortic medial hemorrhage and dissection. SMCs were not apoptotic but displayed senescence associated-ß-galactosidase activity and upregulated p16, indicating premature senescence. Furthermore, there was evidence for oxidized DNA lesions, double-strand DNA strand breaks, and pronounced susceptibility to single-strand breakage. This was linked to suppressed poly(ADP-ribose) polymerase-1 activity and was reversible on resupplying NAD + with nicotinamide riboside. Remarkably, we discovered unrepaired DNA strand breaks in SMCs within the human ascending aorta, which were specifically enriched in SMCs with low NAMPT. NAMPT promoter analysis revealed CpG hypermethylation within the dilated human thoracic aorta and in SMCs cultured from these tissues, which inversely correlated with NAMPT expression. The aortic media depends on an intrinsic NAD + fueling system to protect against DNA damage and premature SMC senescence, with relevance to human thoracic aortopathy. © 2017 American Heart

  3. More than just a urinary catheter — Haemorrhage control by using a Foley catheter in a penetrating aortic root injury

    Directory of Open Access Journals (Sweden)

    Adel Elmoghrabi

    2016-08-01

    Full Text Available The prevalence of great vessel injuries in thoracic trauma is reported at 0.3–10%, predominantly due to a penetrating mechanism. Thoracic aortic injuries, more specifically those within proximity of the aortic root are challenging to manage and unceasing bleeding hampers adequate visualisation for performing repair. We report a case of a 31-year-old male that presented to the emergency department 1 h after sustaining a stab wound injury within proximity of the left upper sternal border. Vital signs were stable on presentation. Physical examination revealed a 1.5 cm laceration in the 3rd intercostal space. Chest X-ray revealed a small left pneumothorax, FAST scan was negative, and CT of the chest revealed left sided haemopneumothorax and haemopericardium. The patient was emergently transferred to the operating room where median thoracotomy was performed. A significant amount of bleeding was observed originating from a 1 cm laceration of the aortic root. Bleeding was controlled using a Foley catheter after unsuccessful attempts of digital compression, and the laceration was repaired using pledgeted sutures. Postoperative echocardiography and CT scan of the chest revealed normal cardiac functions with resolution of haemopericardium and haemopneumothorax, and the patient was discharged in a stable condition. High index of suspicion should be maintained for injury to the great vessels in patients with penetrating chest injuries, despite apparent haemodynamic stability. In this case, balloon tamponade using a Foley catheter served as a quick and simple technique that resulted in an almost bloodless field, facilitating adequate visualisation for definitive repair. Although the use of this technique has been previously described, this report serves as a reminder that a Foley catheter can be successfully used for balloon catheter tamponade in injuries to the aortic root. Keywords: Aortic root, Injury, Foley's catheter, Balloon catheter, Aorta

  4. Elevated expression levels of lysyl oxidases protect against aortic aneurysm progression in Marfan syndrome.

    Science.gov (United States)

    Busnadiego, O; Gorbenko Del Blanco, D; González-Santamaría, J; Habashi, J P; Calderon, J F; Sandoval, P; Bedja, D; Guinea-Viniegra, J; Lopez-Cabrera, M; Rosell-Garcia, T; Snabel, J M; Hanemaaijer, R; Forteza, A; Dietz, H C; Egea, G; Rodriguez-Pascual, F

    2015-08-01

    Patients with Marfan syndrome (MFS) are at high risk of life-threatening aortic dissections. The condition is caused by mutations in the gene encoding fibrillin-1, an essential component in the formation of elastic fibers. While experimental findings in animal models of the disease have shown the involvement of transforming growth factor-β (TGF-β)- and angiotensin II-dependent pathways, alterations in the vascular extracellular matrix (ECM) may also play a role in the onset and progression of the aortic disease. Lysyl oxidases (LOX) are extracellular enzymes, which initiates the formation of covalent cross-linking of collagens and elastin, thereby contributing to the maturation of the ECM. Here we have explored the role of LOX in the formation of aortic aneurysms in MFS. We show that aortic tissue from MFS patients and MFS mouse model (Fbn1(C1039G/+)) displayed enhanced expression of the members of the LOX family, LOX and LOX-like 1 (LOXL1), and this is associated with the formation of mature collagen fibers. Administration of a LOX inhibitor for 8weeks blocked collagen accumulation and aggravated elastic fiber impairment, and these effects correlated with the induction of a strong and rapidly progressing aortic dilatation, and with premature death in the more severe MFS mouse model, Fbn1(mgR/mgR), without any significant effect on wild type animals. This detrimental effect occurred preferentially in the ascending portion of the aorta, with little or no involvement of the aortic root, and was associated to an overactivation of both canonical and non-canonical TGF-β signaling pathways. The blockade of angiotensin II type I receptor with losartan restored TGF-β signaling activation, normalized elastic fiber impairment and prevented the aortic dilatation induced by LOX inhibition in Fbn1(C1039G/+) mice. Our data indicate that LOX enzymes and LOX-mediated collagen accumulation play a critical protective role in aneurysm formation in MFS. Copyright © 2015 Elsevier

  5. Aortic root operations for Marfan syndrome: a comparison of the Bentall and valve-sparing procedures.

    Science.gov (United States)

    Patel, Nishant D; Weiss, Eric S; Alejo, Diane E; Nwakanma, Lois U; Williams, Jason A; Dietz, Harry C; Spevak, Philip J; Gott, Vincent L; Vricella, Luca A; Cameron, Duke E

    2008-06-01

    We compared results of the Bentall procedure with valve-sparing aortic root replacement (VSRR) for aortic root aneurysm in Marfan syndrome. Marfan syndrome patients who had the Bentall procedure or VSRR at our institution between April 1997 and September 2006 were identified. Follow-up information was obtained from hospital charts and contact with patients or their physicians. Kaplan-Meier survival and propensity score analyses were performed. One hundred forty Marfan syndrome patients had either the Bentall procedure (n = 56) or VSRR (n = 84; 40 remodeling and 44 reimplantation). Bentall patients were older than VSRR patients (38 versus 29 years; p = 0.0001) and had more aortic dissections (16% versus 1%; p = 0.0012); more urgent/emergent surgery (20% versus 2%; p = 0.0008); larger preoperative sinus diameter (5.7 versus 5.1 cm; p = 0.0004); and more preoperative 3+/4+ aortic insufficiency (59% versus 10%; p valve replacement was 90% for VSRR patients. Eight-year survival was 90% for Bentall and 100% for VSRR patients (p = 0.01). Propensity-adjusted regression showed that the Bentall procedure did not predict mortality (p = 1.00) and did not protect from reoperation (odds ratio = 0.28; 95% confidence interval: 0.01 to 4.33; p = 0.36). The Bentall procedure and VSRR have similar operative results in Marfan syndrome. The procedures are distinguished by higher rates of thromboembolism among Bentall patients and higher rates of reoperation among VSRR patients. Lower late survival among Bentall patients probably reflects the preferential use of the Bentall procedure for higher risk patients.

  6. Aortic root segmentation in 4D transesophageal echocardiography

    Science.gov (United States)

    Chechani, Shubham; Suresh, Rahul; Patwardhan, Kedar A.

    2018-02-01

    The Aortic Valve (AV) is an important anatomical structure which lies on the left side of the human heart. The AV regulates the flow of oxygenated blood from the Left Ventricle (LV) to the rest of the body through aorta. Pathologies associated with the AV manifest themselves in structural and functional abnormalities of the valve. Clinical management of pathologies often requires repair, reconstruction or even replacement of the valve through surgical intervention. Assessment of these pathologies as well as determination of specific intervention procedure requires quantitative evaluation of the valvular anatomy. 4D (3D + t) Transesophageal Echocardiography (TEE) is a widely used imaging technique that clinicians use for quantitative assessment of cardiac structures. However, manual quantification of 3D structures is complex, time consuming and suffers from inter-observer variability. Towards this goal, we present a semiautomated approach for segmentation of the aortic root (AR) structure. Our approach requires user-initialized landmarks in two reference frames to provide AR segmentation for full cardiac cycle. We use `coarse-to-fine' B-spline Explicit Active Surface (BEAS) for AR segmentation and Masked Normalized Cross Correlation (NCC) method for AR tracking. Our method results in approximately 0.51 mm average localization error in comparison with ground truth annotation performed by clinical experts on 10 real patient cases (139 3D volumes).

  7. Sequential Hybrid Repair of Aorta and Bilateral Common Iliac Arteries Secondary to Chronic Aortic Dissection with Extensive Aneurysmal Degeneration in a Marfan Patient.

    Science.gov (United States)

    Hinojosa, Carlos A; Anaya-Ayala, Javier E; Laparra-Escareno, Hugo; Lizola, Rene; Torres-Machorro, Adriana

    2017-09-01

    Marfan syndrome is a connective tissue disorder associated with aortic dissection, aneurysmal degeneration and rupture. These cardiovascular complications represent the main cause of mortality, therefore repair is indicated. We present a 35-year-old woman who experienced acute onset of chest pain. Her imaging revealed a chronic DeBakey type I dissection with aortic root dilation and descending thoracic aneurysmal degeneration. She underwent a Bentall procedure and endovascular exclusion of the descending thoracic aortic aneurysm. She was closely followed and 2 years later a computed tomography angiography (CTA) revealed the aneurysmal degeneration of the thoracoabominal aorta and bilateral iliac arteries. The patient underwent a composite reconstruction using multi-visceral branched and bifurcated Dacron grafts. At 5 years from her last surgery, a CTA revealed no new dissection or further aneurysmal degenerations. Aortic disease in Marfan patients is a complex clinical problem that may lead to secondary or tertiary aortic reconstructions; close follow-up is mandatory.

  8. Systemic aspergilloma post aortic root surgery following coronary artery stenting: diagnostic and management dilemma

    Science.gov (United States)

    Hussein, Nabil; Qamar, Sombul; Abid, Qamar

    2015-01-01

    Aspergillus infections such as Aspergillus endocarditis were once relatively rare occurrences, however, due to the increased use of intracardiac devices, the incidence has grown. With mortality rates close to 100%, in medically treated cases, it is paramount that early diagnosis and treatment are performed. An immunocompetent aviculturist presented 8 months post aortic root replacement for severe aortic regurgitation with a composite graft, with central crushing chest pain. Investigations confirmed ST elevation inferior myocardial infarction due to stenosis of the origin of the right coronary artery, which was stented. Echocardiogram demonstrated a mobile mass posterior to the left ventricular outflow tract. Following referral to our cardiothoracic surgeons, a polypoidal mass covering the right ostial button was noted along with systemic complications of the disease. Emergency redo aortic valve replacement with a homograft and coronary artery bypass was performed. Histological analysis confirmed A. fumigatus and the patient was started on intravenous voriconazole. PMID:26025972

  9. A non-contrast self-navigated 3-dimensional MR technique for aortic root and vascular access route assessment in the context of transcatheter aortic valve replacement: proof of concept

    Energy Technology Data Exchange (ETDEWEB)

    Renker, Matthias [Medical University of South Carolina, Heart and Vascular Center, Charleston, SC (United States); University Hospital Giessen and Marburg, Department of Medicine I, Giessen (Germany); Varga-Szemes, Akos; Rier, Jeremy D.; Steinberg, Daniel H. [Medical University of South Carolina, Heart and Vascular Center, Charleston, SC (United States); Schoepf, U.J. [Medical University of South Carolina, Heart and Vascular Center, Charleston, SC (United States); Medical University of South Carolina, Department of Radiology and Radiological Science, Charleston, SC (United States); Baumann, Stefan [Medical University of South Carolina, Heart and Vascular Center, Charleston, SC (United States); University of Heidelberg, 1st Department of Medicine, Faculty of Medicine Mannheim, University Medical Centre Mannheim (UMM), Mannheim (Germany); Piccini, Davide [Siemens Healthcare IM BM PI, Advanced Clinical Imaging Technology, Lausanne (Switzerland); University Hospital (CHUV) and University of Lausanne (UNIL), Department of Radiology, Lausanne (Switzerland); Zenge, Michael O.; Mueller, Edgar [Siemens AG Healthcare Sector, Erlangen (Germany); Rehwald, Wolfgang G. [Duke University Medical Center, Cardiovascular MR Center, Durham, NC (United States); Moellmann, Helge [Kerckhoff Heart and Thorax Center, Bad Nauheim (Germany); Hamm, Christian W. [University Hospital Giessen and Marburg, Department of Medicine I, Giessen (Germany); De Cecco, Carlo N. [Medical University of South Carolina, Heart and Vascular Center, Charleston, SC (United States); University of Rome ' ' Sapienza' ' -Polo Pontino, Department of Radiological Sciences, Oncology and Pathology, Latina (Italy)

    2016-04-15

    Due to the high prevalence of renal failure in transcatheter aortic valve replacement (TAVR) candidates, a non-contrast MR technique is desirable for pre-procedural planning. We sought to evaluate the feasibility of a novel, non-contrast, free-breathing, self-navigated three-dimensional (SN3D) MR sequence for imaging the aorta from its root to the iliofemoral run-off in comparison to non-contrast two-dimensional-balanced steady-state free-precession (2D-bSSFP) imaging. SN3D [field of view (FOV), 220-370 mm{sup 3}; slice thickness, 1.15 mm; repetition/echo time (TR/TE), 3.1/1.5 ms; and flip angle, 115 ] and 2D-bSSFP acquisitions (FOV, 340 mm; slice thickness, 6 mm; TR/TE, 2.3/1.1 ms; flip angle, 77 ) were performed in 10 healthy subjects (all male; mean age, 30.3 ± 4.3 yrs) using a 1.5-T MRI system. Aortic root measurements and qualitative image ratings (four-point Likert-scale) were compared. The mean effective aortic annulus diameter was similar for 2D-bSSFP and SN3D (26.7 ± 0.7 vs. 26.1 ± 0.9 mm, p = 0.23). The mean image quality of 2D-bSSFP (4; IQR 3-4) was rated slightly higher (p = 0.03) than SN3D (3; IQR 2-4). The mean total acquisition time for SN3D imaging was 12.8 ± 2.4 min. Our results suggest that a novel SN3D sequence allows rapid, free-breathing assessment of the aortic root and the aortoiliofemoral system without administration of contrast medium. (orig.)

  10. A non-contrast self-navigated 3-dimensional MR technique for aortic root and vascular access route assessment in the context of transcatheter aortic valve replacement: proof of concept

    International Nuclear Information System (INIS)

    Renker, Matthias; Varga-Szemes, Akos; Rier, Jeremy D.; Steinberg, Daniel H.; Schoepf, U.J.; Baumann, Stefan; Piccini, Davide; Zenge, Michael O.; Mueller, Edgar; Rehwald, Wolfgang G.; Moellmann, Helge; Hamm, Christian W.; De Cecco, Carlo N.

    2016-01-01

    Due to the high prevalence of renal failure in transcatheter aortic valve replacement (TAVR) candidates, a non-contrast MR technique is desirable for pre-procedural planning. We sought to evaluate the feasibility of a novel, non-contrast, free-breathing, self-navigated three-dimensional (SN3D) MR sequence for imaging the aorta from its root to the iliofemoral run-off in comparison to non-contrast two-dimensional-balanced steady-state free-precession (2D-bSSFP) imaging. SN3D [field of view (FOV), 220-370 mm 3 ; slice thickness, 1.15 mm; repetition/echo time (TR/TE), 3.1/1.5 ms; and flip angle, 115 ] and 2D-bSSFP acquisitions (FOV, 340 mm; slice thickness, 6 mm; TR/TE, 2.3/1.1 ms; flip angle, 77 ) were performed in 10 healthy subjects (all male; mean age, 30.3 ± 4.3 yrs) using a 1.5-T MRI system. Aortic root measurements and qualitative image ratings (four-point Likert-scale) were compared. The mean effective aortic annulus diameter was similar for 2D-bSSFP and SN3D (26.7 ± 0.7 vs. 26.1 ± 0.9 mm, p = 0.23). The mean image quality of 2D-bSSFP (4; IQR 3-4) was rated slightly higher (p = 0.03) than SN3D (3; IQR 2-4). The mean total acquisition time for SN3D imaging was 12.8 ± 2.4 min. Our results suggest that a novel SN3D sequence allows rapid, free-breathing assessment of the aortic root and the aortoiliofemoral system without administration of contrast medium. (orig.)

  11. Aortic Dissection in a Healthy Male Athlete: A Unique Case with Comprehensive Literature Review

    Directory of Open Access Journals (Sweden)

    Balraj Singh

    2016-01-01

    Full Text Available A young otherwise healthy 27-year-old male who has been using anabolic steroids for a long time developed Type I aortic dissection associated with heavy weightlifting. The patient did not have a recent history of trauma to the chest, no history of hypertension, and no illicit drug use. He presented with severe chest pain radiating to back and syncopal event with exertion. Initial vitals were significant for blood pressure of 80/50 mmHg, pulse of 80 beats per minute, respirations of 24 per minute, and oxygen saturation of 92% on room air. Physical exam was significant for elevated jugular venous pressure, muffled heart sounds, and cold extremities with diminished pulses in upper and absent pulses in lower extremities. Bedside echocardiogram showed aortic root dilatation and cardiac tamponade. STAT computed tomography (CT scan of chest revealed dissection of ascending aorta. Cardiothoracic surgery was consulted and patient underwent successful repair of ascending aorta. Hemodynamic stress of weightlifting can predispose to aortic dissection. Aortic dissection is a rare but often catastrophic condition if not diagnosed and managed acutely. Although rare, aortic dissection needs to be in the differential when a young weightlifter presents with chest pain as a delay in diagnosis may be fatal.

  12. Heart failure and sudden cardiac death in heritable thoracic aortic disease caused by pathogenic variants in the SMAD3 gene.

    Science.gov (United States)

    Backer, Julie De; Braverman, Alan C

    2018-05-01

    Predominant cardiovascular manifestations in the spectrum of Heritable Thoracic Aortic Disease include by default aortic root aneurysms- and dissections, which may be associated with aortic valve disease. Mitral- and tricuspid valve prolapse are other commonly recognized features. Myocardial disease, characterized by heart failure and/or malignant arrhythmias has been reported in humans and in animal models harboring pathogenic variants in the Fibrillin1 gene. Description of clinical history of three cases from one family in Ghent (Belgium) and one family in St. Louis (US). We report on three cases from two families presenting end-stage heart failure (in two) and lethal arrhythmias associated with moderate left ventricular dilatation (in one). All three cases harbor a pathogenic variant in the SMAD3 gene, known to cause aneurysm osteoarthritis syndrome, Loeys-Dietz syndrome type 3 or isolated Heritable Thoracic Aortic Disease. These unusual presentations warrant awareness for myocardial disease in patients harboring pathogenic variants in genes causing Heritable Thoracic Aortic Disease and indicate the need for prospective studies in larger cohorts. © 2018 The Authors. Molecular Genetics & Genomic Medicine published by Wiley Periodicals, Inc.

  13. Arch translocation and the intra-arch elephant-trunk technique with collared graft for extended chronic dissecting aortic aneurysm

    Directory of Open Access Journals (Sweden)

    Ikenaga Shigeru

    2013-01-01

    Full Text Available Abstract Management of extensive, chronic, dissecting aortic aneurysms after prior repair of the ascending aorta presents a technical challenge for surgeons. A symptomatic 64-year-old patient was admitted for elective surgical repair of an aortic annular dilatation, causing severe aortic regurgitation, and a Crawford type II extended thoracoabdominal aneurysm, 4 years after he underwent primary repair of an acute aortic dissection. The aorta was diffusely dilated, and there were no sites beyond the distal aortic arch where anastomosis could be performed. We successfully performed total aortic replacement with a 2-stage strategy, using an arch translocation technique and an intra-arch elephant-trunk technique.

  14. Multi detector computed tomography (MDCT) of the aortic root; ECG-gated verses non-ECG-gated examinations

    International Nuclear Information System (INIS)

    Kristiansen, Joanna; Guenther, Anne; Aalokken, Trond Mogens; Andersen, Rune

    2011-01-01

    Purpose: Motion artifacts may degrade a conventional CT examination of the ascending aorta and hinder accurate diagnosis. We quantitatively compared retrospectively electrocardiographic (ECG) -gated multi detector computed tomography (MDCT) with non-ECG-gated MDCT in order to demonstrate whether or not one of the methods should be preferred. Method: The study included seventeen patients with surgically reconstructed aortic root and reimplanted coronary arteries. All patients had undergone both non-gated MDCT and retrospectively ECG-gated MDCT employing a stringently modulated tube current with single phase image reconstruction. The incidence of motion artifacts in the left main coronary artery (LM), proximal right coronary artery (RCA), and aortic root and ascending aorta were rated using a four point scale. The effective dose for each scan was calculated and normalized to a 15 cm scan length. Statistical analysis of motion artifacts and radiation dose was performed using Wilcoxon matched pairs signed rank sum test. Results: A significant reduction in motion artifacts was found in all three vessels in images from the retrospectively ECG-gated scans (LM: P = 0.005, RCA: P = 0.015, aorta: P = 0.003). The mean normalized effective radiation dose was 3.69 mSv (±1.03) for the non-ECG-gated scans and 16.37 mSv (±2.53) for the ECG-gated scans. Conclusion: Retrospective ECG-gating with single phase reconstruction significantly reduces the incidence of motion artifacts in the aortic root and the proximal portion of the coronary arteries but at the expense of a fourfold increase in radiation dose.

  15. Facial and Ocular Features of Marfan Syndrome

    Directory of Open Access Journals (Sweden)

    Juan C. Leoni

    2014-10-01

    Full Text Available Marfan syndrome is the most common inherited disorder of connective tissue affecting multiple organ systems. Identification of the facial, ocular and skeletal features should prompt referral for aortic imaging since sudden death by aortic dissection and rupture remains a major cause of death in patients with unrecognized Marfan syndrome. Echocardiography is recommended as the initial imaging test, and once a dilated aortic root is identified magnetic resonance or computed tomography should be done to assess the entire aorta. Prophylactic aortic root replacement is safe and has been demonstrated to improve life expectancy in patients with Marfan syndrome. Medical therapy for Marfan syndrome includes the use of beta blockers in older children and adults with an enlarged aorta. Addition of angiotensin receptor antagonists has been shown to slow the progression of aortic root dilation compared to beta blockers alone. Lifelong and regular follow up in a center for specialized care is important for patients with Marfan syndrome. We present a case of a patient with clinical features of Marfan syndrome and discuss possible therapeutic interventions for her dilated aorta.

  16. Quantified degree of eccentricity of aortic valve calcification predicts risk of paravalvular regurgitation and response to balloon post-dilation after self-expandable transcatheter aortic valve replacement.

    Science.gov (United States)

    Park, Jun-Bean; Hwang, In-Chang; Lee, Whal; Han, Jung-Kyu; Kim, Chi-Hoon; Lee, Seung-Pyo; Yang, Han-Mo; Park, Eun-Ah; Kim, Hyung-Kwan; Chiam, Paul T L; Kim, Yong-Jin; Koo, Bon-Kwon; Sohn, Dae-Won; Ahn, Hyuk; Kang, Joon-Won; Park, Seung-Jung; Kim, Hyo-Soo

    2018-05-15

    Limited data exist regarding the impact of aortic valve calcification (AVC) eccentricity on the risk of paravalvular regurgitation (PVR) and response to balloon post-dilation (BPD) after transcatheter aortic valve replacement (TAVR). We investigated the prognostic value of AVC eccentricity in predicting the risk of PVR and response to BPD in patients undergoing TAVR. We analyzed 85 patients with severe aortic stenosis who underwent self-expandable TAVR (43 women; 77.2±7.1years). AVC was quantified as the total amount of calcification (total AVC load) and as the eccentricity of calcium (EoC) using calcium volume scoring with contrast computed tomography angiography (CTA). The EoC was defined as the maximum absolute difference in calcium volume scores between 2 adjacent sectors (bi-partition method) or between sectors based on leaflets (leaflet-based method). Total AVC load and bi-partition EoC, but not leaflet-based EoC, were significant predictors for the occurrence of ≥moderate PVR, and bi-partition EoC had a better predictive value than total AVC load (area under the curve [AUC]=0.863 versus 0.760, p for difference=0.006). In multivariate analysis, bi-partition EoC was an independent predictor for the risk of ≥moderate PVR regardless of perimeter oversizing index. The greater bi-partition EoC was the only significant parameter to predict poor response to BPD (AUC=0.775, p=0.004). Pre-procedural assessment of AVC eccentricity using CTA as "bi-partition EoC" provides useful predictive information on the risk of significant PVR and response to BPD in patients undergoing TAVR with self-expandable valves. Copyright © 2017 Elsevier B.V. All rights reserved.

  17. Dynamics of the aortic annulus in 4D CT angiography for transcatheter aortic valve implantation patients

    NARCIS (Netherlands)

    Elattar, Mustafa A.; Vink, Leon W.; van Mourik, Martijn S.; Baan, Jan; Vanbavel, Ed T.; Planken, R. Nils; Marquering, Henk A.

    2017-01-01

    Transcatheter aortic valve implantation (TAVI) is a well-established treatment for patients with severe aortic valve stenosis. This procedure requires pre-operative planning by assessment of aortic dimensions on CT Angiography (CTA). It is well-known that the aortic root dimensions vary over the

  18. Bentall operation, total aortic replacement and mitral valve replacement for a young adult with Marfan syndrome: a case of three-staged operation.

    Science.gov (United States)

    Inui, K; Shimazaki, Y; Watanabe, T; Kuraoka, S; Minowa, T; Miura, M; Oshikiri, S; Toyama, H

    1998-08-01

    In Marfan syndrome, the most common cardiovascular abnormalities are dilatation of the aorta and aortic valve regurgitation in adult patients. Mitral valve dysfunction is the most common cause of morbidity and mortality in infants and children with Marfan syndrome, and is not frequently operated on in adult Marfan patients who undergo surgery for diseases of the aortic root and total aorta. This report describes a successfully three-staged operation for a 24 year-old man with Marfan syndrome who underwent an emergent Bentall operation and aortic arch replacement, total aortic replacement and mitral valve replacement over 2 years. Mitral valve regurgitation was mild but increased after the second operation. The graft was tightly adhesive and invasive to the sternum. Endoscopic view was helpful to avoid graft damage at resternotomy. The postoperative course was uneventful in each operation. Microscopic examination of the mitral valve leaflets showed abnormal increase of mucopolysaccharides, and disruption and fragmentation of elastic fibers.

  19. Assessment and feasibility of the four landmarks of the aortic root in a cohort of very preterm infants

    Directory of Open Access Journals (Sweden)

    Sam Phillips

    2015-01-01

    Conclusion : We present reliability and reference values for all four anatomic landmarks of the aortic root in very preterm infants and demonstrated the importance of standardizing and reporting cardiac output measurements in preterm infants.

  20. Cardiovascular Benefits of Moderate Exercise Training in Marfan Syndrome: Insights From an Animal Model.

    Science.gov (United States)

    Mas-Stachurska, Aleksandra; Siegert, Anna-Maria; Batlle, Monsterrat; Gorbenko Del Blanco, Darya; Meirelles, Thayna; Rubies, Cira; Bonorino, Fabio; Serra-Peinado, Carla; Bijnens, Bart; Baudin, Julio; Sitges, Marta; Mont, Lluís; Guasch, Eduard; Egea, Gustavo

    2017-09-25

    Marfan syndrome (MF) leads to aortic root dilatation and a predisposition to aortic dissection, mitral valve prolapse, and primary and secondary cardiomyopathy. Overall, regular physical exercise is recommended for a healthy lifestyle, but dynamic sports are strongly discouraged in MF patients. Nonetheless, evidence supporting this recommendation is lacking. Therefore, we studied the role of long-term dynamic exercise of moderate intensity on the MF cardiovascular phenotype. In a transgenic mouse model of MF ( Fbn1 C1039G/+ ), 4-month-old wild-type and MF mice were subjected to training on a treadmill for 5 months; sedentary littermates served as controls for each group. Aortic and cardiac remodeling was assessed by echocardiography and histology. The 4-month-old MF mice showed aortic root dilatation, elastic lamina rupture, and tunica media fibrosis, as well as cardiac hypertrophy, left ventricular fibrosis, and intramyocardial vessel remodeling. Over the 5-month experimental period, aortic root dilation rate was significantly greater in the sedentary MF group, compared with the wild-type group (∆mm, 0.27±0.07 versus 0.13±0.02, respectively). Exercise significantly blunted the aortic root dilation rate in MF mice compared with sedentary MF littermates (∆mm, 0.10±0.04 versus 0.27±0.07, respectively). However, these 2 groups were indistinguishable by aortic root stiffness, tunica media fibrosis, and elastic lamina ruptures. In MF mice, exercise also produced cardiac hypertrophy regression without changes in left ventricular fibrosis. Our results in a transgenic mouse model of MF indicate that moderate dynamic exercise mitigates the progression of the MF cardiovascular phenotype. © 2017 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley.

  1. Personalised external aortic root support (PEARS) in Marfan syndrome: Analysis of 1-9 year outcomes by intention-to-treat in a cohort of the first 30 consecutive patients to receive a novel tissue and valve-conserving procedure, compared with the published results of aortic root replacement

    NARCIS (Netherlands)

    T. Treasure (Tom); J.J.M. Takkenberg (Hanneke); T. Golesworthy (Tal); F. Rega (Filip); M. Petrou (Mario); U. Rosendahl (Ulrich); R. Mohiaddin (Raad); M. Rubens (Michael); W. Thornton (Warren); B. Lees (Belinda); J. Pepper (John)

    2014-01-01

    textabstractObjective: Among people with Marfan syndrome who have a typical aortic root aneurysm, dissection is a characteristic cause of premature death. To pre-empt Type A dissection, composite root replacement with a mechanical valve became the standard of care in the 1980s and 1990s. This is

  2. Compression of the right coronary artery by an aortic pseudoaneurysm after infective endocarditis: an unusual case of myocardial ischemia.

    Science.gov (United States)

    Lacalzada-Almeida, Juan; De la Rosa-Hernández, Alejandro; Izquierdo-Gómez, María Manuela; García-Niebla, Javier; Hernández-Betancor, Iván; Bonilla-Arjona, Juan Alfonso; Barragán-Acea, Antonio; Laynez-Cerdeña, Ignacio

    2018-01-01

    A 61-year-old male with a prosthetic St Jude aortic valve size 24 presented with heart failure symptoms and minimal-effort angina. Eleven months earlier, the patient had undergone cardiac surgery because of an aortic root dilatation and bicuspid aortic valve with severe regurgitation secondary to infectious endocarditis by Coxiela burnetii and coronary artery disease in the left circumflex coronary artery. Then, a prosthesis valve and a saphenous bypass graft to the left circumflex coronary artery were placed. The patient was admitted to the Cardiology Department of Hospital Universitario de Canarias, Tenerife, Spain and a transthoracic echocardiography was performed that showed severe paraprosthetic aortic regurgitation and an aortic pseudoaneurysm. The 64-slice multidetector computed tomography confirmed the pseudoaneurysm, originating from the right sinus of Valsalva, with a compression of the native right coronary artery and a normal saphenous bypass graft. On the basis of these findings, we performed surgical treatment with a favorable postoperative evolution. In our case, results from complementary cardiac imaging techniques were crucial for patient management. The multidetector computed tomography allowed for a confident diagnosis of an unusual mechanism of coronary ischemia.

  3. Valve-sparing and valve-replacing techniques for aortic root replacement in patients with Marfan syndrome: Analysis of early outcome.

    Science.gov (United States)

    Volguina, Irina V; Miller, D Craig; LeMaire, Scott A; Palmero, Laura C; Wang, Xing Li; Connolly, Heidi M; Sundt, Thoralf M; Bavaria, Joseph E; Dietz, Harry C; Milewicz, Dianna M; Coselli, Joseph S

    2009-05-01

    A prospective, international registry study was initiated to provide contemporary comparative data on short-term clinical outcomes after aortic valve-sparing and aortic valve-replacing root operations in patients with Marfan syndrome. The purpose of this initial report is to describe the study design and to compare early outcomes in the first 151 enrolled patients. We assessed 30-day outcomes in 151 patients who met strict Ghent diagnostic criteria for Marfan syndrome and underwent aortic root replacement with either valve-replacing (n = 46) or valve-sparing techniques (n = 105) at one of 18 participating centers. In the valve replacement group, a mechanical composite valve graft was used in 39 (85%) patients and a bioprosthetic valve in 7 (15%). In the valve-sparing group, David V procedures were performed in 57 (54%) patients, David I in 38 (36%), David IV in 8 (8%), Florida sleeve in 1 (1%), and Yacoub remodeling in 1 (1%). No in-hospital or 30-day deaths occurred. Despite longer crossclamp and cardiopulmonary bypass times in the valve-sparing group, there were no significant between-group differences in postoperative complications. Thirty-day valve-related complications occurred in 2 (4%) patients undergoing valve replacement and in 3 (3%) undergoing valve-sparing procedures (P = .6). The analysis of early outcomes revealed that valve-sparing techniques were the most common approach to root replacement in patients with Marfan syndrome in these centers. The complexity of valve-sparing root replacement did not translate into any demonstrable adverse early outcomes. Subsequent analysis will compare the 3-year durability of these two surgical approaches.

  4. Three-dimensional flow structures past a bio-prosthetic valve in an in-vitro model of the aortic root

    OpenAIRE

    Hasler, David; Obrist, Dominik

    2018-01-01

    The flow field past a prosthetic aortic valve comprises many details that indicate whether the prosthesis is functioning well or not. It is, however, not yet fully understood how an optimal flow scenario would look, i.e. which subtleties of the fluid dynamics in place are essential regarding the durability and compatibility of a prosthetic valve. In this study, we measured and analyzed the 3D flow field in the vicinity of a bio-prosthetic heart valve in function of the aortic root size. The m...

  5. An Erupted Dilated Odontoma: A Rare Presentation

    Directory of Open Access Journals (Sweden)

    Gaurav Sharma

    2016-01-01

    Full Text Available A dilated odontoma is an extremely rare developmental anomaly represented as a dilatation of the crown and root as a consequence of a deep, enamel-lined invagination and is considered a severe variant of dens invaginatus. An oval shape of the tooth lacking morphological characteristics of a crown or root implies that the invagination happened in the initial stages of morphodifferentiation. Spontaneous eruption of an odontoma is a rare occurrence and the occurrence of a dilated odontoma in a supernumerary tooth is even rarer with only a few case reports documented in the English literature. We present an extremely rare case of erupted dilated odontoma occurring in the supernumerary tooth in anterior maxillary region in an 18-year-old male, which, to the best of our knowledge, is the first ever case reported in English literature.

  6. Modelling and numerical simulation of the in vivo mechanical response of the ascending aortic aneurysm in Marfan syndrome.

    Science.gov (United States)

    García-Herrera, Claudio M; Celentano, Diego J; Herrera, Emilio A

    2017-03-01

    Marfan syndrome (MFS) is a genetic disorder that affects connective tissue, impairing cardiovascular structures and function, such as heart valves and aorta. Thus, patients with Marfan disease have a higher risk of developing circulatory problems associated with mitral and aortic valves prolapse, manifested as dilated aorta and aortic aneurysm. However, little is known about the biomechanical characteristics of these structures affected with MFS. This study presents the modelling and simulation of the mechanical response of human ascending aortic aneurysms in MFS under in vivo conditions with intraluminal pressures within normotensive and hypertensive ranges. We obtained ascending aortic segments from five adults with MFS subjected to a vascular prosthesis implantation replacing an aortic aneurysm. We characterised the arterial samples via ex vivo tensile test measurements that enable fitting the material parameters of a hyperelastic isotropic constitutive model. Then, these material parameters were used in a numerical simulation of an ascending aortic aneurysm subjected to in vivo normotensive and hypertensive conditions. In addition, we assessed different constraints related to the movement of the aortic root. Overall, our results provide not only a realistic description of the mechanical behaviour of the vessel, but also useful data about stress/stretch-based criteria to predict vascular rupture. This knowledge may be included in the clinical assessment to determine risk and indicate surgical intervention.

  7. Aortic valve replacement and prosthesis-patient mismatch in the era of trans-catheter aortic valve implantation.

    Science.gov (United States)

    Morita, Shigeki

    2016-08-01

    The treatment strategy for aortic stenosis (AS) has been changing due to newly developed valvular prostheses and trans-catheter aortic valve implantation (TAVI). To determine the role of new modalities for AS with a small aortic root, papers using the concept of prosthesis-patient mismatch (PPM) were reviewed. First, to determine the cut-off value of the indexed effective orifice area (IEOA) for defining PPM, the studies of surgical aortic valve replacement (SAVR) with a follow-up longer than 5 years and a patient number larger than 500 were reviewed. Second, the papers comparing TAVI and SAVR were reviewed. Furthermore, the prevalence of PPM was reviewed, with the addition of papers on aortic root enlargement, sutureless AVR, and aortic valve reconstruction with autologous pericardium. The results of the long-term survival after aortic valve replacement (AVR) have indicated that an IEOA less than 0.65 cm(2)/m(2) should be avoided in all cases, whereas the indications for patients with an IEOA between 065 and 0.85 cm(2)/m(2) should be determined by considering multiple factors. A large body size and younger age have a significantly negative influence on the long-term survival. In Asian population, the prevalence of PPM was low, despite the fact that the size of the aortic annulus was small. The IEOA after TAVI was larger than after surgical AVR in population-matched studies. To evaluate the role of TAVI and other modalities for a small aortic root, studies with a longer follow-up and larger volume are thus warranted.

  8. Elastin-derived peptides promote abdominal aortic aneurysm formation by modulating M1/M2 macrophage polarization1

    Science.gov (United States)

    Dale, Matthew A; Xiong, Wanfen; Carson, Jeffrey S; Suh, Melissa K; Karpisek, Andrew D.; Meisinger, Trevor M.; Casale, George P.; Baxter, B. Timothy

    2016-01-01

    Abdominal aortic aneurysm (AAA) is a dynamic vascular disease characterized by inflammatory cell invasion and extracellular matrix (ECM) degradation. Damage to elastin in the ECM results in release of elastin-derived peptides (EDPs), which are chemotactic for inflammatory cells such as monocytes. Their effect on macrophage polarization is less well known. Pro-inflammatory M1 macrophages initially are recruited to sites of injury but, if their effects are prolonged, they can lead to chronic inflammation that prevents normal tissue repair. Conversely, anti-inflammatory M2 macrophages reduce inflammation and aid in wound healing. Thus, a proper M1/M2 ratio is vital for tissue homeostasis. AAA tissue reveals a high M1/M2 ratio where pro-inflammatory cells and their associated markers dominate. In the present study, in vitro treatment of bone marrow-derived macrophages with EDPs induced M1 macrophage polarization. By using C57Bl/6 mice, antibody-mediated neutralization of EDPs reduced aortic dilation, matrix metalloproteinase activity, and pro-inflammatory cytokine expression at early and late time points after aneurysm induction. Furthermore, direct manipulation of the M1/M2 balance altered aortic dilation. Injection of M2 polarized macrophages reduced aortic dilation after aneurysm induction. EDPs promoted a pro-inflammatory environment in aortic tissue by inducing M1 polarization and neutralization of EDPs attenuated aortic dilation. The M1/M2 imbalance is vital to aneurysm formation. PMID:27183603

  9. Bicuspid aortic valve syndrome and fibrillinopathies: potential impact on clinical approach

    Directory of Open Access Journals (Sweden)

    Rosina De Cario

    2014-01-01

    Full Text Available Bicuspid aortic valve (BAV is a common heterogeneous disorder whose natural history is determined by hemodynamic valvular impairment and/or increased prevalence of aortic abnormalities ranging from dilatation to aneurysm and dissection. BAV-related aortopathy is frequently associated with relevant aortic pathologic changes leading to structural alterations, characteristic degenerative lesions and histological changes of the aorta very similar to those identified and described in patients with Marfan syndrome (MFS, an inherited connective tissue disorder associated with mutations in fibrillin 1 (FBN1 gene in more than 90% of patients. Recently, a 4-fold increase in the prevalence of BAV in MFS patients has been reported. Subsequently, pathogenetic FBN1 mutations in patients with BAV and aortic dilatation/aneurysm in whom MFS and other more severe type 1 fibrillinopathies were clinically excluded have been identified. In this review we discuss how this evidence, together with that of the wide heterogeneity in pathogenetic mechanisms of BAV-related aortopathy, may impact the clinical management of BAV.

  10. Altered Aortic Upper Wall TDI Velocity Is Inversely Related with Left Ventricular Diastolic Function in Operated Tetralogy of Fallot.

    Science.gov (United States)

    Bassareo, Pier Paolo; Saba, Luca; Marras, Andrea R; Mercuro, Giuseppe

    2016-12-01

    Postoperative tetralogy of Fallot (TOF) patients often develop progressive aortic root dilatation due to an impairment in aortic elastic properties. (1) to assess aortic elasticity at the level of the aortic upper wall by tissue Doppler imaging (TDI); (2) to evaluate the influence of aortic elasticity on left ventricular (LV) diastolic function in TOF patients. Twenty-eight postoperative TOF patients (14 males, 14 females. Mean age: 25.7 ± 1.6 years) and 28 age- and sex-matched normal subjects were examined. Aortic distensibility and stiffness index were calculated. Aortic wall systolic and diastolic velocities, LV systolic and diastolic parameters were assessed by TDI. Aortic distensibility was significantly lower (P = .024), and aortic stiffness index significantly higher (P = .036) in TOF patients compared to controls. E/E' was significantly higher in TOF than in control group (P < .001). Aortic upper wall early diastolic velocity (AWEDV) was significantly correlated with aortic stiffness index (r: -0.42; P < .03), aortic distensibility (r = 0.54; P < .004), left atrial volume (r = -0.62; P = .0004), and E/E' ratio (r = -0.87; P < .0001). The latter relationship remained significant even when excluding the influence of age at surgery (r = -0.60; P < .0007) and of previous palliative surgery (r = -0.53; P < .02). Aortic elastic properties can be directly assessed using TDI to measure AWEDV. Aortic elasticity is significantly lower in postoperative TOF patients, exerting a negative effect also on LV diastolic function, with a potential long-term influence on clinical status. © 2016 Wiley Periodicals, Inc.

  11. Altered aortic shape in bicuspid aortic valve relatives influences blood flow patterns.

    Science.gov (United States)

    Schnell, Susanne; Smith, Danielle A; Barker, Alex J; Entezari, Pegah; Honarmand, Amir R; Carr, Maria L; Malaisrie, S Chris; McCarthy, Patrick M; Collins, Jeremy; Carr, James C; Markl, Michael

    2016-11-01

    Bicuspid aortic valve (BAV) is known to exhibit familial inheritance and is associated with aortopathy and altered aortic haemodynamics. However, it remains unclear whether BAV-related aortopathy can be inherited independently of valve morphology. Four-dimensional flow magnetic resonance imaging for the in vivo assessment of thoracic aortic 3D blood flow was performed in 24 BAV relatives with trileaflet aortic valves (age = 40 ± 14 years) and 15 healthy controls (age = 37 ± 10 years). Data analysis included aortic dimensions, shape (round/gothic/cubic), and 3D blood flow characteristics (semi-quantitative vortex/helix grading and peak velocities). Cubic and gothic aortic shapes were markedly more prevalent in BAV relatives compared with controls (38 vs. 7%). Ascending aorta (AAo) vortex flow in BAV relatives was significantly increased compared with controls (grading = 1.5 ± 1.0 vs. 0.6 ± 0.9, P = 0.015). Aortic haemodynamics were influenced by aortic shape: peak velocities were reduced for gothic aortas vs. round aortas (P = 0.003); vortex flow was increased for cubic aortas in the AAo (P gothic aortas in the AAo and descending aorta (P = 0.003, P = 0.029). Logistic regression demonstrated significant associations of shape with severity of vortex flow in AAo (P < 0.001) and aortic arch (P = 0.016) in BAV relatives. BAV relatives expressed altered aortic shape and increased vortex flow despite the absence of valvular disease or aortic dilatation. These data suggest a heritable component of BAV-related aortopathy affecting aortic shape and aberrant blood flow, independent of valve morphology. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2016. For permissions please email: journals.permissions@oup.com.

  12. Aortic and Cardiac Structure and Function Using High-Resolution Echocardiography and Optical Coherence Tomography in a Mouse Model of Marfan Syndrome.

    Directory of Open Access Journals (Sweden)

    Ling Lee

    Full Text Available Marfan syndrome (MFS is an autosomal-dominant disorder of connective tissue caused by mutations in the fibrillin-1 (FBN1 gene. Mortality is often due to aortic dissection and rupture. We investigated the structural and functional properties of the heart and aorta in a [Fbn1C1039G/+] MFS mouse using high-resolution ultrasound (echo and optical coherence tomography (OCT. Echo was performed on 6- and 12-month old wild type (WT and MFS mice (n = 8. In vivo pulse wave velocity (PWV, aortic root diameter, ejection fraction, stroke volume, left ventricular (LV wall thickness, LV mass and mitral valve early and atrial velocities (E/A ratio were measured by high resolution echocardiography. OCT was performed on 12-month old WT and MFS fixed mouse hearts to measure ventricular volume and mass. The PWV was significantly increased in 6-mo MFS vs. WT (366.6 ± 19.9 vs. 205.2 ± 18.1 cm/s; p = 0.003 and 12-mo MFS vs. WT (459.5 ± 42.3 vs. 205.3 ± 30.3 cm/s; p< 0.0001. PWV increased with age in MFS mice only. We also found a significantly enlarged aortic root and decreased E/A ratio in MFS mice compared with WT for both age groups. The [Fbn1C1039G/+] mouse model of MFS replicates many of the anomalies of Marfan patients including significant aortic dilation, central aortic stiffness, LV systolic and diastolic dysfunction. This is the first demonstration of the direct measurement in vivo of pulse wave velocity non-invasively in the aortic arch of MFS mice, a robust measure of aortic stiffness and a critical clinical parameter for the assessment of pathology in the Marfan syndrome.

  13. Surgical correction of mitral valve prolapse : a cure for recurrent ventricular tachycardia in Marfan syndrome?

    Science.gov (United States)

    Beroukhim, Rebecca S; Reed, John H; Schaffer, Michael S; Yetman, Anji T

    2006-01-01

    We describe the case of a 3-year-old child with neonatal Marfan syndrome complicated by mitral valve prolapse with regurgitation, marked aortic root dilatation, and ventricular tachycardia. The patient had resolution of ventricular tachycardia following surgical intervention consisting of a valve-sparing aortic root replacement and mitral valve annuloplasty.

  14. Aortic root replacement in 372 Marfan patients: evolution of operative repair over 30 years.

    Science.gov (United States)

    Cameron, Duke E; Alejo, Diane E; Patel, Nishant D; Nwakanma, Lois U; Weiss, Eric S; Vricella, Luca A; Dietz, Harry C; Spevak, Philip J; Williams, Jason A; Bethea, Brian T; Fitton, Torin P; Gott, Vincent L

    2009-05-01

    We reviewed the evolution of practice and late results of aortic root replacement (ARR) in Marfan syndrome patients at our institution. A retrospective clinical review of Marfan patients undergoing ARR at our institution was performed. Follow-up data were obtained from hospital and office records and from telephone contact with patients or their physicians. Between September 1976 and September 2006, 372 Marfan syndrome patients underwent ARR: 269 had a Bentall composite graft, 85 had valve-sparing ARR, 16 had ARR with homografts, and 2 had ARR with porcine xenografts. In the first 24 years of the study, 85% received a Bentall graft; during the last 8 years, 61% had a valve-sparing procedure. There was no operative or hospital mortality among the 327 patients who underwent elective repair; there were 2 deaths among the 45 patients (4.4%) who underwent emergent or urgent operative repair. There were 74 late deaths (70 Bentalls, 2 homograft, and 2 valve-sparing ARRs). The most frequent causes of late death were dissection or rupture of the residual aorta (10 of 74) and arrhythmia (9 of 74). Of the 85 patients who had a valve-sparing procedure, 40 had a David II remodeling operation; there was 1 late death in this group, and 5 patients required late aortic valve replacement for aortic insufficiency. A David I reimplantation procedure using the De Paulis Valsalva graft has been used exclusively since May 2002. All 44 patients in this last group have 0 to 1+ aortic insufficiency. Prophylactic surgical replacement of the ascending aorta in patients with Marfan syndrome has low operative risk and can prevent aortic catastrophe in most patients. Valve-sparing procedures, particularly using the reimplantation technique with the Valsalva graft, show promise but have not yet proven as durable as the Bentall.

  15. Mild aerobic exercise blocks elastin fiber fragmentation and aortic dilatation in a mouse model of Marfan syndrome associated aortic aneurysm.

    Science.gov (United States)

    Gibson, Christine; Nielsen, Cory; Alex, Ramona; Cooper, Kimbal; Farney, Michael; Gaufin, Douglas; Cui, Jason Z; van Breemen, Cornelis; Broderick, Tom L; Vallejo-Elias, Johana; Esfandiarei, Mitra

    2017-07-01

    Regular low-impact physical activity is generally allowed in patients with Marfan syndrome, a connective tissue disorder caused by heterozygous mutations in the fibrillin-1 gene. However, being above average in height encourages young adults with this syndrome to engage in high-intensity contact sports, which unfortunately increases the risk for aortic aneurysm and rupture, the leading cause of death in Marfan syndrome. In this study, we investigated the effects of voluntary (cage-wheel) or forced (treadmill) aerobic exercise at different intensities on aortic function and structure in a mouse model of Marfan syndrome. Four-week-old Marfan and wild-type mice were subjected to voluntary and forced exercise regimens or sedentary lifestyle for 5 mo. Thoracic aortic tissue was isolated and subjected to structural and functional studies. Our data showed that exercise improved aortic wall structure and function in Marfan mice and that the beneficial effect was biphasic, with an optimum at low intensity exercise (55-65% V̇o 2max ) and tapering off at a higher intensity of exercise (85% V̇o 2max ). The mechanism underlying the reduced elastin fragmentation in Marfan mice involved reduction of the expression of matrix metalloproteinases 2 and 9 within the aortic wall. These findings present the first evidence of potential beneficial effects of mild exercise on the structural integrity of the aortic wall in Marfan syndrome associated aneurysm. Our finding that moderate, but not strenuous, exercise protects aortic structure and function in a mouse model of Marfan syndrome could have important implications for the medical care of young Marfan patients. NEW & NOTEWORTHY The present study provides conclusive scientific evidence that daily exercise can improve aortic health in a mouse model of Marfan syndrome associated aortic aneurysm, and it establishes the threshold for the exercise intensity beyond which exercise may not be as protective. These findings establish a platform

  16. Sinus of Valsalva aneurysm and bicuspid aortic valve: detection and mechanism by cardiac magnetic resonance imaging

    Directory of Open Access Journals (Sweden)

    Jen Li Looi

    2011-09-01

    Full Text Available Cardiac magnetic resonance imaging (CMR demonstrated a sinus of Valsalva aneurysm (SVA with severe dilatation of the right coronary sinus in association with a congenital bicuspid aortic valve (BAV and subaortic membrane. The SVA had not been apparent on echocardiography as the dilatation was outside standard echo image planes. On both CMR and echo, blood flow was eccentrically directed into the right coronary sinus by the domed posterior leaflet of the BAV. The impact of the aortic jet on the wall of the right coronary sinus is probably important in the aetiology of the sinus dilatation. CMR proved valuable in demonstrating the SVA and understanding its aetiology.

  17. Sinus of Valsalva aneurysm and bicuspid aortic valve: detection and mechanism by cardiac magnetic resonance imaging

    Directory of Open Access Journals (Sweden)

    Jen Li Looi

    2011-10-01

    Full Text Available Cardiac magnetic resonance imaging (CMR demonstrated a sinus of Valsalva aneurysm (SVA with severe dilatation of the right coronary sinus in association with a congenital bicuspid aortic valve (BAV and subaortic membrane. The SVA had not been apparent on echocardiography as the dilatation was outside standard echo image planes. On both CMR and echo, blood flow was eccentrically directed into the right coronary sinus by the domed posterior leaflet of the BAV. The impact of the aortic jet on the wall of the right coronary sinus is probably important in the aetiology of the sinus dilatation. CMR proved valuable in demonstrating the SVA and understanding its aetiology.

  18. Study of phenotype evolution during childhood in Marfan syndrome to improve clinical recognition.

    Science.gov (United States)

    Stheneur, Chantal; Tubach, Florence; Jouneaux, Marlène; Roy, Carine; Benoist, Gregoire; Chevallier, Bertrand; Boileau, Catherine; Jondeau, Guillaume

    2014-03-01

    Because diagnosis of Marfan syndrome is difficult during infancy, we used a large cohort of children to describe the evolution of the Marfan syndrome phenotype with age. Two hundred and fifty-nine children carrying an FBN1 gene mutation and fulfilling Ghent criteria were compared with 474 non-Marfan syndrome children. Prevalence of skeletal features changed with aging: prevalence of pectus deformity increased from 43% at 0-6 years to 62% at 15-17 years, wrist signs increased from 28 to 67%, and scoliosis increased from 16 to 59%. Hypermobility decreased from 67 to 47% and pes planus decreased from 73 to 65%. Striae increased from 2 to 84%. Prevalence of ectopia lentis remained stable, varying from 66 to 72%, similar to aortic root dilatation (varying from 75 to 80%). Aortic root dilatation remained stable during follow-up in this population receiving β-blocker therapy. When comparing Marfan syndrome children with non-Marfan syndrome children, height appeared to be a simple and discriminant criterion when it was >3.3 SD above the mean. Ectopia lentis and aortic dilatation were both similarly discriminating. Ectopia lentis and aortic dilatation are the best-discriminating features, but height remains a simple discriminating variable for general practitioners when >3.3 SD above the mean. Mean aortic dilatation remains stable in infancy when children receive a β-blocker.

  19. Effect of Pregnancy on Ventricular and Aortic Dimensions in Repaired Tetralogy of Fallot.

    Science.gov (United States)

    Cauldwell, Matthew; Quail, Michael A; Smith, Gillian S; Heng, Ee Ling; Ghonim, Sarah; Uebing, Anselm; Swan, Lorna; Li, Wei; Patel, Roshni R; Pennell, Dudley J; Steer, Philip J; Johnson, Mark R; Gatzoulis, Michael A; Babu-Narayan, Sonya V

    2017-07-23

    The aim was to assess whether cardiovascular adaptation to pregnancy in women with repaired tetralogy of Fallot (TOF) adversely affects hemodynamic stability, in particular with respect to right ventricular (RV) dilatation, pulmonary regurgitation, or aortic root dilatation. This was a retrospective cohort study of women with repaired TOF with paired cardiovascular magnetic resonance scans before and after their first pregnancy (baseline RV end systolic volume index 49 mL/m 2 and RV end diastolic volume index 118 mL/m 2 ) matched with a comparison group of nulliparous women with TOF. Cases were matched for age at baseline cardiovascular magnetic resonance scan, time between follow-up of cardiovascular magnetic resonance scans, QRS duration, RV ejection fraction, and indexed RV end systolic and diastolic volume at baseline. Effect of pregnancy and time on parameters was assessed using mixed-effects modelling. Nineteen women with repaired TOF who had completed their first pregnancy were identified and matched with 38 nulliparous women. We observed no deleterious effects of pregnancy on RV volumes, aortic dimensions, or exercise data. There was an effect of pregnancy observed in both left ventricular end diastolic volume and left ventricular stroke volume, consistent with a sustained small increase in left ventricular stroke volume attributed to pregnancy (53-55 mL/m 2 ). Women with repaired TOF and with mild-to-moderate RV dilatation considering pregnancy can be reassured that pregnancy is unlikely to cause deterioration in their cardiovascular status. We recommend that women are routinely assessed and followed up before and after pregnancy and that prepregnancy counseling is tailored to their individual clinical status. © 2017 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley.

  20. Aorto-aortic intrathoracic bypass in surgical treatment of aortic

    International Nuclear Information System (INIS)

    Gutierrez Perez, F.; Duran Reyes, A.; Bigalli, D.; Filgueira Berobide, J.

    1998-01-01

    The prevalence of coarctation of the aorta is 6.5 percent of all congenital heart defects, according to national and international data. There is a restenosis rate of patients after surgery. Factors that influence this evolution depends on the age at which patients underwent surgery for the first time the anatomy of the aortic arch and type of surgical technique. Several procedures can be used to correct the coarctation, which include surgery and balloon catheter dilation. We present here a case of a patient of 22 years old, with a recurrent coarctation of the aorta studied by echocardiography and magnetic resonance imaging. The patient underwent surgery a third time. We used an anterior approach (median sternotomy) and performed an aortic bypass graft, intrathoracic, under cardiopulmonary bypass. Evolved favorably and was discharged on the sixth day of post operative day (Author) [es

  1. Cardiological assessment of a cohort of Egyptian patients with ...

    African Journals Online (AJOL)

    Heba Salah A. ElAbd

    2015-12-30

    Dec 30, 2015 ... tricuspid regurge was found in 9 patients, this was considered normal finding. There was no ... ously include valvular (aortic, and mitral) insufficiency, aortic root dilation, atrial .... independent mechanisms. Hum Mol Genet ...

  2. Aortic annulus and ascending aorta: Comparison of preoperative and periooperative measurement in patients with aortic stenosis

    International Nuclear Information System (INIS)

    Smid, Michal; Ferda, Jiri; Baxa, Jan; Cech, Jakub; Hajek, Tomas; Kreuzberg, Boris; Rokyta, Richard

    2010-01-01

    Background: Precise determination of the aortic annulus size constitutes an integral part of the preoperative evaluation prior to aortic valve replacement. It enables the estimation of the size of prosthesis to be implanted. Knowledge of the size of the ascending aorta is required in the preoperative analysis and monitoring of its dilation enables the precise timing of the operation. Our goal was to compare the precision of measurement of the aortic annulus and ascending aorta using magnetic resonance (MR), multidetector-row computed tomography (MDCT), transthoracic echocardiography (TTE), and transoesophageal echocardiography (TEE) in patients with degenerative aortic stenosis. Methods and results: A total of 15 patients scheduled to have aortic valve replacement were enrolled into this prospective study. TTE was performed in all patients and was supplemented with TEE, CT and MR in the majority of patients. The values obtained were compared with perioperative measurements. For the measurement of aortic annulus, MR was found to be the most precise technique, followed by MDCT, TTE, and TEE. For the measurement of ascending aorta, MR again was found to be the most precise technique, followed by MDCT, TEE, and TTE. Conclusion: In our study, magnetic resonance was found to be the most precise technique for the measurement of aortic annulus and ascending aorta in patients with severe degenerative aortic stenosis.

  3. Identification of fibrillin 1 gene mutations in patients with bicuspid aortic valve (BAV) without Marfan syndrome

    Science.gov (United States)

    2014-01-01

    Background Bicuspid aortic valve (BAV) is the most frequent congenital heart disease with frequent involvement in thoracic aortic dilatation, aneurysm and dissection. Although BAV and Marfan syndrome (MFS) share some clinical features, and some MFS patients with BAV display mutations in FBN1, the gene encoding fibrillin-1, the genetic background of isolated BAV is poorly defined. Methods Ten consecutive BAV patients [8 men, age range 24–42 years] without MFS were clinically characterized. BAV phenotype and function, together with evaluation of aortic morphology, were comprehensively assessed by Doppler echocardiography. Direct sequencing of each FBN1 exon with flanking intron sequences was performed on eight patients. Results We detected three FBN1 mutations in two patients (aged 24 and 25 years) displaying aortic root aneurysm ≥50 mm and moderate aortic regurgitation. In particular, one patient had two mutations (p.Arg2726Trp and p.Arg636Gly) one of which has been previously associated with variable Marfanoid phenotypes. The other patient showed a pArg529Gln substitution reported to be associated with an incomplete MFS phenotype. Conclusions The present findings enlarge the clinical spectrum of isolated BAV to include patients with BAV without MFS who have involvement of FBN1 gene. These results underscore the importance of accurate phenotyping of BAV aortopathy and of clinical characterization of BAV patients, including investigation of systemic connective tissue manifestations and genetic testing. PMID:24564502

  4. Neonatal aortic stenosis.

    Science.gov (United States)

    Drury, Nigel E; Veldtman, Gruschen R; Benson, Lee N

    2005-09-01

    Neonatal aortic stenosis is a complex and heterogeneous condition, defined as left ventricular outflow tract obstruction at valvular level, presenting and often requiring treatment in the first month of life. Initial presentation may be catastrophic, necessitating hemodynamic, respiratory and metabolic resuscitation. Subsequent management is focused on maintaining systemic blood flow, either via a univentricular Norwood palliation or a biventricular route, in which the effective aortic valve area is increased by balloon dilation or surgical valvotomy. In infants with aortic annular hypoplasia but adequately sized left ventricle, the Ross-Konno procedure is also an attractive option. Outcomes after biventricular management have improved in recent years as a consequence of better patient selection, perioperative management and advances in catheter technology. Exciting new developments are likely to significantly modify the natural history of this disorder, including fetal intervention for the salvage of the hypoplastic left ventricle; 3D echocardiography providing better definition of valve morphology and aiding patient selection for a surgical or catheter-based intervention; and new transcutaneous approaches, such as duel beam echo, to perforate the valve.

  5. In vivo 3-dimensional Magnetic Resonance Wall Shear Stress Estimation in Ascending Aortic Dilatation

    Science.gov (United States)

    Bieging, Erik T.; Frydrychowicz, Alex; Wentland, Andrew; Landgraf, Benjamin R.; Johnson, Kevin M.; Wieben, Oliver; François, Christopher J.

    2011-01-01

    Purpose To estimate surface-based wall shear stress (WSS) and evaluate flow patterns in ascending aortic dilatation (AscAD) using a high-resolution, time-resolved, three-dimensional (3D), three-directional velocity encoded, radially undersampled phase contrast magnetic resonance sequence (4D PC-MRI). Materials and Methods 4D PC-MRI was performed in 11 patients with AscAD (46.3±22.0 years) and 10 healthy volunteers (32.9±13.4 years) after written informed consent and IRB-approval. Following manual vessel wall segmentation of the ascending aorta (MATLAB, The Mathworks, Natick, MA), a 3D surface was created using spline interpolation. Spatial WSS variation based on surface division in 12 segments and temporal variation were evaluated in AscAD and normal aortas. Visual analysis of flow patterns was performed based on streamlines and particle traces using EnSight (v9.0, CEI, Apex, NC). Results AscAD was associated with significantly increased diastolic WSS, decreased systolic to diastolic WSS ratio, and delayed onset of peak WSS (all P wall of the ascending aorta. Vortical flow with highest velocities along the anterior wall and increased helical flow during diastole were observed in AscAD compared to controls. Conclusion Changes in WSS in the ascending aorta of AscAD correspond to observed alterations in flow patterns compared to controls. PMID:21563242

  6. Valve-sparing root and ascending aorta replacement after heart transplantation.

    Science.gov (United States)

    Elhenawy, Abdelsalam M; Feindel, Christopher M; Ross, Heather; Butany, Jagdish; Yau, Terrence M

    2012-12-01

    A 45-year-old female underwent heart transplantation 17 years ago, with a heart from a 15-year-old donor. Recently, she had developed an aneurysm of the donor aortic root and ascending aorta, with severe aortic insufficiency. Two surgical options were considered; retransplantation versus replacement of the aortic root and ascending aorta. A valve-sparing replacement of the aortic root and ascending aortic aneurysm was performed. The donor aorta showed pathologic changes typical of Marfan syndrome. Nineteen months postoperatively, the patient remains in functional class I, with trivial aortic insufficiency. Copyright © 2012 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  7. Comparison of Cine-MRI and Transthoracic Echocardiography for the Assessment of Aortic Root Diameters in Patients with Suspected Marfan Syndrome.

    Science.gov (United States)

    Bannas, P; Rybczynski, M; Sheikhzadeh, S; von Kodolitsch, Y; Derlin, T; Yamamura, J; Lund, G; Adam, G; Groth, M

    2015-11-01

    Patients with Marfan syndrome require repeated imaging for monitoring of aortic root aneurysms. Therefore, we evaluated the agreement and reproducibility of cine-MRI and echocardiography measurements of the sinuses of Valsalva in patients with suspected Marfan syndrome. 51 consecutive patients with suspected Marfan syndrome were prospectively examined using cine-MRI and echocardiography. Two readers independently measured aortic root diameters at the level of the sinuses of Valsalva in both cine-MRI and echocardiography. Statistics included intraclass correlation coefficient, Pearson correlation coefficient, Bland-Altman analysis, and two-sided t-test. In 38 of the 51 individuals (74.5 %), the diagnosis of Marfan syndrome was established according to the criteria of the Ghent-2 nosology. Cine-MRI measurements of the sinuses of Valsalva revealed a strong correlation with echocardiography (r = 0.929), but a statistically significant bias of -1.0  mm (p Marfan syndrome. © Georg Thieme Verlag KG Stuttgart · New York.

  8. Familial Abdominal Aortic Aneurysm : Clinical Features and Genetics

    NARCIS (Netherlands)

    K.M. van de Luijtgaarden (Koen)

    2016-01-01

    markdownabstractAbstract Cardiovascular disease is the most important cause of death in the world and encompasses occlusive as well as aneurysmal disease. The most common aneurysm in humans is the abdominal aortic aneurysm (AAA). The question is why the aorta dilates in aneurysmal disease and

  9. Association of aortic and main left coronary aneurysms with severe aortic insufficiency in Takayasu’s arteritis

    Directory of Open Access Journals (Sweden)

    Marcelo Ferraz de Freitas

    2011-04-01

    Full Text Available Takayasu’s arteritis is a granulomatous vasculitis of unknown etiology that affects mainly the aorta and its branches. As a result of intimal fibroproliferation, segmental stenosis, occlusion, dilatation, and aneurysmal formation of the involved vessels may develop. It is an uncommon disease and usually affects young Asian female patients during the second and third decades of life. Coronary arteries are exceptionally affected and coronary aneurysm formation is a very rare finding. We describe a case of a previously healthy 26-year-old Caucasian female whose Takayasu’s arteritis presented as a previously undescribed association of aortic and main left coronary aneurysms with severe aortic insufficiency.

  10. Association of aortic and main left coronary aneurysms with severe aortic insufficiency in Takayasu’s arteritis

    Science.gov (United States)

    Schafranski, Marcelo Derbli; Ferraz de Freitas, Marcelo; Valladão de Carvalho, Marcelo

    2011-01-01

    Takayasu’s arteritis is a granulomatous vasculitis of unknown etiology that affects mainly the aorta and its branches. As a result of intimal fibroproliferation, segmental stenosis, occlusion, dilatation, and aneurysmal formation of the involved vessels may develop. It is an uncommon disease and usually affects young Asian female patients during the second and third decades of life. Coronary arteries are exceptionally affected and coronary aneurysm formation is a very rare finding. We describe a case of a previously healthy 26-year-old Caucasian female whose Takayasu’s arteritis presented as a previously undescribed association of aortic and main left coronary aneurysms with severe aortic insufficiency. PMID:24765288

  11. Outcomes of Aortic Valve-Sparing Operations in Marfan Syndrome.

    Science.gov (United States)

    David, Tirone E; David, Carolyn M; Manlhiot, Cedric; Colman, Jack; Crean, Andrew M; Bradley, Timothy

    2015-09-29

    In many cardiac units, aortic valve-sparing operations have become the preferred surgical procedure to treat aortic root aneurysm in patients with Marfan syndrome, based on relatively short-term outcomes. This study examined the long-term outcomes of aortic valve-sparing operations in patients with Marfan syndrome. All patients with Marfan syndrome operated on for aortic root aneurysm from 1988 through 2012 were followed prospectively for a median of 10 years. Follow-up was 100% complete. Time-to-event analyses were calculated using the Kaplan-Meier method with log-rank test for comparisons. A total of 146 patients with Marfan syndrome had aortic valve-sparing operations. Reimplantation of the aortic valve was performed in 121 and remodeling of the aortic root was performed in 25 patients. Mean age was 35.7 ± 11.4 years and two-thirds were men. Nine patients had acute, 2 had chronic type A, and 3 had chronic type B aortic dissections before surgery. There were 1 operative and 6 late deaths, 5 caused by complications of dissections. Mortality rate at 15 years was 6.8 ± 2.9%, higher than the general population matched for age and sex. Five patients required reoperation on the aortic valve: 2 for endocarditis and 3 for aortic insufficiency. Three patients developed severe, 4 moderate, and 3 mild-to-moderate aortic insufficiency. Rate of aortic insufficiency at 15 years was 7.9 ± 3.3%, lower after reimplantation than remodeling. Nine patients developed new distal aortic dissections during follow-up. Rate of dissection at 15 years was 16.5 ± 3.4%. Aortic valve-sparing operations in patients with Marfan syndrome were associated with low rates of valve-related complications in long-term follow-up. Residual and new aortic dissections were the leading cause of death. Copyright © 2015 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

  12. Long-Term Outcomes of Homografts in the Aortic Valve and Root Position: A 20-Year Experience

    Directory of Open Access Journals (Sweden)

    Joo Yeon Kim

    2016-08-01

    Full Text Available Background: The advantages of using a homograft in valve replacement surgery are the excellent hemodynamic profile, low risk of thromboembolism, and low risk of prosthetic valve infection. The aim of this study was to evaluate the long-term outcomes of homograft implantation in the aortic valve position. Methods: This is a retrospective study of 33 patients (>20 years old who underwent aortic valve replacement or root replacement with homografts between April 1995 and May 2015. Valves were collected within 24 hours from explanted hearts of heart transplant recipients (<60 years and organ donors who were not suitable for heart transplantation. The median follow-up duration was 35.6 months (range, 0 to 168 months. Results: Aortic homografts were used in all patients. The 30-day mortality rate was 9.1%. The 1- and 5-year survival rates were 80.0%±7.3% and 60.8%±10.1%, respectively. The 1-, 5-, and 10-year freedom from reoperation rates were 92.3%±5.2%, 68.9%±10.2%, and 50.3%±13.6%, respectively. The 1-, 5-, and 10-year freedom from significant aortic dysfunction rates were 91.7%±8.0%, 41.7%±14.2%, and 25.0%±12.5%, respectively. Conclusion: Homografts had the advantages of a good hemodynamic profile and low risk of thromboembolic events, and with good outcomes in cases of aortitis.

  13. Beneficial Outcome of Losartan Therapy Depends on Type of FBN1 Mutation in Marfan Syndrome.

    Science.gov (United States)

    Franken, Romy; den Hartog, Alexander W; Radonic, Teodora; Micha, Dimitra; Maugeri, Alessandra; van Dijk, Fleur S; Meijers-Heijboer, Hanne E; Timmermans, Janneke; Scholte, Arthur J; van den Berg, Maarten P; Groenink, Maarten; Mulder, Barbara J M; Zwinderman, Aeilko H; de Waard, Vivian; Pals, Gerard

    2015-04-01

    It has been shown that losartan reduces aortic dilatation in patients with Marfan syndrome. However, treatment response is highly variable. This study investigates losartan effectiveness in genetically classified subgroups. In this predefined substudy of COMPARE, Marfan patients were randomized to daily receive losartan 100 mg or no losartan. Aortic root dimensions were measured by MRI at baseline and after 3 years. FBN1 mutations were classified based on fibrillin-1 protein effect into (1) haploinsufficiency, decreased amount of normal fibrillin-1, or (2) dominant negative, normal fibrillin-1 abundance with mutant fibrillin-1 incorporated in the matrix. A pathogenic FBN1 mutation was found in 117 patients, of whom 79 patients were positive for a dominant negative mutation (67.5%) and 38 for a mutation causing haploinsufficiency (32.5%). Baseline characteristics between treatment groups were similar. Overall, losartan significantly reduced aortic root dilatation rate (no losartan, 1.3±1.5 mm/3 years, n=59 versus losartan, 0.8±1.4 mm/3 years, n=58; P=0.009). However, losartan reduced only aortic root dilatation rate in haploinsufficient patients (no losartan, 1.8±1.5 mm/3 years, n=21 versus losartan 0.5±0.8 mm/3 years, n=17; P=0.001) and not in dominant negative patients (no losartan, 1.2±1.7 mm/3 years, n=38 versus losartan 0.8±1.3 mm/3 years, n=41; P=0.197). Marfan patients with haploinsufficient FBN1 mutations seem to be more responsive to losartan therapy for inhibition of aortic root dilatation rate compared with dominant negative patients. Additional treatment strategies are needed in Marfan patients with dominant negative FBN1 mutations. http://www.trialregister.nl/trialreg/index.asp; Unique Identifier: NTR1423. © 2015 American Heart Association, Inc.

  14. Aortic atresia with normal sized left ventricle

    OpenAIRE

    Priya Jagia; Arun Sharma; Saurabh K Gupta; Munish Guleria

    2016-01-01

    Aortic atresia with an associated ventricular septal defect and adequate sized left ventricle is extremely rare. We present two cases in which an alternate diagnosis was suggested on echocardiography because the hypoplastic aortic trunk was missed due to its small caliber. The final diagnosis was, however, clinched on dual source computed tomography, which not only showed the thin aortic trunk but also clearly depicted the coronary artery origins from the hypoplastic aortic root. To the best ...

  15. Perturbed sympatho-vagal balance in Turner syndrome - relation to aortic dilation

    DEFF Research Database (Denmark)

    Trolle, Christian; Mortensen, Kristian Havmand; Andersen, Niels Holmark

    Objective: The risk of aortic dissection is 100 fold increased in Turner syndrome (TS). Increased blood pressure (BP) and heart rate is present as well as an increased risk of ischemic heart disease and diabetes. This study aimed to prospectively assess heart rate variability (HRV) in TS and its...... relation to aortic dimensions. Methods: Adults with TS (n=91, aged 37.4±10.4 years) recruited through the Danish National Society of Turner Syndrome Contact Group and an endocrine outpatient clinic were examined thrice (mean follow-up of 4.7±0.5 years). Healthy controls (n=64, aged 39.4±12.1 years) were...

  16. Quantification of plaque lipids in the aortic root of ApoE-deficient mice by 3D DIXON magnetic resonance imaging in an ex vivo model

    International Nuclear Information System (INIS)

    Dietel, Barbara; Kuehn, Constanze; Achenbach, Stephan; Budinsky, Lubos; Uder, Michael; Hess, Andreas

    2015-01-01

    To establish a dedicated protocol for the three-dimensional (3D) quantification of plaque lipids in apolipoprotein E-deficient (apoE -/- ) mice using ex vivo MRI. ApoE -/- mice were fed a high-fat diet (n = 10) or normal food (n = 10) for 3 months. Subsequently, a 3D FLASH MRI sequence was used to view the anatomy of the aortic root in the isolated hearts, where a 3D double-echo two-excitation pulse sequence (DIXON sequence) was used to selectively image plaque lipids. The vessel wall, lumen and plaque lipid volumes were quantified by MRI and histology for correlation analysis. DIXON MRI allowed visualisation and accurate quantification of plaque lipids. When comparing the vessel wall, lumen and plaque lipid sizes in the aortic root, Bland-Altman and linear regression analysis revealed a close correlation between MRI results and the histological data both on a slice-by-slice basis and of the volumetric measurements (vessel wall: r 2 = 0.775, p 2 = 0.875; p = 0.002; plaque lipid: r 2 = 0.819, p = 0.003). The combination of 3D FLASH and DIXON-sequence MRI permits an accurate ex vivo assessment of the investigated plaque parameters in the aortic root of mice, particularly the lipid content. (orig.)

  17. Challenging Friesian horse diseases : aortic rupture and megaesophagus

    NARCIS (Netherlands)

    Ploeg, M.

    2015-01-01

    Aortic rupture is quite rare in Warmblood horses and is best known as an acute and fatal rupture of the aortic root in older breeding stallions. It has now become clear that aortic rupture, which is diagnosed around an age of 4 years, is more frequent in the Friesian breed than in others. The high

  18. Aortic valve replacement with sutureless prosthesis: better than root enlargement to avoid patient-prosthesis mismatch?

    Science.gov (United States)

    Beckmann, Erik; Martens, Andreas; Alhadi, Firas; Hoeffler, Klaus; Umminger, Julia; Kaufeld, Tim; Sarikouch, Samir; Koigeldiev, Nurbol; Cebotari, Serghei; Schmitto, Jan Dieter; Haverich, Axel; Shrestha, Malakh

    2016-06-01

    Aortic valve replacement in patients with a small aortic annulus may result in patient-prosthesis mismatch (PPM). Aortic root enlargement (ARE) can reduce PPM, but leads to extended cardiac ischaemia times. Sutureless valves have the potential to prevent PPM while reducing cardiac ischaemia times. Between January 2007 and December 2011, a total of 128 patients with a small aortic annulus underwent surgery for aortic valve stenosis at our centre. Thirty-six (17% male, n = 6) patients received conventional valve replacement with ARE and 92 (16% male, n = 18) subjects received sutureless valve implantation (Sorin Perceval). We conducted a comparative, retrospective study with follow-up. The sutureless group showed a significantly higher age (79 years) than the ARE patients (62 years, P body surface area was 0.91 ± 0.2 cm(2)/m(2) in ARE patients and 0.83 ± 0.14 cm(2)/m(2) in sutureless patients (P = 0.040). The rate of patients with severe PPM was 6% (n = 2) in ARE patients and 11% (n = 8%) in sutureless patients (not significant, n.s.). The 30-day mortality rates were 2% (n = 2) in sutureless patients and 6% (n = 2) in ARE patients (n.s.). The 1- and 5-year survival rates of the sutureless group were 92 and 54% years, respectively, whereas the 1- and 5-year survival rates of the ARE group were 76% (n.s.). Although the sutureless valve patients received significantly more concomitant procedures, all operation-associated times were significantly shorter. Despite sutureless valve patients being older, the 30-day mortality and survival rates were comparable in the two groups. Since the indexed EOA was only slightly lower and the incidence of severe PPM was not significantly higher in the sutureless valve patients, we conclude that sutureless valve implantation is an alternative to conventional ARE to treat a small aortic annulus and avoid PPM, especially in geriatric patients who benefit from the quick implantation process. © The Author 2016. Published by Oxford

  19. Dilation of the ascending aorta in Turner syndrome - a prospective cardiovascular magnetic resonance study

    Directory of Open Access Journals (Sweden)

    Pedersen Erik M

    2011-04-01

    Full Text Available Abstract Background The risk of aortic dissection is 100-fold increased in Turner syndrome (TS. Unfortunately, risk stratification is inadequate due to a lack of insight into the natural course of the syndrome-associated aortopathy. Therefore, this study aimed to prospectively assess aortic dimensions in TS. Methods Eighty adult TS patients were examined twice with a mean follow-up of 2.4 ± 0.4 years, and 67 healthy age and gender-matched controls were examined once. Aortic dimensions were measured at nine predefined positions using 3D, non-contrast and free-breathing cardiovascular magnetic resonance. Transthoracic echocardiography and 24-hour ambulatory blood pressure were also performed. Results At baseline, aortic diameters (body surface area indexed were larger at all positions in TS. Aortic dilation was more prevalent at all positions excluding the distal transverse aortic arch. Aortic diameter increased in the aortic sinus, at the sinotubular junction and in the mid-ascending aorta with growth rates of 0.1 - 0.4 mm/year. Aortic diameters at all other positions were unchanged. The bicuspid aortic valve conferred higher aortic sinus growth rates (p Conclusion A general aortopathy is present in TS with enlargement of the ascending aorta, which is accelerated in the presence of a bicuspid aortic valve.

  20. Endovascular management of an acquired aortobronchial fistula following aortic bypass for coarctation.

    LENUS (Irish Health Repository)

    O'Sullivan, Katie E

    2013-09-20

    Aortobronchial fistula (ABF) in the setting of aortic coarctation repair is very rare but uniformly fatal if untreated. Endovascular stenting of the descending aorta is now the first-choice approach for ABF presenting with haemoptysis and offers a less-invasive technique with improved outcomes, compared with open repair. We report a case of late ABF occurring following bypass for aortic coarctation. Management focused on two key manoeuvres: use of a covered endovascular stent to occlude the aortic bypass thus controlling the fistula and dilatation and stenting of native coarctation.

  1. Transaortic edge-to-edge mitral valve repair for moderate secondary/functional mitral regurgitation in patients undergoing aortic root/valve intervention.

    Science.gov (United States)

    Choudhary, Shiv Kumar; Abraham, Atul; Bhoje, Amol; Gharde, Parag; Sahu, Manoj; Talwar, Sachin; Airan, Balram

    2017-11-01

    The present study evaluates the feasibility, safety, and efficacy of edge-to-edge repair for moderate secondary/functional mitral regurgitation in patients undergoing aortic valve/root interventions. Sixteen patients underwent transaortic edge-to-edge mitral valve repair. Mitral regurgitation was 2+ in 8 patients and 3+ in 6 patients. Two patients in whom cardiac arrest developed preoperatively had severe (4+) mitral regurgitation. Patients underwent operation for severe aortic regurgitation ± aortic root lesions. The mean left ventricular systolic and diastolic diameters were 51.5 ± 12.8 mm and 70.7 ± 10.7 mm, respectively. Left ventricular ejection fraction ranged from 20% to 60%. Primary surgical procedure included Bentall's ± hemiarch replacement in 10 patients, aortic valve replacement in 5 patients, and noncoronary sinus replacement with aortic valve repair in 1 patient. Severity of mitral regurgitation decreased to trivial or zero in 13 patients, 1+ in 2 patients, and 2+ in 1 patient. There were no gradients across the mitral valve in 9 patients, less than 5 mm Hg in 6 patients, and 9 mm Hg in 1 patient. There was no operative mortality. Follow-up ranged from 2 weeks to 54 months. Echocardiography showed trivial or no mitral regurgitation in 12 patients, 1+ in 2 patients, and 2+ in 2 patients. None of the patients had significant mitral stenosis. The mean left ventricular systolic and diastolic diameters decreased to 40.5 ± 10.3 mm and 58.7 ± 11.6 mm, respectively. Ejection fraction also improved slightly (22%-65%). Transaortic edge-to-edge mitral valve repair is a safe and effective technique to abolish secondary/functional mitral regurgitation. However, its impact on overall survival needs to be studied. Copyright © 2017 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.

  2. [Surgical results for aortic involvement in Marfan syndrome].

    Science.gov (United States)

    Shiiya, N; Matsuzaki, K; Maruyama, R; Kunihara, T; Murashita, T; Aoki, H; Yasuda, K

    2002-07-01

    From 1991 through 2001, 21 Marfan patients underwent aortic operations in our hospital. They received a total of 36 aortic operations, 31 by ourselves including 4 non-elective operations and 2 operations before 1991. Extent of replacement was Bentall + total arch (4), Bentall (8), valve sparing aortic root (reimplantation) (2), re-anastomosis + coronary aortic bypass grafting (CABG) after Bentall (1), ascending + total arch (3), ascending (1), total arch (1), total thoracoabdominal (10), thoracoabdominal (1), descending thoracic (2), distal arch (1), abdominal (2). Multiple operations were required in 11 patients (2 operations in 7, 3 operations in 4). Eight reoperations in 6 patients were for adjacent lesion, 5 reoperations were for remote lesion, and 2 others were for complication of Bentall (initial operation elsewhere). Among the 8 reoperations for adjacent lesion, 3 were scheduled operation (2 with elephant trunk), 4 were for residual dissection, and 1 was for annulo-aortic ectasia (AAE). Total aortic replacement was achieved in 4 and subtotal replacement excluding the root in 2. There was no hospital mortality. Paraparesis occurred in 1 who died 4.7 years after operation. The remaining patients are currently alive. No other aortic event occurred. Aortic reoperation-free survival was 83% at 5 year and 28% at 10 year.

  3. Successful surgical treatment of intramural aortoatrial fistula, severe aortic regurgitation, mitral prolapse, and tricuspid insufficiency in a patient with Ehlers-Danlos syndrome type IV.

    Science.gov (United States)

    Jiang, Shengli; Gao, Changqing; Ren, Chonglei; Zhang, Tao

    2012-06-01

    Patients with Ehlers-Danlos syndrome (EDS) type IV, an inherited connective tissue disorder, are predisposed to vascular and digestive ruptures, and arterial ruptures account for the majority of deaths. A 31-year-old man with EDS presented with an intramural aortoatrial fistula, severe aortic regurgitation, mitral valve prolapse, and severe tricuspid valve insufficiency combined with a severely dilated left ventricle. Determining the best surgical option for the patient was not easy, especially regarding the course of action for the aortic root with a tear in the sinus of Valsalva. The fistula tract was closed at the aorta with suture and with a patch in the right atrium, the mitral valve was repaired with edge-to-edge suture and then annuloplasty with a Cosgrove ring, the aortic valve was replaced with a mechanical prosthesis, and a modified De Vega technique was used for the tricuspid valvuloplasty. The postoperative course was uncomplicated, and the patient was discharged 2 weeks later. The considerations made to arrive at the chosen surgical course of action in this complex case are reviewed.

  4. Relationship between fibrillin-1 genotype and severity of cardiovascular involvement in Marfan syndrome.

    Science.gov (United States)

    Franken, Romy; Teixido-Tura, Gisela; Brion, Maria; Forteza, Alberto; Rodriguez-Palomares, Jose; Gutierrez, Laura; Garcia Dorado, David; Pals, Gerard; Mulder, Barbara Jm; Evangelista, Artur

    2017-11-01

    The effect of FBN1 mutation type on the severity of cardiovascular manifestations in patients with Marfan syndrome (MFS) has been reported with disparity results. This study aims to determine the impact of the FBN1 mutation type on aortic diameters, aortic dilation rates and on cardiovascular events (ie, aortic dissection and cardiovascular mortality). MFS patients with a pathogenic FBN1 mutation followed at two specialised units were included. FBN1 mutations were classified as being dominant negative (DN; incorporation of non-mutated and mutated fibrillin-1 in the extracellular matrix) or having haploinsufficiency (HI; only incorporation of non-mutated fibrillin-1, thus a decreased amount of fibrillin-1 protein). Aortic diameters and the aortic dilation rate at the level of the aortic root, ascending aorta, arch, descending thoracic aorta and abdominal aorta by echocardiography and clinical endpoints comprising dissection and death were compared between HI and DN patients. Two hundred and ninety patients with MFS were included: 113 (39%) with an HI- FBN1 mutation and 177 (61%) with a DN- FBN1 . At baseline, patients with HI- FBN1 had a larger aortic root diameter than patients with DN- FBN1 (HI: 39.3±7.2 mm vs DN: 37.3±6.8 mm, p=0.022), with no differences in age or body surface area. After a mean follow-up of 4.9±2.0 years, aortic root and ascending dilation rates were increased in patients with HI- FBN1 (HI: 0.57±0.8 vs DN: 0.28±0.5 mm/year, p=0.004 and HI: 0.59±0.9 vs DN: 0.30±0.7 mm/year, p=0.032, respectively). Furthermore, patients with HI- FBN1 tended to be at increased risk for the combined endpoint of dissection and death compared with patients with DN- FBN1 (HR: 3.3, 95% CI 1.0 to 11.4, p=0.060). Patients with an HI mutation had a more severely affected aortic phenotype, with larger aortic root diameters and a more rapid dilation rate, and tended to have an increased risk of death and dissections compared with patients with a DN

  5. Type A aortic dissection in Marfan syndrome: extent of initial surgery determines long-term outcome.

    Science.gov (United States)

    Rylski, Bartosz; Bavaria, Joseph E; Beyersdorf, Friedhelm; Branchetti, Emanuela; Desai, Nimesh D; Milewski, Rita K; Szeto, Wilson Y; Vallabhajosyula, Prashanth; Siepe, Matthias; Kari, Fabian A

    2014-04-01

    Data on outcomes after Stanford type A aortic dissection in patients with Marfan syndrome are limited. We investigated the primary surgery and long-term results in patients with Marfan syndrome who suffered aortic dissection. Among 1324 consecutive patients with aortic dissection type A, 74 with Marfan syndrome (58% men; median age, 37 years [first and third quartiles, 29 and 48 years]) underwent surgical repair (85% acute dissections; 68% DeBakey I; 55% composite valved graft, 30% supracoronary ascending replacement, 15% valve-sparing aortic root replacement; 12% total arch replacement; 3% in-hospital mortality) at 2 tertiary centers in the United States and Europe over the past 25 years. The rate of aortic reintervention with resternotomy was 24% (18 of 74) and of descending aorta (thoracic+abdominal) intervention was 30% (22 of 74) at a median follow-up of 8.4 years (first and third quartiles, 2.2 and 12.7 years). Freedom from need for aortic root reoperation in patients who underwent primarily a composite valved graft or valve-sparing aortic root replacement procedure was 95±3%, 88±5%, and 79±5% and in patients who underwent supracoronary ascending replacement was 83±9%, 60±13%, 20±16% at 5, 10, and 20 years. Secondary aortic arch surgery was necessary only in patients with initial hemi-arch replacement. Emergency surgery for type A dissection in patients with Marfan syndrome is associated with low in-hospital mortality. Failure to extend the primary surgery to aortic root or arch repair leads to a highly complex clinical course. Aortic root replacement or repair is highly recommended because supracoronary ascending replacement is associated with a high need (>40%) for root reintervention.

  6. A quarter of a century of experience with aortic valve-sparing operations.

    Science.gov (United States)

    David, Tirone E; Feindel, Christopher M; David, Carolyn M; Manlhiot, Cedric

    2014-09-01

    To examine the late outcomes of aortic valve-sparing operations to treat patients with aortic root aneurysm with and without aortic insufficiency (AI) in a cohort of patients followed up prospectively since 1988. A total of 371 consecutive patients had undergone aortic valve-sparing surgery (mean age, 47 ± 15 years; 78% men) from 1988 through 2010. In addition to the aortic root aneurysm, 47% had moderate or severe AI, 35.5% had Marfan syndrome, 12.1% had type A aortic dissection, 9.2% had bicuspid aortic valve, 8.4% had mitral insufficiency, 16.1% had aortic arch aneurysm, and 10.2% had coronary artery disease. Reimplantation of the aortic valve was used in 296 patients and remodeling of the aortic root in 75. Cusp repair by plication of the free margin along the nodule of Arantius was used in 36.6% of patients, and reinforcement of the free margin with a double layer of fine Gore-Tex suture in 24.2%. The patients were followed up prospectively with images of the aortic root for a median follow-up of 8.9 ± 5.2 years. A total of 4 operative and 39 late deaths occurred. Survival at 18 years was 76.8% ± 4.31%, lower than that for the general population matched for age and gender. Age, type A aortic dissection, impaired ventricular function, and preoperative AI were associated with increased mortality on multivariable analysis. Reoperations on the aortic valve were performed in 8 patients for recurrent AI and in 2 for infective endocarditis. Freedom from reoperation on the aortic valve at 18 years was 94.8% ± 2.0%. No predictors of the need for reoperation were found on multivariable analysis. Eighteen patients developed AI greater than mild. Freedom from AI greater than mild at 18 years was 78.0% ± 4.8%. No predictors of recurrent AI were identified on multivariable analysis. Aortic valve-sparing operations continue to provide excellent clinical outcomes, although a slow but progressive deterioration of aortic valve function seems to occur during the first 2

  7. Structure and function of the tricuspid and bicuspid regurgitant aortic valve: an echocardiographic study.

    Science.gov (United States)

    Rönnerfalk, Mattias; Tamás, Éva

    2015-07-01

    The emerging new treatment options for aortic valve disease call for more sophisticated diagnostics. We aimed to describe the echocardiographic pathophysiology and characteristics of the purely regurgitant aortic valve in detail. Twenty-nine men, with chronic aortic regurgitation without concomitant heart disease referred for aortic valve intervention, underwent 2D transoesophageal echocardiographic (TEE) examination prior to surgery according to a previously published matrix. Measurements of the aortic valve apparatus in long and short axis view were made in systole and diastole and analysed off-line. The aortic valves were grouped as tricuspid (TAV) or bicuspid (BAV), and classified by regurgitation mechanism. Twenty-four examinations were eligible for analysis of which 13 presented TAV and 11 BAV. The regurgitation mechanism was classified as dilatation of the aorta in 6 cases, as prolapse in 11 cases and as poor cusp tissue quality or quantity in 7 cases. The ventriculo-aortic junction (VAJ) and valve opening were closely related (TAV r = 0.5, BAV r = 0.73) but no correlation was found between the VAJ and the maximal sinus diameter (maxSiD) or the sinotubular junction (STJ). However, the STJ and maxSiD were significantly related (TAV vs BAV: systole r = 0.9, r = 0.8; diastole r = 0.9, r = 0.7), forming an entity. The conjoined BAV cusps were shorter than the anterior cusps when closed (P = 0.002); the inter-commissural distances of the cusps in the BAV group were significantly different (P = 0.001 resp. 0.03) in both systole and diastole. The VAJ was independent of other aortic dimensions and should thereby be considered as a separate entity with influence on valve opening. The detailed 2D TEE measurements of this study add further important information to our knowledge about the function and echocardiographic anatomy of the pathological aortic valve and root either as a stand-alone examination or as a benchmark and complement to 3D echocardiography. This may

  8. Loyes-Dietz syndrome presenting with severe aortic insufficiency – case report

    International Nuclear Information System (INIS)

    Kunovsky, P.; Dinka, R.; Krissakova, A.; Culen, M.; Nosal, M.; Kovacik, J.; Ilencikova, D.; Outrata, R.

    2013-01-01

    Severe aortic insufficiency (AI) in childhood is very rare cause of heart failure. Mostly is associated with connective tissue disorders as Marfan syndrome (MFS) or recently described Loyes-Dietz syndrome (LDS) (1). Authors present a case report of 9 years old girl with severe AI caused by aneurysm of aortic root. Typical findings associated with LDS are wide spread aneurysms of aorta, hypertelorism, cleft palate or split uvula (bifida uvula) and generalized arterial tortuosity. LDS is an autosomal dominant genetic disorder of the connective tissue; caused by mutation in the genes encoding the transforming growth factor beta receptor 1 and 2 (TGFBR1 and TGFRB2). Afflicted patients demonstrate different involvement of cardiovascular, musculoskeletal and central nervous system. From prognostic point of view the most consequential is widespread involvement of arterial system, when in addition to ascending aorta other parts of aorta and their branches might be also afflicted. Life threatening dissection and ruptures can occur earlier and at less dilated aneurysms than in MFS, requiring more aggressive diagnostic and therapeutic management with timely surgical intervention. Incidence of LDS is less frequent than serious congenital heart defects but due to its catastrophic potential even in early childhood as well as possible preventive intervention the importance of early diagnosis and treatment should be emphasized. (author)

  9. Sciatica caused by a dilated epidural vein: MR findings

    International Nuclear Information System (INIS)

    Demaerel, P.; Petre, C.; Wilms, G.; Plets, C.

    1999-01-01

    We report the MR imaging findings in a 41-year-old woman presenting with sudden low back pain and sciatica. At surgery a dilated epidural vein was found compressing the nerve root. The MR findings may suggest the diagnosis. Magnetic resonance imaging of a dilated epidural vein or varix causing sciatica has not been reported until now. (orig.) (orig.)

  10. Aorta-atria-septum combined incision for aortic valve re-replacement

    Science.gov (United States)

    Xu, Yiwei; Ye, Xiaofeng; Li, Zhaolong

    2018-01-01

    This case report illustrates a patient who underwent supra-annular mechanical aortic valve replacement then suffered from prosthesis dysfunction, increasing pressure gradient with aortic valve. She was successfully underwent aortic valve re-replacement, sub-annular pannus removing and aortic annulus enlargement procedures through combined cardiac incision passing through aortic root, right atrium (RA), and upper atrial septum. This incision provides optimal visual operative field and simplifies dissection. PMID:29850170

  11. Aortic valve-sparing surgery in Marfan syndrome.

    Science.gov (United States)

    Nachum, Eyal; Shinfeld, Amichay; Kogan, Alexander; Preisman, Sergey; Levin, Shany; Raanani, Ehud

    2013-08-01

    Patients with Marfan syndrome are referred for cardiac surgery due to root aneurysm with or without aortic valve regurgitation. Because these patients are young and frequently present with normal-appearing aortic cusps, valve sparing is often recommended. However, due to the genetic nature of the disease, the durability of such surgery remains uncertain. Between February 2004 and June 2012, 100 patients in our department suffering from aortic aneurysm with aortic valve regurgitation underwent elective aortic valve-sparing surgery. Of them, 30 had Marfan syndrome, were significantly younger (30 +/- 13 vs. 53 +/- 16 years), and had a higher percentage of root aneurysm, compared with ascending aorta aneurysm in their non-Marfan counterparts. We evaluated the safety, durability, clinical and echocardiographic mid-term results of these patients. While no early deaths were reported in either group, there were a few major early complications in both groups. At follow-up (reaching 8 years with a mean of 34 +/- 26 months) there were no late deaths, and few major late complications in the Marfan group. Altogether, 96% and 78% of the patients were in New York Heart Association functional class I-II in the Marfan and non-Marfan groups respectively. None of the Marfan patients needed reoperation on the aortic valve. Freedom from recurrent aortic valve regurgitation > 3+ was 94% in the Marfan patients. Aortic valve-sparing surgery in Marfan symdrome patients is safe and yields good mid-term clinical outcomes.

  12. Perioperative management of endovascular abdominal aortic aneurysm repair

    International Nuclear Information System (INIS)

    Wang Haofu; Wang Yuwei; Li Jun; Zhao Zonggang; Qi Sen

    2010-01-01

    Objective: To summarize the clinical experience of perioperative management in performing endovascular abdominal aortic aneurysm repair (EVAR). Methods: EVAR was performed in 22 patients with abdominal aortic aneurysm. The clinical data were retrospectively analyzed. Before treatment the functions of main organs were evaluated and certain measures were adopted in order to protect them. Useful parameters, including the length, diameter, angle and configuration of the proximal and distal aneurysmal neck, the relationship of the aneurysm to aortic branches, the distance from the lowest renal artery to the bifurcation of abdominal aorta, and the quality of access vessels (such as diameter, tortuosity and calcification degree) were determined and assessed with CTA. According to the parameters thus obtained, the suitable stent-graft with ideal diameter and length was selected, and the optimal surgery pattern was employed. Local anesthesia was employed in 20 patients, among them the local anesthesia had to be changed to general anesthesia in one. Epidural anesthesia was carried out in one patient through the surgically-reconstructed iliac artery access,and general anesthesia was employed in one patient who had Stanford type A aortic dissection. The lowest renal artery must be accurately localized before deployment of stent-graft was started. At least one patent internal iliac artery should be reserved when bilateral internal iliac arteries needed to be covered, to be covered by stages or to be reconstructed. After stent-graft placement, angiography must be performed to find out if there was any endoleak and, if any, to determine the type of endoleak and to deal with it properly. Two cases had proximal type I endoleak, so balloon dilation was employed in one and cuff implantation in another one. Distal type I endoleak occurred in one case, but, unfortunately, the iliac artery ruptured when balloon dilation was employed, therefore the patient had to receive vascular repair

  13. Aortic atresia with normal sized left ventricle

    Directory of Open Access Journals (Sweden)

    Priya Jagia

    2016-01-01

    Full Text Available Aortic atresia with an associated ventricular septal defect and adequate sized left ventricle is extremely rare. We present two cases in which an alternate diagnosis was suggested on echocardiography because the hypoplastic aortic trunk was missed due to its small caliber. The final diagnosis was, however, clinched on dual source computed tomography, which not only showed the thin aortic trunk but also clearly depicted the coronary artery origins from the hypoplastic aortic root. To the best of our knowledge, use of multi-detector computed tomography in aortic atresia with well developed left ventricle has not been reported in literature till date.

  14. Biological aortic valve replacement: advantages and optimal indications of stentless compared to stented valve substitutes. A review.

    Science.gov (United States)

    Tavakoli, Reza; Danial, Pichoy; Oudjana, Ahmed Hamid; Jamshidi, Peiman; Gassmann, Max; Leprince, Pascal; Lebreton, Guillaume

    2018-05-01

    Controversy still surrounds the optimal biological valve substitute for aortic valve replacement. In light of the current literature, we review advantages and optimal indications of stentless compared to stented aortic bio-prostheses. Recent meta-analyses, prospective randomized controlled trials and retrospective studies comparing the most frequently used stentless and stented aortic bio-prostheses were analyzed. In the present review, the types and implantation techniques of the bio-prosthesis that are seldom taken into account by most studies and reviews were integrated in the interpretation of the relevant reports. For stentless aortic root bio-prostheses, full-root vs. sub-coronary implantation offered better early transvalvular gradients, effective orifice area and left ventricular mass regression as well as late freedom from structural valve deterioration in retrospective studies. Early mortality and morbidity did not differ between the stentless and stented aortic bio-prostheses. Early transvalvular gradients, effective orifice area and regression of left ventricular hypertrophy were significantly better for stentless, especially as full-root, compared to stented bio-prostheses. The long-term valve-related survival for stentless aortic root and Toronto SPV bio-prosthesis was as good as that for stented pericardial aortic bio-prostheses. For full-root configuration this survival advantage was statistically significant. There seems to be not one but different ideal biological valve substitutes for different subgroups of patients. In patients with small aortic root or exposed to prosthesis-patient mismatch full-root implantation of stentless bio-prostheses may better meet functional needs of individual patients. Longer follow-ups on newer generation of stented bio-prostheses are needed for comparison of their hemodynamic performance with stentless counterparts especially in full-root configuration.

  15. Effect of the Antioxidant Lipoic Acid in Aortic Phenotype in a Marfan Syndrome Mouse Model

    Directory of Open Access Journals (Sweden)

    Maria C. Guido

    2018-01-01

    Full Text Available Marfan syndrome (MFS cardiovascular manifestations such as aortic aneurysms and cardiomyopathy carry substantial morbidity/mortality. We investigated the effects of lipoic acid, an antioxidant, on ROS production and aortic remodeling in a MFS mgΔloxPneo mouse model. MFS and WT (wild-type 1-month-old mice were allocated to 3 groups: untreated, treated with losartan, and treated with lipoic acid. At 6 months old, echocardiography, ROS production, and morphological analysis of aortas were performed. Aortic ROS generation in 6-month-old MFS animals was higher at advanced stages of disease in MFS. An unprecedented finding in MFS mice analyzed by OCT was the occurrence of focal inhomogeneous regions in the aortic arch, either collagen-rich extremely thickened or collagen-poor hypotrophic regions. MFS animals treated with lipoic acid showed markedly reduced ROS production and lower ERK1/2 phosphorylation; meanwhile, aortic dilation and elastic fiber breakdown were unaltered. Of note, lipoic acid treatment associated with the absence of focal inhomogeneous regions in MFS animals. Losartan reduced aortic dilation and elastic fiber breakdown despite no change in ROS generation. In conclusion, oxidant generation by itself seems neutral with respect to aneurysm progression in MFS; however, lipoic acid-mediated reduction of inhomogeneous regions may potentially associate with less anisotropy and reduced chance of dissection/rupture.

  16. Automated Normalized Cut Segmentation of Aortic Root in CT Angiography

    NARCIS (Netherlands)

    Elattar, Mustafa; Wiegerinck, Esther; Planken, Nils; VanBavel, Ed; van Assen, Hans; Baan, Jan Jr; Marquering, Henk

    2014-01-01

    Transcatheter Aortic Valve Implantation (TAVI) is a new minimal-invasive intervention for implanting prosthetic valves in patients with aortic stenosis. This procedure is associated with adverse effects like paravalvular leakage, stroke, and coronary obstruction. Accurate automated sizing for

  17. Reoperative reimplantation procedure after previous remodeling in a patient with Marfan syndrome.

    Science.gov (United States)

    Hechadi, Jawad; De Kerchove, Laurent; Vohra, Hunaid A; El Khoury, Gebrine

    2013-05-01

    A 43-year-old man with Marfan syndrome presented with recurrent aortic root dilatation and aortic regurgitation at ten years after a remodeling (Yacoub) procedure. Herein is described a reoperative valve-sparing procedure employing the reimplantation technique (David procedure) used in the treatment of this patient.

  18. Feasibility of Angioplasty and Stenting for Abdominal Aortic Lesions Adjacent to Previously Stented Visceral Artery Lesions in Patients with Takayasu Arteritis

    International Nuclear Information System (INIS)

    Joseph, George; George, Paul V.; Pati, Purendra Kumar; Chandy, Sunil Thomas

    2007-01-01

    Two young female patients with Takayasu arteritis presented with symptomatic long-segment abdominal aortic stenosis in the vicinity of previously deployed celiac and renal artery stents that projected markedly into the narrowed aortic lumen. Crushing or distortion of the visceral artery stents during aortic angioplasty was avoided by performing simultaneous or alternating balloon dilatations in the aorta and in the visceral artery stents. Consequently, the visceral artery stents remained patent and shortened longitudinally, allowing unhindered deployment of Wallstents in the adjacent aorta and abolition of a pressure gradient across the aortic lesions. Access to side branches covered by the Wallstent was obtained without difficulty, enabling the performance of balloon dilatation in multiple side branches and ostial stent deployment in a renal artery. These techniques could increase the scope of endovascular therapy in the treatment of patients with Takayasu arteritis

  19. Long-term results of aortic valve-sparing operations in patients with Marfan syndrome.

    Science.gov (United States)

    David, Tirone E; Armstrong, Sue; Maganti, Manjula; Colman, Jack; Bradley, Timothy J

    2009-10-01

    The appropriateness of aortic valve-sparing operations in patients with Marfan syndrome has been questioned. This study examines the long-term results of these operations in patients with Marfan syndrome. From 1988 to 2006, 103 consecutive patients with Marfan syndrome (mean age, 37 +/- 12 years) and aortic root aneurysm had aortic valve-sparing operations. Emergency surgery was performed in 11 patients: 8 for acute type A aortic dissection and 3 for unexplained persistent chest pain. Fourteen patients also had mitral valve surgery. The technique of aortic valve reimplantation was used in 77 patients, and aortic root remodeling was used in 26 patients. Patients were followed prospectively and underwent annual echocardiographic studies. The mean follow-up was 7.3 +/- 4.2 years and 100% complete. There was 1 operative death and 5 late deaths. Four of the 6 deaths were due to complications of aortic dissections. The patients' survival at 15 years was 87.2% compared with 95.6% for the general population of Ontario matched for age and sex. Seven patients had important aortic insufficiency: 4 mild to moderate, 2 moderate, and 1 moderate to severe. Freedom from greater than mild aortic insufficiency at 15 years was 79.2%. Three patients, all after aortic root remodeling, had aortic valve replacement, 2 for aortic insufficiency and 1 for endocarditis. At the most recent follow-up, 97 patients were alive: 86 were in functional class I, and 11 were in functional class II. Aortic valve-sparing operations provided excellent clinical outcomes in this series of patients with Marfan syndrome. Postoperatively, complications of aortic dissections were the leading cause of death.

  20. Etiology and pathogenesis of the Marfan syndrome: current understanding.

    Science.gov (United States)

    Pyeritz, Reed E

    2017-11-01

    Much has changed regarding Marfan syndrome (MFS) over the past few decades. Once described solely as a heritable disorder of connective tissue, MFS is now one of a number of conditions recognized to be a disorder of abnormal signalling in the TGF-β pathway. The cardinal features of MFS, once encompassed by the ocular, skeletal and cardiovascular systems, are now known to encompass many more organ systems, especially as people with MFS grow older. They are growing older by several decades compared to the 1970's because of profound improvements in diagnosis and management of the cardiovascular features, especially dilatation of the aortic root. This dilatation can be detected first in infancy and followed up by echocardiography. Progressive enlargement increases the risk of type A dissection and aortic regurgitation, the major causes of early mortality, in untreated patients today. Medical therapy with β-adrenergic blockade, first shown to be effective in the 1980's, can retard this dilatation. In the past decade, angiotensin receptor blockade, which reduces aberrant signalling through one of the TGF-β pathways, also can be effective. However, when dilatation of the root becomes such that the risk of dissection increases to an unacceptable degree, surgical therapy becomes necessary. In the mid-1970's, the composite graft, introduced by Hugh Bentall, markedly reduced mortality. In the past decade, a valve-spring aortic root replacement, advanced by Tirone David, has become widely adopted. Mid-term results are quite encouraging. Other cardiovascular involvement, such as mitral valve prolapse, type B dissection, and dilatation and dissection of aortic branches, also require close monitoring. Currently, life-expectancy in people with MFS who are diagnosed early and treated prophylactically is approaching that of the general population.

  1. Automatic segmentation of the aortic root in CT angiography of candidate patients for transcatheter aortic valve implantation

    NARCIS (Netherlands)

    Elattar, M.A.; Wiegerinck, E.; Planken, R.N.; VanBavel, E.T.; Assen, van H.C.; Baan Jr., J.; Marquering, H.A.

    2014-01-01

    Transcatheter aortic valve implantation is a minimal-invasive intervention for implanting prosthetic valves in patients with aortic stenosis. Accurate automated sizing for planning and patient selection is expected to reduce adverse effects such as paravalvular leakage and stroke. Segmentation of

  2. Sciatica caused by a dilated epidural vein: MR findings

    Energy Technology Data Exchange (ETDEWEB)

    Demaerel, P.; Petre, C.; Wilms, G. [Dept. of Radiology, Catholic University of Leuven (Belgium); Plets, C. [Dept. of Neurosurgery, Catholic University of Leuven (Belgium)

    1999-02-01

    We report the MR imaging findings in a 41-year-old woman presenting with sudden low back pain and sciatica. At surgery a dilated epidural vein was found compressing the nerve root. The MR findings may suggest the diagnosis. Magnetic resonance imaging of a dilated epidural vein or varix causing sciatica has not been reported until now. (orig.) (orig.) With 1 fig., 4 refs.

  3. Interobserver variability of CT angiography for evaluation of aortic annulus dimensions prior to transcatheter aortic valve implantation (TAVI)

    Energy Technology Data Exchange (ETDEWEB)

    Schmidkonz, C., E-mail: christian.schmidkonz@gmail.com [Department of Internal Medicine 2 (Cardiology), University of Erlangen, Ulmenweg 18, D-91054 Erlangen (Germany); Marwan, M.; Klinghammer, L.; Mitschke, M.; Schuhbaeck, A.; Arnold, M. [Department of Internal Medicine 2 (Cardiology), University of Erlangen, Ulmenweg 18, D-91054 Erlangen (Germany); Lell, M. [Radiological Institute, University of Erlangen, Maximiliansplatz 1, D-91054 Erlangen (Germany); Achenbach, S.; Pflederer, T. [Department of Internal Medicine 2 (Cardiology), University of Erlangen, Ulmenweg 18, D-91054 Erlangen (Germany)

    2014-09-15

    Highlights: • Cardiac CT provides highly reproducible measurements of aortic annulus and root dimensions prior to TAVI. • The perimeter-derived aortic annulus diameter shows the lowest interobserver variability. • If all three CT sizing methods are considered and stated as a “consensus result”, mismatches in prosthesis size selection can be further reduced. - Abstract: Objective: Assessment of aortic annulus dimensions prior to transcatheter aortic valve implantation (TAVI) is crucial for accurate prosthesis sizing in order to avoid prosthesis–annulus-mismatch possibly resulting in complications like valve dislodgement, paravalvular regurgitation or annulus rupture. Contrast-enhanced multidetector computed tomography allows 3-dimensional assessment of aortic annulus dimensions. Only limited data exist about its interobserver variability. Methods: In 100 consecutive patients with symptomatic severe aortic stenosis (51 male, BMI 27 ± 5 kg/m{sup 2}, age 81 ± 7 years, heart rate 72 ± 15 bpm, Logistic Euroscore 31 ± 14%, STS-Score 7 ± 4%), pre-interventional aortic annulus assessment was performed by dual source computed tomography (collimation 2 × 128 × 0.6 mm, high pitch spiral data acquisition mode, 40–60 ml contrast agents, radiation dose 3.5 ± 0.9 mSv). The following aortic annulus characteristics were determined by three independent observers: aortic annulus maximum, minimum and mean diameters (D{sub max}, D{sub min}, D{sub mean}), eccentricity index (EI), effective aortic annulus diameter according to its circumference (D{sub circ}), effective aortic annulus diameter according to its area (D{sub area}), distance from the aortic annulus plane to the left (LCA) and right coronary artery (RCA) ostia, maximum (D{sub max}AR) and minimum aortic root diameter (D{sub min}AR), maximum (D{sub max}STJ) and minimum diameter of the sinotubular junction (D{sub min}STJ). Subsequently, interobserver variabilities were assessed. Results: Correlation between

  4. Redox stress in Marfan syndrome: Dissecting the role of the NADPH oxidase NOX4 in aortic aneurysm.

    Science.gov (United States)

    Jiménez-Altayó, Francesc; Meirelles, Thayna; Crosas-Molist, Eva; Sorolla, M Alba; Del Blanco, Darya Gorbenko; López-Luque, Judit; Mas-Stachurska, Aleksandra; Siegert, Ana-Maria; Bonorino, Fabio; Barberà, Laura; García, Carolina; Condom, Enric; Sitges, Marta; Rodríguez-Pascual, Fernando; Laurindo, Francisco; Schröder, Katrin; Ros, Joaquim; Fabregat, Isabel; Egea, Gustavo

    2018-04-01

    Marfan syndrome (MFS) is characterized by the formation of ascending aortic aneurysms resulting from altered assembly of extracellular matrix fibrillin-containing microfibrils and dysfunction of TGF-β signaling. Here we identify the molecular targets of redox stress in aortic aneurysms from MFS patients, and investigate the role of NOX4, whose expression is strongly induced by TGF-β, in aneurysm formation and progression in a murine model of MFS. Working models included aortae and cultured vascular smooth muscle cells (VSMC) from MFS patients, and a NOX4-deficient Marfan mouse model (Fbn1 C1039G/+ -Nox4 -/- ). Increased tyrosine nitration and reactive oxygen species levels were found in the tunica media of human aortic aneurysms and in cultured VSMC. Proteomic analysis identified nitrated and carbonylated proteins, which included smooth muscle α-actin (αSMA) and annexin A2. NOX4 immunostaining increased in the tunica media of human Marfan aorta and was transcriptionally overexpressed in VSMC. Fbn1 C1039G/+ -Nox4 -/- mice aortas showed a reduction of fragmented elastic fibers, which was accompanied by an amelioration in the Marfan-associated enlargement of the aortic root. Increase in the contractile phenotype marker calponin in the tunica media of MFS mice aortas was abrogated in Fbn1 C1039G/+ -Nox4 -/- mice. Endothelial dysfunction evaluated by myography in the Marfan ascending aorta was prevented by the absence of Nox4 or catalase-induced H 2 O 2 decomposition. We conclude that redox stress occurs in MFS, whose targets are actin-based cytoskeleton members and regulators of extracellular matrix homeostasis. Likewise, NOX4 have an impact in the progression of the aortic dilation in MFS and in the structural organization of the aortic tunica media, the VSMC phenotypic modulation, and endothelial function. Copyright © 2018 Elsevier Inc. All rights reserved.

  5. The role of annular dimension and annuloplasty in tricuspid aortic valve repair.

    Science.gov (United States)

    de Kerchove, Laurent; Mastrobuoni, Stefano; Boodhwani, Munir; Astarci, Parla; Rubay, Jean; Poncelet, Alain; Vanoverschelde, Jean-Louis; Noirhomme, Philippe; El Khoury, Gebrine

    2016-02-01

    Valve sparing reimplantation can improve the durability of bicuspid aortic valve repair compared with subcommissural annuloplasty, especially in patients with a large basal ring. This study analyses the effect of basal ring size and annuloplasty on valve repair in the setting of a tricuspid aortic valve. From 1995 to 2013, 382 patients underwent elective tricuspid aortic valve repair. We included only those undergoing subcommissural annuloplasty, valve sparing reimplantation or no annuloplasty and in whom intraoperative transoesophageal echocardiography images were available for retrospective pre- and post-repair basal ring measurements (n = 323, subcommissural annuloplasty: 146, valve sparing reimplantation: 154, no annuloplasty: 23). In a subgroup of patients with available echocardiographic images, basal ring was retrospectively measured at the latest follow-up or prior to reoperation. subcommissural annuloplasty and valve sparing reimplantation were compared after matching for degree of aortic regurgitation and root size. All three groups differed significantly for most of preoperative characteristics. Hospital mortality was 0.9%. The median follow-up was 4.7 years. At 8 years, overall survival was 80 ± 5%. Freedom from reoperation and freedom from aortic regurgitation >1+ were 92 ± 5% and 71 ± 8%, respectively. In multivariate analysis, predictors of aortic regurgitation >1+ were left ventricular end-diastolic diameter (P = 0.003), cusp repair (P = 0.006), body surface area (P = 0.01) and subcommissural annuloplasty (P = 0.05). In subcommissural annuloplasty, freedom from aortic regurgitation >1+ was lower for patients with basal ring ≥28 mm compared with patients with basal ring 1+ was independent of basal ring size (P = 0.38). In matched comparison between subcommissural annuloplasty and valve sparing reimplantation, freedom from aortic regurgitation >1+ was not significantly different (P = 0.06), but in patients with basal ring ≥28 mm, valve sparing

  6. Obstructive pneumonia: an indication for surgery in mega aorta syndrome.

    Science.gov (United States)

    Kalkat, Maninder S; Bonser, Robert S

    2003-04-01

    A 76-year-old woman with annuloaortic ectasia, aortic regurgitation, and aneurysmal dilatation extending from the root to mid descending aorta presented with symptoms of bronchial compression and obstructive pneumonitis. Despite vigorous antibiotic treatment and physiotherapy, the pneumonitis persisted with clinical and radiologic deterioration. An emergency operation was performed to relieve bronchial compression. The operation comprised homograft aortic root replacement with prosthetic replacement of the arch and descending aorta. Postoperatively the pneumonia rapidly resolved.

  7. Bicuspid aortic valves are associated with increased wall and turbulence shear stress levels compared to trileaflet aortic valves.

    Science.gov (United States)

    Saikrishnan, Neelakantan; Mirabella, Lucia; Yoganathan, Ajit P

    2015-06-01

    Congenital bicuspid aortic valves (BAVs) are associated with accelerated disease progression, such as leaflet calcification and ascending aorta dilatation. Although common underlying genetic factors have been implicated in accelerated disease in BAV patients, several studies have suggested that altered hemodynamics also play a role in this disease process. The present study compares turbulence and wall shear stress (WSS) measurements between various BAV and trileaflet aortic valve (TAV) models to provide information for mechanobiological models of BAV disease. BAV and TAV models were constructed from excised porcine aortic valves to simulate parametric variations in BAV stenosis, hemodynamics and geometry. Particle image velocimetry experiments were conducted at physiological pressure conditions to characterize velocity fields in the ascending aorta. The velocity fields were post-processed to calculate turbulence, viscous and wall shear stresses in the ascending aorta. Stenosed BAV models showed the presence of eccentric systolic jets, causing increased WSS. Lower cardiac output resulted in a narrower jet, lower turbulence and lower viscous shear stress (VSS). The specific severe stenosis BAV model studied here showed reduced WSS due to reduction in non-fused leaflet mobility. Dilation of the aorta did not affect any turbulence or VSS, but reduced the WSS. In comparison with BAVs, TAVs have similar VSS values, but much smaller WSS and turbulence levels. These increased turbulence  and WSS levels in BAVs may play a key role in amplifying the biological responses of the ascending aorta wall and valvular leaflets, and support the hemodynamic underpinnings of BAV disease processes.

  8. Generation and Characterization of Vascular Smooth Muscle Cell Lines Derived from a Patient with a Bicuspid Aortic Valve

    Directory of Open Access Journals (Sweden)

    Pamela Lazar-Karsten

    2016-04-01

    Full Text Available Thoracic aortic dilation is the most common malformation of the proximal aorta and is responsible for 1%–2% of all deaths in industrialized countries. In approximately 50% of patients with a bicuspid aortic valve (BAV, dilation of any or all segments of the aorta occurs. BAV patients with aortic dilation show an increased incidence of cultured vascular smooth muscle cell (VSMC loss. In this study, VSMC, isolated from the ascending aorta of BAV, was treated with Simian virus 40 to generate a BAV-originated VSMC cell line. To exclude any genomic DNA or cross-contamination, highly polymorphic short tandem repeats of the cells were profiled. The cells were then characterized using flow cytometry and karyotyping. The WG-59 cell line created is the first reported VSMC cell line isolated from a BAV patient. Using an RT2 Profiler PCR Array, genes within the TGFβ/BMP family that are dependent on losartan treatment were identified. Endoglin was found to be among the regulated genes and was downregulated in WG-59 cells following treatment with different losartan concentrations, when compared to untreated WG-59 cells.

  9. Reduction of mouse atherosclerosis by urokinase inhibition or with a limited-spectrum matrix metalloproteinase inhibitor

    DEFF Research Database (Denmark)

    Hu, Jie Hong; Touch, Phanith; Zhang, Jingwan

    2015-01-01

    -accelerated atherosclerosis) to investigate whether systemic inhibition of proteolytic activity of uPA or a subset of MMPs can reduce protease-induced atherosclerosis and aortic dilation. METHODS AND RESULTS: SR-uPA mice were fed a high-fat diet for 10 weeks and treated either with an antibody inhibiting mouse uPA (mU1...... surface lesion coverage. Several lines of evidence identified MMP-13 as a mediator of uPA-induced aortic MMP activity. CONCLUSIONS: Pharmacological inhibition of either uPA or selected MMPs decreased atherosclerosis in SR-uPA mice. uPA inhibition decreased aortic dilation. Differential effects of both...... agents on aortic root vs. distal aortic atherosclerosis suggest prevention of atherosclerosis progression vs. initiation. Systemic inhibition of uPA or a subset of MMPs shows promise for treating atherosclerosis....

  10. Overview of current surgical strategies for aortic disease in patients with Marfan syndrome.

    Science.gov (United States)

    Miyahara, Shunsuke; Okita, Yutaka

    2016-09-01

    Marfan syndrome is a heritable, systemic disorder of the connective tissue with a high penetrance, named after Dr. Antoine Marfan. The most clinically important manifestations of this syndrome are cardiovascular pathologies which cause life-threatening events, such as acute aortic dissections, aortic rupture and regurgitation of the aortic valve or other artrioventricular valves leading to heart failure. These events play important roles in the life expectancy of patients with this disorder, especially prior to the development of effective surgical approaches for proximal ascending aortic disease. To prevent such catastrophic aortic events, a lower threshold has been recommended for prophylactic interventions on the aortic root. After prophylactic root replacement, disease in the aorta beyond the root and distal to the arch remains a cause for concern. Multiple surgeries are required throughout a patient's lifetime that can be problematic due to distal lesions complicated by dissection. Many controversies in surgical strategies remain, such as endovascular repair, to manage such complex cases. This review examines the trends in surgical strategies for the treatment of cardiovascular disease in patients with Marfan syndrome, and current perspectives in this field.

  11. Relation of Left Atrial Size, Cardiac Morphology, and Clinical Outcome in Asymptomatic Aortic Stenosis

    DEFF Research Database (Denmark)

    Christensen, Nicolaj Lyhne; Dahl, Jordi; Carter-Storch, Rasmus

    2017-01-01

    characterized by higher LV mass index (73 ± 17 g/m² vs. 66 ± 16 g/m² , p=0.03), increased right ventricle (70 ± 14 ml/m² vs. 63 ± 12 ml/m², p=0.01) and LV end-diastolic volume index (84 ± 18 ml/m² vs. 77 ± 16 ml/m², p=0.05), and higher brain natriuretic peptide (BNP). No difference in late enhancement was seen......Left atrial (LA) dilatation in asymptomatic severe aortic stenosis (AS) may be an indicator of advanced disease. The aim was to investigate the association between LA volume index (LAVi) and left ventricular (LV) morphology assessed with cardiac magnetic resonance imaging (cMRI), and to assess...... underwent echocardiography, cMRI, exercise test, and patients were followed for the composite endpoint of death, readmission or aortic valve replacement. AVA index was similar (0.45 ± 0.08 cm² /m² vs. 0.45 ± 0.09 cm², p=0.85) in patients with a dilated and normal LA. On cMRI patients with dilated LA were...

  12. Abdominal Aortic Dissection with Acute Mesenteric Ischemia in a Patient with Marfan Syndrome

    Directory of Open Access Journals (Sweden)

    Chii-Shyan Lay

    2006-07-01

    Full Text Available Marfan syndrome is an autosomal dominant inherited disorder of connective tissue, with various complications manifested primarily in the cardiovascular system. It potentially leads to aortic dissection and rupture, these being the major causes of death. We report a patient who complained of acute abdominal pain, which presented as acute mesenteric ischemia combined with abdominal aortic dissection. Echocardiography showed enlargement of the aortic root and mitral valve prolapse. Abdominal computed tomography scan revealed acute mesenteric ischemia due to abdominal aortic dissection. Finally, the patient underwent surgery of aortic root replacement and had a successful outcome. Therefore, we suggest that for optimal risk assessment and monitoring of patients with Marfan syndrome, both aortic stiffness and the diameter of the superior mesenteric vein compared with that of the superior mesenteric artery are useful screening methods to detect acute mesenteric ischemia secondary to abdominal aortic dissection. Early diagnosis and early treatment can decrease the high mortality rate of patients with Marfan syndrome.

  13. Diminutive Porcelain Ascending Aorta With Supravalvular Aortic Stenosis.

    Science.gov (United States)

    Houmsse, Mustafa; McDavid, Asia; Kilic, Ahmet

    2018-05-01

    This report describes the case of a 49-year-old man with a medical history significant for congenital aortic stenosis. The patient presented with progressive shortness of breath and decreased stamina and was found to have a concentric, diminutive porcelain ascending aorta with diffuse supravalvular aortic stenosis. We describe treatment with an aortic root augmentation and Bentall procedure using hypothermic circulatory arrest. Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  14. Aortic Involvement in Pediatric Marfan syndrome: A Review.

    Science.gov (United States)

    Ekhomu, Omonigho; Naheed, Zahra J

    2015-06-01

    Outlining specific protocols for the management of pediatric patients with Marfan syndrome has been challenging. This is mostly due to a dearth of clinical studies performed in pediatric patients. In Marfan syndrome, the major sources of morbidity and mortality relate to the cardiovascular system. In this review, we focus on aortic involvement seen in pediatric patients with Marfan syndrome, ranging from aortic dilatation to aortic rupture and heart failure. We discuss the histological, morphological, and pathogenetic basis of the cardiac manifestations seen in pediatric Marfan syndrome and use a specific case to depict our experienced range of cardiovascular manifestations. The survival for patients with Marfan syndrome may approach the expected survival for non-affected patients, with optimal management. With this potentiality in mind, we explore possible and actual management considerations for pediatric Marfan syndrome, examining both medical and surgical therapy modalities that can make the possibility of improved survival a reality.

  15. 3D echocardiographic analysis of aortic annulus for transcatheter aortic valve replacement using novel aortic valve quantification software: Comparison with computed tomography.

    Science.gov (United States)

    Mediratta, Anuj; Addetia, Karima; Medvedofsky, Diego; Schneider, Robert J; Kruse, Eric; Shah, Atman P; Nathan, Sandeep; Paul, Jonathan D; Blair, John E; Ota, Takeyoshi; Balkhy, Husam H; Patel, Amit R; Mor-Avi, Victor; Lang, Roberto M

    2017-05-01

    With the increasing use of transcatheter aortic valve replacement (TAVR) in patients with aortic stenosis (AS), computed tomography (CT) remains the standard for annulus sizing. However, 3D transesophageal echocardiography (TEE) has been an alternative in patients with contraindications to CT. We sought to (1) test the feasibility, accuracy, and reproducibility of prototype 3DTEE analysis software (Philips) for aortic annular measurements and (2) compare the new approach to the existing echocardiographic techniques. We prospectively studied 52 patients who underwent gated contrast CT, procedural 3DTEE, and TAVR. 3DTEE images were analyzed using novel semi-automated software designed for 3D measurements of the aortic root, which uses multiplanar reconstruction, similar to CT analysis. Aortic annulus measurements included area, perimeter, and diameter calculations from these measurements. The results were compared to CT-derived values. Additionally, 3D echocardiographic measurements (3D planimetry and mitral valve analysis software adapted for the aortic valve) were also compared to the CT reference values. 3DTEE image quality was sufficient in 90% of patients for aortic annulus measurements using the new software, which were in good agreement with CT (r-values: .89-.91) and small (software can accurately measure aortic annulus in patients with severe AS undergoing TAVR, in better agreement with CT than the existing methodology. Accordingly, intra-procedural TEE could potentially replace CT in patients where CT carries significant risk. © 2017, Wiley Periodicals, Inc.

  16. Compression of the right coronary artery by an aortic pseudoaneurysm after infective endocarditis: an unusual case of myocardial ischemia

    Directory of Open Access Journals (Sweden)

    Lacalzada-Almeida J

    2017-12-01

    Full Text Available Juan Lacalzada-Almeida,1 Alejandro De la Rosa-Hernández,1 María Manuela Izquierdo-Gómez,1 Javier García-Niebla,2 Iván Hernández-Betancor,1 Juan Alfonso Bonilla-Arjona,3 Antonio Barragán-Acea,1 Ignacio Laynez-Cerdeña1 1Cardiology Department, Hospital Universitario de Canarias, Tenerife, 2Health services from the Health Area of El Hierro, Valle del Golfo Health Center, El Hierro, 3Radiology Department, Hospital Universitario de Canarias, Tenerife, Spain Abstract: A 61-year-old male with a prosthetic St Jude aortic valve size 24 presented with heart failure symptoms and minimal-effort angina. Eleven months earlier, the patient had undergone cardiac surgery because of an aortic root dilatation and bicuspid aortic valve with severe regurgitation secondary to infectious endocarditis by Coxiela burnetii and coronary artery disease in the left circumflex coronary artery. Then, a prosthesis valve and a saphenous bypass graft to the left circumflex coronary artery were placed. The patient was admitted to the Cardiology Department of Hospital Universitario de Canarias, Tenerife, Spain and a transthoracic echocardiography was performed that showed severe paraprosthetic aortic regurgitation and an aortic pseudoaneurysm. The 64-slice multidetector computed tomography confirmed the pseudoaneurysm, originating from the right sinus of Valsalva, with a compression of the native right coronary artery and a normal saphenous bypass graft. On the basis of these findings, we performed surgical treatment with a favorable postoperative evolution. In our case, results from complementary cardiac imaging techniques were crucial for patient management. The multidetector computed tomography allowed for a confident diagnosis of an unusual mechanism of coronary ischemia. Keywords: pseudoaneurysm, infective endocarditis, myocardial ischemia, aortic valve prosthesis

  17. Rupture of an Abdominal Aortic Aneurysm in a Young Man with Marfan Syndrome.

    Science.gov (United States)

    Pedersen, Maria Weinkouff; Huynh, Khiem Dinh; Baandrup, Ulrik Thorngren; Nielsen, Dorte Guldbrand; Andersen, Niels Holmark

    2018-04-01

    Abdominal aortic aneurysms (AAAs) are very rare in Marfan syndrome. We present a case with a young nonsmoking and normotensive male with Marfan syndrome, who developed an infrarenal AAA that presented with rupture to the retroperitoneal cavity causing life-threatening bleeding shock. The patient had acute aortic surgery and survived. Five months before this incident, the patient had uneventful elective aortic root replacement (ad modum David) due to an enlarged aortic root. At that time, his abdominal aorta was assessed with a routine ultrasound scan that showed a normal-sized abdominal aorta. This documents that the aneurysm had evolved very rapidly despite young age and absence of risk factors. Copyright © 2018 Elsevier Inc. All rights reserved.

  18. The David V Valve-Sparing Root Replacement Provides Improved Survival Compared With Mechanical Valve-conduits in the Treatment of Young Patients With Aortic Root Pathology.

    Science.gov (United States)

    Esaki, Jiro; Leshnower, Bradley G; Binongo, Jose N; Lasanajak, Yi; McPherson, LaRonica; Halkos, Michael E; Guyton, Robert A; Chen, Edward P

    2016-11-01

    Valve-sparing root replacement (VSRR) is an attractive therapy for aortic root aneurysms; however, there is a paucity of data comparing VSRR with conventional root replacement using a mechanical valve-conduit (MECH). This study evaluates and compares outcomes of VSRR and MECH. A retrospective review from 2002 to 2015 at a US academic center identified 444 patients who underwent VSRR (282 patients) or MECH (162 patients). Propensity score matching was performed, based on 22 preoperative and intraoperative characteristics, and 87 matched pairs were identified. There was no difference in mean age between the groups (VSRR 45.0 years, MECH 44.2 years, p = 0.59). The incidence of Marfan syndrome (VSRR 10.3%, MECH 12.6%, p = 0.63), type A acute aortic dissection (VSRR 25.3%, MECH 27.6%, p = 0.73), reoperation (VSRR 23.0%, MECH 21.8%, p = 0.86), and arch replacement (VSRR 54.0%, MECH 52.9%, p = 0.88) were similar in both groups. Ejection fraction was similar (VSRR 52.8% ± 10.9%, MECH 52.4% ± 11.7%, p = 0.83). Operative mortality was 2.3% with VSRR and 8.0% with MECH (p = 0.10). There were no significant differences in renal failure requiring dialysis (VSRR 1.1%, MECH 4.6%, p = 0.24), permanent neurologic dysfunction (VSRR 2.3%, MECH 6.9%, p = 0.16), and pacemaker implantation (VSRR 1.1%, MECH 1.1%, p = 0.99) between the groups. Survival at 7 years was significantly improved in patients who underwent VSSR (VSRR 85.5%, MECH 73.6%, p = 0.03). In comparison with patients undergoing MECH, there is improved midterm survival among patients undergoing VSRR, with similar operative mortality and morbidity. For appropriately selected patients, VSRR provides an attractive and potentially superior alternative to MECH. Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  19. Beta-blockers for preventing aortic dissection in Marfan syndrome.

    Science.gov (United States)

    Koo, Hyun-Kyoung; Lawrence, Kendra Ak; Musini, Vijaya M

    2017-11-07

    Marfan syndrome is a hereditary disorder affecting the connective tissue and is caused by a mutation of the fibrillin-1 (FBN1) gene. It affects multiple systems of the body, most notably the cardiovascular, ocular, skeletal, dural and pulmonary systems. Aortic root dilatation is the most frequent cardiovascular manifestation and its complications, including aortic regurgitation, dissection and rupture are the main cause of morbidity and mortality. To assess the long-term efficacy and safety of beta-blocker therapy as compared to placebo, no treatment or surveillance only in people with Marfan syndrome. We searched the following databases on 28 June 2017; CENTRAL, MEDLINE, Embase, Science Citation Index Expanded and the Conference Proceeding Citation Index - Science in the Web of Science Core Collection. We also searched the Online Metabolic and Molecular Bases of Inherited Disease (OMMBID), ClinicalTrials.gov and the World Health Organization (WHO) International Clinical Trials Registry Platform (ICTRP) on 30 June 2017. We did not impose any restriction on language of publication. All randomised controlled trials (RCTs) of at least one year in duration assessing the effects of beta-blocker monotherapy compared with placebo, no treatment or surveillance only, in people of all ages with a confirmed diagnosis of Marfan syndrome were eligible for inclusion. Two review authors independently screened titles and abstracts for inclusion, extracted data and assessed trial quality. Trial authors were contacted to obtain missing data. Dichotomous outcomes will be reported as relative risk and continuous outcomes as mean differences with 95% confidence intervals. We assessed the quality of evidence using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach. One open-label, randomised, single-centre trial including 70 participants with Marfan syndrome (aged 12 to 50 years old) met the inclusion criteria. Participants were randomly assigned to

  20. Análise do tratamento cirúrgico da raiz da aorta com o tubo valvulado e com a preservação da valva aórtica Analysis of aortic root surgery with composite mechanical aortic valve conduit and valve-sparing reconstruction

    Directory of Open Access Journals (Sweden)

    Ricardo Ribeiro Dias

    2010-12-01

    Full Text Available OBJETIVO: Análise comparativa dos resultados imediatos e tardios da reconstrução da raiz da aorta com o tubo valvulado e com a preservação da valva aórtica. MÉTODOS: No período de novembro de 2002 a setembro de 2009, 164 pacientes com idade média de 54 ± 15 anos, sendo 115 do sexo masculino, foram submetidos ao tratamento cirúrgico da raiz da aorta. Foram 125 tubos valvulados e 39 reconstruções da raiz da aorta com preservação da valva aórtica. Dezesseis por cento dos pacientes eram portadores de síndrome de Marfan e 4,3% apresentavam valva aórtica bivalvulada. Cento e quarenta e quatro (88% pacientes foram acompanhados durante tempo médio de seguimento de 41,1 ± 20,8 meses. RESULTADOS: A mortalidade hospitalar total foi de 4,9%; sendo 5,6% nas operações com tubo valvulado e 2,6% nas preservações da valva aórtica (POBJECTIVE: Comparative analysis of early and late results of aortic root reconstruction with aortic valve sparing operations and the composite mechanical valve conduit replacement. METHODS: From November 2002 to September 2009, 164 consecutive patients with mean age 54 ± 15 years, 115 male, underwent the aortic root reconstruction (125 mechanical valve conduit replacements and 39 valve sparing operations. Sixteen percent of patients had Marfan syndrome and 4.3% had bicuspid aortic valve. One hundred and forty-four patients (88% were followed for a mean period of 41.1 ± 20.8 months. RESULTS: The hospital mortality was 4.9%, 5.6% in operations with valved conduits and 2.6% in the valve sparing procedures (P <0.05. There was no difference neither in survival (95% CI = 86% - 96%, P= 0.1 nor in reoperation-free survival (95% CI = 85% - 90%, P = 0.29. The survival free of complications such as bleeding, thromboembolism and endocarditis were favorable to the valve sparing operations, respectively (95% CI = 70% - 95%, P = 0.001, (95% CI = 82% - 95% P = 0.03 and (95% CI = 81% - 95%, P = 0.03. Multivariate analysis

  1. A case of megadolichobasilar anomaly complicated with abdominal aortic aneurysm

    International Nuclear Information System (INIS)

    Ohta, Sumio; Yamaguchi, Takenori; Ogata, Jun; Ito, Mamoru; Kikuchi, Haruhiko

    1985-01-01

    A 41 year-old hypertensive male was admitted because of progressing left hemiparesis and dysarthria. CT demonstrated hyperdense mass with partial contast enhancement, extending from the level of lower pons to that of suprasellar cistern. Reconstructed imaging of CT showed a huge mass lesion, in which a wide curvilinear hyperdensity was demonstrated by contrast enhancement. Cerebral angiography revealed markedly elongated and dilated basilar and carotid arteries. From these findings, the prepontine hyperdense mass lesion was diagnosed as megadolichobasilar anomaly with marked wall thickening. Findings of abdominal aortic angiography and abdominal CT suggested the presence of marked atherosclerosis and abdominal aortic aneurysm with mural thrombi. Six months after initial admission, neurological symptoms gradually deteriorated and CT showed dilatation of the 3rd and lateral ventricles, suggesting the development of hydrocephalus due to compression of the aqueduct by the megadolichobasilar anomaly. Magnetic resonance imaging at this time demonstrated more details of the lesion and the deformity of the brain stem, which was not detected by conventional CT. Complications of vascular anomalies other than intracranial vasculature, such as aortic aneurysm, have also been repoted. After the introduction of CT, demonstration of a long, wide, curvilinear structure with abnormal density in the prepontine region has been reported to be diagnostic for the megadolichobasilar anomaly. This patient has had hypertension for 10 years, which probably due to chronic nephritis. He had no definite findings for angitis, but had abdominal aortic aneurysm with mural thrombi. From these findings, atherosclerosis of large vessels may have played one of the roles in the pathogenesis of this anomaly in the present case. (J.P.N.)

  2. The risk for type B aortic dissection in Marfan syndrome.

    Science.gov (United States)

    den Hartog, Alexander W; Franken, Romy; Zwinderman, Aeilko H; Timmermans, Janneke; Scholte, Arthur J; van den Berg, Maarten P; de Waard, Vivian; Pals, Gerard; Mulder, Barbara J M; Groenink, Maarten

    2015-01-27

    Aortic dissections involving the descending aorta are a major clinical problem in patients with Marfan syndrome. The purpose of this study was to identify clinical parameters associated with type B aortic dissection and to develop a risk model to predict type B aortic dissection in patients with Marfan syndrome. Patients with the diagnosis of Marfan syndrome and magnetic resonance imaging or computed tomographic imaging of the aorta were followed for a median of 6 years for the occurrence of type B dissection or the combined end point of type B aortic dissection, distal aortic surgery, and death. A model using various clinical parameters as well as genotyping was developed to predict the risk for type B dissection in patients with Marfan syndrome. Between 1998 and 2013, 54 type B aortic dissections occurred in 600 patients with Marfan syndrome (mean age 36 ± 14 years, 52% male). Independent variables associated with type B aortic dissection were prior prophylactic aortic surgery (hazard ratio: 2.1; 95% confidence interval: 1.2 to 3.8; p = 0.010) and a proximal descending aorta diameter ≥27 mm (hazard ratio: 2.2; 95% confidence interval: 1.1 to 4.3; p = 0.020). In the risk model, the 10-year occurrence of type B aortic dissection in low-, moderate-, and high-risk patients was 6%, 19%, and 34%, respectively. Angiotensin II receptor blocker therapy was associated with fewer type B aortic dissections (hazard ratio: 0.3; 95% confidence interval: 0.1 to 0.9; p = 0.030). Patients with Marfan syndrome with prior prophylactic aortic surgery are at substantial risk for type B aortic dissection, even when the descending aorta is only slightly dilated. Angiotensin II receptor blocker therapy may be protective in the prevention of type B aortic dissections. Copyright © 2015 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

  3. Bentall Procedure Using Cryopreserved Valved Aortic Homografts

    Science.gov (United States)

    Christenson, Jan T.; Sierra, Jorge; Trindade, Pedro T.; Didier, Dominique; Kalangos, Afksendiyos

    2004-01-01

    The Bentall procedure is the standard operation for patients who have lesions of the ascending aorta associated with aortic valve disease. In many cases, however, mechanical prosthetic conduits are not suitable. There are few reports in the English-language medical literature concerning the mid- to long-term outcome of Bentall operations with cryopreserved homografts. Therefore, we reviewed our experience with this procedure and valved homografts. From January 1997 through December 2002, 21 patients underwent a Bentall operation with cryopreserved homografts at our institution. There were 14 males and 7 females; the mean age was 36 ± 21 years (range, 15–74 years). Eleven patients had undergone previous aortic valve surgery. All patients had aortic dilatation or aneurysms involving the ascending aorta. Indications for surgery included aortic valve stenosis or insufficiency, and aortic valve endocarditis (native valve or prosthetic). One patient had Takayasu's arteritis and 3 had Marfan syndrome. There was 1 hospital death (due to sepsis), but no other major postoperative complications. The mean hospital stay was 14 ± 7 days. Follow-up echocardiographic and computed tomographic scans were performed yearly. The mean follow-up was 34 months (6–72 months). Follow-up imaging revealed no calcifications or degenerative processes related to the homograft. Four patients had minimal valve regurgitation. Two patients died during follow-up. The 3-year actuarial survival rate was 85.7%. Our data suggest that the Bentall procedure with a valved homograft conduit is a safe procedure with excellent mid- to long-term results, comparable to results reported with aortic valve replacement with a homograft. PMID:15745290

  4. Ascending aorta dilatation rates in patients with tricuspid and bicuspid aortic stenosis: the COFRASA/GENERAC study.

    Science.gov (United States)

    Kerneis, Caroline; Pasi, Nicoletta; Arangalage, Dimitri; Nguyen, Virginia; Mathieu, Tiffany; Verdonk, Constance; Codogno, Isabelle; Ou, Phalla; Duval, Xavier; Tubiana, Sarah; Cimadevilla, Claire; Vahanian, Alec; Messika-Zeitoun, And David

    2017-07-25

    Ascending aorta (AA) dilatation is common in aortic valve stenosis (AS) but data regarding AA progression, its determinants and impact of valve anatomy [bicuspid (BAV), or tricuspid (TAV)] are scarce. Asymptomatic AS patients enrolled in a prospective cohort (COFRASA/GENERAC) with at least 2 years of follow-up were considered in the present analysis. A transthoracic echocardiography (TTE) and a computed tomography (CT) scan were performed at inclusion and yearly thereafter. We enrolled 195 patients [mean gradient 22 ± 11 mmHg, 42 BAV patients (22%)]. Mean aorta diameters assessed using TTE were 35 ± 4 and 36 ± 5 mm at the sinuses of Valsalva and tubular level, respectively. Ascending aorta diameter was >40 mm in 29% of patients (24% in TAV vs. 52% in BAV, P  0.05). Only four patients presented an AA progression ≥2 mm/year. Correlations between TTE and CT scan were excellent (all r >0.74) and similar results were obtained using CT. During follow-up, two BAV patients underwent a combined AA surgery; no surgery was primarily performed for AA aneurysm and no dissection was observed. In this prospective cohort of AS patients determinants of AA diameters were age, sex, BSA, and valve anatomy but not AS severity. AA progression rates were low and not influenced by AS severity or valve anatomy. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2017. For permissions, please email: journals.permissions@oup.com.

  5. Characteristics of aortic valve dysfunction and ascending aorta dimensions according to bicuspid aortic valve morphology

    Energy Technology Data Exchange (ETDEWEB)

    Shin, Hong Ju [Konkuk University Medical Center, Konkuk University School of Medicine, Department of Cardiovascular Surgery, Seoul (Korea, Republic of); Yonsei University College of Medicine, Department of Cardiovascular Surgery, Seoul (Korea, Republic of); Shin, Je Kyoun; Chee, Hyun Kun; Kim, Jun Suk [Konkuk University Medical Center, Konkuk University School of Medicine, Department of Cardiovascular Surgery, Seoul (Korea, Republic of); Ko, Sung Min [Konkuk University Medical Center, Konkuk University School of Medicine, Department of Radiology, Seoul (Korea, Republic of)

    2015-07-15

    To characterize aortic valve dysfunction and ascending aorta dimensions according to bicuspid aortic valve (BAV) morphology using computed tomography (CT) and surgical findings. We retrospectively enrolled 209 patients with BAVs who underwent transthoracic echocardiography (TTE) and CT. BAVs were classified as anterior-posterior (BAV-AP) or lateral (BAV-LA) orientation of the cusps and divided according to the presence (raphe+) or absence (raphe-) of a raphe. Ascending aortic dimensions were measured by CT at four levels. BAV-AP was present in 129 patients (61.7 %) and raphe+ in 120 (57.4 %). Sixty-nine patients (33.0 %) had aortic regurgitation (AR), 70 (33.5 %) had aortic stenosis (AS), and 58 (27.8 %) had combined AS and AR. AR was more common in patients with BAV-AP and raphe+; AS was more common with BAV-LA and raphe-.Annulus/body surface area and tubular portion/body surface area diameters in patients with BAV-AP (17.1 ± 2.3 mm/m{sup 2} and 24.2 ± 5.3 mm/m{sup 2}, respectively) and raphe+ (17.3 ± 2.2 mm/m{sup 2} and 24.2 ± 5.5 mm/m{sup 2}, respectively) were significantly different from those with BAV-LA (15.8 ± 1.9 mm/m{sup 2} and 26.4 ± 5.5 mm/m{sup 2}, respectively) and raphe- (15.7 ± 1.9 mm/m{sup 2} and 26.2 ± 5.4 mm/m{sup 2}, respectively). The morphological characteristics of BAV might be associated with the type of valvular dysfunction, and degree and location of an ascending aorta dilatation. (orig.)

  6. Machine-learning phenotypic classification of bicuspid aortopathy.

    Science.gov (United States)

    Wojnarski, Charles M; Roselli, Eric E; Idrees, Jay J; Zhu, Yuanjia; Carnes, Theresa A; Lowry, Ashley M; Collier, Patrick H; Griffin, Brian; Ehrlinger, John; Blackstone, Eugene H; Svensson, Lars G; Lytle, Bruce W

    2018-02-01

    Bicuspid aortic valves (BAV) are associated with incompletely characterized aortopathy. Our objectives were to identify distinct patterns of aortopathy using machine-learning methods and characterize their association with valve morphology and patient characteristics. We analyzed preoperative 3-dimensional computed tomography reconstructions for 656 patients with BAV undergoing ascending aorta surgery between January 2002 and January 2014. Unsupervised partitioning around medoids was used to cluster aortic dimensions. Group differences were identified using polytomous random forest analysis. Three distinct aneurysm phenotypes were identified: root (n = 83; 13%), with predominant dilatation at sinuses of Valsalva; ascending (n = 364; 55%), with supracoronary enlargement rarely extending past the brachiocephalic artery; and arch (n = 209; 32%), with aortic arch dilatation. The arch phenotype had the greatest association with right-noncoronary cusp fusion: 29%, versus 13% for ascending and 15% for root phenotypes (P < .0001). Severe valve regurgitation was most prevalent in root phenotype (57%), followed by ascending (34%) and arch phenotypes (25%; P < .0001). Aortic stenosis was most prevalent in arch phenotype (62%), followed by ascending (50%) and root phenotypes (28%; P < .0001). Patient age increased as the extent of aneurysm became more distal (root, 49 years; ascending, 53 years; arch, 57 years; P < .0001), and root phenotype was associated with greater male predominance compared with ascending and arch phenotypes (94%, 76%, and 70%, respectively; P < .0001). Phenotypes were visually recognizable with 94% accuracy. Three distinct phenotypes of bicuspid valve-associated aortopathy were identified using machine-learning methodology. Patient characteristics and valvular dysfunction vary by phenotype, suggesting that the location of aortic pathology may be related to the underlying pathophysiology of this disease. Copyright © 2017 The American

  7. Dual source multidetector CT-angiography before transcatheter aortic valve implantation (TAVI) using a high-pitch spiral acquisition mode

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    Wuest, W.; Anders, K.; May, M.S.; Uder, M. [University of Erlangen, Department of Radiology, Erlangen (Germany); Schuhbaeck, A.; Gauss, S.; Marwan, M.; Arnold, M.; Muschiol, G.; Daniel, W.G.; Achenbach, S. [University of Erlangen, Department of Cardiology, Erlangen (Germany); Ensminger, S. [University of Erlangen, Department of Cardiac Surgery, Erlangen (Germany)

    2012-01-15

    Transcatheter Aortic Valve Implantation (TAVI) is an alternative to surgical valve replacement in high risk patients. Angiography of the aortic root, aorta and iliac arteries is required to select suitable candidates, but contrast agents can be harmful due to impaired renal function. We evaluated ECG-triggered high-pitch spiral dual source Computed Tomography (CT) with minimized volume of contrast agent to assess aortic root anatomy and vascular access. 42 patients (82 {+-} 6 years) scheduled for TAVI underwent dual source (DS) CT angiography (CTA) of the aorta using a prospectively ECG-triggered high-pitch spiral mode (pitch = 3.4) with 40 mL iodinated contrast agent. We analyzed aortic root/iliac dimensions, attenuation, contrast to noise ratio (CNR), image noise and radiation exposure. Aortic root/iliac dimensions and distance of coronary ostia from the annulus could be determined in all cases. Mean aortic and iliac artery attenuation was 320 {+-} 70 HU and 340 {+-} 77 HU. Aortic/iliac CNR was 21.7 {+-} 6.8 HU and 14.5 {+-} 5.4 HU using 100 kV (18.8 {+-} 4.1 HU and 8.7 {+-} 2.6 HU using 120 kV). Mean effective dose was 4.5 {+-} 1.2 mSv. High-pitch spiral DSCTA can be used to assess the entire aorta and iliac arteries in TAVI candidates with a low volume of contrast agent while preserving diagnostic image quality. (orig.)

  8. Different transcatheter strategies for aortic coarctation associated with patent ductus arteriosus

    Directory of Open Access Journals (Sweden)

    Anil Kumar Singhi

    2012-07-01

    Conclusions: In coarctation with patent ductus arteriosus associated with good sized aortic isthmus, closure of duct with duct occluder device and balloon aortoplasty would correct the lesions. If there is isthmic hypoplasia, device closure of the duct and stenting of the coarctation is needed. Covered stent is a reasonable alternative only in presence of non dilated descending aorta.

  9. Operated DeBakey type III dissecting aortic aneurysm: review of 12 cases

    International Nuclear Information System (INIS)

    Moon, Hi Eun; Lee, Ghi Jai; Oh, Sang Joon; Yoon, Sei Ra; Shim, Jae Chan; Kim, Ho Kyun; Han, Chang Yul

    1995-01-01

    We evaluated the indications of operation and radiologic findings in 12 operated DeBakey type III aortic dissections. We retrospectively reviewed radiologic findings of 12 operated DeBakey type III aortic dissections, using CT, MRI, or aortography, and correlations were made with clinical course of the patients. Three cases were uncomplicated dissections. There were aneurysm rupture in 4 cases, impending rupture in 4 cases, occlusion of common iliac artery in 2 cases, occlusion of renal artery in 1 case, and compression of bronchus and esophagus by dilated aorta in 1 case. Associated clinical sign and symptoms were chest and back pain in 12 cases, claudication in 3 cases, dyspnea and dysphagia in 1 case, hoarseness in 1 case, and hemoptysis in 1 case. Post-operative complications were death from aneurysm rupture in 1 case, paraplegia in 2 cases, acute renal failure in 3 cases, and hemopericardium in 1 case. Although medical therapy is preferred in management of DeBakey type III aortic dissection, surgical treatment should be considered in patients with radiological findings of aortic rupture, impending rupture, occlusion of aortic major branches

  10. 3D printing based on cardiac CT assists anatomic visualization prior to transcatheter aortic valve replacement.

    Science.gov (United States)

    Ripley, Beth; Kelil, Tatiana; Cheezum, Michael K; Goncalves, Alexandra; Di Carli, Marcelo F; Rybicki, Frank J; Steigner, Mike; Mitsouras, Dimitrios; Blankstein, Ron

    2016-01-01

    3D printing is a promising technique that may have applications in medicine, and there is expanding interest in the use of patient-specific 3D models to guide surgical interventions. To determine the feasibility of using cardiac CT to print individual models of the aortic root complex for transcatheter aortic valve replacement (TAVR) planning as well as to determine the ability to predict paravalvular aortic regurgitation (PAR). This retrospective study included 16 patients (9 with PAR identified on blinded interpretation of post-procedure trans-thoracic echocardiography and 7 age, sex, and valve size-matched controls with no PAR). 3D printed models of the aortic root were created from pre-TAVR cardiac computed tomography data. These models were fitted with printed valves and predictions regarding post-implant PAR were made using a light transmission test. Aortic root 3D models were highly accurate, with excellent agreement between annulus measurements made on 3D models and those made on corresponding 2D data (mean difference of -0.34 mm, 95% limits of agreement: ± 1.3 mm). The 3D printed valve models were within 0.1 mm of their designed dimensions. Examination of the fit of valves within patient-specific aortic root models correctly predicted PAR in 6 of 9 patients (6 true positive, 3 false negative) and absence of PAR in 5 of 7 patients (5 true negative, 2 false positive). Pre-TAVR 3D-printing based on cardiac CT provides a unique patient-specific method to assess the physical interplay of the aortic root and implanted valves. With additional optimization, 3D models may complement traditional techniques used for predicting which patients are more likely to develop PAR. Copyright © 2016 Society of Cardiovascular Computed Tomography. Published by Elsevier Inc. All rights reserved.

  11. Participation of Arachidonic Acid Metabolism in the Aortic Aneurysm Formation in Patients with Marfan Syndrome

    Directory of Open Access Journals (Sweden)

    María E. Soto

    2018-02-01

    Full Text Available Marfan syndrome (MFS is a pleiotropic genetic disease involving the cardiovascular system where a fibrillin-1 mutation is present. This mutation is associated with accelerated activation of transforming growth factor β (TGFβ1 which contributes to the formation of aneurysms in the root of the aorta. There is an imbalance in the synthesis of thromboxane A2 (TXA2 and prostacyclin, that is a consequence of a differential protein expression of the isoforms of cyclooxygenases (COXs, suggesting an alteration of arachidonic acid (AA metabolism. The aim of this study was to analyze the participation of AA metabolism associated with inflammatory factors in the dilation and dissection of the aortic aneurysm in patients with MFS. A decrease in AA (p = 0.02, an increase in oleic acid (OA, TGFβ1, tumor necrosis factor alpha (TNFα, prostaglandin E2 (PGE2 (p < 0.05, and COXs activity (p = 0.002 was found. The expressions of phospholipase A2 (PLA2, cytochrome P450 (CYP450 4A, 5-lipoxygenase (5-LOX, COX2 and TXA2R (p < 0.05 showed a significant increase in the aortic aneurysm of patients with MFS compared to control subjects. COX1, 6-keto-prostaglandin 1 alpha (6-keto-PG1α and 8-isoprostane did not show significant changes. Histological examination of the aortas showed an increase of cystic necrosis, elastic fibers and collagen in MFS. The results suggest that there are inflammatory factors coupled to genetic factors that predispose to aortic endothelial dysfunction in the aortic tissue of patients with MFS. There is a decrease in the percentage of AA, associated with an increase of PLA2, COX2/TXA2R, CYP450 4A, and 5-LOX which leads to a greater synthesis of PGE2 than of 6-keto-PGF1α, thus contributing to the formation of the aortic aneurysm. The evident loss of the homeostasis in these mechanisms confirms that there is a participation of the AA pathway in the aneurysm progression in MFS.

  12. Low-level 45,X/46,XX mosaicism is not associated with congenital heart disease and thoracic aorta dilatation:prospective magnetic resonance imaging and ultrasound study.

    Science.gov (United States)

    Klásková, E; Tüdös, Z; Sobek, A; Zapletalová, J; Dostál, J; Zbořilová, B; Sobek, A; Adamová, K; Lattová, V; Dostálová, Z; Procházka, M

    2015-06-01

    To establish the prevalence of risk factors for aortic dissection, such as bicuspid aortic valve, aortic coarctation and ascending aorta dilatation, in women with low-level 45,X/46,XX mosaicism undergoing an in-vitro fertilization (IVF) procedure. The study group comprised 25 women with low-level 45,X/46,XX mosaicism (ranging from 3.3% to 10.0%) who were referred to two reproductive medicine units between 2009 and 2013 because of infertility and who underwent subsequent karyotyping. In accordance with the recommendation of the Practice Committee of the American Society for Reproductive Medicine for patients with Turner syndrome (TS), prior to the IVF procedure, all women underwent careful cardiovascular screening for congenital heart disease and thoracic aorta dilatation, including standard cardiac examination, echocardiography and non-contrast cardiac magnetic resonance imaging. Aortic size index (ASI, diameter of the ascending aorta normalized to body surface area) and the prevalence of coarctation of the aorta and of bicuspid aortic valve were compared with findings previously reported in women with TS and the general population. Bicuspid aortic valve without any stenosis or regurgitation was found in one woman in the study group with low-level 45,X/46,XX mosaicism, a statistically significantly lower prevalence of bicuspid aortic valve than that reported in women with TS. Aortic coarctation was not identified in any individual. The ASI was below the  95th percentile in all cases and the mean value was significantly lower than the mean reference values for both the general population and women with TS. Compared with the general population, the prevalence of risk factors for aortic dissection was not found to be higher in women with low-level 45,X/46,XX mosaicism without any noticeable features except infertility. Copyright © 2014 ISUOG. Published by John Wiley & Sons Ltd.

  13. Frequency of echocardiographic complications of dilated cardiomyopathy at a tertiary care hospital

    International Nuclear Information System (INIS)

    Rashid, A.; Ahmed, H.N.; Ahmed, N.

    2012-01-01

    Dilated cardiomyopathy can lead to a variety of complications recognisable on clinical, echocardiographic, electrocardiographic and radiographic assessment. Among this, transthoracic echocardiography has the dual advantage of being helpful in making the diagnosis of dilated cardiomyopathy as well as an effective tool in early recognition of certain complications for timely management to improve the quality of life of these patients. Methods: This descriptive (case series) study was undertaken at departments of medicine, cardiology, paediatrics and obs/gyn, Ayub Teaching Hospital, Abbottabad from July to December, 2008. fifty patients of dilated cardiomyopathy without age and gender discrimination were selected by convenience sampling. Those with hypertrophic and restrictive cardiomyopathies, valvular and congenital heart disease, hypertension and ischemic heart disease were excluded. Results: mean age was 47.12 +- 17.9 year with male predominance (males=34, females=16). Mean ejection fraction was 30.6 +- 6.9%. complications revealed on echocardiography were intracardiac thrombi (5, 10%), spontaneous echo contrast (5, 10%), pericardial effusion (6, 12%), mitral regurgitation (46, 92%), tricuspid (25, 50%), aortic (5, 10%), pulmonary (2, 4%) multi-valvular regurgitation (28, 56%), and left atrial dilatation (36, 72%). Conclusion: lv systolic dysfunction, cardiac thrombi, spontaneous echo contrast, mitral and tricuspid regurgitation and left atrial enlargement are important complications of dilated cardiomyopathy. echocardiography is important tool towards identification of these complications. (author)

  14. Preoperative computer tomography evaluation in transcatheter aortic valve replacement (TAVI)

    International Nuclear Information System (INIS)

    Groudeva, V.; Stoynova, V.; Trendafilova, D.; Dzhorgova, Y.; Nachev, G.

    2014-01-01

    Transcatheter aortic valve replacement is rapidly emerging technique alternative to surgery in high risk patients. Imaging and especially computer tomography is important in preoperative assessment of the aortic ring and the prosthetic valve choice. The aim of this study is to share authors initial experience in CT assessment of the aortic ring prior to Transcatheter aortic valve replacement. 49 patients (mean age 76,55) underwent 320 rows MDCT (Acquilon One) prior TAVI. Protocol involved scanning from thoracic inlet to common femoral arteries. Aortic root size, aortic diameter at the level of coronary sinuses and the sinotubular junction and distance to coronary ostia were evaluated on a Vitrea work station. MDCT established maximal aortic ring diameter from 18 to 31 mm mean 25,04 mm while the lesser rate was from 16 to 21 mm. Accordingly positioned prostheses were in 34,75% No. 23, in 49% - No. 26 and in16,3% - No. 29. MDCT is crucial in aortic valve assessment prior to TAVI in experienced hands and multidisciplinary team. (authors) Key words: TRANSCATHETER AORTIC VALVE REPLACEMENT (TAVI). MDCT AORTIC VALVE ASSESSMENT

  15. 21 CFR 884.4260 - Hygroscopic Laminaria cervical dilator.

    Science.gov (United States)

    2010-04-01

    ... 21 Food and Drugs 8 2010-04-01 2010-04-01 false Hygroscopic Laminaria cervical dilator. 884.4260 Section 884.4260 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES... conical and expansible material made from the root of a seaweed (Laminaria digitata or Laminaria japonica...

  16. Epidemiology and contemporary management of abdominal aortic aneurysms.

    Science.gov (United States)

    Ullery, Brant W; Hallett, Richard L; Fleischmann, Dominik

    2018-05-01

    Abdominal aortic aneurysm (AAA) is most commonly defined as a maximal diameter of the abdominal aorta in excess of 3 cm in either anterior-posterior or transverse planes or, alternatively, as a focal dilation ≥ 1.5 times the diameter of the normal adjacent arterial segment. Risk factors for the development of AAA include age > 60, tobacco use, male gender, Caucasian race, and family history of AAA. Aneurysm growth and rupture risk appear to be associated with persistent tobacco use, female gender, and chronic pulmonary disease. The majority of AAAs are asymptomatic and detected incidentally on various imaging studies, including abdominal ultrasound, and computed tomographic angiography. Symptoms associated with AAA may include abdominal or back pain, thromboembolization, atheroembolization, aortic rupture, or development of an arteriovenous or aortoenteric fistula. The Screening Abdominal Aortic Aneurysms Efficiently (SAAAVE) Act provides coverage for a one-time screening abdominal ultrasound at age 65 for men who have smoked at least 100 cigarettes and women who have family history of AAA disease. Medical management is recommended for asymptomatic patients with AAAs  5 mm/6 months), or presence of a fusiform aneurysm with maximum diameter of 5.5 cm or greater. Intervention for AAA includes conventional open surgical repair and endovascular aortic stent graft repair.

  17. Expanding the clinical spectrum of chromosome 15q26 terminal deletions associated with IGF-1 resistance.

    Science.gov (United States)

    O'Riordan, Aisling M; McGrath, Niamh; Sharif, Farhana; Murphy, Nuala P; Franklin, Orla; Lynch, Sally Ann; O'Grady, Michael J

    2017-01-01

    Haploinsufficiency of the insulin-like growth factor-1 receptor (IGF1R) gene on chromosome 15q26.3 is associated with impaired prenatal and postnatal growth, developmental delay, dysmorphic features and skeletal abnormalities. Terminal deletions of chromosome 15q26 arising more proximally may also be associated with congenital heart disease, epilepsy, diaphragmatic hernia and renal anomalies. We report three additional cases of 15q26 terminal deletions with novel features which may further expand the spectrum of this rarely reported contiguous gene syndrome. Phenotypic features including neonatal lymphedema, aplasia cutis congenita and aortic root dilatation have not been reported previously. Similarly, laboratory features of insulin-like growth factor 1 (IGF-1) resistance are described, including markedly elevated IGF-1 of up to +4.7 SDS. In one patient, the elevated IGF-1 declined over time and this coincided with a period of spontaneous growth acceleration. Deletions of 15q26 are a potential risk factor for aortic root dilatation, neonatal lymphedema and aplasia cutis in addition to causing growth restriction. What is Known: • Terminal deletions of chromosome 15q26 are associated with impaired prenatal and postnatal growth, developmental delay, dysmorphic features and skeletal abnormalities. What is New: • Neonatal lymphedema, aplasia cutis congenita and aortic root dilatation have not been previously described in 15q26 terminal deletions and may represent novel features. • IGF-1 levels may be increased up to 4.7 SDS.

  18. Acute aortic dissection type A discloses Corpus alienum

    Directory of Open Access Journals (Sweden)

    Kolat Philipp

    2009-01-01

    Full Text Available Abstract We report an unusual case of an aortic type A dissection with a corpus alienum which compresses the right ventricle. The patient successfully underwent an aortic root replacement in deep hypothermia with re-implantation of the coronary arteries using a modified Bentall procedure and the resection of the corpus alienum. Intraoperative finding reveals 3 greatly adhered gauze compresses, which were most likely forgotten in the operation 34 years ago.

  19. Early and late outcomes of 1000 minimally invasive aortic valve operations.

    Science.gov (United States)

    Tabata, Minoru; Umakanthan, Ramanan; Cohn, Lawrence H; Bolman, Ralph Morton; Shekar, Prem S; Chen, Frederick Y; Couper, Gregory S; Aranki, Sary F

    2008-04-01

    Minimal access cardiac valve surgery is increasingly utilized. We report our 11-year experience with minimally invasive aortic valve surgery. From 07/96 to 12/06, 1005 patients underwent minimally invasive aortic valve surgery. Early and late outcomes were analyzed. Median patient age was 68 years (range: 24-95), 179 patients (18%) were 80 years or older, 130 patients (13%) had reoperative aortic valve surgery, 86 (8.4%) had aortic root replacement, 62 (6.1%) had concomitant ascending aortic replacement, and 26 (2.6%) had percutaneous coronary intervention on the day of surgery (hybrid procedure). Operative mortality was 1.9% (19/1005). The incidences of deep sternal wound infection, pneumonia and reoperation for bleeding were 0.5% (5/1005), 1.3% (13/1005) and 2.4% (25/1005), respectively. Median length of stay was 6 days and 733 patients (72%) were discharged home. Actuarial survival was 91% at 5 years and 88% at 10 years. In the subgroup of the elderly (> or =80 years), operative mortality was 1.7% (3/179), median length of stay was 8 days and 66 patients (37%) were discharged home. Actuarial survival at 5 years was 84%. There was a significant decreasing trend in cardiopulmonary bypass time, the incidence of bleeding, and operative mortality over time. Minimal access approaches in aortic valve surgery are safe and feasible with excellent outcomes. Aortic root replacement, ascending aortic replacement, and reoperative surgery can be performed with these approaches. These procedures are particularly well-tolerated in the elderly.

  20. MCP-1, ICAM-1 and VCAM-1 are present in early aneurysmal dilatation in experimental rats

    International Nuclear Information System (INIS)

    Jun Fan; Hao-Tong; Jing Di; Fang Liu; Hai-Hua Zhao; Shu-Ling Bai; Xiang Li; Linlin Zhong

    2010-01-01

    Recent studies have suggested that inflammation actively participates in ascending aortic aneurysm formation. The aim of the present study was to evaluate the expression changes of adhesion molecules and MMPs in an experimental model of ascending aortic aneurysm induced by ascending aorta banding in Wistar rats. Twelve rats developed aortic dilation after ascending aorta banding treatment, while nine normal animals underwent surgery without banding were used as controls. Light microscope and scanning electron microscope showed that the wall of the ascending aorta became disorganized as well as infiltration by inflammatory cells in aneurysmal rats. By using immunohistochemical techniques, a significant increase in the immunostaining of MCP-1 was observed in the aneurysmal wall as compared to the normal aortic wall. Under similar experimental conditions, we also found that the immunostaining of ICAM-1 and VCAM-1 was markedly increased in the aneurysmal wall. In addition, gelatin zymo graphic analysis showed that the expression and activities of MMP-2 and MMP-9 were remarkably enhanced in the ascending aorta of ascending aortic aneurysmal rats as compared to normal rats. These results demonstrate that MCP-1, ICAM-1 and VCAM-1 are involved in the pathogenesis of ascending aortic aneurysm and an increase in the immunostaining and activity of MMP-2 and MMP-9 may promote the progression of ascending aortic aneurysm. (authors)

  1. Right circumflex retro-oesophageal aortic arch with coarctation of a high-positioned right arch

    International Nuclear Information System (INIS)

    Ahn, Kyung-Sik; Yong, Hwan Seok; Woo, Ok Hee; Kang, Eun-Young; Lee, Joo-Won

    2007-01-01

    We present a rare case of right circumflex retro-oesophageal aortic arch with coarctation of a high-positioned right arch. A 7-month-old boy presented with a cardiac murmur. Cardiac situs was normal and there was no evidence of an intracardiac shunt or patent ductus arteriosus. MR aortography revealed a right aortic arch that was high-positioned, tortuous and narrowed. This right aortic arch crossed the midline behind the oesophagus and continued as a left-sided descending aorta. The left common carotid and subclavian arteries arose from a large branching vascular structure that derived from the top of the left-sided descending aorta. The right common carotid artery arose from the ascending aorta. The proximal portion of the right common carotid artery showed very severe stenosis and poststenotic dilatation. The right subclavian artery originated distal to the narrowed and tortuous segment of the aortic arch. (orig.)

  2. Aortic stenosis and vascular calcifications in alkaptonuria.

    Science.gov (United States)

    Hannoush, Hwaida; Introne, Wendy J; Chen, Marcus Y; Lee, Sook-Jin; O'Brien, Kevin; Suwannarat, Pim; Kayser, Michael A; Gahl, William A; Sachdev, Vandana

    2012-02-01

    Alkaptonuria is a rare metabolic disorder of tyrosine catabolism in which homogentisic acid (HGA) accumulates and is deposited throughout the spine, large joints, cardiovascular system, and various tissues throughout the body. In the cardiovascular system, pigment deposition has been described in the heart valves, endocardium, pericardium, aortic intima and coronary arteries. The prevalence of cardiovascular disease in patients with alkaptonuria varies in previous reports. We present a series of 76 consecutive adult patients with alkaptonuria who underwent transthoracic echocardiography between 2000 and 2009. A subgroup of 40 patients enrolled in a treatment study underwent non-contrast CT scans and these were assessed for vascular calcifications. Six of the 76 patients had aortic valve replacement. In the remaining 70 patients, 12 patients had aortic sclerosis and 7 patients had aortic stenosis. Unlike degenerative aortic valve disease, we found no correlation with standard cardiac risk factors. There was a modest association between the severity of aortic valve disease and joint involvement, however, we saw no correlation with urine HGA levels. Vascular calcifications were seen in the coronaries, cardiac valves, aortic root, descending aorta and iliac arteries. These findings suggest an important role for echocardiographic screening of alkaptonuria patients to detect valvular heart disease and cardiac CT to detect coronary artery calcifications. Published by Elsevier Inc.

  3. Optimal results of aortic valve replacement with small mechanical valves (< 19 mm).

    Science.gov (United States)

    Kato, Yasuyuki; Hattori, Koji; Motoki, Manabu; Takahashi, Yosuke; Kotani, Shinsuke; Nishimura, Shinsuke; Shibata, Toshihiko

    2013-07-01

    Controversy exists regarding the optimal operative method or type of prosthesis for patients with a small aortic root. The aim of this retrospective study was to investigate the early and mid-term outcomes of standard aortic valve replacement (AVR) using 16 mm or 18 mm ATS Advanced Performance (AP) or 17 mm St. Jude Medical (SJM) Regent valves for a small aortic root. Between April 2003 and August 2009, 78 patients (age range: 50-86 years; 86% aged > or = 65 years) underwent AVR with 16 mm or 18 mm ATS AP valves (16AP group: n = 21, 18AP group: n = 32), or a 17 mm SJM Regent valve (17Regent group: n = 25). Fifty-six patients (72%) had a body surface area (BSA) of regression was similar among the groups (-30%, -25% and -28% in the 16AP, 17Regent and 18AP groups, respectively; p = 0.844). The early and mid-term results of AVR with 16 mm or 18 mm ATS AP valves, or with a 17 mm SJM Regent valve, were satisfactory. Therefore, standard AVR using these small mechanical prostheses, which avoids the need to enlarge the annulus or to conduct stentless bioprosthesis implantation, might represent an acceptable method, especially in elderly patients with a small aortic root.

  4. Aortic allografts in treatment of aortic valve and ascending aorta prosthetic endocarditis

    Directory of Open Access Journals (Sweden)

    S.V. Spiridonov

    2017-03-01

    Full Text Available The aim – to assess short- and long-term results of aortic root replacement using aortic allografts in patients with prosthetic endocarditis. Materials and methods. Since February 2009 until June 2016 aortic valve and ascending aorta replacement using aortic allografts was performed in 26 patients with prosthetic endocarditis. In 50 % of cases at initial operation aortic valve replacement was performed, in another 50 % of cases – aortic valve and ascending aorta replacement. Echocardiography was performed 10 days, 3, 6 and 12 months, 2, 3 and 5 years after surgery. Analysis of long-term results included all cases of deaths, prosthesis-related complications and recurrence of endocarditis. Results. 30-day mortality was 23.1 %. Extracorporeal membranous oxygenation (ECMO was used only in 5 patients (19.2 %. Four patients were weaned from ECMO. We did not observe any allograft-related complications. During follow-up period there were no cases of reoperation due to structural allograft failure. Relapse of infection occurred in 1 patient (3.8 % four years after the operation and led to lethal outcome. Conclusion. Reoperations using allografts are an effective surgical treatment of prosthetic endocarditis. In majority of cases prosthetic endocarditis was caused by gram-positive cocci (Staphylococcus. In 84.6 % of cases it was associated with destruction of paravalvular structures and abscesses formation. Heart failure was a causative factor of different complications in these patients, which required ECMO in 19.2 % of patients. In 80 % of cases patients were weaned from ECMO. Allografts using for the treatment of prosthetic endocarditis is associated with high resistance to infection and with a significant rate of freedom from recurrence of endocarditis within 3 years after surgery.

  5. CD1d deficiency inhibits the development of abdominal aortic aneurysms in LDL receptor deficient mice

    NARCIS (Netherlands)

    van Puijvelde, Gijs H. M.; Foks, Amanda C.; van Bochove, Rosemarie E.; Bot, Ilze; Habets, Kim L. L.; de Jager, Saskia C.; ter Borg, Mariëtte N. D.; van Osch, Puck; Boon, Louis; Vos, Mariska; de Waard, Vivian; Kuiper, Johan

    2018-01-01

    An abdominal aortic aneurysm (AAA) is a dilatation of the abdominal aorta leading to serious complications and mostly to death. AAA development is associated with an accumulation of inflammatory cells in the aorta including NKT cells. An important factor in promoting the recruitment of these

  6. Long-term results of modified Bentall procedure using flanged composite aortic prosthesis.

    Science.gov (United States)

    Tamura, Kiyoshi; Arai, Hirokuni; Kawaguchi, Satoru; Makita, Satoru; Miyagi, Naoto; Watanabe, Taiju; Fujiwara, Tatsuki

    2013-01-01

    We have been using the flanged composite aortic prosthesis and Carrel button technique to re-attach the coronary ostia in aortic root replacement procedures at our institution over the last twenty five years. Our objective was to evaluate the long-term results of aortic root replacement with this technique. A total of 73 patients from January 1984 to August 2010 were included in this study. The median age was 52.7 ± 14.4 years (range 28-80 years). There were 48 male and 25 female patients. 44 patients (60.3%) had annuloaortic ectasia, and 15 patients (20.5%) had acute type A aortic dissection. Marfan syndrome was recognized in 12 patients (16.5%). The early mortality rate was 5.5% (n = 4). Causes of death were multiple organ failures in two patients and sepsis in another two patients. The actuarial survival rate was 84.2% at 5 years, 64.3% at 15 years and 51.9% at 25 years. Only one patient with aortitis needed a reoperation because of coronary pseudoaneurysm after 23 years from the previous operation. This modified Bentall procedure is reliable and safe, with superior long-term survival and a low rate of aortic reoperation.

  7. Lagrangian coherent structures in the left ventricle in the presence of aortic valve regurgitation

    Science.gov (United States)

    di Labbio, Giuseppe; Vetel, Jerome; Kadem, Lyes

    2017-11-01

    Aortic valve regurgitation is a rather prevalent condition where the aortic valve improperly closes, allowing filling of the left ventricle of the heart to occur partly from backflow through the aortic valve. Although studies of intraventricular flow are rapidly gaining popularity in the fluid dynamics research community, much attention has been given to the left ventricular vortex and its potential for early detection of disease, particularly in the case of dilated cardiomyopathy. Notably, the subsequent flow in the left ventricle in the presence of aortic valve regurgitation ought to be appreciably disturbed and has yet to be described. Aortic valve regurgitation was simulated in vitro in a double-activation left heart duplicator and the ensuing flow was captured using two-dimensional time-resolved particle image velocimetry. Further insight into the regurgitant flow is obtained by computing attracting and repelling Lagrangian coherent structures. An interesting interplay between the two inflowing jets and their shear layer roll-up is observed for various grades of regurgitation. This study highlights flow features which may find use in further assessing regurgitation severity.

  8. Combined Bentall and modified Ravitch procedures in a patient with Marfan syndrome.

    Science.gov (United States)

    Kansara, Bhuvnesh; Singh, Ajmer; Girotra, Samir; Iyer, K S

    2013-01-01

    Marfan syndrome is an inherited, connective-tissue disorder transmitted as an autosomal dominant trait. Cardinal features of the disorder include tall stature, ectopia lentis, mitral valve prolapse, aortic root dilatation, and aortic dissection. Pectus excavatum may exist as an isolated lesion or in association with a genetic syndrome such as Marfan syndrome. We report the successful management of a simultaneous correction of pectus excavatum and the underlying cardiovascular diseases.

  9. Combined Bentall and modified Ravitch procedures in a patient with Marfan syndrome

    OpenAIRE

    Kansara, Bhuvnesh; Singh, Ajmer; Girotra, Samir; Iyer, K S

    2013-01-01

    Marfan syndrome is an inherited, connective-tissue disorder transmitted as an autosomal dominant trait. Cardinal features of the disorder include tall stature, ectopia lentis, mitral valve prolapse, aortic root dilatation, and aortic dissection. Pectus excavatum may exist as an isolated lesion or in association with a genetic syndrome such as Marfan syndrome. We report the successful management of a simultaneous correction of pectus excavatum and the underlying cardiovascular diseases.

  10. CT imaging of aortic intramural hematoma: Report of two cases, and review of literature.

    Science.gov (United States)

    Serafin, Zbigniew; Laskowska, Katarzyna; Marzec, Małgorzata; Lasek, Władysław

    2011-01-01

    Aortic intramural hematoma (IMH) is a relatively rare, but potentially fatal pathology, which is most commonly diagnosed under emergency conditions with computed tomography angiography (CTA). We present two cases of IMH examined with different CTA protocols, which resulted in diverse diagnoses.In the first patient, contrast-enhanced CTA revealed dilatation of thoracic aorta and polymorphic wall thickening. It was misdiagnosed as being a mural thrombus and was complicated by acute dissection. In the second patient, non-enhanced CT revealed hyperdense aortic wall thickening. The patient was diagnosed as having type B IMH but he died due to aortic rupture.In our opinion, CT examination of the aorta in emergency cases should always include non-enhanced scanning. Any delay in proper diagnosis and treatment of IMH may result in serious complications.

  11. CT imaging of aortic intramural hematoma: Report of two cases, and review of literature

    International Nuclear Information System (INIS)

    Serafin, Z.; Laskowska, K.; Marzec, M.; Lasek, W.

    2011-01-01

    Aortic intramural hematoma (IMH) is a relatively rare, but potentially fatal pathology, which is most commonly diagnosed under emergency conditions with computed tomography angiography (CTA). We present two cases of IMH examined with different CTA protocols, which resulted in diverse diagnoses. In the first patient, contrast-enhanced CTA revealed dilatation of thoracic aorta and polymorphic wall thickening. It was misdiagnosed as being a mural thrombus and was complicated by acute dissection. In the second patient, non-enhanced CT revealed hyperdense aortic wall thickening. The patient was diagnosed as having type B IMH but he died due to aortic rupture. In our opinion, CT examination of the aorta in emergency cases should always include non enhanced scanning. Any delay in proper diagnosis and treatment of IMH may result in serious complications. (authors)

  12. Abscisic acid ameliorates atherosclerosis by suppressing macrophage and CD4+ T cell recruitment into the aortic wall.

    Science.gov (United States)

    Guri, Amir J; Misyak, Sarah A; Hontecillas, Raquel; Hasty, Alyssa; Liu, Dongmin; Si, Hongwei; Bassaganya-Riera, Josep

    2010-12-01

    Abscisic acid (ABA) is a natural phytohormone which improves insulin sensitivity and reduces adipose tissue inflammation when supplemented into diets of obese mice. The objective of this study was to investigate the mechanisms by which ABA prevents or ameliorates atherosclerosis. apolipoprotein E-deficient (ApoE(-/-)) mice were fed high-fat diets with or without ABA for 84 days. Systolic blood pressure was assessed on Days 0, 28, 56 and 72. Gene expression, immune cell infiltration and histological lesions were evaluated in the aortic root wall. Human aortic endothelial cells were used to examine the effect of ABA on 3',5'-cyclic adenosine monophosphate (cAMP) and nitric oxide (NO) production in vitro. We report that ABA-treated mice had significantly improved systolic blood pressure and decreased accumulation of F4/80(+)CD11b(+) macrophages and CD4(+) T cells in aortic root walls. At the molecular level, ABA significantly enhanced aortic endothelial nitric oxide synthase (eNOS) and tended to suppress aortic vascular cell adhesion molecule-1 (VCAM-1) and monocyte chemoattractant protein-1 (MCP-1) expression and plasma MCP-1 concentrations. ABA also caused a dose-dependent increase in intracellular concentrations of cAMP and NO and up-regulated eNOS mRNA expression in human aortic endothelial cells. This is the first report showing that ABA prevents or ameliorates atherosclerosis-induced hypertension, immune cell recruitment into the aortic root wall and up-regulates aortic eNOS expression in ApoE(-/-) mice. Copyright © 2010 Elsevier Inc. All rights reserved.

  13. Aortic root, not valve, calcification correlates with coronary artery calcification in patients with severe aortic stenosis

    DEFF Research Database (Denmark)

    Henein, Michael; Hällgren, Peter; Holmgren, Anders

    2015-01-01

    calcification (AVC), due to tissue similarity between the two types of vessel rather than with the valve leaflet tissue. MATERIAL AND METHODS: We studied 212 consecutive patients (age 72.5 ± 7.9 years, 91 females) with AS requiring aortic valve replacement (AVR) in two Heart Centers, who underwent multidetector...... cardiac CT preoperatively. CAC, AVC and ARC were quantified using Agatston scoring. Correlations were tested by Spearman's test and Mann-Whitney U-test was used for comparing different subgroups; bicuspid (BAV) vs tricuspid (TAV) aortic valve. RESULTS: CAC was present in 92%, AVC in 100% and ARC in 82......% of patients. CAC correlated with ARC (rho = 0.51, p AVC. The number of calcified coronary arteries correlated with ARC (rho = 0.45, p AVC. 29/152 patients had echocardiographic evidence of BAV and 123 TAV, who were older (p

  14. Esophageal dilatation using the Eder Puestow dilators.

    Science.gov (United States)

    Royston, C M; Dowling, B L; Gear, M W

    1976-06-01

    We have performed fifty-one dilatations in twenty-six patients using an end-viewing fiberoptic endoscope and Eder Puestow dilators. All (except two) were performed using intravenous diazepam, the majority on an outpatient basis. The only complication has been a single case of aspiration pneumonia. We have found this method of esophageal dilatation particularly useful in the preoperative dilatation of benign strictures, and in those elderly frail patients who are unsuitable for surgery. Transthoracic resection of the stricture is avoided and thus transabdominal repair of the hiatus hernia may be undertaken.

  15. Uremia modulates the phenotype of aortic smooth muscle cells

    DEFF Research Database (Denmark)

    Madsen, Marie; Pedersen, Annemarie Aarup; Albinsson, Sebastian

    2017-01-01

    the phenotype of aortic SMCs in vivo. METHODS: Moderate uremia was induced by 5/6 nephrectomy in apolipoprotein E knockout (ApoE(-/-)) and wildtype C57Bl/6 mice. Plasma analysis, gene expression, histology, and myography were used to determine uremia-mediated changes in the arterial wall. RESULTS: Induction...... of moderate uremia in ApoE(-/-) mice increased atherosclerosis in the aortic arch en face 1.6 fold (p = 0.04) and induced systemic inflammation. Based on histological analyses of aortic root sections, uremia increased the medial area, while there was no difference in the content of elastic fibers or collagen...... in the aortic media. In the aortic arch, mRNA and miRNA expression patterns were consistent with a uremia-mediated phenotypic modulation of SMCs; e.g. downregulation of myocardin, α-smooth muscle actin, and transgelin; and upregulation of miR146a. Notably, these expression patterns were observed after acute (2...

  16. The value of echocardiography in follow-up of human tissue valves in aortic position

    NARCIS (Netherlands)

    T.P. Willems (Tineke)

    1999-01-01

    textabstractThe application of human tissue valves for aortic valve or root replacement was introduced during the 19608. The first successful clinical orthotopic implantation of an aortic allograft was performed by Ross and Barrat-Boyes independently in 19621,2, In 1967 Ross first reported the use

  17. Aortic rupture during aortoplasty in Takayasu arteritis – A rare complication: Case report and review of literature

    Directory of Open Access Journals (Sweden)

    Vimal Mehta

    2014-05-01

    Full Text Available Balloon angioplasty of the stenosed aorta is usually a relatively simple, yet potentially a catastrophic procedure. Aortic rupture during aortoplasty, though uncommon, carries a high mortality. We report case of a 39-year-old female with aortoarteritis with multiple arterial stenoses whose infra-renal abdominal aorta ruptured during balloon dilatation of the stent deployed in that segment. The site of aortic rupture was temporarily occluded by low-pressure inflation of the same balloon and then was sealed using a stent-graft introduced by contra-lateral femoral arterial access.

  18. Long-term follow-up of patients undergoing aortic root enlargement for insertion of a larger prosthesis.

    Science.gov (United States)

    Correia, Pedro M; Coutinho, Gonçalo F; Branco, Carlos; Antunes, Manuel J

    2016-07-01

    To evaluate the long-term survival of patients undergoing aortic root enlargement (ARE) compared with those with small aortic root (SAR), exploring risk factors for late mortality as well as the influence of patient-prosthesis mismatch (PPM). From January 1999 through December 2010, a total of 3724 patients underwent isolated or combined aortic valve replacement at our institution. From these, 239 (6.4%) had transannular ARE with a pericardial patch, to permit implantation of a larger prosthesis. This study population was compared with a control group of 767 patients (20.6%) who were considered to have SAR, as a prosthesis of size 21 or less was implanted. Mean age was comparable: 70.4 ± 12.5 vs 69.9 ± 9.6 years for ARE and SAR groups, respectively (P = 0.552). Female sex predominated in the control group (81.6 vs 88.0%; P = 0.011). Patients of the ARE group tended to have higher mean body surface area (1.59 ± 0.15 vs 1.57 ± 0.13 m(2); P = 0.061) and were less symptomatic (NYHA III-IV: 49.4 vs 57.9%; P = 0.021). Implantation of bioprostheses was more frequent in the ARE group (76.2 vs 52.3%; P < 0.001), while concomitant procedures were more frequent in the SAR group (25.5 vs 32.2%; P = 0.050). Patients in the SAR group had higher moderate PPM (29.7 vs 50.1%; P < 0.001), but no patient was left with severe PPM. Hospital mortality was not statistically different between ARE and SAR groups (0.8 vs 0.5%; P = 0.632). The overall survival rate for ARE group patients at 5, 10 and 15 years was 82.7 ± 2.5, 64.8 ± 3.8 and 36.0 ± 7.5%, respectively, in comparison with 86.2 ± 1.3, 62.9 ± 2.3 and 38.4 ± 4.3% for the SAR group (P = 0.741). There was no significant difference in long-term survival of ARE patients compared with the age- and gender-matched general population (P = 0.794). Long-term survival was not affected by the presence of PPM. Increasing age, male sex, atrial fibrillation, LV end-systolic dimension, preoperative creatinine and NYHA class III-IV were

  19. Vasorelaxant Effect of Osterici Radix Ethanol Extract on Rat Aortic Rings

    Directory of Open Access Journals (Sweden)

    Kyungjin Lee

    2013-01-01

    Full Text Available The root of Ostericum koreanum Maximowicz has been used as a traditional medicine called “Kanghwal” in Korea (or “Qianghuo” in China. The purpose of this study was to investigate the vasorelaxant activity and mechanism of action of an ethanol extract of the O. koreanum root (EOK. We used isolated rat aortic rings to assess the effects of EOK on various vasorelaxant or vasoconstriction factors. EOK induced vasorelaxation in phenylephrine hydrochloride (PE or KCl precontracted aortic rings in a concentration-dependent manner. However, the vasorelaxant effects of EOK on endothelium-intact aortic rings were reduced by pretreatment with L-NAME or methylene blue. In Ca2+-free Krebs-Henseleit solution, pretreatment with EOK (0.3 mg/mL completely inhibited PE-induced constriction. In addition, EOK (0.3 mg/mL also completely inhibited vasoconstriction induced by supplemental Ca2+ in aortic rings that were precontracted with PE or KCl. Furthermore, the EOK-induced vasorelaxation in PE-contracted aortic rings was inhibited by preincubation with nifedipine. These results indicate that the vasorelaxant effects of EOK are responsible for the induction of NO formation from L-Arg and NO-cGMP pathways, blockage of the extracellular Ca2+ entry via the receptor-operative Ca2+ channel and voltage-dependent calcium channel, and blockage of sarcoplasmic reticulum Ca2+ release via the inositol triphosphate pathway.

  20. Combined Bentall and modified Ravitch procedures in a patient with Marfan syndrome

    Directory of Open Access Journals (Sweden)

    Bhuvnesh Kansara

    2013-01-01

    Full Text Available Marfan syndrome is an inherited, connective-tissue disorder transmitted as an autosomal dominant trait. Cardinal features of the disorder include tall stature, ectopia lentis, mitral valve prolapse, aortic root dilatation, and aortic dissection. Pectus excavatum may exist as an isolated lesion or in association with a genetic syndrome such as Marfan syndrome. We report the successful management of a simultaneous correction of pectus excavatum and the underlying cardiovascular diseases.

  1. Pregnancy-related acute aortic dissection in Marfan syndrome: A review of the literature.

    Science.gov (United States)

    Smith, Katherine; Gros, Bernard

    2017-05-01

    A well-established association exists between acute aortic dissection and pregnancy, particularly in women with Marfan syndrome. However, there is debate regarding appropriate management guidelines. In particular, there are differing opinions regarding when prophylactic aortic root repair should be recommended as well as the efficacy of beta blockers in this clinical scenario. The current study evaluated 10 years of published literature (2005-2015) in the PubMed/Medline database. Fifty articles, describing 72 cases of women who presented with aortic dissection in the antepartum or postpartum period were identified. Comparisons on demographic variables and clinical outcomes between cases of women with Marfan syndrome (n = 36) and without Marfan syndrome (n = 36) were conducted. There were no significant differences in demographics (age, gravidity, parity) between the Marfan and non-Marfan cases. Marfan patients presented with antepartum dissections significantly earlier in pregnancy than those without Marfan syndrome (P = .002). However, there were no significant difference between the 2 groups in maternal mortality, fetal mortality, or obstetric outcomes (mode of delivery and gestational age at delivery). Eight cases described events in Marfan women with an aortic root diameter ≤40 mm. Six events occurred in Marfan women who were managed with beta blockers. Current guidelines rely on aortic root diameter for stratification of Marfan women into risk categories, but we identified several cases that would be missed by these guidelines. Specifically, the existing literature suggest that women with Marfan syndrome should take precautions throughout pregnancy, rather than the third trimester. © 2017 Wiley Periodicals, Inc.

  2. The Externally Supported Ross Operation: Early Outcomes and Intermediate Follow-Up.

    Science.gov (United States)

    Jacobsen, Roni M; Earing, Michael G; Hill, Garick D; Barnes, Michael; Mitchell, Michael E; Woods, Ronald K; Tweddell, James S

    2015-08-01

    The externally supported Ross (supported Ross), consisting of a Dacron (DuPont, Wilmington, DE) graft to support the neoaortic root, has been used in adolescent and adult patients to prevent neoaortic dilatation. Outcomes after the supported Ross technique were compared with the Ross procedure using the standard aortic root replacement technique (standard Ross). This was a retrospective analysis of 36 adolescent and young adult patients who underwent the Ross procedure between 1992 and 2013. The outcomes of supported Ross procedures in 26 patients were compared with the Ross procedure in 10 patients. End points included survival, neoaortic root dilatation, development of neoaortic regurgitation, and the need for reintervention. The median age at operation was 14 years (range, 11 to 31 years), and indications for the operation were mixed lesions (47%), followed by aortic regurgitation (42%) and stenosis (11%). There were no early deaths. The mean follow-up was 2.2 years (range, 1 to 11 years). At the 1-year (p = 0.01) and 3-year (p Ross cohort had a smaller neoaortic root z-score. Neither cohort had a large number of patients with significant neoaortic regurgitation, with 1 patient in the supported cohort compared with 3 patients in the standard cohort. Overall, 4 patients (40%) in the standard Ross cohort had required reintervention, including 3 directed at the neoaortic root. One patient in the supported Ross cohort required early reintervention for revision of the right coronary artery. At intermediate follow-up, patients who underwent the supported Ross technique were less likely to have neoaortic root dilatation compared with patients who underwent a standard Ross procedure. Further studies are needed to evaluate the long-term durability of this technique, particularly in regards to the development of significant aortic regurgitation, the rate of reintervention, and application to younger and smaller patients. Copyright © 2015 The Society of Thoracic Surgeons

  3. Oil Red O and Hematoxylin and Eosin Staining for Quantification of Atherosclerosis Burden in Mouse Aorta and Aortic Root.

    Science.gov (United States)

    Andrés-Manzano, M Jesús; Andrés, Vicente; Dorado, Beatriz

    2015-01-01

    Methods for staining tissues with Oil Red O and hematoxylin-eosin are classical histological techniques that are widely used to quantify atherosclerotic burden in mouse tissues because of their ease of use, reliability, and the large amount of information they provide. These stains can provide quantitative data about the impact of a genetic or environmental factor on atherosclerotic burden and on the initiation, progression, or regression of the disease, and can also be used to evaluate the efficacy of drugs designed to prevent or treat atherosclerosis. This chapter provides protocols for quantifying atherosclerotic burden in mouse aorta and aortic root, including methods for dissection, Oil Red O staining, hematoxylin-eosin staining, and image analysis.

  4. Association Between Left Atrial Dilatation and Invasive Hemodynamics at Rest and During Exercise in Asymptomatic Aortic Stenosis

    DEFF Research Database (Denmark)

    Christensen, Nicolaj Lyhne; Dahl, Jordi Sanchez; Carter-Storch, Rasmus

    2016-01-01

    BACKGROUND: Transition from an asymptomatic to symptomatic state in severe aortic stenosis is often difficult to assess. Identification of a morphological sign of increased hemodynamic load may be important in asymptomatic aortic stenosis to identify patients at risk. METHODS AND RESULTS: Thirty...... was similar between groups (0.81±0.15 versus 0.84±0.18 cm(2); P=0.58). PCWP was higher at rest and during exercise in patients with LA volume index ≥35 mL/m(2) (Prest, PCWP was

  5. Aortic valve-sparing operation in Marfan syndrome: what do we know after a decade?

    Science.gov (United States)

    Kallenbach, Klaus; Baraki, Hassina; Khaladj, Nawid; Kamiya, Hiroyuki; Hagl, Christian; Haverich, Axel; Karck, Matthias

    2007-02-01

    We assessed the outcome in patients with Marfan syndrome operated on exclusively with the aortic valve-sparing reimplantation technique for aortic root aneurysms during more than a decade. Between July 1993 and April 2005, the aortic valve-sparing reimplantation technique (David I) was used in 325 patients. In 59 patients with clinical evidence of Marfan syndrome, procedures were done for aortic root aneurysm (n = 55) or aortic dissection type A (n = 4). Their mean age was 30 +/- 12 years (range, 9 to 62 years), and 37 (63%) were male. Additional procedures were arch replacement in 4 patients, coronary artery bypass grafting in 1, mitral valve surgery in 9, and closure of atrial septal defect in 3. Mean follow-up was 54 +/- 37 months (range, 0 to 139 months). No patient died during the first 30 days postoperatively. Mean bypass time was 163 +/- 34 minutes (range, 99 to 248 minutes), and mean aortic cross clamp time was 126 +/- 28 minutes (range, 78 to 202 minutes). Four patients (6.8%) required rethoracotomy for postoperative bleeding. Five late deaths (8.5%) occurred during follow-up. Reoperation of the reconstructed valve was required in 7 patients. Freedom from reoperation was 88% +/- 5% at 5 years and 80% +/- 9% at 10 years. Mean grade of aortic insufficiency was 1.81 preoperatively compared with 0.20 early postoperatively (p valve should encourage use of this technique in patients with Marfan syndrome.

  6. Arterial elastic fiber structure. Function and potential roles in acute aortic dissection.

    Science.gov (United States)

    Pratt, B; Curci, J

    2010-10-01

    The lethality of acute aortic dissection is well recognized. Successful treatment and prevention of aortic dissection is going to be dependent upon an improved understanding of the molecular and physiologic events which predispose to dissection development and propagation. In this review, we will focus on the elastic fiber, one of the critical elements of the aortic wall matrix. Mechanical or functional failure of the elastin in the wall of the aorta likely predisposes to dissection as well as the post-dissection aortic degeneration with aneurysm formation. Insight into the role of the elastin and the elastic fiber in aortic dissection has recently been accelerated by research into the molecular mechanisms associated with hereditary propensity for aortic dissection, such as Marfan syndrome. These studies have implicated both structural and metabolic contributions of alterations in the scaffolding proteins in matrix elastic fibers. In particular, increased transforming growth factor-β (TGF-β) activity may play a prominent role in predisposing the aortic wall to dissection. The events which predispose to post-dissection aortic degeneration are somewhat less well defined. However, the loss of the structural integrity of the remaining elastic fibers leaves the wall weaker and prone to dilatation and rupture. It appears likely that the upregulation of several potent proteases, particularly those of the matrix metalloproteinase (MMP) family such as MMP-9, are participating in the subsequent matrix damage. Novel medical treatments based on this pathologic data have been proposed and in some cases have made it to clinical trials. The ongoing study evaluating whether therapeutic inhibition of TGF-β may be useful in reducing the risk of aortic dissection in patients at high risk represents one promising new strategy in the treatment of this deadly disease.

  7. Aorta measurements are heritable and influenced by bicuspid aortic valve

    Directory of Open Access Journals (Sweden)

    Lisa J Martin

    2011-09-01

    Full Text Available Abstract: Word Count 266, 1609 charactersObjectives: To determine whether the contributions of genetics and bicuspid aortic valve (BAV independently influence aortic (Ao dimensions.Background: Ao dilation is a risk factor for aneurysm, dissection, and sudden cardiac death. Frequent association of BAV with Ao dilation implicates a common underlying defect possibly due to genetic factors. Methods: Families enriched for BAV underwent standardized transthoracic echocardiography. In addition to BAV status, echocardiographic measures of Ao (annulus to descending Ao, pulmonary artery and mitral valve annulus diameters were obtained. Using variance components analysis, heritability was estimated with and without BAV status. Additionally, bivariate genetic analyses between Ao dimensions and BAV were performed.Results: Our cohort was obtained from 209 families enriched for BAV. After adjusting for age, body surface area and sex, individuals with BAV had a statistically significant increase in all echocardiographic measurements (p < 0.006 except descending Ao and mitral valve annulus. Individuals with BAV were at greater odds of having Ao dilation (OR = 4.44, 95% CI 2.93 – 6.72 than family members without BAV. All echocardiographic measurements exhibited moderate to strong heritability (0.25 to 0.53, and these estimates were not influenced by inclusion of BAV as a covariate. Bivariate genetic analyses supported that the genetic correlation between BAV and echo measures were not significantly different from zero.Conclusions: We show for the first time that echocardiographic measurements of Ao, pulmonary artery and mitral valve annulus diameters are quantitative traits that exhibit significant heritability. In addition, our results suggest the presence of BAV independently influences the proximal Ao and pulmonary artery measures but not those in the descending Ao or mitral valve annulus.

  8. Computed tomography angiography reveals stenosis and aneurysmal dilation of an aberrant right subclavian artery causing systemic blood pressure misreading in an old Pekinese dog.

    Science.gov (United States)

    Kim, Jaehwan; Eom, Kidong; Yoon, Hakyoung

    2017-06-16

    A 14-year-old dog weighing 4 kg presented with hypotension only in the right forelimb. Thoracic radiography revealed a round soft tissue opacity near the aortic arch and below the second thoracic vertebra on a lateral view. Three-dimensional computed tomography angiography clearly revealed stenosis and aneurysmal dilation of an aberrant right subclavian artery. Stenosis and aneurysm of an aberrant subclavian artery should be included as a differential diagnosis in dogs showing a round soft tissue opacity near the aortic arch and below the thoracic vertebra on the lateral thoracic radiograph.

  9. 3D Bioprinting of Heterogeneous Aortic Valve Conduits with Alginate/Gelatin Hydrogels

    Science.gov (United States)

    Duan, Bin; Hockaday, Laura A.; Kang, Kevin H.; Butcher, Jonathan T.

    2013-01-01

    Heart valve disease is a serious and growing public health problem for which prosthetic replacement is most commonly indicated. Current prosthetic devices are inadequate for younger adults and growing children. Tissue engineered living aortic valve conduits have potential for remodeling, regeneration, and growth, but fabricating natural anatomical complexity with cellular heterogeneity remain challenging. In the current study, we implement 3D bioprinting to fabricate living alginate/gelatin hydrogel valve conduits with anatomical architecture and direct incorporation of dual cell types in a regionally constrained manner. Encapsulated aortic root sinus smooth muscle cells (SMC) and aortic valve leaflet interstitial cells (VIC) were viable within alginate/gelatin hydrogel discs over 7 days in culture. Acellular 3D printed hydrogels exhibited reduced modulus, ultimate strength, and peak strain reducing slightly over 7-day culture, while the tensile biomechanics of cell-laden hydrogels were maintained. Aortic valve conduits were successfully bioprinted with direct encapsulation of SMC in the valve root and VIC in the leaflets. Both cell types were viable (81.4±3.4% for SMC and 83.2±4.0% for VIC) within 3D printed tissues. Encapsulated SMC expressed elevated alpha-smooth muscle actin when printed in stiff matrix, while VIC expressed elevated vimentin in soft matrix. These results demonstrate that anatomically complex, heterogeneously encapsulated aortic valve hydrogel conduits can be fabricated with 3D bioprinting. PMID:23015540

  10. Echocardiographic aortic valve calcification and outcomes in women and men with aortic stenosis.

    Science.gov (United States)

    Thomassen, Henrik K; Cioffi, Giovanni; Gerdts, Eva; Einarsen, Eigir; Midtbø, Helga Bergljot; Mancusi, Costantino; Cramariuc, Dana

    2017-10-01

    Sex differences in risk factors of aortic valve calcification (AVC) by echocardiography have not been reported from a large prospective study in aortic stenosis (AS). AVC was assessed using a prognostically validated visual score and grouped into none/mild or moderate/severe AVC in 1725 men and women with asymptomatic AS in the Simvastatin Ezetimibe in Aortic Stenosis study. The severity of AS was assessed by the energy loss index (ELI) taking pressure recovery in the aortic root into account. More men than women had moderate/severe AVC at baseline despite less severe AS by ELI (pAVC at baseline was independently associated with lower aortic compliance and more severe AS in both sexes, and with increased high-sensitive C reactive protein (hs-CRP) only in men (all pAVC at baseline was associated with a 2.5-fold (95% CI 1.64 to 3.80) higher hazard rate of major cardiovascular events in women, and a 2.2-fold higher hazard rate in men (95% CI 1.54 to 3.17) (both pAVC at baseline also predicted a 1.8-fold higher hazard rate of all-cause mortality in men (95% CI 1.04 to 3.06, pAVC scored by echocardiography has sex-specific characteristics in AS. Moderate/severe AVC is associated with higher cardiovascular morbidity in both sexes, and with higher all-cause mortality in men. ClinicalTrials.gov identifier: NCT00092677. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  11. The dilatation of main pulmonary artery and right ventricle observed by enhanced chest computed tomography predict poor outcome in inoperable chronic thromboembolic pulmonary hypertension.

    Science.gov (United States)

    Ema, Ryogo; Sugiura, Toshihiko; Kawata, Naoko; Tanabe, Nobuhiro; Kasai, Hajime; Nishimura, Rintaro; Jujo, Takayuki; Shigeta, Ayako; Sakao, Seiichiro; Tatsumi, Koichiro

    2017-09-01

    Dilatation of the pulmonary artery and right ventricle on chest computed tomography images is often observed in patients with pulmonary hypertension. The clinical significance of these image findings has not been defined in chronic thromboembolic pulmonary hypertension. We investigated whether the pulmonary arterial and right ventricle dilatation was associated with poor outcome in chronic thromboembolic pulmonary hypertension. This was a retrospective cohort investigation in 60 subjects with inoperable chronic thromboembolic pulmonary hypertension diagnosed consecutively between 1997 and 2010 at Chiba University Hospital. Digital scout multi-detector chest computed tomography images were obtained. The main pulmonary arterial to ascending aortic diameter ratio and the right ventricular to left ventricular diameter ratio were calculated. Main pulmonary arterial to ascending aortic diameter ratio ranged from 0.85 to 1.84, and right ventricular to left ventricular diameter ratio ranged from 0.71 to 2.88. During the observation period of 1284.5days (range, 21-4550days), 13 patients required hospitalization due to worsening; 6 of them died. Kaplan-Meier analysis showed significant differences in hospitalization between the patients with main pulmonary arterial to ascending aortic diameter ratio of ≥1.1 and pulmonary hypertension. Copyright © 2017 The Authors. Published by Elsevier B.V. All rights reserved.

  12. Bovine aortic arch and idiopathic pulmonary artery aneurysm associated with bronchial compression

    Directory of Open Access Journals (Sweden)

    Süleyman Sezai Yıldız

    2015-09-01

    Full Text Available The left common carotid artery originating from the brachiocephalic trunk is termed the bovine aortic arch. Although it is the third most-common normal variant found in 9% humans, the origin of this term remains unclear. Until now, It has not been reported in the literature bovine aortic arch togetherness with pulmonary aneurysm and bronchial compression. Herein, we present a case with bovine aorta arch and pulmonary artery aneurysm associated with bronchial compression, which is incidentally detected by X-ray film. A 56-year-old Caucasian female admitted to the cardiology clinic with complaint of chest pain. Physical examination was unremarkable. Blood biochemistry values and cardiac markers were in normal range. Chest radiography revealed a widened mediastinum and prominent pulmonary conus with no active pulmonary disease. A subsequent transthoracic echocardiography revealed left ventricular hypertrophy, left atrial enlargement (diameter: 41 mm, mild mitral and tricuspid valve insufficiency, dilatation of main pulmonary artery (parasternal short-axis view diameter: 33 mm, normal pulmonary artery pressure and normal left ventricular systolic function. Computed tomography revealed bovine aortic arch associated with pulmonary artery aneurysm (diameter: 53 mm. And left main bronch of trachea was critically squeezed by aortic arch. Aortic and pulmonary vascular anomalies should be considered in patients with chest pain. And, identification with imaging modalities is important for prevention of chronic and irreversible complications.

  13. Magnetic resonance imaging 4-D flow-based analysis of aortic hemodynamics in Turner syndrome.

    Science.gov (United States)

    Arnold, Raoul; Neu, Marie; Hirtler, Daniel; Gimpel, Charlotte; Markl, Michael; Geiger, Julia

    2017-04-01

    Cardiovascular surveillance is important in Turner syndrome because of the increased risk of aortic dilation and dissection with consecutively increased mortality. To compare 4-D flow MRI for the characterization of aortic 3-D flow patterns, dimensions and vessel wall parameters in pediatric patients with Turner syndrome and age-matched controls. We performed 4-D flow MRI measuring in vivo 3-D blood flow with coverage of the thoracic aorta in 25 patients with Turner syndrome and in 16 female healthy controls (age mean ± standard deviation were 16 ± 5 years and 17 ± 4 years, respectively). Blood flow was visualized by time-resolved 3-D path lines. Visual grading of aortic flow in terms of helices and vortices was performed by two independent observers. Quantitative analysis included measurement of aortic diameters, quantification of peak systolic wall shear stress, pulsatility index and oscillatory shear index at eight defined sites. Patients with Turner syndrome had significantly larger aortic diameters normalized to BSA, increased vortices in the ascending aorta and elevated helix flow in the ascending and descending aorta compared to controls (all PTurner patients compared to controls (p=0.02, p=0.002 and p=0.01 respectively). Four-dimensional flow MRI provides new insights into the altered aortic hemodynamics and wall shear stress that could have an impact on the development of aortic dissections.

  14. Dilatation of the ascending aorta is associated with presence of aortic regurgitation in patients after repair of tetralogy of Fallot

    Science.gov (United States)

    Ordovas, Karen Gomes; Keedy, Alexander; Naeger, David M.; Kallianos, Kimberly; Foster, Elyse; Liu, Jing; Saloner, David; Hope, Michael D.

    2016-01-01

    To evaluate the association between aortic morphology and elasticity with aortic regurgitation in surgically corrected of tetralogy of Fallot (TOF) patients. We retrospectively identified 72 consecutive patients with surgically corrected TOF and 27 healthy controls who underwent cardiac MRI evaluation. Velocity-encoded cine MRI was used to quantify degree of aortic regurgitation (AR) in TOF patients. Ascending aorta diameters were measured at standard levels on MRA images. Aortic pulse-wave velocity (PWV) was quantified with MRI. Morphological and functional MRI variables were compared between groups of TOF patients with and without clinically relevant AR and controls. The association between aortic morphology and elasticity with the presence of AR was evaluated using univariate and multivariate logistic regression. The majority of TOF patients had only trace AR. Nine TOF patients (12 %) had an AR fraction higher than 15 %. Indexed aorta diameter at the sinotubular junction (p = 0.007), at the RPA level (p = 0.006), and low left ventricular ejection fraction (LVEF) (p = 0.015) showed the strongest associations with the presence of at least mild AR, which persisted after controlling for age and gender. Increased ascending aorta dimension is associated with AR in patients after repair of TOF. LVEF was also low in the group of patients with relevant AR compared to those without, suggesting even mild to moderate AR may contribute to LV dysfunction in these patients. Enlarged ascending aorta may be an indication for precise quantification of regurgitant fraction with MRI, since symptomatic patients may need aortic valve repair when moderate regurgitation is present. PMID:27240599

  15. Outcomes after valve-preserving root surgery for patients with Marfan syndrome.

    Science.gov (United States)

    Kunihara, Takashi; Aicher, Diana; Rodionycheva, Svetlana; Asano, Mitsuru; Tochii, Masato; Sata, Fumihiro; Schäfers, Hans-Joachim

    2012-09-01

    In patients with Marfan syndrome (MFS), valve reimplantation has been proposed as superior to root remodeling. In the present study, both forms of valve-preserving root repair were applied and mid-term results analyzed in MFS patients compared to a propensity score-matched cohort. Among 604 patients who underwent valve-preserving aortic root surgery between 1995 and 2011 at the authors' institution, 33 MFS patients (16 males, 17 females; mean age 31 +/- 12 years) underwent either remodeling (n=21) or reimplantation (n=12). All patients were followed up echocardiographically, and the outcome with regard to late aortic valve regurgitation (AR) grade EII and reoperation on the aortic valve was compared between MFS patients and the matched cohort (n=33). Baseline characteristics and operative data were similar between the groups. Actuarial freedom from AR > or = II at seven years was 86 +/- 8% in MFS patients and 90 +/- 10% in matched non-MFS patients (p = 0.94). Actuarial freedom from reoperation at seven years was 90 +/- 7% in MFS patients and 100% in non-MFS patients (p = 0.79). In Cox's proportional hazard's model, no independent risk factor, including MFS, was found for recurrent AR or reoperation. Within the MFS patients, remodeling and reimplantation provided an almost identical freedom from late AR > or = II and reoperation up to five years postoperatively (p = 0.55 and 0.99, respectively). The stability of valve-preserving aortic root repair was comparable between patients with or without MFS. Both forms of valve-preserving root repair can provide similar mid-term results for MFS patients, primarily according to their root geometry. However, additional long-term follow up data based on a larger number of patients are required to confirm the evidence obtained to date.

  16. Contemporary Management of Type B Aortic Dissection in the Endovascular Era.

    Science.gov (United States)

    Bannazadeh, Mohsen; Tadros, Rami O; McKinsey, James; Chander, Rajiv; Marin, Michael L; Faries, Peter L

    2016-04-01

    Aortic dissection (AD) is one of the most common catastrophic pathologies affecting the aorta. Anatomic classification is based on the origin of entry tear and its extension. Type A dissections originate in the ascending aorta, whereas the entry tear in Type B dissections starts distal to the left subclavian artery. The patients with aortic dissection who manifest complications such as rupture, malperfusion, aneurysmal degeneration, and intractable pain are classified as complicated AD. Risk factors for developing aortic dissection include age, male gender, and aortic wall structural abnormalities. The most common presenting symptom of acute aortic dissection is pain. Malperfusion occurs as a result of end-organ ischemia due to involvement of aortic branches from the dissecting process. This can happen in various locations causing mesenteric ischemia (mesenteric vessels), stroke (aortic arch vessels), renal failure (renal arteries), spinal ischemia, and limb ischemia (iliac or subclavian arteries). Aneurysmal degeneration is the most common complication of patients with chronic Type B dissection who are managed with medical therapy. Management of Type B aortic dissection (TBAD) remains controversial. Many groups recommend conservative therapy for newly diagnosed TBAD and reserve surgical management for patients who develop complications such as rupture, malperfusion, aneurysmal dilatation, and refractory pain. The mainstay of medical therapy includes antihypertensive medication to reduced ΔP/ ΔT by lowering blood pressure and heart rate. With the continued success of thoracic endovascular aortic repair (TEVAR), this procedure has been extended to treat TBAD in selected patients. The outcomes of TEVAR are promising, with early mortality rates from 10% to 20%. With promising results from these series, some groups recommend early TEVAR in uncomplicated TBAD to prevent future adverse events. The goals of endovascular treatment of TBAD are to cover the entry tear

  17. Repair-oriented classification of aortic insufficiency: impact on surgical techniques and clinical outcomes.

    Science.gov (United States)

    Boodhwani, Munir; de Kerchove, Laurent; Glineur, David; Poncelet, Alain; Rubay, Jean; Astarci, Parla; Verhelst, Robert; Noirhomme, Philippe; El Khoury, Gébrine

    2009-02-01

    Valve repair for aortic insufficiency requires a tailored surgical approach determined by the leaflet and aortic disease. Over the past decade, we have developed a functional classification of AI, which guides repair strategy and can predict outcome. In this study, we analyze our experience with a systematic approach to aortic valve repair. From 1996 to 2007, 264 patients underwent elective aortic valve repair for aortic insufficiency (mean age - 54 +/- 16 years; 79% male). AV was tricuspid in 171 patients bicuspid in 90 and quadricuspid in 3. One hundred fifty three patients had type I dysfunction (aortic dilatation), 134 had type II (cusp prolapse), and 40 had type III (restrictive). Thirty six percent (96/264) of the patients had more than one identified mechanism. In-hospital mortality was 1.1% (3/264). Six patients experienced early repair failure; 3 underwent re-repair. Functional classification predicted the necessary repair techniques in 82-100% of patients, with adjunctive techniques being employed in up to 35% of patients. Mid-term follow up (median [interquartile range]: 47 [29-73] months) revealed a late mortality rate of 4.2% (11/261, 10 cardiac). Five year overall survival was 95 +/- 3%. Ten patients underwent aortic valve reoperation (1 re-repair). Freedoms from recurrent Al (>2+) and from AV reoperation at 5 years was 88 +/- 3% and 92 +/- 4% respectively and patients with type I (82 +/- 9%; 93 +/- 5%) or II (95 +/- 5%; 94 +/- 6%) had better outcomes compared to type III (76 +/- 17%; 84 +/- 13%). Aortic valve repair is an acceptable therapeutic option for patients with aortic insufficiency. This functional classification allows a systematic approach to the repair of Al and can help to predict the surgical techniques required as well as the durability of repair. Restrictive cusp motion (type III), due to fibrosis or calcification, is an important predictor for recurrent Al following AV repair.

  18. [Aortic valve preservation in Marfan syndrome. Initial experience].

    Science.gov (United States)

    Forteza, Alberto; Cortina, Jose M; Sánchez, Violeta; Centeno, Jorge; López, M Jesús; Pérez de la Sota, Enrique; Rufilanchas, Juan J

    2007-05-01

    Preservation of the aortic valve using the technique described by David has been shown to be as effective as the Bentall-De Bono procedure. It avoids both the need for long-term anticoagulation and the complications associated with mechanical prostheses. We report our initial experience using this technique in patients with Marfan syndrome. Between April 2004 and April 2006, we used the David reimplantation technique in 40 patients with an aortic root aneurysm. Eighteen patients had Marfan syndrome. Their median age was 29 years (13-55 years). Echocardiography showed that the median diameter of the aortic sinus was 53 mm (46-59 mm). In 17 patients, aortic valve preservation was possible. No patient died during hospitalization and there were no significant complications. On echocardiography at discharge, no patient had greater than grade-II aortic regurgitation. During a median follow-up period of 8 months (1-24 months), one patient died due to rupture of an abdominal aneurysm. The others are all in New York Heart Association class I. Preservation of the aortic valve by means of valve reimplantation produced excellent results. It avoided both the thromboembolic and hemorrhagic complications associated with prostheses and the need for long-term anticoagulation. If reimplanted valves continue to function adequately over the long term, this technique should become the treatment of choice for aneurysms of the ascending aorta in patients with Marfan syndrome.

  19. Cardiovascular Disease in Ageing: An Overview on Thoracic Aortic Aneurysm as an Emerging Inflammatory Disease

    Directory of Open Access Journals (Sweden)

    Calogera Pisano

    2017-01-01

    Full Text Available Medial degeneration associated with thoracic aortic aneurysm and acute aortic dissection was originally described by Erdheim as a noninflammatory lesion related to the loss of smooth muscle cells and elastic fibre fragmentation in the media. Recent evidences propose the strong role of a chronic immune/inflammatory process in aneurysm evocation and progression. The coexistence of inflammatory cells with markers of apoptotic vascular cell death in the media of ascending aorta with aneurysms and type A dissections raises the possibility that activated T cells and macrophages may contribute to the elimination of smooth muscle cells and degradation of the matrix. On the other hand, several inflammatory pathways (including TGF-β, TLR-4 interferon-γ, chemokines, and interferon-γ seem to be involved in the medial degeneration related to aged and dilated aorta. This is an overview on thoracic aortic aneurysm as an emerging inflammatory disease.

  20. Endograft Collapse After Endovascular Treatment for Thoracic Aortic Disease

    International Nuclear Information System (INIS)

    Bandorski, Dirk; Brueck, Martin; Guenther, Hans-Ulrich; Manke, Christoph

    2010-01-01

    Endovascular treatment is an established therapy for thoracic aortic disease. Collapse of the endograft is a potentially fatal complication. We reviewed 16 patients with a thoracic endograft between 2001 and 2006. Medical records of the treated patients were studied. Data collected include age, gender, diagnosis, indication for endoluminal treatment, type of endograft, and time of follow up. All patients (n = 16; mean age, 61 years; range, 21-82 years) underwent computed tomography (CT) for location of the lesion and planning of the intervention. Time of follow-up with CT scan ranged from 1 to 61 months. Indications for endovascular treatment were degenerative aneurysm (n = 7; 44%), aortic dissection (n = 2; 12%), perforated aortic ulcer (n = 4; 25%), and traumatic aortic injury (n = 3; 19%). Three patients suffered from a collapse of the endograft (one patient distal, two patients proximal) between 3 and 8 days after endovascular treatment. These patients were younger (mean age, 37 ± 25 years vs. 67 ± 16 years; P 0.05]; distal, 45 ± 23.5% vs. 38 ± 21.7% [P > 0.05]). Proximal collapse was corrected by placing a bare stent. In conclusion, risk factors for stent-graft collapse are a small lumen of the aorta and a small radius of the aortic arch curvature (young patients), as well as oversizing, which is an important risk factor and is described for different types of endografts and protheses (Gore TAG and Cook Zenith). Dilatation of the collapsed stent-graft is not sufficient. Following therapy implantation of a second stent or surgery is necessary in patients with a proximal endograft collapse. Distal endograft collapse can possibly be treated conservatively under close follow-up.

  1. Elective reconstruction of thoracoabdominal aortic aneurysm type IV by transabdominal approach

    Directory of Open Access Journals (Sweden)

    Marjanović Ivan

    2012-01-01

    Full Text Available Introduction. Thoracoabdominal aortic aneurysm (TAAA type IV represents an aortic dilatation from the level of the diaphragmatic hiatus to the iliac arteries branches, including visceral branches of the aorta. In the traditional procedure of TAAA type IV repair, the body is opened using thoractomy and laparotomy in order to provide adequate exposure of the descending thoracic and abdominal aorta for safe aortic reconstruction. Case report. We reported a 71-yearold man with elective reconstruction of the TAAA type IV performed by transabdominal approach. Computed tomography scans angiography revealed a TAAA type IV with diameter of 62 mm in the region of celiac trunk and superior mesenteric artery branching, and the largest diameter of 75 mm in the infrarenal aortic level. The patient comorbidity included a chronic obstructive pulmonary disease and hypertension, therefore he was treated for a prolonged period. In preparation for the planned aortic reconstruction asymptomatic carotid disease (occlusion of the left internal carotid artery and subtotal stenosis of the right internal carotid artery was diagnosed. Within the same intervention percutaneous transluminal angioplasty with stent placement in right internal carotid artery was made. In general, under endotracheal anesthesia and epidural analgesia, with transabdominal approach performed aortic reconstruction with tubular dakron graft 24 mm were, and reimplantation of visceral aortic branches into the graft performed. Postoperative course was uneventful, and the patient was discharged on the postoperative day 17. Control computed tomography scan angiography performed three months after the operation showed vascular state of the patient to be in order. Conclusion. Complete transabdominal approach to TAAA type IV represents an appropriate substitute for thoracoabdominal approach, without compromising safety of the patient. This approach is less traumatic, especially in patients with impaired

  2. Durability of Aortic Valve Cusp Repair With and Without Annular Support.

    Science.gov (United States)

    Zeeshan, Ahmad; Idrees, Jay J; Johnston, Douglas R; Rajeswaran, Jeevanantham; Roselli, Eric E; Soltesz, Edward G; Gillinov, A Marc; Griffin, Brian; Grimm, Richard; Hammer, Donald F; Pettersson, Gösta B; Blackstone, Eugene H; Sabik, Joseph F; Svensson, Lars G

    2018-03-01

    To determine the value of aortic valve repair rather than replacement for valve dysfunction, we assessed late outcomes of various repair techniques in the contemporary era. From January 2001 to January 2011, aortic valve repair was planned in 1,124 patients. Techniques involved commissural figure-of-8 suspension sutures (n = 63 [6.2%]), cusp repair with commissuroplasty (n = 481 [48%]), debridement (n = 174 [17%]), free-margin plication (n = 271 [27%]) or resection (n = 75) or both, or annulus repair with resuspension (n = 230 [23%]), root reimplantation (n = 252 [25%]), or remodeling (n = 35 [3.5%]). Planned repair was aborted for replacement in 115 patients (10%); risk factors included greater severity of aortic regurgitation (AR; p = 0.0002) and valve calcification (p < 0.0001). In-hospital outcomes for the remaining 1,009 patients included death (12 [1.2%]), stroke (13 [1.3%]), and reoperation for valve dysfunction (14 [1.4%]). Freedom from aortic valve reoperation at 1, 5, and 10 years was 97%, 93%, and 90%, respectively. Figure-of-8 suspension sutures, valve resuspension, and root repair and replacement were least likely to require reoperation; cusp repair with commissural sutures, plication, and commissuroplasty was most likely (p < 0.05). Survival at 1, 5, and 10 years was 96%, 92%, and 83%. Immediate postoperative AR grade was none-mild (94%), moderate (5%), and severe (1%). At 10 years after repair, AR grade was none (20%), mild (33%), moderate (26%), and severe (21%). Patients undergoing root procedures were less likely to have higher-grade postoperative AR (p < 0.0001). Valve repair is effective and durable for treating aortic valve dysfunction. Greater severity of AR preoperatively is associated with higher likelihood of repair failure. Commissural figure-of-8 suspension sutures and repair with annular support have the best long-term durability. Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights

  3. Abdominal aortic occlusion and vascular compromise secondary to acute gastric dilatation in a patient with bulimia.

    Science.gov (United States)

    Elsharif, M; Doulias, T; Aljundi, W; Balchandra, S

    2014-11-01

    Acute gastric dilation is a rare but recognised complication in patients with bulimia and anorexia following binge episodes owing to decreased bowel motility. We present a rare case of acute gastric dilation secondary to bulimia in an otherwise healthy 18-year-old female patient that resulted in compression and complete occlusion of the abdominal aorta, leading to acute mesenteric and bilateral lower limb ischaemia. This resolved immediately following a laparotomy and gastric decompression. Management of these patients is very challenging owing to the lack of a successful precedent. To our knowledge, such a catastrophic complication has only ever been reported once in the literature and the outcome was fatal. Our case is of additional importance as it offers a successful management strategy for these patients.

  4. Bentall procedure using cryopreserved valved aortic homografts: mid- to long-term results.

    Science.gov (United States)

    Christenson, Jan T; Sierra, Jorge; Trindade, Pedro T; Dominique, Didier; Kalangos, Afksendiyos

    2004-01-01

    The Bentall procedure is the standard operation for patients who have lesions of the ascending aorta associated with aortic valve disease. In many cases, however, mechanical prosthetic conduits are not suitable. There are few reports in the English-language medical literature concerning the mid- to long-term outcome of Bentall operations with cryopreserved homografts. Therefore, we reviewed our experience with this procedure and valved homografts. From January 1997 through December 2002, 21 patients underwent a Bentall operation with cryopreserved homografts at our institution. There were 14 males and 7 females; the mean age was 36 +/- 21 years (range, 15-74 years). Eleven patients had undergone previous aortic valve surgery. All patients had aortic dilatation or aneurysms involving the ascending aorta. Indications for surgery included aortic valve stenosis or insufficiency, and aortic valve endocarditis (native valve or prosthetic). One patient had Takayasu's arteritis and 3 had Marfan syndrome. There was 1 hospital death (due to sepsis), but no other major postoperative complications. The mean hospital stay was 14 +/- 7 days. Follow-up echocardiographic and computed tomographic scans were performed yearly. The mean follow-up was 34 months (6-72 months). Follow-up imaging revealed no calcifications or degenerative processes related to the homograft. Four patients had minimal valve regurgitation. Two patients died during follow-up. The 3-year actuarial survival rate was 85.7%. Our data suggest that the Bentall procedure with a valved homograft conduit is a safe procedure with excellent mid- to long-term results, comparable to results reported with aortic valve replacement with a homograft.

  5. Direct percutaneous transaortic approach for treatment of aortic pseudoaneurysms.

    Science.gov (United States)

    Pirelli, Luigi; Kliger, Chad; Fontana, Gregory P; Ruiz, Carlos E

    2015-05-01

    Aortic pseudoaneurysms (APAs) can develop months or years after aortic and cardiac surgery. If not treated appropriately, APAs can lead to fatal complications and ultimately death. We describe a case of a 61-year old patient with a diagnosed large pseudoaneurysm 5 years after his aortic valve surgery, who was treated with a novel transcatheter direct transaortic approach. The patient had dilated cardiomyopathy with an APA adjacent to the lower sternal plate. An Amplatzer septal occlusion device followed by coils was delivered transcutaneously through the APA to close its neck and fill the false aneurysm, respectively. Triple fusion multimodality imaging was used to guide the placement of the occlusion devices. The merging of computed tomography (CT) and echocardiography with real-time fluoroscopy was fundamental in procedural planning and guidance. Post-procedural transoesophageal echocardiogram (TOE) and CT angiography showed complete exclusion of the APA. A direct transaortic approach is a valid option for closure of an APA if the surgical risk is prohibitive, and the use of triple fusion technology is an essential tool in the hands of interventionalists and surgeons for preoperative planning and conduction of these procedures. © The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

  6. Mechanisms of recurrent aortic regurgitation after aortic valve repair: predictive value of intraoperative transesophageal echocardiography.

    Science.gov (United States)

    le Polain de Waroux, Jean-Benoît; Pouleur, Anne-Catherine; Robert, Annie; Pasquet, Agnès; Gerber, Bernhard L; Noirhomme, Philippe; El Khoury, Gébrine; Vanoverschelde, Jean-Louis J

    2009-08-01

    The aim of the present study was to examine the intraoperative echocardiographic features associated with recurrent severe aortic regurgitation (AR) after an aortic valve repair surgery. Surgical valve repair for AR has significant advantages over valve replacement, but little is known about the predictors and mechanisms of its failure. We blindly reviewed all clinical, pre-operative, intraoperative, and follow-up transesophageal echocardiographic data of 186 consecutive patients who underwent valve repair for AR during a 10-year period and in whom intraoperative and follow-up echo data were available. After a median follow-up duration of 18 months, 41 patients had recurrent 3+ AR, 23 patients presented with residual 1+ to 2+ AR, and 122 had no or trivial AR. In patients with recurrent 3+ AR, the cause of recurrent AR was the rupture of a pericardial patch in 3 patients, a residual cusp prolapse in 26 patients, a restrictive cusp motion in 9 patients, an aortic dissection in 2 patients, and an infective endocarditis in 1 patient. Pre-operatively, all 3 groups were similar for aortic root dimensions and prevalence of bicuspid valve (overall 37%). Patients with recurrent AR were more likely to display Marfan syndrome or type 3 dysfunction pre-operatively. At the opposite end, patients with continent AR repair at follow-up were more likely to have type 2 dysfunction pre-operatively. After cardiopulmonary bypass, a shorter coaptation length, the degree of cusp billowing, a lower level of coaptation (relative to the annulus), a larger diameter of the aortic annulus and the sino-tubular junction, the presence of a residual AR, and the width of its vena contracta were associated with the presence of AR at follow-up. Multivariate Cox analysis identified a shorter coaptation length (odds ratio [OR]: 0.8, p = 0.05), a coaptation occurring below the level of the aortic annulus (OR: 7.9, p < 0.01), a larger aortic annulus (OR: 1.2, p = 0.01), and residual aortic regurgitation

  7. Surgical treatment and thoracic endovascular aortic repair in type A aortic dissection in a pregnant patient with Marfan syndrome.

    Science.gov (United States)

    Sterner, Doerthe; Probst, Chris; Mellert, Friedrich; Schiller, Wolfgang

    2014-07-01

    We report an acute aortic dissection type Stanford A extending down to both iliac arteries affecting a 32-year-old woman suspected to have Marfan syndrome during week 37 of pregnancy. In a multidisciplinary approach, and emergency Cesarean section was performed followed by an abdominal hysterectomy and a valve-sparing aortic root replacement using a reimplantation technique. The aorta was replaced up to the hemi arch. Because of the high suspicion of visceral ischemia as confirmed ex juvantibus, an endovascular stent graft was implanted. Molecular testing revealed a frameshift mutation and confirmed the diagnosis of Marfan syndrome. Both the patient and her healthy child underwent an uneventful recovery. Copyright © 2014 Elsevier Inc. All rights reserved.

  8. [Right-side aortic arch with aberrant left subclavian artery and Kommerell's diverticulum. A cause of vascular ring].

    Science.gov (United States)

    Tamayo-Espinosa, Tania; Erdmenger-Orellana, Julio; Becerra-Becerra, Rosario; Balderrabano-Saucedo, Norma; Segura-Standford, Begoña

    The right-side aortic arch may be associated with aberrant left subclavian artery, in some cases this artery originates from an aneurismal dilation of the aorta called Kommerell's diverticulum. A report is presented on 2 cases of vascular ring formed by a right-side aortic arch, anomalous left subclavian artery, Kommerell's diverticulum and left patent ductus arteriosus. A review the literature was also performed as regards the embryological development and the imaging methods used to help in the diagnosis of this rare vascular anomaly. Copyright © 2017 Instituto Nacional de Cardiología Ignacio Chávez. Publicado por Masson Doyma México S.A. All rights reserved.

  9. Sixteen-Year Experience of David and Bentall Procedures in Acute Type A Aortic Dissection.

    Science.gov (United States)

    Yang, Bo; Patel, Himanshu J; Sorek, Claire; Hornsby, Whitney E; Wu, Xiaoting; Ward, Sarah; Thomas, Marc; Driscoll, Anisa; Waidley, Victoria A; Norton, Elizabeth L; Likosky, Donald S; Deeb, G Michael

    2018-03-01

    To examine short-term and midterm outcomes after the David and Bentall procedures in patients with an acute type A aortic dissection. Between 2001 and 2017, patients (n = 135) with acute type A aortic dissection underwent an aortic root replacement with either the David (n = 40) or Bentall (n = 95) procedure. Perioperative outcome, reoperation rate, aortic valve function, and long-term survival were evaluated. The median age of the entire cohort was 56 years. Rates of malperfusion (21%), shock (16%), history of renal failure (4%), and extent of surgery were similar between David and Bentall groups. However, the David group was significantly younger (45 versus 61 years) with less hypertension (45% versus 66%), coronary artery disease (0% versus 17%), valvulopathy (5% versus 19%), and prior cardiac surgery (5% versus 21%). Overall operative mortality was 9.6% (David 3% and Bentall 13%). Composite outcome comprising myocardial infarction, stroke, new-onset renal failure, and operative mortality was 18% in the entire cohort (David 5% and Bentall 23%). In the David group, the freedom of moderate aortic insufficiency was 95% at 10 years. The rate of reoperation for pathology of the proximal aorta or aortic valve was 0% and 2% for the David and Bentall groups, respectively. Ten-year Kaplan-Meier survival was 66% (95% confidence interval: 51% to 77%) for the entire cohort, with 98% (95% confidence interval: 84% to 99%) survival in the David group and 57% (95% confidence interval: 42% to 70%) survival in the Bentall group. Both the David and Bentall procedures are appropriate surgical approaches for aortic root replacement in select patients with an acute type A aortic dissection. Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  10. Rapid Two-stage Versus One-stage Surgical Repair of Interrupted Aortic Arch with Ventricular Septal Defect in Neonates

    Directory of Open Access Journals (Sweden)

    Meng-Lin Lee

    2008-11-01

    Conclusion: The outcome of rapid two-stage repair is comparable to that of one-stage repair. Rapid two-stage repair has the advantages of significantly shorter cardiopulmonary bypass duration and AXC time, and avoids deep hypothermic circulatory arrest. LVOTO remains an unresolved issue, and postoperative aortic arch restenosis can be dilated effectively by percutaneous balloon angioplasty.

  11. Endovascular repair or medical treatment of acute type B aortic dissection? A comparison

    Energy Technology Data Exchange (ETDEWEB)

    Chemelli-Steingruber, I. [Department of Radiology, Innsbruck Medical University (Austria); Chemelli, A. [Department of Radiology, Innsbruck Medical University (Austria)], E-mail: andreas.chemelli@i-med.ac.at; Strasak, A. [Department of Medical Statistics, Informatics and Health Economics, Innsbruck Medical University (Austria); Hugl, B. [Department of Vascular Surgery, Innsbruck Medical University (Austria); Hiemetzberger, R. [Department of Cardiology, Innsbruck Medical University (Austria); Jaschke, W.; Glodny, B.; Czermak, B.V. [Department of Radiology, Innsbruck Medical University (Austria)

    2010-01-15

    Introduction: The aim of this retrospective study was to compare the outcome of thoracic endovascular aortic repair (TEVAR) to that of medical therapy in patients with acute type B aortic dissection (TBD). Materials and methods: From July 1996 to April 2008, 88 patients presenting with acute TBD underwent either TEVAR (group A, n = 38) or medical therapy (group B, n = 50). Indications for TEVAR were intractable pain, aortic branch compromise resulting in end-organ ischemia, rapid aortic dilatation and rupture. Follow-up was performed postinterventionally, at 3, 6 and 12 months and yearly thereafter and included clinical examinations and computed tomography (CT), as well as aortic diameter measurements and assessment of thrombosis. Results: Mean follow-up was 33 months in group A and 36 months in group B. The overall mortality rate was 23.7% in group A and 24% in group B, where 4 patients died of late aortic rupture. In group A, complications included 9 endoleaks and 4 retrograde type A dissections, 3 patients were converted to open surgery and 2 needed secondary intervention. None of the patients developed paraplegia. In group B, 4 patients were converted to open surgery and 2 to TEVAR. The maximal aortic diameter increased in both groups. Regarding the extent of thrombosis, our analyses showed slightly better overall results after TEVAR, but they also showed a tendency towards approximation between the two groups during follow-up. Conclusion: TEVAR is a feasible treatment option in acute TBD. However, several serious complications may occur during and after TEVAR and it should therefore be reserved to patients with life-threatening symptoms.

  12. Doubly committed subarterial ventricular septal defect with prolapsed right coronary cusp with moderate aortic regurgitation

    Directory of Open Access Journals (Sweden)

    Redoy Ranjan

    2017-11-01

    Full Text Available A 4 year old girl was presented with the respiratory tract infection, breathlessness after taking meal, failure to thrive, abnormal movement of the chest on left side overlying the area of heart and systolic murmur. She developed these symptoms gradually for the last 3.5 years. Echocardiography revealed doubly committed subarterial ventricular septal defect with moderate aortic regurgitation. The size of the ventricular septal defect was 7 x 9 mm at the left ventricular outflow tract. The right coronary cusp of the aortic valve was prolapsed. Left atrium and left ventricle were dilated. The pulmonary artery systolic pressure was 35 mm Hg. The ventricular septal defect was closed with the standard surgical procedure using cardiopulmonary bypass followed by aortotomy and right atriotomy. Immediate post-operative period of this case was uneventful and the patient was discharged on 9th post-operative day. Follow-up echocardiography showed no residual ventricular septal defect or aortic regurgitation and the ventricular function was good.

  13. Cryopreserved human aortic root allografts arterial wall: Structural changes occurring during thawing.

    Directory of Open Access Journals (Sweden)

    Robert Novotny

    Full Text Available The aim of our experimental work was to assess morphological changes of arterial wall that arise during different thawing protocols of a cryopreserved human aortic root allograft (CHARA arterial wall.The experiment was performed on CHARAs. Two thawing protocols were tested: 1, CHARAs were thawed at a room temperature at +23°C; 2, CHARAs were placed directly into a water bath at +37°C.After fixation, all samples were washed in distilled water for 5 min, and dehydrated in a graded ethanol series (70, 85, 95, and 100% for 5 min at each level. The tissue samples were then immersed in 100% hexamethyldisilazane for 10 minutes and air dried in an exhaust hood at room temperature. Processed samples were mounted on stainless steel stubs, coated with gold.Thawing protocol 1: All 6 (100% samples showed loss of the endothelium and damage to the subendothelial layers with randomly dispersed circular defects and micro-fractures without smooth muscle cells contractions in the tunica media. Thawing protocol 2: All 6 (100% samples showed loss of endothelium from the luminal surface, longitudinal corrugations in the direction of blood flow caused by smooth muscle cells contractions in the tunica media with frequent fractures in the subendothelial layer.All the samples thawed at the room temperature showed smaller structural damage to the CHARA arterial wall with no smooth muscle cell contraction in tunica media when compared to the samples thawed in a water bath.

  14. Inflammation aggravates disease severity in Marfan syndrome patients.

    Directory of Open Access Journals (Sweden)

    Teodora Radonic

    Full Text Available BACKGROUND: Marfan syndrome (MFS is a pleiotropic genetic disorder with major features in cardiovascular, ocular and skeletal systems, associated with large clinical variability. Numerous studies reveal an involvement of TGF-β signaling. However, the contribution of tissue inflammation is not addressed so far. METHODOLOGY/PRINCIPAL FINDINGS: Here we showed that both TGF-β and inflammation are up-regulated in patients with MFS. We analyzed transcriptome-wide gene expression in 55 MFS patients using Affymetrix Human Exon 1.0 ST Array and levels of TGF-β and various cytokines in their plasma. Within our MFS population, increased plasma levels of TGF-β were found especially in MFS patients with aortic root dilatation (124 pg/ml, when compared to MFS patients with normal aorta (10 pg/ml; p = 8×10(-6, 95% CI: 70-159 pg/ml. Interestingly, our microarray data show that increased expression of inflammatory genes was associated with major clinical features within the MFS patients group; namely severity of the aortic root dilatation (HLA-DRB1 and HLA-DRB5 genes; r = 0.56 for both; False Discovery Rate(FDR = 0%, ocular lens dislocation (RAET1L, CCL19 and HLA-DQB2; Fold Change (FC = 1.8; 1.4; 1.5, FDR = 0% and specific skeletal features (HLA-DRB1, HLA-DRB5, GZMK; FC = 8.8, 7.1, 1.3; FDR = 0%. Patients with progressive aortic disease had higher levels of Macrophage Colony Stimulating Factor (M-CSF in blood. When comparing MFS aortic root vessel wall with non-MFS aortic root, increased numbers of CD4+ T-cells were found in the media (p = 0.02 and increased number of CD8+ T-cells (p = 0.003 in the adventitia of the MFS patients. CONCLUSION/SIGNIFICANCE: In conclusion, our results imply a modifying role of inflammation in MFS. Inflammation might be a novel therapeutic target in these patients.

  15. Dilated Cardiomyopathy

    Science.gov (United States)

    ... Family history of dilated cardiomyopathy Inflammation of heart muscle from immune system disorders, such as lupus Neuromuscular disorders, such as muscular dystrophy Complications Complications from dilated cardiomyopathy include: Heart ...

  16. The influence of emotional stress on Doppler-derived aortic peak velocity in boxer dogs.

    Science.gov (United States)

    Pradelli, D; Quintavalla, C; Crosta, M C; Mazzoni, L; Oliveira, P; Scotti, L; Brambilla, P; Bussadori, C

    2014-01-01

    Subaortic stenosis (SAS) is a common congenital heart disease in Boxers. Doppler-derived aortic peak velocity (AoPV) is a diagnostic criterion for the disease. To investigate the influence of emotional stress during echocardiographic examination on AoPV in normal and SAS-affected Boxers. To evaluate the effects of aortic root diameters on AoPV in normal Boxers. DOGS: Two hundred and fifteen normal and 19 SAS-affected Boxers. The AoPV was recorded at the beginning of echocardiographic examination (T0), and when the emotional stress of the dog was assumed to decrease based on behavioral parameters and heart rate (T1). AoPV0-AoPV1 was calculated. In normal dogs, stroke volume index was calculated at T0 and T1. Aortic root diameters were measured and their relationship with AoPV and AoPV0-AoPV1 was evaluated. In normal dogs, AoPV was higher at T0 (median, 1.95 m/s; range, 1.60-2.50 m/s) than at T1 (median, 1.76 m/s; range, 1.40-2.20 m/s; P dogs, AoPV0 was higher than AoPV1 (P < .0001; reduction 7.3%). Aortic peak velocity was affected by emotional stress during echocardiographic examination both in SAS-affected and normal Boxers. In normal Boxers, aortic root size weakly affected AoPVs, but did not affect AoPV0-AoPV1. Stroke volume seems to play a major role in stress-related AoPV increases in normal Boxers. Emotional stress should be taken into account when screening for SAS in the Boxer breed. Copyright © 2014 by the American College of Veterinary Internal Medicine.

  17. [Arterial involvements in hereditary dysplasia of the connective tissue].

    Science.gov (United States)

    Beylot, C; Doutre, M S; Beylot-Barry, M; Busquet, M

    1994-03-01

    Arterial involvement is an important feature of the diagnosis and, above all, prognosis of heritable disorders of connective tissue. In pseudoxanthoma elasticum, a progressive occlusive syndrome is associated with hemorrhage and especially with gastrointestinal bleeding. Aneurysms are uncommon. Hypertension occurs frequently. Cutaneous signs (yellowish pseudo xanthomatous papules of the large folds) the ocular changes (angioid streaks) and pathology showing numerous, thickened, fragmented, disorganized, calcified elastic fibers in the deep dermis and arterial walls, allow the diagnosis to be made. In the heterogeneous group of Ehlers-Danlos syndromes, type IV is characterized by sudden spontaneous rupture of the large arteries. Aneurysms and carotido-cavernous fistulae are rather frequent. Owing to friability of the arterial walls, arteriograms and other procedure requiring arterial puncture may prove hazardous and surgery difficult. Such patients have an acrogeric morphotype, and thin, fragile skin, but cutaneous hyperelasticity and joint hyperlaxity are usually minimal. Pathology evidences collagen hypoplasia in the skin and arterial walls. The severity of Marfan syndrome is due to aortic involvement. A fusiform aneurysm of the ascending aorta represents a vital risk of rupture. Aortic root dilatation is associated and responsible of severe aortic regurgitation. Aortic dissection is also a serious threat. Improved surgical techniques for repairing a dilated or dissected aortic root with simultaneous replacement of the aortic valve increases the life expectancy of such patients. Dolichomorphism is the characteristic skeletal abnormality, particularly with arachnodactyly and upward ectopia lentis, which is almost bilateral, is a very frequent feature of Marfan syndrome. The most typical histological finding is aortic cystic median necrosis. The basic defect in Marfan syndrome concerns the fibrillin, whose gene is located on chromosome 15. The three diseases

  18. Acute myocardial ischemia after aortic valve replacement: A comprehensive diagnostic evaluation using dynamic multislice spiral computed tomography

    Energy Technology Data Exchange (ETDEWEB)

    Lembcke, Alexander [Department of Radiology, Charite-Universitaetsmedizin Berlin, Freie Universitaet Berlin and Humboldt-Universitaet zu Berlin, Berlin (Germany)]. E-mail: alexander.lembcke@gmx.de; Hein, Patrick A. [Department of Radiology, Charite-Universitaetsmedizin Berlin, Freie Universitaet Berlin and Humboldt-Universitaet zu Berlin, Berlin (Germany); Enzweiler, Christian N.H. [Department of Radiology, Charite-Universitaetsmedizin Berlin, Freie Universitaet Berlin and Humboldt-Universitaet zu Berlin, Berlin (Germany); Hoffmann, Udo [Department of Radiology, Massachusetts General Hospital and Harvard Medical School, Boston, MA (United States); Klessen, Christian [Department of Radiology, Charite-Universitaetsmedizin Berlin, Freie Universitaet Berlin and Humboldt-Universitaet zu Berlin, Berlin (Germany); Dohmen, Pascal M. [Department of Cardiovascular Surgery, Charite-Universitaetsmedizin Berlin, Freie Universitaet Berlin and Humboldt-Universitaet zu Berlin, Berlin (Germany)

    2006-03-15

    We describe the case of a 72-year-old man presenting with endocarditis and clinical signs of acute myocardial ischemia after biological aortic valve replacement. A comprehensive cardiac dynamic multislice spiral computed tomography demonstrated: (1) an endocarditic vegetation of the aortic valve; (2) a subvalvular leakage feeding a paravalvular pseudoaneurysm based on an aortic root abscess with subsequent compromise of the systolic blood flow in the left main coronary artery and the resulting myocardial perfusion deficit.

  19. Acute myocardial ischemia after aortic valve replacement: A comprehensive diagnostic evaluation using dynamic multislice spiral computed tomography

    International Nuclear Information System (INIS)

    Lembcke, Alexander; Hein, Patrick A.; Enzweiler, Christian N.H.; Hoffmann, Udo; Klessen, Christian; Dohmen, Pascal M.

    2006-01-01

    We describe the case of a 72-year-old man presenting with endocarditis and clinical signs of acute myocardial ischemia after biological aortic valve replacement. A comprehensive cardiac dynamic multislice spiral computed tomography demonstrated: (1) an endocarditic vegetation of the aortic valve; (2) a subvalvular leakage feeding a paravalvular pseudoaneurysm based on an aortic root abscess with subsequent compromise of the systolic blood flow in the left main coronary artery and the resulting myocardial perfusion deficit

  20. Dilations and interaction

    International Nuclear Information System (INIS)

    Buchholz, D.; Fredenhagen, K.

    1976-07-01

    As a consequence of the geometrical features of dilations massless particles do not interact in a local, dilationally invariant quantum theory. This result also holds in models in which dilations are only an asymptotically visible symmetry of the S-matrix. (orig.) [de

  1. Aortic valve-sparing in 37 patients with Marfan syndrome: midterm results with David operation.

    Science.gov (United States)

    Forteza, Alberto; De Diego, Javier; Centeno, Jorge; López, Maria Jesus; Pérez, Enrique; Martín, Carlos; Sánchez, Violeta; Rufilanchas, Juan J; Cortina, Jose

    2010-01-01

    We reviewed our experience with aortic valve-sparing operations in Marfan syndrome during last 5 years. Between March 2004 and June 2009, 94 patients with aortic root aneurysms underwent valve-sparing operations. Of these, 37 (68% male) were diagnosed with Marfan syndrome, according to the Ghent diagnostic criteria. Mean age was 30 +/- 10 years (range, 11 to 59 years). Moderate/severe aortic regurgitation was present in 13%, and the mean diameter of the Valsalva sinuses was 50 +/- 4 mm (range, 42 to 62 mm). The David V modification was performed in the last 28 patients. Additional procedures were mitral valve repair in 6, tricuspid valve repair in 3, closure of septal atrial defect in 2, and closure of a patent foramen ovale in 13. Mean follow-up was 27 +/- 16 months (range, 1 to 61 months). There were no in-hospital deaths and no major adverse outcomes. One patient required implantation of a mechanical prosthesis during the same procedure because of moderate aortic regurgitation. One late death occurred. No patients required reoperation. In the last follow-up, 23 patients did not have aortic regurgitation, 12 had grade I, and 1 had grade II. No thromboembolic complications have been documented, and 97% of the patients are free from anticoagulation. Short-term and midterm results with the reimplantation technique for aortic root aneurysms in Marfan patients are excellent. If long-term results are similar, this technique could be the treatment of choice for these patients. 2010 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  2. Finite element analysis of helical flows in human aortic arch: A novel index

    OpenAIRE

    Lee, Cheng-Hung; Liu, Kuo-Sheng; Jhong, Guan-Heng; Liu, Shih-Jung; Hsu, Ming-Yi; Wang, Chao-Jan; Hung, Kuo-Chun

    2014-01-01

    This study investigates the helical secondary flows in the aortic arch using finite element analysis. The relationship between helical flow and the configuration of the aorta in patients of whose three-dimensional images constructed from computed tomography scans was examined. A finite element model of the pressurized root, arch, and supra-aortic vessels was developed to simulate the pattern of helical secondary flows. Calculations indicate that most of the helical secondary flow was formed i...

  3. The Arteries Root from the Aortic Arch in the Sparrowhawk (Accipiter nisus, Falconiformes: Accipitridae)

    OpenAIRE

    Balkaya, Hulya

    2016-01-01

    This study was aimed at determining the morphology of the aortic arch in the sparrowhawk. For this purpose, arteries near the heart of six sparrowhawks were assessed. Latex injection method was applied to the three materials and barium sulphate solution was injected into the aorta for angiography in three other materials. It was observed that two major arteries arose from aortic arch in the sparrowhawk: the left brachiocephalic trunk and the right brachiocephalic trunk. These trunks were cont...

  4. Dimensional correlates of left ventricular dilation in the presence of hypertrophy.

    Science.gov (United States)

    Al-Nouri, M B; Ford, L E; Wix, H

    1983-01-01

    Twelve normal subjects, 50 patients with valvular heart disease, and 14 with hypertension were studied. Those with valvular disease were divided into two groups: 28 with angiographically measured ejection fractions greater than or equal to 0.6 and 22 with ejection fractions less than 0.6. The echocardiographically measured ventricular thickness divided by radius ratio (t/r) was approximately proportional to peak systolic pressure (P) in all groups having ejection fractions greater than or equal to 0.6, so that the t/r divided by P ratios were nearly the same. Patients with ejection fractions less than 0.6 had significantly lower t/r divided by P values. No single component of the t/r divided by P ratio would identify the patients with lower ejection fractions. The t/r divided by P ratios in 14 hypertensive patients were nearly identical to the ratios in six patients with aortic stenosis and ejection fractions greater than or equal to 0.6, indicating that an aortic valve gradient does not cause a grossly abnormal form of pressure hypertrophy. The t/r ratio is thus a double sensitive, noninvasive index of dilation when correlated with systolic pressure.

  5. Acute Aortic Arch Perforation During Transcatheter Aortic Valve Replacement in Bicuspid Aortic Stenosis and a Gothic Aortic Arch.

    Science.gov (United States)

    Millan-Iturbe, Oscar; Sawaya, Fadi J; Bieliauskas, Gintautas; Chow, Danny H F; De Backer, Ole; Søndergaard, Lars

    2017-09-01

    Transcatheter aortic valve replacement (TAVR) has evolved from a novel technology to an established therapy for high/intermediate-risk patients with severe symptomatic aortic stenosis (AS). Although TAVR is used to treat bicuspid severe AS, the large randomized trials typically excluded bicuspid AS because of its unique anatomic features. This case report describes an acute aortic perforation during delivery of a transcatheter heart valve to treat a severe bicuspid AS with a "gothic aortic arch"; more careful evaluation of the preprocedural multislice computed tomographic scan would have unveiled a sharply angulated aortic arch. This life-threatening complication was successfully treated by thoracic endovascular aortic repair. Copyright © 2017 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.

  6. Computational modeling of blood flow in the aorta--insights into eccentric dilatation of the ascending aorta after surgery for coarctation.

    Science.gov (United States)

    Szopos, Marcela; Poussineau, Nicole; Maday, Yvon; Canniffe, Carla; Celermajer, David S; Bonnet, Damien; Ou, Phalla

    2014-10-01

    To assess whether combining a computational modeling technique with data from patient magnetic resonance imaging studies can detect different fluid dynamics and vascular biomechanical properties of the ascending and horizontal aorta in patients with angulated "gothic" arch geometry compared with those with normal "Romanesque" arch geometry after aortic coarctation repair. Advanced computational fluid dynamics techniques (coupled Navier-Stokes and elastodynamics equations) were used to predict the fluid-wall interactions in large arteries. We modeled the fluid dynamics and shear stress in the ascending and horizontal aorta in cases of "gothic" arch and normal "Romanesque" aortic arch geometry. A total of 30 patients after aortic coarctation repair prospectively underwent 3-dimensional magnetic resonance imaging angiography of the thoracic aorta. Measurements of the ascending and horizontal aorta were assessed using multiplanar reformatting images. Our computational model demonstrated that wall shear stress is greater in those with an angulated "gothic" aortic arch than in those with a "Romanesque" arch. In particular, wall shear stress affected the anterior and posterior segments of the ascending aorta and the inferior and superior segments of the horizontal aorta (vs the left and right segments). In vivo, a "gothic" arch was associated with dilatation of the ascending and horizontal aorta, which was eccentric rather than concentric (Pgothic" aortic arch after repair of coarctation. This suggests that patients with an angulated "gothic" aortic arch might warrant increased surveillance for aortic complications. Copyright © 2014 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.

  7. Evidence-based Surgery of Aortic Regurgitation: Results of a Questionnaire in German-speaking Countries.

    Science.gov (United States)

    Dinges, Christian; Steindl, Johannes; Hitzl, Wolfgang; Kiesslich, Tobias; Seitelberger, Rainald

    2017-03-04

    Background  evidence-based medicine (EBM) approaches have reached broad acceptance, both in conservative and surgical disciplines. The aim of this study is to clarify the role of EBM in a rare condition of aortic regurgitation (AR) with surgical indication. Methods  A purpose-built Internet-based questionnaire was sent to 607 cardiovascular surgeons in Germany, Austria, and Switzerland. A virtual 64-year-old patient's medical history was presented, including two ultrasound images and one computed tomography scan, showing a 58-mm aortic root aneurysm and a severe trileaflet regurgitant aortic valve. Participants had to choose their preferred therapeutic strategy from a list. Additionally, demographics including nationality, the center size, and the frequency of similar types of patients referred to their departments were collected. Results  Of 607 questionnaires, 100 were returned (16%). One participant was excluded due to conflicting answers. Most surgeons ( n  = 84; 84%) chose a valve-sparing root replacement (VSRR). A Bentall procedure was preferred by 13 surgeons (13%). Two surgeons voted for aortic valve replacement combined with partial root resection. The decision-making process was not significantly influenced by center size, nationality, or frequency of patients. Conclusion  Applying the current guidelines to our virtual study patient, 84% of participants acted accordingly choosing VSRR. Remarkably, 14% of these surgeons see less than 10 and 43% see not more than 20 comparable patients per year. Since the guidelines reserve VSRR for competent centers, those numbers as well as the guidelines themselves should be further discussed. Georg Thieme Verlag KG Stuttgart · New York.

  8. Residual and Progressive Aortic Regurgitation After Valve-Sparing Root Replacement: A Propensity-Matched Multi-Institutional Analysis in 764 Patients.

    Science.gov (United States)

    Kari, Fabian A; Doll, Kai-Nicolas; Hemmer, Wolfgang; Liebrich, Markus; Sievers, Hans-Hinrich; Richardt, Doreen; Reichenspurner, Hermann; Detter, Christian; Siepe, Matthias; Czerny, Martin; Beyersdorf, Friedhelm

    2016-04-01

    Residual/progressive aortic regurgitation (rAR, pAR) after valve-sparing aortic root replacement (V-SARR) can lead to reoperations. We sought to characterize risk factors of mild rAR and pAR after V-SARR in a multicenter cohort. The effect of additional cusp repair on valve function was analyzed using propensity matching. A total of 1,015 patients after V-SARR were identified with (n = 288, 28%) or without additional cusp/commissure repair (n = 727, 72%) at four cardiac units in Germany. A total of 764 patients fulfilling transthoracic echocardiography follow-up-criteria comprised the study cohort. Logistic regression was used for risk factor analysis with endpoints rAR, new onset AR, and pAR. t tests and analyses of variance were used for between-group differences. The effects of additional cusp repair on valve function were studied comparing propensity-matched quintiles. The incidence of rAR was 29%, with influencing factors aneurysm size (p = 0.07) and preoperative aortic valve function (p = 0.08). It was found more often among nonsyndromic patients (34% vs. 14%; OR, 0.4; p < 0.001). Progression of rAR was detectable in 30% after a mean of 4.3 years. The progression rate of rAR ∼ 0.3 grades per patient-year within the first 5 years. When quintiles identified by propensity score were compared, additional cusp repair was linked to new onset AR (p = 0.016) while it was not linked to rAR (p = 0.14) or pAR (p = 0.5). The incidences of rAR and pAR are considerable after V-SARR. Patients should be operated on before large aneurysms are present. New onset AR after an initially good functional result is more likely after an additional cusp repair, while rAR and pAR are not influenced by cusp repair. Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  9. Identification of Reference Genes for Quantitative Real Time PCR Assays in Aortic Tissue of Syrian Hamsters with Bicuspid Aortic Valve.

    Science.gov (United States)

    Rueda-Martínez, Carmen; Fernández, M Carmen; Soto-Navarrete, María Teresa; Jiménez-Navarro, Manuel; Durán, Ana Carmen; Fernández, Borja

    2016-01-01

    Bicuspid aortic valve (BAV) is the most frequent congenital cardiac malformation in humans, and appears frequently associated with dilatation of the ascending aorta. This association is likely the result of a common aetiology. Currently, a Syrian hamster strain with a relatively high (∼40%) incidence of BAV constitutes the only spontaneous animal model of BAV disease. The characterization of molecular alterations in the aorta of hamsters with BAV may serve to identify pathophysiological mechanisms and molecular markers of disease in humans. In this report, we evaluate the expression of ten candidate reference genes in aortic tissue of hamsters in order to identify housekeeping genes for normalization using quantitative real time PCR (RT-qPCR) assays. A total of 51 adult (180-240 days old) and 56 old (300-440 days old) animals were used. They belonged to a control strain of hamsters with normal, tricuspid aortic valve (TAV; n = 30), or to the affected strain of hamsters with TAV (n = 45) or BAV (n = 32). The expression stability of the candidate reference genes was determined by RT-qPCR using three statistical algorithms, GeNorm, NormFinder and Bestkeeper. The expression analyses showed that the most stable reference genes for the three algorithms employed were Cdkn1β, G3pdh and Polr2a. We propose the use of Cdkn1β, or both Cdkn1β and G3pdh as reference genes for mRNA expression analyses in Syrian hamster aorta.

  10. Identification of Reference Genes for Quantitative Real Time PCR Assays in Aortic Tissue of Syrian Hamsters with Bicuspid Aortic Valve.

    Directory of Open Access Journals (Sweden)

    Carmen Rueda-Martínez

    Full Text Available Bicuspid aortic valve (BAV is the most frequent congenital cardiac malformation in humans, and appears frequently associated with dilatation of the ascending aorta. This association is likely the result of a common aetiology. Currently, a Syrian hamster strain with a relatively high (∼40% incidence of BAV constitutes the only spontaneous animal model of BAV disease. The characterization of molecular alterations in the aorta of hamsters with BAV may serve to identify pathophysiological mechanisms and molecular markers of disease in humans. In this report, we evaluate the expression of ten candidate reference genes in aortic tissue of hamsters in order to identify housekeeping genes for normalization using quantitative real time PCR (RT-qPCR assays. A total of 51 adult (180-240 days old and 56 old (300-440 days old animals were used. They belonged to a control strain of hamsters with normal, tricuspid aortic valve (TAV; n = 30, or to the affected strain of hamsters with TAV (n = 45 or BAV (n = 32. The expression stability of the candidate reference genes was determined by RT-qPCR using three statistical algorithms, GeNorm, NormFinder and Bestkeeper. The expression analyses showed that the most stable reference genes for the three algorithms employed were Cdkn1β, G3pdh and Polr2a. We propose the use of Cdkn1β, or both Cdkn1β and G3pdh as reference genes for mRNA expression analyses in Syrian hamster aorta.

  11. Mid-term results of different aortic valve-sparing procedures in Marfan syndrome.

    Science.gov (United States)

    Schmidtke, Claudia; Karluss, Antje; Sier, Holger; Hüppe, Michael; Brauer, Kirk; Sievers, Hans-H

    2012-03-01

    Marfan patients with aortic root aneurysm are typically treated with the Bentall procedure, though aortic valve-sparing procedures (AVSPs) are also possible. The study aim was to compare the authors' experience with two such techniques performed at their institution, namely a reimplantation according to David (David I) and remodeling according to Yacoub. Between 1996 and 2009, a total of 37 Marfan patients underwent an AVSP at the authors' institution. Of these patients, 25 (mean age 32 +/- 14.9 years) underwent surgery according to David (group D), and 12 (mean age 35 +/- 10.9 years) according to Yacoub (group Y). The patients underwent both clinical and echocardiographic follow up examinations at a mean of 42.0 +/- 36.4 months after surgery. One patient from each group had moved abroad and was lost to follow up. The remaining 35 patients were alive at follow up, and none presented with any major neurological or bleeding complications. In addition, no significant differences were noted between the groups in terms of NYHA classification, left ventricular function, or left ventricular diameter. At follow up, aortic valve function was also comparable between groups, with a peak/mean gradient of 9.4 +/- 6.4/5.3 +/- 3.5 mmHg and 5.1 +/- 3.3/2.8 +/- 1.5 mmHg for groups D and Y, respectively (p = 0.081/0.058). The measured mean grades of aortic valve regurgitation were comparable in groups D and Y (0.6 +/- 0.7 and 1.1 +/- 0.6, respectively; p = 0.055). However, aortic root dimensions obtained via M-mode were smaller in group D patients (29.6 +/- 2.3 mm) than in group Y patients (36.1 +/- 6.6 mm) (p = 0.027). Only three patients from group Y required reoperation on the aortic valve due to valvular regurgitation (p = 0.028); two of these had presented with aortic dissection at the first operation. Both types of AVSP can be performed with comparably good interim clinical results, and also low mortality and morbidity, in patients with Marfan syndrome.

  12. Computed tomography-based anatomic characterization of proximal aortic dissection with consideration for endovascular candidacy.

    Science.gov (United States)

    Moon, Michael C; Greenberg, Roy K; Morales, Jose P; Martin, Zenia; Lu, Qingsheng; Dowdall, Joseph F; Hernandez, Adrian V

    2011-04-01

    Proximal aortic dissections are life-threatening conditions that require immediate surgical intervention to avert an untreated mortality rate that approaches 50% at 48 hours. Advances in computed tomography (CT) imaging techniques have permitted increased characterization of aortic dissection that are necessary to assess the design and applicability of new treatment paradigms. All patients presenting during a 2-year period with acute proximal aortic dissections who underwent CT scanning were reviewed in an effort to establish a detailed assessment of their aortic anatomy. Imaging studies were assessed in an effort to document the location of the primary proximal fenestration, the proximal and distal extent of the dissection, and numerous morphologic measurements pertaining to the aortic valve, root, and ascending aorta to determine the potential for an endovascular exclusion of the ascending aorta. During the study period, 162 patients presented with proximal aortic dissections. Digital high-resolution preoperative CT imaging was performed on 76 patients, and 59 scans (77%) were of adequate quality to allow assessment of anatomic suitability for treatment with an endograft. In all cases, the dissection plane was detectable, yet the primary intimal fenestration was identified in only 41% of the studies. Scans showed 24 patients (32%) appeared to be anatomically amenable to such a repair (absence of valvular involvement, appropriate length and diameter of proximal sealing regions, lack of need to occlude coronary vasculature). Of the 42 scans that were determined not to be favorable for endovascular repair, the most common exclusion finding was the absence of a proximal landing zone (n = 15; 36%). Appropriately protocoled CT imaging provides detailed anatomic information about the aortic root and ascending aorta, allowing the assessment of which dissections have proximal fenestrations that may be amenable to an endovascular repair. Copyright © 2011 Society for

  13. Magnetic resonance imaging 4-D flow-based analysis of aortic hemodynamics in Turner syndrome

    International Nuclear Information System (INIS)

    Arnold, Raoul; Neu, Marie; Hirtler, Daniel; Gimpel, Charlotte; Markl, Michael; Geiger, Julia

    2017-01-01

    Cardiovascular surveillance is important in Turner syndrome because of the increased risk of aortic dilation and dissection with consecutively increased mortality. To compare 4-D flow MRI for the characterization of aortic 3-D flow patterns, dimensions and vessel wall parameters in pediatric patients with Turner syndrome and age-matched controls. We performed 4-D flow MRI measuring in vivo 3-D blood flow with coverage of the thoracic aorta in 25 patients with Turner syndrome and in 16 female healthy controls (age mean ± standard deviation were 16 ± 5 years and 17 ± 4 years, respectively). Blood flow was visualized by time-resolved 3-D path lines. Visual grading of aortic flow in terms of helices and vortices was performed by two independent observers. Quantitative analysis included measurement of aortic diameters, quantification of peak systolic wall shear stress, pulsatility index and oscillatory shear index at eight defined sites. Patients with Turner syndrome had significantly larger aortic diameters normalized to BSA, increased vortices in the ascending aorta and elevated helix flow in the ascending and descending aorta compared to controls (all P<0.03). Patients with abnormal helical or vortical flow in the ascending aorta had significantly larger diameters of the ascending aorta (P<0.03). Peak systolic wall shear stress, pulsatility index and oscillatory shear index were significantly lower in Turner patients compared to controls (p=0.02, p=0.002 and p=0.01 respectively). Four-dimensional flow MRI provides new insights into the altered aortic hemodynamics and wall shear stress that could have an impact on the development of aortic dissections. (orig.)

  14. Magnetic resonance imaging 4-D flow-based analysis of aortic hemodynamics in Turner syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Arnold, Raoul [University Medical Center Heidelberg, Department of Congenital Heart Disease and Pediatric Cardiology, Heidelberg (Germany); Neu, Marie [University Medical Center, Department of Pediatric Hematology/Oncology/Hemostaseology, Mainz (Germany); Hirtler, Daniel [University of Freiburg, Department of Congenital Heart Defects and Pediatric Cardiology, Heart Center, Freiburg im Breisgau (Germany); Gimpel, Charlotte [Center for Pediatrics, Medical Center - University of Freiburg, Department of General Pediatrics, Adolescent Medicine and Neonatology, Freiburg im Breisgau (Germany); Markl, Michael [Northwestern University, Department of Radiology, Feinberg School of Medicine, Chicago, IL (United States); Northwestern University, Department of Biomedical Engineering, McCormick School of Engineering, Chicago, IL (United States); Geiger, Julia [Northwestern University, Department of Radiology, Feinberg School of Medicine, Chicago, IL (United States); University Children' s Hospital, Department of Radiology, Zuerich (Switzerland)

    2017-04-15

    Cardiovascular surveillance is important in Turner syndrome because of the increased risk of aortic dilation and dissection with consecutively increased mortality. To compare 4-D flow MRI for the characterization of aortic 3-D flow patterns, dimensions and vessel wall parameters in pediatric patients with Turner syndrome and age-matched controls. We performed 4-D flow MRI measuring in vivo 3-D blood flow with coverage of the thoracic aorta in 25 patients with Turner syndrome and in 16 female healthy controls (age mean ± standard deviation were 16 ± 5 years and 17 ± 4 years, respectively). Blood flow was visualized by time-resolved 3-D path lines. Visual grading of aortic flow in terms of helices and vortices was performed by two independent observers. Quantitative analysis included measurement of aortic diameters, quantification of peak systolic wall shear stress, pulsatility index and oscillatory shear index at eight defined sites. Patients with Turner syndrome had significantly larger aortic diameters normalized to BSA, increased vortices in the ascending aorta and elevated helix flow in the ascending and descending aorta compared to controls (all P<0.03). Patients with abnormal helical or vortical flow in the ascending aorta had significantly larger diameters of the ascending aorta (P<0.03). Peak systolic wall shear stress, pulsatility index and oscillatory shear index were significantly lower in Turner patients compared to controls (p=0.02, p=0.002 and p=0.01 respectively). Four-dimensional flow MRI provides new insights into the altered aortic hemodynamics and wall shear stress that could have an impact on the development of aortic dissections. (orig.)

  15. Echocaridography, electrocardiography, and radiography of cats with dilatation cardiomyopathy, hypertrophic cardiomyopathy, and hyperyroidism

    International Nuclear Information System (INIS)

    Moise, N.S.; Dietze, A.E.; Mezza, L.E.; Strickland, D.; Erb, H.N.; Edwards, N.J.

    1986-01-01

    The echocardiographic, ECG, and radiographic findings of sequentially examined cats with dilatation cardiomyopathy (DCM, n = 7), hypertrophic cardiomyopathy (HCM, n = 8), and hyperthyroidism (HT, n = 20) were compared with those of healthy control cats (n = 11). Cats with DCM were easily differentiated from healthy cats by echocardiography and from cats with HCM and HT by a dilated left ventricle at end-diastole with a mean +/- SD of 2.20 +/- 0.36 cm, reduced fractional shortening (2.9% +/- 3.7%), reduced aortic amplitude (0.07 +/- 0.05 cm), reduced left ventricular wall amplitude (0.09 +/- 0.09 cm), and increased E-point septal separation (0.83 +/- 0.29 cm). The cats with HCM were most consistently recognized echocardiographically by increased left ventricular wall thickness at end-diastole (0.75 +/- 0.12 cm). Some cats with HT had abnormal echocardiograms with left ventricular wall hypertrophy. These cats could usually be differentiated from the cats with HCM because of normal or increased ventricular wall amplitude, aortic amplitude, or percentage of thickening of the left ventricular wall and interventricular septum. Left atrial enlargement (left atrial diameter greater than 1.57 cm or left atrium/aorta greater than 1.75) was commonly detected by the echocardiogram in cats with DCM, HCM, or HT. The echocardiogram was helpful in differentiating the type of cardiomyopathy (DCM, HCM, or HT) when plain thoracic radiographs indicated that cardiomegaly existed. The ECG may have indicated incorrectly that there was left ventricular enlargement in some cats with HT, and it did not indicate consistently that left ventricular enlargement existed when present in cats with DCM or HCM. The ECG was a poor indicator of left atrial enlargement in all cats

  16. Vortex dynamics in Patient-Specific Stenotic Tricuspid and Bicuspid Aortic Valves pre- and post- Trans-catheter Aortic Valve Replacement

    Science.gov (United States)

    Hatoum, Hoda; Dasi, Lakshmi Prasad

    2017-11-01

    Understanding blood flow related adverse complications such as leaflet thrombosis post-transcatheter aortic valve implantation (TAVI) requires a deeper understanding of how patient-specific anatomic and hemodynamic factors, and relative valve positioning dictate sinus vortex flow and stasis regions. High resolution time-resolved particle image velocimetry measurements were conducted in compliant and transparent 3D printed patient-specific models of stenotic bicuspid and tricuspid aortic valve roots from patients who underwent TAVI. Using Lagrangian particle tracking analysis of sinus vortex flows and probability distributions of residence time and blood damage indices we show that (a) patient specific modeling provides a more realistic assessment of TAVI flows, (b) TAVI deployment alters sinus flow patterns by significantly decreasing sinus velocity and vorticity, and (c) relative valve positioning can control critical vortex structures that may explain preferential leaflet thrombosis corresponding to separated flow recirculation, secondary to valve jet vectoring relative to the aorta axis. This work provides new methods and understanding of the spatio-temporal aortic sinus vortex dynamics in post TAVI pathology. This study was supported by the Ohio State University DHLRI Trifit Challenge award.

  17. Stent-assisted, balloon-induced intimal disruption and relamination of aortic dissection in patients with Marfan syndrome: Midterm outcomes and aortic remodeling.

    Science.gov (United States)

    Faure, Elsa Madeleine; El Batti, Salma; Abou Rjeili, Marwan; Ben Abdallah, Iannis; Julia, Pierre; Alsac, Jean-Marc

    2018-05-17

    The study objective was to assess the midterm outcomes and aortic remodeling in patients with Marfan syndrome with complicated acute type B aortic dissection treated with stent-assisted, balloon-induced intimal disruption and relamination. We reviewed all patients treated with stent-assisted, balloon-induced intimal disruption and relamination for a complicated acute type B aortic dissection associated with Marfan syndrome according to the revised Ghent criteria. Between 2015 and November 2017, 7 patients with Marfan syndrome underwent stent-assisted, balloon-induced intimal disruption and relamination for a complicated acute type B aortic dissection. The median age of patients was 47 years (range, 23-70). Four patients had a history of aortic root replacement. Technical success was achieved in 100%. Three patients required an adjunctive procedure for renal artery stenting (n = 2) and iliac artery stenting (n = 1). There was no in-hospital death, 30-day postoperative stroke, spinal cord ischemia, ischemic colitis, or renal failure requiring dialysis. At a median follow-up of 15 months (range, 7-28), 1 patient required aortic arch replacement for aneurysmal degeneration associated with a type Ia endoleak at 2 years, giving a late reintervention rate of 14%. There was no other secondary endoleak. The primary visceral patency rate was 100%. There were no all-cause deaths reported. At last computed tomography scan, all patients had complete aortic remodeling of the treated thoracoabdominal aorta. Distally, at the nonstented infrarenal aortoiliac level, 6 patients had persistent false lumen flow with stable aorto-iliac diameter in 5. One patient had iliac diameter growth (27 mm diameter at last computed tomography scan). Stent-assisted, balloon-induced intimal disruption and relamination of aortic dissection in patients with Marfan syndrome is feasible, safe, and associated with an immediate and midterm persisting thoracoabdominal aortic remodeling. Copyright

  18. Outcomes of Reintervention on the Autograft After Ross Procedure.

    Science.gov (United States)

    Kumar, S Ram; Bansal, Neeraj; Wells, Winfield J; Starnes, Vaughn A

    2016-11-01

    After a Ross procedure, a small subset of patients requires reintervention for autograft dilatation or valve insufficiency. We sought to determine the indications, nature, and outcomes of autograft reinterventions in the left ventricular outflow tract after a Ross procedure. We retrospectively reviewed the charts of 316 consecutive patients, age 4 days to 70 years, who underwent a Ross procedure at our institution. Of these, 47 patients (15%) required autograft reintervention during a median follow-up of 8.2 years. Forty-seven patients, median age 26 years (interquartile range [IQR], 14.4 to 44.8 years), 32 (68%) men, required autograft reintervention a median 5.5 years (IQR, 1.8 to 9.4 years) after a Ross procedure. In 47% (22 of 47 patients), the autograft valve could be salvaged (15 valve-sparing aortic replacements, 7 valve repairs). The remaining 53% underwent replacement of the root (10 mechanical, 5 homograft) or valve alone (7 mechanical, 3 bioprosthetic). Twenty patients presented without autograft root dilatation 1 year (IQR, 0.5 to 2.9 years) after Ross. Fifteen of them (75%) required valve replacement. The 27 patients who demonstrated root dilatation presented 6.9 years (IQR, 4.5 to 9.7 years; p Ross, and 17 (63%) of these valves could be spared (p = 0.01). There was no surgical mortality and 5 (11%) major morbidity events. Patients were followed up for 4.9 years (IQR, 2.1 to 7 years) after left ventricular outflow tract reintervention. For patients whose autograft valve could be spared, 3-, 5-, and 8-year freedom from valve replacement was 92%, 86%, and 86%, respectively. At last follow-up, only 1 patient had greater than mild aortic insufficiency, and all but 1 had normal ejection fraction. Autograft reintervention after a Ross appears to follow a bimodal distribution. Patients with primary autograft leaflet problems tend to present early without root dilatation and frequently require valve replacement. The autograft valve can be salvaged in the

  19. Re-interventions on the thoracic and thoracoabdominal aorta in patients with Marfan syndrome.

    Science.gov (United States)

    Schoenhoff, Florian S; Carrel, Thierry P

    2017-11-01

    The advent of multi-gene panel genetic testing and the discovery of new syndromic and non-syndromic forms of connective tissue disorders have established thoracic aortic aneurysms as a genetically mediated disease. Surgical results in patients with Marfan syndrome (MFS) provide an important benchmark for this patient population. Prophylactic aortic root surgery prevents acute dissection and has contributed to the improved survival of MFS patients. In the majority of patients, re-interventions are driven by a history of dissection. Patients undergoing elective root repair have a low risk for re-interventions on the root itself. Experienced centers have results after valve-sparing procedures at 10 years comparable with those seen after a modified Bentall procedure. In patients where only the ascending aorta was replaced during the initial surgery, re-intervention rates are high as the root continues to dilate. The fate of the aortic arch in MFS patients presenting with dissection is strongly correlated with the extent of the initial surgery. Not replacing the entire ascending aorta and proximal aortic arch results in a high rate of re-interventions. Nevertheless, the additional burden of replacing the entire aortic arch during emergent proximal repair is not very well defined and makes comparisons with patients undergoing elective arch replacement difficult. Interestingly, replacing the entire aortic arch during initial surgery for acute dissection does not protect from re-interventions on downstream aortic segments. MFS patients suffering from type B dissection have a high risk for re-interventions ultimately leading up to replacement of the entire thoracoabdominal aorta even if the dissection was deemed uncomplicated by conventional criteria. While current guidelines do not recommend the implantation of stent grafts in MFS patients, implantation of a frozen-elephant-trunk to create a stable proximal landing zone for future endovascular or open procedures has

  20. The outcome after aortic valve-sparing (David) operation in 179 patients: a single-centre experience.

    Science.gov (United States)

    Leontyev, Sergey; Trommer, Constanze; Subramanian, Sreekumar; Lehmann, Sven; Dmitrieva, Yaroslava; Misfeld, Martin; Mohr, Friedrich W; Borger, Michael A

    2012-08-01

    The David aortic valve-sparing reimplantation (AVr-D) operation is increasingly being used in patients with aortic root aneurysmal disease and pliable aortic cusps. The objective of this study was to assess our early and medium-term outcomes with the AVr-D operation. Between 2003 and 2011, a total of 179 patients underwent AVr-D procedures. The mean patient age was 49.7 ± 15.1 years, and 23.5% (n = 42) were females. Marfan syndrome was present in 17.3% of patients (n = 31), and acute Type A aortic dissection in 15.6% (n = 28). Clinical follow-up was 100% complete and was 1.8 ± 1.6 years (0 days to 7.5 years) long. Echocardiographic follow-up was performed 2.2 ± 1.5 years (0 days to 7.5 years) postoperatively and was 77% complete. Early mortality was 1.1% (n = 2), with both deaths occurring in patients with Type A dissection. Pre-discharge echocardiography revealed no patients with >2+ aortic insufficiency (AI), 19.6% of patients (n = 34) with 1+ or 2+ AI and 80.4% of patients (n = 145) with trace or no AI. Left ventricular end-diastolic diameters decreased significantly from 5.6 ± 0.9 to 5.1 ± 0.8 cm early postoperatively (P valve re-replacement during follow-up, two due to early endocarditis and two due to non-coronary leaflet prolapse in Marfan patients. Five-year freedom from aortic valve reoperation was 95.9 ± 2.0%. AVr-D is associated with a low mortality and morbidity rate, even in patients with Type A aortic dissection. Although a slightly higher rate of recurrent AI may be present in patients with Marfan syndrome, freedom from recurrent AI and reoperation remains excellent during medium-term follow-up. The David operation should be considered the gold standard for patients with proximal aortic root pathology (aneurysm or dissection) and pliable aortic cusps.

  1. Colombian experience with transcatheter aortic valve implantation of medtronic CoreValve.

    Science.gov (United States)

    Dager, Antonio E; Nuis, Rutger-Jan; Caicedo, Bernardo; Fonseca, Jaime A; Arana, Camilo; Cruz, Lidsa; Benitez, Luis M; Nader, Carlos A; Duenas, Eduardo; de Marchena, Eduardo J; O'Neill, William W; de Jaegere, Peter P

    2012-01-01

    At our institutions, increasing numbers of aortic stenosis patients were not candidates for surgical aortic valve replacement. Accordingly, we initiated the Cali Colombian Transcatheter Aortic Valve Implantation (TAVI) program. From March 2008 through January 2011, 53 consecutive patients (mean age, 79 ± 6 yr; men, 58%) underwent TAVI with the Medtronic CoreValve System, and data were prospectively collected. Our study's endpoints conformed with Valve Academic Research Consortium recommendations. We report our clinical results.Predicted mortality rates were 25% (interquartile range, 17%-34%) according to logistic EuroSCORE and 6% (interquartile range, 3%-8%) according to the Society of Thoracic Surgeons score. The 30-day mortality rate was 9% (3 intraprocedural deaths, 5 total). The combined 30-day safety endpoint was 30% (major vascular sequelae, 23%; life-threatening bleeding, 12%; myocardial infarction, 4%; major stroke, 4%; and acute kidney injury [stage 3], 2%). Eight patients (15%) required post-implantation balloon dilation and 2 (4%) required valve-in-valve implantation, for a technical device success rate of 77%. Mean peak transvalvular gradient decreased from 74 ± 29 to 17 ± 8 mmHg and mean transvalvular gradient from 40 ± 17 to 8 ± 4 mmHg (both P=0.001). Moderate or severe aortic regurgitation decreased from 32% to 18% (P=0.12) and mitral regurgitation from 32% to 13% (P=0.002). The 1-year survival rate was 81%.We found that TAVI with the CoreValve prosthesis was safe and feasible, with sustained long-term results, for treating aortic stenosis in patients at excessive surgical risk; nonetheless, serious adverse events occurred in 30% of the patients.

  2. Early results after implantation of a new geometric annuloplasty ring for aortic valve repair.

    Science.gov (United States)

    Mazzitelli, Domenico; Nöbauer, Christian; Rankin, J Scott; Badiu, Catalin C; Krane, Markus; Crooke, Philip S; Cohn, William E; Opitz, Anke; Schreiber, Christian; Lange, Rüdiger

    2013-01-01

    Aortic valve repair is associated with fewer long-term valve-related complications as compared with valve replacement, and repair is being performed increasingly. A current problem is the lack of a geometric annuloplasty ring to facilitate reconstruction. This paper describes the first clinical application of such a device designed to permanently restore physiologic annular size and geometry during aortic valve repair. Based on mathematical studies of human cadaver valves, as well as computed tomography angiographic analyses of awake patients with normal valves, a three-dimensional annuloplasty ring has been developed, consisting of low-profile, one-piece titanium construction and Dacron cloth covering. The ring design incorporates 2:3 elliptical base geometry and 10-degree outwardly flaring subcommissural posts. Appropriately sized rings were implanted in 5 patients with severe aortic insufficiency due to annular dilation and anatomic leaflet defects. The rings restored annular geometry and facilitated leaflet repairs in all patients. Each recovered excellent valve function with minimal residual leak. All patients convalesced uneventfully, were discharged within 7 days after surgery, and continue with stable valve function as long as 6 months after implantation. Initial clinical application of a geometric aortic annuloplasty ring was associated with excellent device performance and perhaps better repairs. Further clinical series and patient follow-up should identify potential benefits of the device, including improved applicability and stability of aortic valve repair. Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  3. Valve-sparing root replacement for freestanding pulmonary autograft aneurysm after the Ross procedure.

    Science.gov (United States)

    Ratschiller, Thomas; Eva, Sames-Dolzer; Schimetta, Wolfgang; Paulus, Patrick; Müller, Hannes; Zierer, Andreas; Mair, Rudolf

    2018-02-20

    Autograft dilatation is the main long-term complication following the Ross procedure using the freestanding root replacement technique. We reviewed our 25-year experience with the Ross procedure with a special emphasis on valve-sparing reoperations. From 1991 to 2016, 153 patients (29.6 ± 16.6 years; 29.4% pediatric) underwent a Ross operation at our institution with implantation of the autograft as freestanding root replacement. The follow-up is 98.7% complete with a mean of 12.2 ± 5.5 years. Mortality at 30-days was 2.0%. Echocardiography documented no or trivial aortic regurgitation in 99.3% of the patients at discharge. Survival probability at 20 years was 85.4%. No case of autograft endocarditis occurred. Autograft deterioration rate was 2.01% per patient-year, and freedom from autograft reoperation was 75.3% at 15 years. A reoperation for autograft aneurysm was required in 35 patients (22.9%) at a mean interval of 11.1 ± 4.6 years after the Ross procedure. A valve-sparing root replacement was performed in 77% of patients, including 10 David and 17 Yacoub procedures with no early mortality. Three patients required prosthetic valve replacement within 2 years after a Yacoub operation. At latest follow-up, 92% of all surviving patients still carry the pulmonary autograft valve. Freedom from autograft valve replacement was 92.1% at 15 years. Using the David or Yacoub techniques, the autograft valve can be preserved in the majority of patients with root aneurysms after the Ross procedure. Reoperations can be performed with no early mortality, a good functional midterm result, and an acceptable reintervention rate. Copyright © 2018 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.

  4. Three-dimensional finite volume modelling of blood flow in simulated angular neck abdominal aortic aneurysm

    Science.gov (United States)

    Algabri, Y. A.; Rookkapan, S.; Chatpun, S.

    2017-09-01

    An abdominal aortic aneurysm (AAA) is considered a deadly cardiovascular disease that defined as a focal dilation of blood artery. The healthy aorta size is between 15 and 24 mm based on gender, bodyweight, and age. When the diameter increased to 30 mm or more, the rupture can occur if it is kept growing or untreated. Moreover, the proximal angular neck of aneurysm is categorized as a significant morphological feature with prime harmful effects on endovascular aneurysm repair (EVAR). Flow pattern in pathological vessel can influence the vascular intervention. The aim of this study is to investigate the blood flow behaviours in angular neck abdominal aortic aneurysm with simulated geometry based on patient’s information using computational fluid dynamics (CFD). The 3D angular neck AAA models have been designed by using SolidWorks Software. Consequently, CFD tools are used for simulating these 3D models of angular neck AAA in ANSYS FLUENT Software. Eventually, based on the results, we summarized that the CFD techniques have shown high performance in explaining and investigating the flow patterns for angular neck abdominal aortic aneurysm.

  5. Midterm Results of Aortic Valve Replacement with Cryopreserved Homografts

    Directory of Open Access Journals (Sweden)

    Emre Özker

    2012-06-01

    Full Text Available Objective: The aim of this study was to analyze the midterm clinical results of aortic valve replacement with cryopreserved homografts.Materials and Methods: Aortic valve replacement was performed in 40 patients with cryopreserved homograft. The indications were aortic valve endocarditis in 20 patients (50%, truncus arteriosus in 6 patients (15%, and re-stenosis or regurtitation after aortic valve reconstruction in 14 (35% patients. The valve sizes ranged from 10 to 27mm. A full root replacement technique was used for homograft replacement in all patients.Results: The 30-day postoperative mortality rate was 12.5% (5 patients. There were four late deaths. Only one of them was related to cardiac events. Overall mortality was 22.5%. Thirty-three patients were followed up for 67±26 months. Two patients needed reoperation due to aortic aneurysm caused by endocarditis. The mean transvalvular gradient significantly decreased after valve replacement (p<0.003. The last follow up showed that the 27 (82% patients had a normal left ventricular function.Conclusion: Cryopreserved homografts are safe alternatives to mechanical valves that can be used when there are proper indications. Although it has a high perioperative mortality rate, cryopreserved homograft implantation is an alternative for valve replacement, particularly in younger patients and for complex surgical problems such as endocarditis that must be minimalized.

  6. Aortic insufficiency

    Science.gov (United States)

    ... page, please enable JavaScript. Aortic insufficiency is a heart valve disease in which the aortic valve does not close ... aortic insufficiency Images Aortic insufficiency References Carabello BA. Valvular heart disease. In: Goldman L, Schafer AI, eds. Goldman-Cecil ...

  7. Dilation and Curettage (D&C)

    Science.gov (United States)

    ... For Patients About ACOG Dilation and Curettage (D&C) Home For Patients Search FAQs Dilation and Curettage ( ... February 2016 PDF Format Dilation and Curettage (D&C) Special Procedures What is dilation and curettage (D& ...

  8. Overexpression of Catalase in Vascular Smooth Muscle Cells Prevents the Formation of Abdominal Aortic Aneurysms

    Science.gov (United States)

    Parastatidis, Ioannis; Weiss, Daiana; Joseph, Giji; Taylor, W Robert

    2013-01-01

    Objective Elevated levels of oxidative stress have been reported in abdominal aortic aneurysms (AAA), but which reactive oxygen species (ROS) promotes the development of AAA remains unclear. Here we investigate the effect of the hydrogen peroxide (H2O2) degrading enzyme catalase on the formation of AAA. Approach and Results AAA were induced with the application of calcium chloride (CaCl2) on mouse infrarenal aortas. The administration of PEG-catalase, but not saline, attenuated the loss of tunica media and protected against AAA formation (0.91±0.1 mm vs. 0.76±0.09 mm). Similarly, in a transgenic mouse model, catalase over-expression in the vascular smooth muscle cells (VSMC) preserved the thickness of tunica media and inhibited aortic dilatation by 50% (0.85±0.14 mm vs. 0.57±0.08 mm). Further studies showed that injury with CaCl2 decreased catalase expression and activity in the aortic wall. Pharmacologic administration or genetic over-expression of catalase restored catalase activity and subsequently decreased matrix metalloproteinase activity. In addition, a profound reduction in inflammatory markers and VSMC apoptosis was evident in aortas of catalase over-expressing mice. Interestingly, as opposed to infusion of PEG-catalase, chronic over-expression of catalase in VSMC did not alter the total aortic H2O2 levels. Conclusions The data suggest that a reduction in aortic wall catalase activity can predispose to AAA formation. Restoration of catalase activity in the vascular wall enhances aortic VSMC survival and prevents AAA formation primarily through modulation of matrix metalloproteinase activity. PMID:23950141

  9. Impaired Central Pulsatile Hemodynamics in Children and Adolescents With Marfan Syndrome.

    Science.gov (United States)

    Grillo, Andrea; Salvi, Paolo; Marelli, Susan; Gao, Lan; Salvi, Lucia; Faini, Andrea; Trifirò, Giuliana; Carretta, Renzo; Pini, Alessandro; Parati, Gianfranco

    2017-11-07

    Marfan syndrome is characterized by aortic root dilation, beginning in childhood. Data about aortic pulsatile hemodynamics and stiffness in pediatric age are currently lacking. In 51 young patients with Marfan syndrome (12.0±3.3 years), carotid tonometry was performed for the measurement of central pulse pressure, pulse pressure amplification, and aortic stiffness (carotid-femoral pulse wave velocity). Patients underwent an echocardiogram at baseline and at 1 year follow-up and a genetic evaluation. Pathogenetic fibrillin-1 mutations were classified between "dominant negative" and "haploinsufficient." The hemodynamic parameters of patients were compared with those of 80 sex, age, blood pressure, and heart-rate matched controls. Central pulse pressure was significantly higher (38.3±12.3 versus 33.6±7.8 mm Hg; P =0.009), and pulse pressure amplification was significantly reduced in Marfan than controls (17.9±15.3% versus 32.3±17.4%; P Marfan and controls (4.98±1.00 versus 4.75±0.67 m/s). In the Marfan group, central pulse pressure and pulse pressure amplification were independently associated with aortic diameter at the sinuses of Valsalva (respectively, β=0.371, P =0.010; β=-0.271, P =0.026). No significant difference in hemodynamic parameters was found according to fibrillin-1 genotype. Patients who increased aortic Z-scores at 1-year follow-up presented a higher central pulse pressure than the remaining (42.7±14.2 versus 32.3±5.9 mm Hg; P =0.004). Central pulse pressure and pulse pressure amplification were impaired in pediatric Marfan syndrome, and associated with aortic root diameters, whereas aortic pulse wave velocity was similar to that of a general pediatric population. An increased central pulse pressure was present among patients whose aortic dilatation worsened at 1-year follow-up. © 2017 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley.

  10. Early results of valve-sparing ascending aortic replacement in type A aortic dissection and aortic insufficiency

    Directory of Open Access Journals (Sweden)

    М. Л. Гордеев

    2016-08-01

    Full Text Available Aim: The study was designed to investigate predictors of effective valve-sparing ascending aortic replacement in patients with Stanford type A aortic dissection combined with aortic insufficiency and to analyze efficacy and safety of this kind of surgery.Methods: From January 2010 to December 2015, 49 patients with Stanford type A aortic dissection combined with aortic insufficiency underwent ascending aortic replacement. All patients were divided into 3 groups: valve-sparing procedures (group 1, n = 11, combined aortic valve and supracoronary ascending aortic replacement (group 2, n = 12, and Bentall procedure (group 3, n = 26. We assessed the initial status of patients, incidence of complications and efficacy of valve-sparing ascending aortic replacement.Results: The hospital mortality rate was 8.2% (4/49 patients. The amount of surgical correction correlated with the initial diameter of the aorta at the level of the sinuses of Valsalva. During the hospital period, none of patients from group 1 developed aortic insufficiency exceeding Grade 2 and the vast majority of patients had trivial aortic regurgitation. The parameters of cardiopulmonary bypass, cross-clamp time and circulatory arrest time did not correlate with the initial size of the ascending aorta and aortic valve blood flow impairment, neither did they influence significantly the incidence and severity of neurological complications. The baseline size of the ascending aorta and degree of aortic regurgitation did not impact the course of the early hospital period.Conclusions: Supracoronary ascending aortic replacement combined with aortic valve repair in ascending aortic dissection and aortic regurgitation is effective and safe. The initial size of the ascending aorta and aortic arch do not influence immediate results. The diameter of the aorta at the level of the sinuses of Valsalva and the condition of aortic valve leaflets could be considered as the limiting factors. Further long

  11. Image quality and diagnostic accuracy of unenhanced SSFP MR angiography compared with conventional contrast-enhanced MR angiography for the assessment of thoracic aortic diseases

    International Nuclear Information System (INIS)

    Krishnam, Mayil S.; Tomasian, Anderanik; Malik, Sachin; Ruehm, Stefan G.; Desphande, Vibhas; Laub, Gerhard

    2010-01-01

    The purpose of this study was to determine the image quality and diagnostic accuracy of three-dimensional (3D) unenhanced steady state free precession (SSFP) magnetic resonance angiography (MRA) for the evaluation of thoracic aortic diseases. Fifty consecutive patients with known or suspected thoracic aortic disease underwent free-breathing ECG-gated unenhanced SSFP MRA with non-selective radiofrequency excitation and contrast-enhanced (CE) MRA of the thorax at 1.5 T. Two readers independently evaluated the two datasets for image quality in the aortic root, ascending aorta, aortic arch, descending aorta, and origins of supra-aortic arteries, and for abnormal findings. Signal-to-noise ratio (SNR) and contrast-to-noise ratio (CNR) were determined for both datasets. Sensitivity, specificity, and diagnostic accuracy of unenhanced SSFP MRA for the diagnosis of aortic abnormalities were determined. Abnormal aortic findings, including aneurysm (n = 47), coarctation (n = 14), dissection (n = 12), aortic graft (n = 6), intramural hematoma (n = 11), mural thrombus in the aortic arch (n = 1), and penetrating aortic ulcer (n = 9), were confidently detected on both datasets. Sensitivity, specificity, and diagnostic accuracy of SSFP MRA for the detection of aortic disease were 100% with CE-MRA serving as a reference standard. Image quality of the aortic root was significantly higher on SSFP MRA (P 0.05). SNR and CNR values were higher for all segments on SSFP MRA (P < 0.01). Our results suggest that free-breathing navigator-gated 3D SSFP MRA with non-selective radiofrequency excitation is a promising technique that provides high image quality and diagnostic accuracy for the assessment of thoracic aortic disease without the need for intravenous contrast material. (orig.)

  12. Type A aortic dissection: Are there CT signs suggestive of valvular involvement?

    Energy Technology Data Exchange (ETDEWEB)

    Platon, Alexandra, E-mail: alexandra.platon@hcuge.ch [Department of Radiology, Emergency Radiology Unit, University Hospital of Geneva, 4 rue Gabrielle-Perret-Gentil, 1211, Geneva (Switzerland); Bernard, Stephane; Perrin, Nils [Department of Radiology, Emergency Radiology Unit, University Hospital of Geneva, 4 rue Gabrielle-Perret-Gentil, 1211, Geneva (Switzerland); Murith, Nicolas [Division of Cardiovascular Surgery, University Hospital of Geneva, 4 rue Gabrielle-Perret-Gentil, 1211, Geneva (Switzerland); John, Gregor [Department of Internal medicine/Geriatrics and Rehabilitation, University Hospital of Geneva, 4 rue Gabrielle-Perret-Gentil, 1211, Geneva (Switzerland); Department of Internal Medicine, Hôpital neuchâtelois, 2300 La Chaux-de-Fonds (Switzerland); Perneger, Thomas [Division of Clinical Epidemiology, University Hospital of Geneva, 4 rue Gabrielle-Perret-Gentil, 1211, Geneva (Switzerland); Rutschmann, Olivier T. [Division of Emergency Medicine, University Hospitals of Geneva and School of Medicine, 4 rue Gabrielle-Perret-Gentil, 1211, Geneva (Switzerland); Poletti, Pierre-Alexandre [Department of Radiology, Emergency Radiology Unit, University Hospital of Geneva, 4 rue Gabrielle-Perret-Gentil, 1211, Geneva (Switzerland)

    2016-11-15

    Highlights: • Non-ECG gated CT rarely shows valve involvement in type A aortic dissection. • Diameters of the aortic root in type A aortic dissection were measured on initial CT. • Sinus of Valsalva diameter >45 mm is 100% specific to predict aortic valve involvement. - Abstract: Aim: To identify the predictive signs of aortic valve involvement on the non-electrocardiogram (ECG)-gated admission computed tomography (CT) of patients with Type A aortic dissection (AD) according to the Stanford classification. Materials and methods: We retrospectively analyzed the non-ECG-gated CT examinations of patients admitted to the emergency department who underwent surgery for Type A AD over a period of 4 years. The diameter of the following structures was calculated as the mean of the smallest and largest diameters (mm) measured in two different planes: aortic annulus, sinus of Valsalva, sinotubular junction, and proximal ascending aorta. These parameters were compared against operative reports in order to determine whether they were predictive of aortic valve involvement. Results: In total, 20 patients (13 men and 7 women) of a mean age of 59.5 years (29–80) were included, 55% of patients (11/20) having surgically proven valvular involvement. The mean diameters (in mm) of the aortic annulus, sinus of Valsalva, sinotubular junction and proximal ascending aorta in the group with (and without, respectively) valvular involvement was 27.7 (26.7), 44.3 (38.1), 42.6 (36.6), and 47.8 (45.9). Only the measurement of the mean diameter of the sinuses of Valsalva was significantly predictive (p = 0.02) of aortic valve involvement. Conclusion: Our findings suggest that measuring the diameter of the sinuses of Valsalva on non-ECG-gated admission CT examinations allows for predicting aortic valve involvement in Type A AD patients.

  13. Type A aortic dissection: Are there CT signs suggestive of valvular involvement?

    International Nuclear Information System (INIS)

    Platon, Alexandra; Bernard, Stephane; Perrin, Nils; Murith, Nicolas; John, Gregor; Perneger, Thomas; Rutschmann, Olivier T.; Poletti, Pierre-Alexandre

    2016-01-01

    Highlights: • Non-ECG gated CT rarely shows valve involvement in type A aortic dissection. • Diameters of the aortic root in type A aortic dissection were measured on initial CT. • Sinus of Valsalva diameter >45 mm is 100% specific to predict aortic valve involvement. - Abstract: Aim: To identify the predictive signs of aortic valve involvement on the non-electrocardiogram (ECG)-gated admission computed tomography (CT) of patients with Type A aortic dissection (AD) according to the Stanford classification. Materials and methods: We retrospectively analyzed the non-ECG-gated CT examinations of patients admitted to the emergency department who underwent surgery for Type A AD over a period of 4 years. The diameter of the following structures was calculated as the mean of the smallest and largest diameters (mm) measured in two different planes: aortic annulus, sinus of Valsalva, sinotubular junction, and proximal ascending aorta. These parameters were compared against operative reports in order to determine whether they were predictive of aortic valve involvement. Results: In total, 20 patients (13 men and 7 women) of a mean age of 59.5 years (29–80) were included, 55% of patients (11/20) having surgically proven valvular involvement. The mean diameters (in mm) of the aortic annulus, sinus of Valsalva, sinotubular junction and proximal ascending aorta in the group with (and without, respectively) valvular involvement was 27.7 (26.7), 44.3 (38.1), 42.6 (36.6), and 47.8 (45.9). Only the measurement of the mean diameter of the sinuses of Valsalva was significantly predictive (p = 0.02) of aortic valve involvement. Conclusion: Our findings suggest that measuring the diameter of the sinuses of Valsalva on non-ECG-gated admission CT examinations allows for predicting aortic valve involvement in Type A AD patients.

  14. Imaging of thoracic aortic dissection

    International Nuclear Information System (INIS)

    Vu, F.H.; Young, N.; Soo, Y.S.

    1994-01-01

    Acute thoracic aortic dissection has a high mortality rate if untreated, so the diagnosis must be rapidly made. Multiple imaging techniques are often used. This retrospective study from 1988 to 1993 assesses the usefulness in diagnosis of chest X-rays, computed tomography (CT) scanning, aortography, magnetic resonance imaging (MRI), trans-thoracic (TTE) and trans-oesophageal (TOE) echocardiography. Forty-two patients with a final clinical diagnosis of dissection were studied. The diagnosis was confirmed in 16 (13 at surgery and three at autopsy). Three died with dissection given as the only cause of death. Chest X-ray abnormalities were seen in all 19 patients with surgery or death from dissection, with a widened mediastinum and/or dilated aorta being present in 17. In the group of 16 patients with surgery or autopsy proof, CT scans found dissections in 9 out of 12 patients studied and correctly classified the type in only five. Aortography was preformed in five, with accurate depiction of dissection and type in all. TTE found dissections in three of eight patients imaged by this method. MRI and TOE were preformed each on two patients, with accurate depiction of dissection and type in each. Because of the relatively low sensitivity of CT scanning in defining aortic dissections Westmead Hospital is currently assessing the use of TOE as the prime imaging modality prior to surgical intervention. 17 refs., 4 tabs., 4 figs

  15. Aortic microcalcification is associated with elastin fragmentation in Marfan syndrome.

    Science.gov (United States)

    Wanga, Shaynah; Hibender, Stijntje; Ridwan, Yanto; van Roomen, Cindy; Vos, Mariska; van der Made, Ingeborg; van Vliet, Nicole; Franken, Romy; van Riel, Luigi Amjg; Groenink, Maarten; Zwinderman, Aeilko H; Mulder, Barbara Jm; de Vries, Carlie Jm; Essers, Jeroen; de Waard, Vivian

    2017-11-01

    Marfan syndrome (MFS) is a connective tissue disorder in which aortic rupture is the major cause of death. MFS patients with an aortic diameter below the advised limit for prophylactic surgery (elastin fragments play a causal role in aortic calcification in MFS, and that microcalcification serves as a marker for aortic disease severity. To address this hypothesis, we analysed MFS patient and mouse aortas. MFS patient aortic tissue showed enhanced microcalcification in areas with extensive elastic lamina fragmentation in the media. A causal relationship between medial injury and microcalcification was revealed by studies in vascular smooth muscle cells (SMCs); elastin peptides were shown to increase the activity of the calcification marker alkaline phosphatase (ALP) and reduce the expression of the calcification inhibitor matrix GLA protein in human SMCs. In murine Fbn1 C1039G/+ MFS aortic SMCs, Alpl mRNA and activity were upregulated as compared with wild-type SMCs. The elastin peptide-induced ALP activity was prevented by incubation with lactose or a neuraminidase inhibitor, which inhibit the elastin receptor complex, and a mitogen-activated protein kinase kinase-1/2 inhibitor, indicating downstream involvement of extracellular signal-regulated kinase-1/2 (ERK1/2) phosphorylation. Histological analyses in MFS mice revealed macrocalcification in the aortic root, whereas the ascending aorta contained microcalcification, as identified with the near-infrared fluorescent bisphosphonate probe OsteoSense-800. Significantly, microcalcification correlated strongly with aortic diameter, distensibility, elastin breaks, and phosphorylated ERK1/2. In conclusion, microcalcification co-localizes with aortic elastin degradation in MFS aortas of humans and mice, where elastin-derived peptides induce a calcification process in SMCs via the elastin receptor complex and ERK1/2 activation. We propose microcalcification as a novel imaging marker to monitor local elastin degradation and

  16. Image quality and diagnostic accuracy of unenhanced SSFP MR angiography compared with conventional contrast-enhanced MR angiography for the assessment of thoracic aortic diseases

    Energy Technology Data Exchange (ETDEWEB)

    Krishnam, Mayil S. [University of California, Cardiovascular and Thoracic Imaging, UCI Medical Center, Irvine, CA (United States); Tomasian, Anderanik; Malik, Sachin; Ruehm, Stefan G. [University of California at Los Angeles, Department of Radiological Sciences, Ronald Reagan Medical Center, Los Angeles, CA (United States); Desphande, Vibhas; Laub, Gerhard [Siemens Medical Solutions, Los Angeles, CA (United States)

    2010-06-15

    The purpose of this study was to determine the image quality and diagnostic accuracy of three-dimensional (3D) unenhanced steady state free precession (SSFP) magnetic resonance angiography (MRA) for the evaluation of thoracic aortic diseases. Fifty consecutive patients with known or suspected thoracic aortic disease underwent free-breathing ECG-gated unenhanced SSFP MRA with non-selective radiofrequency excitation and contrast-enhanced (CE) MRA of the thorax at 1.5 T. Two readers independently evaluated the two datasets for image quality in the aortic root, ascending aorta, aortic arch, descending aorta, and origins of supra-aortic arteries, and for abnormal findings. Signal-to-noise ratio (SNR) and contrast-to-noise ratio (CNR) were determined for both datasets. Sensitivity, specificity, and diagnostic accuracy of unenhanced SSFP MRA for the diagnosis of aortic abnormalities were determined. Abnormal aortic findings, including aneurysm (n = 47), coarctation (n = 14), dissection (n = 12), aortic graft (n = 6), intramural hematoma (n = 11), mural thrombus in the aortic arch (n = 1), and penetrating aortic ulcer (n = 9), were confidently detected on both datasets. Sensitivity, specificity, and diagnostic accuracy of SSFP MRA for the detection of aortic disease were 100% with CE-MRA serving as a reference standard. Image quality of the aortic root was significantly higher on SSFP MRA (P < 0.001) with no significant difference for other aortic segments (P > 0.05). SNR and CNR values were higher for all segments on SSFP MRA (P < 0.01). Our results suggest that free-breathing navigator-gated 3D SSFP MRA with non-selective radiofrequency excitation is a promising technique that provides high image quality and diagnostic accuracy for the assessment of thoracic aortic disease without the need for intravenous contrast material. (orig.)

  17. Modified conduit preparation creates a pseudosinus in an aortic valve-sparing procedure for aneurysm of the ascending aorta.

    Science.gov (United States)

    Cochran, R P; Kunzelman, K S; Eddy, A C; Hofer, B O; Verrier, E D

    1995-06-01

    Mechanical valved conduit replacement of the aortic root is a durable and appropriate procedure for many diseases of the ascending aorta, but may sacrifice an anatomically salvageable aortic valve. For young active patients and for patients with "systemic" arterial disease (atherosclerosis, Marfan's syndrome) who may require future operations, life-long anticoagulation with its attendant thromboembolic versus hemorrhagic risks is not ideal. Several techniques have been suggested as aortic valve-sparing options. Recently, a procedure was described that combines the freehand homograft techniques with the standard Bentall techniques (David procedure). This innovative technique replaces the ascending aorta with a Dacron cylinder, spares the aortic valve, and restores competence and thus offers an excellent alternative. The durability of this procedure that places the aortic valve inside a cylindrical conduit without sinuses of Valsalva is unknown. In selected patients, we have used this technique to spare the aortic valve. On the basis of experimental data and preliminary computer modeling, with the hope of improving the durability, we have modified the conduit to create a "pseudosinus" in our most recent nine patients. We have done the David procedure in 10 patients. The pseudosinus modification was done in the most recent nine patients. Patients' ages ranged from 37 to 71 years (mean 49.9 years). There were five female and five male patients. Five patients had Marfan's syndrome and five patients had annuloaortic ectasia. There has been no mortality and all patients have had both early and late follow-up echocardiography. Five patients have zero to trace aortic insufficiency, four patients have trace to mild aortic insufficiency, and one patient has mild or "1+" aortic insufficiency. Aortic insufficiency has not progressed in any patient during the 18 months of follow-up. The patient with 1+ aortic insufficiency has no activity limits, good ventricular function, and

  18. Is aortopathy in bicuspid aortic valve disease a congenital defect or a result of abnormal hemodynamics? A critical reappraisal of a one-sided argument.

    Science.gov (United States)

    Girdauskas, Evaldas; Borger, Michael A; Secknus, Maria-Anna; Girdauskas, Gracijus; Kuntze, Thomas

    2011-06-01

    Although there is adequate evidence that bicuspid aortic valve (BAV) is an inheritable disorder, there is a great controversy regarding the pathogenesis of dilatation of the proximal aorta. The hemodynamic theory was the first explanation for BAV aortopathy. The genetic theory, however, has become increasingly popular over the last decade and can now be viewed as the clearly dominant one. The widespread belief that BAV disease is a congenital disorder of vascular connective tissue has led to more aggressive treatment recommendations of the proximal aorta in such patients, approaching aortic management recommendations for patients with Marfan syndrome. There is emerging evidence that the 'clinically normal' BAV is associated with abnormal flow patterns and asymmetrically increased wall stress in the proximal aorta. Recent in vitro and in vivo studies on BAV function provide a unique hemodynamic insight into the different phenotypes of BAV disease and asymmetry of corresponding aortopathy even in the presence of a 'clinically normal' BAV. On the other hand, there is a subgroup of young male patients with BAV and a root dilatation phenotype, who may present the predominantly genetic form of BAV disease. In the face of these important findings, we feel that a critical review of this clinical problem is timely and appropriate, as the prevailing BAV-aortopathy theory undoubtedly affects the surgical approach to this common clinical entity. Thorough analysis of the recent literature shows a growing amount of evidence supporting the hemodynamic theory of aortopathy in patients with BAV disease. Data from recent studies requires a reevaluation of our overwhelming support of the genetic theory, and obliges us to acknowledge that hemodynamics plays an important role in the development of this disease process. Given the marked heterogeneity of BAV disease, further studies are required in order to more precisely determine which theory is the 'correct' one for explaining the

  19. Pnematic Dilation in Achalasia

    Directory of Open Access Journals (Sweden)

    Maximilian Bittinger

    2001-01-01

    Full Text Available Pneumatic dilation is the most common first-line therapy for the treatment of achalasia. The aim of dilation is a controlled disruption of circular muscle fibres of the lower esophageal sphincter to reduce the functional obstruction. Several types of dilators and different dilation techniques are used, but the achieved results are similar. The mean success rate is about 80% in the short term, but some patients need redilation in the further course (particularly young patients. Best long term results are obtained if the lower esophageal sphincter pressure can be reduced below 10 mmHg. Major complications are rare after pneumatic dilation; the most serious complication is esophageal perforation, which occurs at a mean rate of about 2.5%. Considering the pros and cons of other effective forms of treatment of achalasia (esophagomyotomy and intrasphincteric injection of botulinum toxin, pneumatic dilation is still the treatment of choice in the majority of patients with achalasia.

  20. Possible extracardiac predictors of aortic dissection in Marfan syndrome

    Science.gov (United States)

    2014-01-01

    Background According to previous studies, aortic diameter alone seems to be insufficient to predict the event of aortic dissection in Marfan syndrome (MFS). Determining the optimal schedule for preventive aortic root replacement (ARR) aortic growth rate is of importance, as well as family history, however, none of them appear to be decisive. Thus, the aim of this study was to search for potential predictors of aortic dissection in MFS. Methods A Marfan Biobank consisting of 79 MFS patients was established. Thirty-nine MFS patients who underwent ARR were assigned into three groups based on the indication for surgery (dissection, annuloaortic ectasia and prophylactic surgery). The prophylactic surgery group was excluded from the study. Transforming growth factor-β (TGF-β) serum levels were measured by ELISA, relative expression of c-Fos, matrix metalloproteinase 3 and 9 (MMP-3 and −9) were assessed by RT-PCR. Clinical parameters, including anthropometric variables - based on the original Ghent criteria were also analyzed. Results Among patients with aortic dissection, TGF-β serum level was elevated (43.78 ± 6.51 vs. 31.64 ± 4.99 ng/l, p < 0.0001), MMP-3 was up-regulated (Ln2α = 1.87, p = 0.062) and striae atrophicae were more common (92% vs. 41% p = 0.027) compared to the annuloaortic ectasia group. Conclusions We found three easily measurable parameters (striae atrophicae, TGF-β serum level, MMP-3) that may help to predict the risk of aortic dissection in MFS. Based on these findings a new classification of MFS, that is benign or malignant is also proposed, which could be taken into consideration in determining the timing of prophylactic ARR. PMID:24720641

  1. Optimal C-arm angulation during transcatheter aortic valve replacement: Accuracy of a rotational C-arm computed tomography based three dimensional heart model.

    Science.gov (United States)

    Veulemans, Verena; Mollus, Sabine; Saalbach, Axel; Pietsch, Max; Hellhammer, Katharina; Zeus, Tobias; Westenfeld, Ralf; Weese, Jürgen; Kelm, Malte; Balzer, Jan

    2016-10-26

    To investigate the accuracy of a rotational C-arm CT-based 3D heart model to predict an optimal C-arm configuration during transcatheter aortic valve replacement (TAVR). Rotational C-arm CT (RCT) under rapid ventricular pacing was performed in 57 consecutive patients with severe aortic stenosis as part of the pre-procedural cardiac catheterization. With prototype software each RCT data set was segmented using a 3D heart model. From that the line of perpendicularity curve was obtained that generates a perpendicular view of the aortic annulus according to the right-cusp rule. To evaluate the accuracy of a model-based overlay we compared model- and expert-derived aortic root diameters. For all 57 patients in the RCT cohort diameter measurements were obtained from two independent operators and were compared to the model-based measurements. The inter-observer variability was measured to be in the range of 0°-12.96° of angular C-arm displacement for two independent operators. The model-to-operator agreement was 0°-13.82°. The model-based and expert measurements of aortic root diameters evaluated at the aortic annulus ( r = 0.79, P optimal C-arm configuration, potentially simplifying current clinical workflows before and during TAVR.

  2. Multiple Re-entry Closures After TEVAR for Ruptured Chronic Post-dissection Thoraco-abdominal Aortic Aneurysm

    Directory of Open Access Journals (Sweden)

    R. Kinoshita

    Full Text Available Introduction: Although thoracic endovascular aortic repair (TEVAR has become a promising treatment for complicated acute type B dissection, its role in treating chronic post-dissection thoraco-abdominal aortic aneurysm (TAA is still limited owing to persistent retrograde flow into the false lumen (FL through abdominal or iliac re-entry tears. Report: A case of chronic post-dissection TAA treatment, in which a dilated descending FL ruptured into the left thorax, is described. The primary entry tear was closed by emergency TEVAR and multiple abdominal re-entries were closed by EVAR. In addition, major re-entries at the detached right renal artery and iliac bifurcation were closed using covered stents. To close re-entries as far as possible, EVAR was carried out using the chimney technique, and additional aortic extenders were placed above the coeliac artery. A few re-entries remained, but complete FL thrombosis of the rupture site was achieved. Follow-up computed tomography showed significant shrinkage of the FL. Discussion: In treating post-dissection TAA, entry closure by TEVAR is sometimes insufficient, owing to persistent retrograde flow into the FL from abdominal or iliac re-entries. Adjunctive techniques are needed to close these distal re-entries to obtain complete FL exclusion, especially in rupture cases. Recently, encouraging results of complete coverage of the thoraco-abdominal aorta with fenestrated or branched endografts have been reported; however, the widespread employment of such techniques appears to be limited owing to technical difficulties. The present method with multiple re-entry closures using off the shelf and immediately available devices is an alternative for the endovascular treatment of post-dissection TAA, especially in the emergency setting. Keywords: Aortic dissection, Ruptured aortic aneurysm, Post-dissection thoracoabdominal aortic aneurysm, Endovascular aortic repair, Reentry closure, Endovascular procedures

  3. Presentation of an uncommon form of aortic dissection and rupture in Marifoan syndrome

    International Nuclear Information System (INIS)

    Delgado, I.; Ruiz, R.; Villanueva, J.M.; Fernandez Cueto, J.L.

    1995-01-01

    In Marfan syndrome, aneurysmatic enlargement of ascending aorta and dissection starting at the root are the most common cardiovascular complications. We present an infrequent case of a 15-year-old patient with a typical case of Marfan syndrome. CT disclosed an aorta and aortic arch of normal size with dissection originating distally with respect to the point where left subclavian artery arises. The disecction extended to descending aorta and to iliac and femoral arteries. Aortic rupture occurred in the arch, with massive hemothorax. The CT findings were confirmed at necropsy. 9 refs

  4. Organizing Thrombus Mimicking a Cardiac Tumor Located at the Mitral-Aortic Intervalvular Fibrosa

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    Ji Seong Lee

    2016-02-01

    Full Text Available Thrombosis at the left ventricular outflow tract occurs without any detectable heart disease or predisposing factors only extremely rarely. A 48-year-old male visited Konkuk University Medical Center with loss of consciousness one month prior to presentation. Before he visited our hospital, he had been diagnosed with a cardiac tumor, which was located between the left atrium and posterior aortic root, and which was adjacent to both the aortic and mitral valves. Cardiac transplantation was recommended at the other hospital because of the high risk of cardiac dysfunction induced by both aortic and mitral valvular dysfunction after surgical resection. Based on preoperative transthoracic echocardiography, cardiac computed tomography, cardiac magnetic resonance imaging, and intraoperative transesophageal echocardiography, we considered it to be a benign tumor. Complete resection was achieved and the pathology confirmed organizing thrombus. We report a case of organizing thrombus mimicking a cardiac tumor, which was located at the mitral-aortic intervalvular fibrosa of the left ventricular outflow tract without any heart disease.

  5. Inter-ethnic differences in valve morphology, valvular dysfunction, and aortopathy between Asian and European patients with bicuspid aortic valve.

    Science.gov (United States)

    Kong, William K F; Regeer, Madelien V; Poh, Kian K; Yip, James W; van Rosendael, Philippe J; Yeo, Tiong C; Tay, Edgar; Kamperidis, Vasileios; van der Velde, Enno T; Mertens, Bart; Ajmone Marsan, Nina; Delgado, Victoria; Bax, Jeroen J

    2018-04-14

    Transcatheter aortic valve replacement (TAVR) has been shown safe and feasible in patients with bicuspid aortic valve (BAV) morphology. Evaluation of inter-ethnic differences in valve morphology and function and aortic root dimensions in patients with BAV is important for the worldwide spread of this therapy in this subgroup of patients. Comparisons between large European and Asian cohorts of patients with BAV have not been performed, and potential differences between populations may have important implications for TAVR. The present study evaluated the differences in valve morphology and function and aortic root dimensions between two large cohorts of European and Asian patients with BAV. Aortic valve morphology was defined on transthoracic echocardiography according to the number of commissures and raphe: type 0 = no raphe and two commissures, type 1 = one raphe and two commissures, type 2 = two raphes and one commissure. Aortic stenosis and regurgitation were graded according to current recommendations. For this study, aortic root dimensions were manually measured on transthoracic echocardiograms at the level of the aortic annulus, sinus of Valsalva (SOV), sinotubular junction (STJ), and ascending aorta (AA). Of 1427 patients with BAV (45.2 ± 18.1 years, 71.9% men), 794 (55.6%) were Europeans and 633 (44.4%) were Asians. The groups were comparable in age and proportion of male sex. Asians had higher prevalence of type 1 BAV with raphe between right and non-coronary cusps than Europeans (19.7% vs. 13.6%, respectively; P < 0.001), whereas the Europeans had higher prevalence of type 0 BAV (two commissures, no raphe) than Asians (14.5% vs. 6.8%, respectively; P < 0.001). The prevalence of moderate and severe aortic regurgitation was higher in Europeans than Asians (44.2% vs. 26.8%, respectively; P < 0.001) whereas there were no differences in BAV with normal function or aortic stenosis. After adjusting for demographics, comorbidities

  6. Frequency of reoperations in patients with Marfan syndrome.

    Science.gov (United States)

    Geisbuesch, Sarah; Schray, Deborah; Bischoff, Moritz S; Lin, Hung-Mo; Di Luozzo, Gabriele; Griepp, Randall B

    2012-05-01

    We undertook a retrospective study of the pattern of reoperations in surgical patients with Marfan disease. Between 1985 and 2008, 83 Marfan patients (60 males, 23 females) underwent 155 aortic operations in our institution. Twenty-eight patients had acute dissection (22 type A, 6 type B), and two had aortic rupture. Mean age at initial operation was 32±13 years. Operations included valve-sparing or Bentall aortic root repair, and ascending aorta, arch, descending thoracic, thoracoabdominal aorta, and infrarenal aortic replacement. Sixty-one patients whose initial operation was elective (Group I) were compared with 22 patients with initial emergency surgery (Group II). Overall, 81/83 patients ultimately underwent root/ascending repair: 64% initially and 36% at reoperation. Operative mortality in Group I was 1.6% for both initial operations and reoperations vs 9.0% and 0% in Group II. Significant differences between Group I and Group II patients included: total reoperations (1 vs 3, p=0.05); arch operations (0 vs 1, p=0.003); descending thoracic aortic operations (0 vs 0.5, p=0.003); and total aortic segments replaced (1.6±1.0 vs 2.4±1.1, p=0.001). Survival at 5 and 10 years did not differ between Group I and II patients (87% and 71% vs 82% and 56%, p=0.19). Although reoperation occurs in about half of surgical Marfan patients, reoperative mortality is low. Patients with initial elective procedures fare better than those with initial emergency surgery: they have fewer subsequent operations, fewer aortic segments replaced, and trend toward improved survival. Elective root replacement should be seriously considered in any Marfan patient with significant root dilatation. Copyright © 2012 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  7. Decreased expression of fibulin-4 in aortic wall of aortic dissection.

    Science.gov (United States)

    Huawei, P; Qian, C; Chuan, T; Lei, L; Laing, W; Wenlong, X; Wenzhi, L

    2014-02-01

    In this research, we will examine the expression of Fibulin-4 in aortic wall to find out its role in aortic dissection development. The samples of aortic wall were obtained from 10 patients operated for acute ascending aortic dissection and five patients for chronic ascending aortic dissection. Another 15 pieces of samples from patients who had coronary artery bypass were as controls. The aortic samples were stained with aldehyde magenta dyeing to evaluate the arrangement of elastic fibers. The Fibulin-4 protein and mRNA expression were both determined by Western blot and realtime quantitative polymerase chain reaction. Compared with the control group, both in acute and chronic ascending aortic dissection, elastic fiber fragments increased and the expression of fibulin-4 protein significantly decreased (P= 0.045 < 0.05). The level of fibulin-4 mRNA decreased in acute ascending aortic dissection (P= 0.034 < 0.05), while it increased in chronic ascending aortic dissection (P=0.004 < 0.05). The increased amounts of elastic fiber fragments were negatively correlated with the expression of fibulin-4 mRNA in acute ascending aortic dissection. In conclusion, in aortic wall of ascending aortic dissection, the expression of fibulin-4 protein decreased and the expression of fibulin-4 mRNA was abnormal. Fibulin-4 may play an important role in the pathogenesis of aortic dissection.

  8. Long-term outcome after the original and simple modified technique of valve-sparing aortic root reimplantation in Marfan-based population, David V University of Tokyo modification.

    Science.gov (United States)

    Ando, Masahiko; Yamauchi, Haruo; Morota, Tetsuro; Taketani, Tsuyoshi; Shimada, Shogo; Nawata, Kan; Umeki, Akihide; Ono, Minoru

    2016-01-01

    In valve-sparing aortic root replacement (VSARR), how to reproduce Valsalva sinus has been an issue. In the original David V procedure, they put plication stitches at sinotubular junction level, although the reefing effect is limited and distal graft remains larger than native. Other modified techniques are two-grafts technique and ready-made Valsalva graft. However, the former needs graft-graft anastomosis and may not be cost-effective, while in the latter, the shape of sinus is fixed and minor adjustment is difficult. David V University of Tokyo modification (David V-UT) is our original solution to that, creating pseudosinus with one straight graft by longitudinal size-reduction running sutures above each pseudosinus. The purpose of the present study is to investigate long-term outcome of David V-UT. We analyzed 59 David V-UT patients from February 2004 to February 2013 and long-term outcomes were investigated by Kaplan-Meier methods. Risk factors for adverse events "death or recurrent aortic insufficiency (AI) with or without aortic valve reoperation" were analyzed by using Cox proportional hazard models. Mean age was 33.1±14.5 years, and 38 patients (64%) were male. Marfan syndrome (MFS) accounts for 47 patients (80%). Only one patient was with bicuspid aortic valve. No in-hospital mortality was observed. Mean follow-up was 4.9±2.4 years. Estimated survival was 94.0±3.4% at 5 years. Freedoms from aortic valve reoperation and recurrent AI greater than mild were 95.7±3.0% and 88.9±4.7% at 5 years, respectively. In Cox proportional hazard analysis, preoperative AI greater than mild and Z score of annular diameter were significant risks for adverse events (p=0.027 and 0.045, hazard ratio 6.084 and 1.432, 95% C.I. 1.225-30.21 and 1.008-2.035, respectively). Even in Marfan-characterized population, David V-UT provided satisfactory long-term outcome, comparable to other VSARR modifications. It is simple but can freely reproduce trilobed sinus with one straight

  9. Aortic, carotid intima-media thickness and flow- mediated dilation as markers of early atherosclerosis in a cohort of pediatric patients with rheumatic diseases.

    Science.gov (United States)

    Del Giudice, Emanuela; Dilillo, Anna; Tromba, Luciana; La Torre, Giuseppe; Blasi, Sara; Conti, Fabrizio; Viola, Franca; Cucchiara, Salvatore; Duse, Marzia

    2018-06-01

    The aims of this study were to identify the presence of endothelial dysfunction as a marker of early atherosclerosis by measuring aortic and carotid intimal-medial thickness (aIMT and cIMT) and flow-mediated dilation (FMD) and their correlation with traditional and no traditional risk factors for atherosclerosis in children with rheumatic diseases. Thirty-nine patients (mean age 15.3 ± 5.7 years), 23 juvenile idiopathic arthritis, 9 juvenile spondyloarthropathies, 7 connective tissue diseases (mean disease duration and onset respectively 5 ± 3.6 and 10 ± 5 years), and 52 healthy children matched for sex and age were enrolled. Demographic data (age, sex, familiarity for cardiovascular disease), traditional risk factors for atherosclerosis (BMI, active and passive smoking, dyslipidemia), activity disease indexes (reactive count protein, erythrocyte sedimentation rate) autoantibodies, and complement tests were collected. aIMT, cIMT, and FMD were assessed following a standardized protocol by high-resolution ultrasonography. Patients resulted significantly more exposed to passive smoking and had a lower BMI and higher homocysteine level than controls. cIMT and aIMT were significantly higher in patients than controls (p disease duration. FMD % was significantly reduced in patients compared to controls (p rheumatic diseases, mainly in early onset forms, and aIMT is an earlier marker of preclinical atherosclerosis. Premature endothelial dysfunction could be included in the follow-up of children with rheumatic disorders to plan prevention strategies of cardiovascular disease already in pediatrics.

  10. Wall stress on ascending thoracic aortic aneurysms with bicuspid compared with tricuspid aortic valve.

    Science.gov (United States)

    Xuan, Yue; Wang, Zhongjie; Liu, Raymond; Haraldsson, Henrik; Hope, Michael D; Saloner, David A; Guccione, Julius M; Ge, Liang; Tseng, Elaine

    2018-03-08

    Guidelines for repair of bicuspid aortic valve-associated ascending thoracic aortic aneurysms have been changing, most recently to the same criteria as tricuspid aortic valve-ascending thoracic aortic aneurysms. Rupture/dissection occurs when wall stress exceeds wall strength. Recent studies suggest similar strength of bicuspid aortic valve versus tricuspid aortic valve-ascending thoracic aortic aneurysms; thus, comparative wall stress may better predict dissection in bicuspid aortic valve versus tricuspid aortic valve-ascending thoracic aortic aneurysms. Our aim was to determine whether bicuspid aortic valve-ascending thoracic aortic aneurysms had higher wall stresses than their tricuspid aortic valve counterparts. Patients with bicuspid aortic valve- and tricuspid aortic valve-ascending thoracic aortic aneurysms (bicuspid aortic valve = 17, tricuspid aortic valve = 19) greater than 4.5 cm underwent electrocardiogram-gated computed tomography angiography. Patient-specific 3-dimensional geometry was reconstructed and loaded to systemic pressure after accounting for prestress geometry. Finite element analyses were performed using the LS-DYNA solver (LSTC Inc, Livermore, Calif) with user-defined fiber-embedded material model to determine ascending thoracic aortic aneurysm wall stress. Bicuspid aortic valve-ascending thoracic aortic aneurysms 99th-percentile longitudinal stresses were 280 kPa versus 242 kPa (P = .028) for tricuspid aortic valve-ascending thoracic aortic aneurysms in systole. These stresses did not correlate to diameter for bicuspid aortic valve-ascending thoracic aortic aneurysms (r = -0.004) but had better correlation to tricuspid aortic valve-ascending thoracic aortic aneurysms diameter (r = 0.677). Longitudinal stresses on sinotubular junction were significantly higher in bicuspid aortic valve-ascending thoracic aortic aneurysms than in tricuspid aortic valve-ascending thoracic aortic aneurysms (405 vs 329 kPa, P = .023). Bicuspid

  11. Characteristic Morphologies of the Bicuspid Aortic Valve in Patients with Genetic Syndromes.

    Science.gov (United States)

    Niaz, Talha; Poterucha, Joseph T; Olson, Timothy M; Johnson, Jonathan N; Craviari, Cecilia; Nienaber, Thomas; Palfreeman, Jared; Cetta, Frank; Hagler, Donald J

    2018-02-01

    In patients with bicuspid aortic valve (BAV), complications including progressive aortic stenosis and aortic dilatation develop over time. The morphology of cusp fusion is one of the determinants of the type and severity of these complications. We present the association of morphology of cusp fusion in BAV patients with distinctive genetic syndromes. The Mayo Clinic echocardiography database was retrospectively reviewed to identify patients (age ≤ 22 years) diagnosed with BAV from 1990 to 2016. Cusp fusion morphology was determined from the echocardiographic studies, while coexisting cardiac defects and genetic syndromes were determined from chart review. A total of 1,037 patients with BAV were identified: 550 (53%) had an isolated BAV, 299 (29%) had BAV and a coexisting congenital heart defect, and 188 (18%) had BAV and a coexisting genetic syndrome or disorder. There were no differences in distribution of morphology across the three groups. However, right-noncoronary (RN) cusp fusion was the predominant morphology associated with Down syndrome (P = .002) and right-left (RL) cusp fusion was the predominant morphology associated with Turner syndrome (P = .02), DiGeorge syndrome (P = .02), and Shone syndrome (P = .0007), when compared with valve morphology in patients with isolated BAV. Isolated BAV patients with RN cusp fusion had larger ascending aorta diameter (P = .001) and higher number of patients with ≥ moderate aortic regurgitation (P = .02), while those with RL cusp fusion had larger sinus of Valsalva diameter (P = .0006). Morphological subtypes of BAV are associated with different genetic syndromes, suggesting distinct perturbations of developmental pathways in aortic valve malformation. Copyright © 2017 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.

  12. Dez anos de experiência com a substituição da valva aórtica com homoenxertos valvares aórticos implantados pela técnica da substituição total da raiz Ten years experience of aortic valve replacement with aortic homograft root replacement

    Directory of Open Access Journals (Sweden)

    Francisco Costa

    2006-06-01

    áximo variando entre 3 a 47 mmHg (média de 14,5 mmHg, sendo que apenas dois pacientes apresentavam gradiente superior a 40mmHg. Insuficiência valvar moderada foi encontrada em quatro pacientes. CONCLUSÕES: Os resultados imediatos e tardios com a substituição da valva aórtica por homoenxerto valvar criopreservado foram excelentes, com boa capacidade funcional e baixa morbi-mortalidade tardia. O único fator de risco para a degeneração tecidual primária foi a idade do paciente menor que 20 anos. Homoenxertos aórticos representam uma excelente opção para pacientes com idade acima de 40-50 anos, especialmente naqueles com contra-indicação ou que não desejem fazer o uso de anticoagulantes.OBJECTIVE: To evaluate the imediate and late results of 10 years of aortic valve root replacement with aortic homografts and to identify possible risk factors related with homograft primary tissue failure. METHODS: Between May 1995 and January 2006, 282 patients with a mean age of 52.8±16.6 years were submitted to aortic valve root replacement with an aortic homografts. The most prevalent etiologies were calcified bicuspid aortic valves and senile degeneration, corresponding to 49% of the cases. Forty-seven patients were reoperations and acute bacterial endocarditis were present in 26. Associated procedures were necessary in 113 patients. The homograft was implanted as a root replacement in all. Follow-up time varied between 1 and 129 months (mean= 41±25 months. RESULTS: Early overall mortality was 7%, with only 2.6% for cases of aortic valve replacement in isolation. Of the 262 patients discharged from hospital, it was possible to obtain clinical and echocardiographic evaluations in 209. Fifty-one patients (20% were lost in the follow-up. There were 17 late deaths between the 2nd and 81st postoperative months, which resulted in an actuarial survival rate of 90% and 80.1% at 5 and 10 years respectively. There were eight thromboembolic events (four early and four late, for a

  13. Aortic Annular Enlargement during Aortic Valve Replacement

    Directory of Open Access Journals (Sweden)

    Selman Dumani

    2016-09-01

    Full Text Available In the surgery of aortic valve replacement is always attempted, as much as possible, to implant the larger prosthesis with the mains goals to enhance the potential benefits, to minimise transvalvular gradient, decrease left ventricular size and avoid the phenomenon of patient-prosthesis mismatch. Implantation of an ideal prosthesis often it is not possible, due to a small aortic annulus. A variety of aortic annulus enlargement techniques is reported to avoid patient-prosthesis mismatch. We present the case that has submitted four three times open heart surgery. We used Manouguian technique to enlarge aortic anulus with excellent results during the fourth time of surgery.

  14. Exertional Angina Due To Fused Aortic Bioprosthesis During Left Ventricular Assist Device Support: Two Cases and Review of the Literature.

    Science.gov (United States)

    Bonios, Michael J; Selzman, Craig H; Gilbert, Edward M; McKellar, Stephen H; Koliopoulou, Antigoni; Strege, Jennifer L; Nativi, Jose N; Fang, James C; Stehlik, Josef; Drakos, Stavros G

    We present the case of two patients with idiopathic dilated cardiomyopathy and moderate aortic valve regurgitation that were treated with a bioprosthetic valve at the time of the left ventricular assist device (LVAD) implantation. A few months later, patients revealed partial recovery in the left ventricle systolic function. Both patients, during the LVAD turndown protocol, reported the onset of chest pain. The transthoracic echocardiography revealed the presence of a new transaortic pressure gradient. We confirmed the presence of a fused bioprosthetic valve by further performing a transesophageal echocardiogram and a left and right heart catheterization. Replacement of aortic valve at the time of an LVAD implantation constitutes a challenging case. Although a mechanical valve is contraindicated due to the increased thromboembolic risk, selecting a bioprosthetic valve increases the risk of valve leaflets fusion. The consequences of this phenomenon should be acknowledged in LVAD patients undergoing aortic valve replacement with a bioprosthetic, especially under the view of LVAD explantation for those revealing myocardial recovery under mechanical unloading.

  15. MR imaging of aortic coarctation

    International Nuclear Information System (INIS)

    Beslic, S.

    2004-01-01

    Purpose. The purpose of this paper is to analyse the contribution of MRI as diagnostic procedure in the preoperative diagnosis of aortic coarctation (CoA), in patients with clinical and echocardiographic suspicion for this disease. Patients and methods. During the period of three years, eight patients were examined, 5 (62.5%) male and 3 (37.5%) female patients with clinical echocardiographic suspicion of CoA. The ratio between male and female patients was 1.7 : 1. The youngest patient was 3 and the oldest 46 years (median age was 15 years). Without administration of contrast media and using body coil the examinations were performed with MR machine Magnetom 1.0 Tesla ( S iemens ) , with the slice thickness of 6 mm, Fast spin-echo (FSE) T1W sequences, Cine gradient echo (GRE) sequence with slab 7 mm and time of flight (TOF) sequence with MIP reconstructions were applied. During the examinations the patients underwent also ECG gating. Examinations were done in axial, coronal and oblique sagittal projections with measuring of the dimensions of cardiovascular structures. Results. CoA was found in 8 (100%) patients. In 7 (87.5%) cases, coarctation developed at isthmus and in one case, coarctation was detected at the horizontal part of aortic arch, between the truncus arteriosus of the left carotid communis artery. Aortal insufficiency was found in 7 (87.5%) patients; in four of them (50%), bicuspidia was confirmed (bicuspid aortic valve), 7 (87.5%) patients had slightly expressed hypertrophy of the left ventricle. Two (25%) patients had dilatation of the ascendant aorta, six (75%) wider outgoing vessels of the aortic arch, four (50%) had well developed arterial collaterals and 2 (25%) patients rib notching. In 2 (25%) patients as side finding thymus persistent was found. Average diameter of coarctation was 10 mm. In one patient, CoA was accompanied with stenosis of pulmonary artery, in one with ventricular septal defect, and one with tricuspid insufficiency. The results

  16. Genetic Bases of Bicuspid Aortic Valve: The Contribution of Traditional and High-Throughput Sequencing Approaches on Research and Diagnosis.

    Science.gov (United States)

    Giusti, Betti; Sticchi, Elena; De Cario, Rosina; Magi, Alberto; Nistri, Stefano; Pepe, Guglielmina

    2017-01-01

    Bicuspid aortic valve (BAV) is a common (0.5-2.0% of general population) congenital heart defect with increased prevalence of aortic dilatation and dissection. BAV has an autosomal dominant inheritance with reduced penetrance and variable expressivity. BAV has been described as an isolated trait or associated with syndromic conditions [e.g., Marfan Marfan syndrome or Loeys-Dietz syndrome (MFS, LDS)]. Identification of a syndromic condition in a BAV patient is clinically relevant to personalize aortic surgery indication. A 4-fold increase in BAV prevalence in a large cohort of unrelated MFS patients with respect to general population was reported, as well as in LDS patients (8-fold). It is also known that BAV is more frequent in patients with thoracic aortic aneurysm (TAA) related to mutations in ACTA2, FBN1 , and TGFBR2 genes. Moreover, in 8 patients with BAV and thoracic aortic dilation, not fulfilling the clinical criteria for MFS, FBN1 mutations in 2/8 patients were identified suggesting that FBN1 or other genes involved in syndromic conditions correlated to aortopathy could be involved in BAV. Beyond loci associated to syndromic disorders, studies in humans and animal models evidenced/suggested the role of further genes in non-syndromic BAV. The transcriptional regulator NOTCH1 has been associated with the development and acceleration of calcium deposition. Genome wide marker-based linkage analysis demonstrated a linkage of BAV to loci on chromosomes 18, 5, and 13q. Recently, a role for GATA4 / 5 in aortic valve morphogenesis and endocardial cell differentiation has been reported. BAV has also been associated with a reduced UFD1L gene expression or involvement of a locus containing AXIN1 / PDIA2 . Much remains to be understood about the genetics of BAV. In the last years, high-throughput sequencing technologies, allowing the analysis of large number of genes or entire exomes or genomes, progressively became available. The latter issue together with the

  17. Genetic Bases of Bicuspid Aortic Valve: The Contribution of Traditional and High-Throughput Sequencing Approaches on Research and Diagnosis

    Directory of Open Access Journals (Sweden)

    Betti Giusti

    2017-08-01

    Full Text Available Bicuspid aortic valve (BAV is a common (0.5–2.0% of general population congenital heart defect with increased prevalence of aortic dilatation and dissection. BAV has an autosomal dominant inheritance with reduced penetrance and variable expressivity. BAV has been described as an isolated trait or associated with syndromic conditions [e.g., Marfan Marfan syndrome or Loeys-Dietz syndrome (MFS, LDS]. Identification of a syndromic condition in a BAV patient is clinically relevant to personalize aortic surgery indication. A 4-fold increase in BAV prevalence in a large cohort of unrelated MFS patients with respect to general population was reported, as well as in LDS patients (8-fold. It is also known that BAV is more frequent in patients with thoracic aortic aneurysm (TAA related to mutations in ACTA2, FBN1, and TGFBR2 genes. Moreover, in 8 patients with BAV and thoracic aortic dilation, not fulfilling the clinical criteria for MFS, FBN1 mutations in 2/8 patients were identified suggesting that FBN1 or other genes involved in syndromic conditions correlated to aortopathy could be involved in BAV. Beyond loci associated to syndromic disorders, studies in humans and animal models evidenced/suggested the role of further genes in non-syndromic BAV. The transcriptional regulator NOTCH1 has been associated with the development and acceleration of calcium deposition. Genome wide marker-based linkage analysis demonstrated a linkage of BAV to loci on chromosomes 18, 5, and 13q. Recently, a role for GATA4/5 in aortic valve morphogenesis and endocardial cell differentiation has been reported. BAV has also been associated with a reduced UFD1L gene expression or involvement of a locus containing AXIN1/PDIA2. Much remains to be understood about the genetics of BAV. In the last years, high-throughput sequencing technologies, allowing the analysis of large number of genes or entire exomes or genomes, progressively became available. The latter issue together with

  18. Inadvertent puncture of the aortic noncoronary cusp during postoperative left atrial tachycardia ablation

    Directory of Open Access Journals (Sweden)

    Dursun Aras, MD

    2015-08-01

    Full Text Available Transseptal catheterization has become part of the interventional electrophysiologist׳s technical armamentarium since the development of left atrial catheter ablation and percutaneous technologies for treating mitral and aortic valve disease. Although frequently performed, the procedure׳s most feared complication is aortic root penetration. Focal atrial tachycardia has been described as the most common late sequela of surgical valve replacements. We present a complicated case involving the inadvertent delivery of an 8 French sheath across the noncoronary cusp during radiofrequency catheter ablation for left atrial tachycardia originating from the mitral annulus in a patient with prior mitral valve replacement.

  19. Aortic coarctation diagnosed by renal Doppler flow patterns in a hypertensive young patient: a case report

    International Nuclear Information System (INIS)

    Sari, S.; Kara, K.; Verim, S.

    2012-01-01

    Full text: Introduction: Aortic coarctation is a congenital malformation, which can cause systemic hypertension and subsequent complications, and causes of secondary hypertension, affecting in differential pressures in the upper and lower extremities. Because hypertension is caused by aortic coarctation, tends to be resistant to medical therapy, early recognition and surgical rectification are important. Objectives and tasks: In this article, we aimed to point out that renal Doppler sonography is a beneficial and frequently used to evaluate secondary hypertension, if there are bilateral tardus-parvus wave patterns are detected. Thus, bilateral renal artery stenosis, aortic stenosis, and coarctation should be considered in this condition. Materials and methods: A 23-year-old male who has six-month history of hypertension. He was referred by a cardiologist for investigation of his secondary hypertension. There was an ascending aortic dilatation, left ventricular hypertrophy in his echocardiography. Results: The patient's blood pressure was measured as 160/90 mm Hg in his both arms. Renal Doppler sonography was performed to identify the potential cause of secondary hypertension, specifically renal artery stenosis, after tardus-parvus pulse waves were noted in both renal intralobar-arteries. Aortic coarctation is suspected and then a chest computed tomography (CT) was performed to evaluate supra-diaphragmatic vessel abnormalities. The CT exposed a stenotic lesion in the isthmus of the aorta. The patient was transferred to cardiovascular surgery department for treatment. Conclusion: Careful physical examination should be performed in all hypertensive patients. If bilateral tardus-parvus wave pattern are seen in patients who has been referred for Doppler evaluation on suspicion of renovascular hypertension, aortic coarctation should be considered as differential diagnosis

  20. Cardiovascular and Musculoskeletal Assessment of Elite US Volleyball Players.

    Science.gov (United States)

    Davis, Christopher K; Dyar, Dan A; Vargas, Lisa A; Grossfeld, Paul D

    2015-11-01

    The aim of this study was to characterize the cardiovascular and musculoskeletal systems of elite volleyball players, including aortic dimensions. Previous studies have shown that the upper limit of normal aortic sinus diameter for male and female athletes is 4 and 3.4 cm, respectively. Cross-sectional analysis. United States Olympic Volleyball Training Facility and Rady Children's Hospital San Diego. Seventy (37 male) members of the US national volleyball team. Athletes underwent evaluation that included medical and family histories, targeted physical examinations specifically focusing on abnormalities present in Marfan syndrome (MFS), and transthoracic echocardiograms. Cardiac chamber and great artery size, valve function, and coronary artery origins were assessed. Three male athletes (8%) had an aortic sinus diameter ≥4 cm, one of whom also had an ascending aorta >4 cm. Two female athletes (6%) had aortic sinus diameter ≥3.4 cm, and another had an ascending aorta of 3.4 cm. There were no other intracardiac or arterial abnormalities. Individual musculoskeletal characteristics of MFS were common among the athletes but not more frequent or numerous in those with aortic dilation. The prevalence of aortic root dilation in this population of athletes was higher than what has previously been reported in other similar populations. Further study is needed to determine whether these represent pathological changes or normal variations in tall athletes. This study adds to the existing knowledge base of athlete's heart, with specific attention to aortic dimensions in elite volleyball players. The data are relevant to similar athletes' medical care and to preparticipation cardiac screening in general.

  1. Aortic valve bypass

    DEFF Research Database (Denmark)

    Lund, Jens T; Jensen, Maiken Brit; Arendrup, Henrik

    2013-01-01

    In aortic valve bypass (AVB) a valve-containing conduit is connecting the apex of the left ventricle to the descending aorta. Candidates are patients with symptomatic aortic valve stenosis rejected for conventional aortic valve replacement (AVR) or transcatheter aortic valve implantation (TAVI). ...

  2. The influence of Marfans and bicuspid valves on outcomes following aortic valve reimplantation.

    Science.gov (United States)

    Martín, Carlos E; García Montero, Carlos; Serrano, Santiago-Fiz; González, Ana; Mingo, Susana; Moñivas, Vanessa; Centeno, Jorge; Forteza, Alberto

    2017-10-01

    We analyzed our early and midterm results with aortic valve reimplantation surgery to determine the influence of Marfan syndrome and bicuspid valves on outcomes with this technique. Between March 2004 and December 2015, 267 patients underwent aortic valve reimplantation operations. The mean diameter of the sinuses of Valsalva was 50 ± 3 mm and moderate/severe aortic regurgitation was present in 34.4% of these patients. A bicuspid aortic valve was present in 21% and 40% had Marfan syndrome. Overall 30-day mortality was 0.37% (1/267). Mean follow-up was 59.7 ± 38.7 months. Overall survival at 1, 3, and 5 years was 98 ± 8%, 98 ± 1%, and 94 ± 2%, respectively. Freedom from reoperation and aortic regurgitation >II was 99 ± 5%, 98 ± 8%, 96.7 ± 8%, and 99 ± 6%, 98 ± 1%, 98 ± 1%, respectively at 1, 3, and 5 years follow-up, with no differences between Marfan and bicuspid aortic valve groups. (p = 0.94 and p = 0.96, respectively). No endocarditis or thromboembolic complications were documented, and 93.6% of the patients did not receive any anticoagulation therapy. The reimplantation technique for aortic root aneurysms is associated with excellent clinical and functional outcomes at short and mid-term follow-up. © 2017 Wiley Periodicals, Inc.

  3. CT-angiography-based evaluation of the aortic annulus for prosthesis sizing in transcatheter aortic valve implantation (TAVI)-predictive value and optimal thresholds for major anatomic parameters.

    Science.gov (United States)

    Schwarz, Florian; Lange, Philipp; Zinsser, Dominik; Greif, Martin; Boekstegers, Peter; Schmitz, Christoph; Reiser, Maximilian F; Kupatt, Christian; Becker, Hans C

    2014-01-01

    To evaluate the predictive value of CT-derived measurements of the aortic annulus for prosthesis sizing in transcatheter aortic valve implantation (TAVI) and to calculate optimal cutoff values for the selection of various prosthesis sizes. The local IRB waived approval for this single-center retrospective analysis. Of 441 consecutive TAVI-patients, 90 were excluded (death within 30 days: 13; more than mild aortic regurgitation: 10; other reasons: 67). In the remaining 351 patients, the CoreValve (Medtronic) and the Edwards Sapien XT valve (Edwards Lifesciences) were implanted in 235 and 116 patients. Optimal prosthesis size was determined during TAVI by inflation of a balloon catheter at the aortic annulus. All patients had undergone CT-angiography of the heart or body trunk prior to TAVI. Using these datasets, the diameter of the long and short axis as well as the circumference and the area of the aortic annulus were measured. Multi-Class Receiver-Operator-Curve analyses were used to determine the predictive value of all variables and to define optimal cutoff-values. Differences between patients who underwent implantation of the small, medium or large prosthesis were significant for all except the large vs. medium CoreValve (all p'sprosthesis size for both manufacturers (multi-class AUC's: 0.80, 0.88, 0.91, 0.88, 0.88, 0.89). Using the calculated optimal cutoff-values, prosthesis size is predicted correctly in 85% of cases. CT-based aortic root measurements permit excellent prediction of the prosthesis size considered optimal during TAVI.

  4. Aortic compliance in patients with aortic regurgitation. Evaluation with magnetic resonance imaging

    International Nuclear Information System (INIS)

    Murai, Sachiko; Hamada, Seiki; Ueguchi, Takashi

    2005-01-01

    The purpose of this study was to assess by means of cine magnetic resonance imaging (MRI) aortic compliance before and after aortic valve replacement (AVR with SJM (St. Jude Medical, St paul, MN) valve) in patients with aortic regurgitation (AR). Two groups (healthy controls and patients with severe isolated AR) of 10 subjects each were included in this study. Cine MRI was performed at three locations of the aorta, and aortic compliance was calculated by dividing the maximum change in the aortic area by pulse pressure. Cine MRI is useful to assess abnormalities of aortic compliance in patients with AR. Compared with the control group, aortic compliance in the AR group was significantly less in the ascending aorta (p<0.05), decreasing in order of aortic location. After AVR, aortic compliance improved for all locations. Cine MRI enables assessment of aortic biophysical properties such as a compliance for evaluating the progression of AR and the efficacy of treatment. (author)

  5. Satisfactory short-term outcomes of the STABILISE technique for type B aortic dissection.

    Science.gov (United States)

    Melissano, Germano; Bertoglio, Luca; Rinaldi, Enrico; Mascia, Daniele; Kahlberg, Andrea; Loschi, Diletta; De Luca, Monica; Monaco, Fabrizio; Chiesa, Roberto

    2018-03-29

    The aim of this study was to evaluate the perioperative and short-term results in a cohort of patients treated during the last year at our institution with the stent-assisted balloon-induced intimal disruption and relamination in aortic dissection repair (STABILISE) technique for acute complicated aortic dissection. Between June 2016 and June 2017, 10 patients (all male; mean age, 62.6 ± 7.4 years) received treatment for acute complicated aortic dissection with the STABILISE technique. After a standard provisional extension to induce complete attachment procedure using the commercially available endovascular dissection system (Cook Medical, Bloomington, Ind), the distal stent graft area and the bare stent area were ballooned to completely exclude the thoracic false lumen (FL) and to obtain a single-channeled abdominal aorta. Computed tomography was routinely performed within the first postoperative week before discharge and then at 3 months, at 6 months, and yearly thereafter. The technical and clinical success rates were analyzed. The 30-day technical and clinical success rates were 100%, with complete thrombosis of the thoracic FL and no type I endoleak. Malperfusion was resolved in all cases. No aortic ruptures were recorded, and no open conversion was required. One case of delayed spinal cord ischemia fully resolved within the discharge period. Predischarge computed tomography showed complete thrombosis of the thoracic FL in all cases. In two cases, some degree of patency of the abdominal FL was observed. At short-term follow-up, the overall aortic diameters remained stable with no further dilation. The STABILISE technique was safe and feasible in this cohort of patients, with complete thrombosis of the thoracic FL and creation of a single-channeled aorta in most cases. Further studies are needed to ascertain the long-term behavior of the treated aorta. Copyright © 2018 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.

  6. Is Decellularized Porcine Small Intestine Sub-mucosa Patch Suitable for Aortic Arch Repair?

    Science.gov (United States)

    Corno, Antonio F.; Smith, Paul; Bezuska, Laurynas; Mimic, Branko

    2018-01-01

    Introduction: We reviewed our experience with decellularized porcine small intestine sub-mucosa (DPSIS) patch, recently introduced for congenital heart defects. Materials and Methods: Between 10/2011 and 04/2016 a DPSIS patch was used in 51 patients, median age 1.1 months (5 days to 14.5 years), for aortic arch reconstruction (45/51 = 88.2%) or aortic coarctation repair (6/51 = 11.8%). All medical records were retrospectively reviewed, with primary endpoints interventional procedure (balloon dilatation) or surgery (DPSIS patch replacement) due to patch-related complications. Results: In a median follow-up time of 1.5 ± 1.1 years (0.6–2.3years) in 13/51 patients (25.5%) a re-intervention, percutaneous interventional procedure (5/51 = 9.8%) or re-operation (8/51 = 15.7%) was required because of obstruction in the correspondence of the DPSIS patch used to enlarge the aortic arch/isthmus, with median max velocity flow at Doppler interrogation of 4.0 ± 0.51 m/s. Two patients required surgery after failed interventional cardiology. The mean interval between DPSIS patch implantation and re-intervention (percutaneous procedure or re-operation) was 6 months (1–17 months). While there were 3 hospital deaths (3/51 = 5.9%) not related to the patch implantation, no early or late mortality occurred for the subsequent procedure required for DPSIS patch interventional cardiology or surgery. The median max velocity flow at Doppler interrogation through the aortic arch/isthmus for the patients who did not require interventional procedure or surgery was 1.7 ± 0.57 m/s. Conclusions: High incidence of re-interventions with DPSIS patch for aortic arch and/or coarctation forced us to use alternative materials (homografts and decellularized gluteraldehyde preserved bovine pericardial matrix). PMID:29900163

  7. Valva aórtica bicúspide: fundamentos teóricos e clínicos para substituição simultânea da aorta ascendente Bicuspid aortic valve: theoretical and clinical aspects of concomitant ascending aorta replacement

    Directory of Open Access Journals (Sweden)

    Mauro Paes Leme De Sá

    2009-06-01

    and vessels of the aortic arch. Previous studies have shown that patients with normal BAV or slight dysfunction may present with dilation of the aortic root. The hemodynamic changes caused by BAV without stenosis or insufficiency seem to be an insufficient explanation for these findings. Several mechanisms have been proposed to explain the molecular and hystological aspects of this disease. We found a reduced fibrillin-1 content in both ascending aorta and pulmonary trunk as a possible cause. Histologically, the ascending aorta can present cystic medial necrosis and elastic fragmentation, similar to Marfan's disease. Some authors concluded that many patients, mainly those with aortic regurgitation, should have the aortic valve and the ascending aorta replaced at the same procedure, even if a mild dilatation (45 mm is present in patients with BAV if life expectancy is anticipated to be greater than 10 years to prevent further aneurysms or ruptures.

  8. Interventional Closure of a Patent Ductus Arteriosus Using an Amplatz Canine Duct Occluder in an Alpaca Cria

    OpenAIRE

    Chapel, E.C.; Lozier, J.; Lakritz, J.; Schober, K.E.

    2017-01-01

    A 6?month old female alpaca cria presented to The Ohio State University for evaluation of a cardiac murmur. Echocardiography revealed a left?to?right shunting patent ductus arteriosus, a restrictive left?to?right shunting perimembranous ventricular septal defect, and secondary moderate left atrial and ventricular dilation. Aortic root angiography demonstrated a type IIA patent ductus arteriosus (PDA). Interventional closure of the PDA was successfully performed, without complication, using an...

  9. Delayed Presentation of a Giant Ascending Aortic Aneurysm following Aortic Valve Replacement

    Directory of Open Access Journals (Sweden)

    Tugrul Göncü

    2009-01-01

    Full Text Available Giant ascending aortic aneurysm formation following aortic valve replacement is rare. A 28-year-old man who underwent aortic valve replacement with a prosthetic valve for aortic regurgitation secondary to congenital bicuspid aortic valve about 10 years ago was diagnosed with a giant ascending aortic aneurysm about 16 cm in diameter in follow-up. The aneurysm was resected leaving the functional old mechanical prosthesis in place and implanted a 34-mm Hemashield woven graft, associated with the left and right coronary artery button implantation. Histological findings of the aortic aneurysm wall showed cystic medial necrosis. The postoperative course was uneventful and postoperative examination demonstrated good surgical results.

  10. Preliminary Evidence for Aortopathy and an X-Linked Parent-of-Origin Effect on Aortic Valve Malformation in a Mouse Model of Turner Syndrome

    Directory of Open Access Journals (Sweden)

    Robert B. Hinton

    2015-07-01

    Full Text Available Turner syndrome (TS, most frequently caused by X-monosomy (45,X, is characterized in part by cardiovascular abnormalities, including aortopathy and bicuspid aortic valve (BAV. There is a need for animal models that recapitulate the cardiovascular manifestations of TS. Extracellular matrix (ECM organization and morphometrics of the aortic valve and proximal aorta were examined in adult 39,XO mice (where the parental origin of the single X was paternal (39,XPO or maternal (39,XMO and 40,XX controls. Aortic valve morphology was normal (tricuspid in all of the 39,XPO and 40,XX mice studied, but abnormal (bicuspid or quadricuspid in 15% of 39,XMO mice. Smooth muscle cell orientation in the ascending aorta was abnormal in all 39,XPO and 39,XMO mice examined, but smooth muscle actin was decreased in 39,XMO mice only. Aortic dilation was present with reduced penetrance in 39,XO mice. The 39,XO mouse demonstrates aortopathy and an X-linked parent-of-origin effect on aortic valve malformation, and the candidate gene FAM9B is polymorphically expressed in control and diseased human aortic valves. The 39,XO mouse model may be valuable for examining the mechanisms underlying the cardiovascular findings in TS, and suggest there are important genetic modifiers on the X chromosome that modulate risk for nonsyndromic BAV and aortopathy.

  11. Endovascular repair of abdominal aortic aneurysms: vascular anatomy, device selection, procedure, and procedure-specific complications.

    Science.gov (United States)

    Bryce, Yolanda; Rogoff, Philip; Romanelli, Donald; Reichle, Ralph

    2015-01-01

    Abdominal aortic aneurysm (AAA) is abnormal dilatation of the aorta, carrying a substantial risk of rupture and thereby marked risk of death. Open repair of AAA involves lengthy surgery time, anesthesia, and substantial recovery time. Endovascular aneurysm repair (EVAR) provides a safer option for patients with advanced age and pulmonary, cardiac, and renal dysfunction. Successful endovascular repair of AAA depends on correct selection of patients (on the basis of their vascular anatomy), choice of the correct endoprosthesis, and familiarity with the technique and procedure-specific complications. The type of aneurysm is defined by its location with respect to the renal arteries, whether it is a true or false aneurysm, and whether the common iliac arteries are involved. Vascular anatomy can be divided more technically into aortic neck, aortic aneurysm, pelvic perfusion, and iliac morphology, with grades of difficulty with respect to EVAR, aortic neck morphology being the most common factor to affect EVAR appropriateness. When choosing among the devices available on the market, one must consider the patient's vascular anatomy and choose between devices that provide suprarenal fixation versus those that provide infrarenal fixation. A successful technique can be divided into preprocedural imaging, ancillary procedures before AAA stent-graft placement, the procedure itself, postprocedural medical therapy, and postprocedural imaging surveillance. Imaging surveillance is important in assessing complications such as limb thrombosis, endoleaks, graft migration, enlargement of the aneurysm sac, and rupture. Last, one must consider the issue of radiation safety with regard to EVAR. (©)RSNA, 2015.

  12. CT-angiography-based evaluation of the aortic annulus for prosthesis sizing in transcatheter aortic valve implantation (TAVI-predictive value and optimal thresholds for major anatomic parameters.

    Directory of Open Access Journals (Sweden)

    Florian Schwarz

    Full Text Available BACKGROUND/OBJECTIVES: To evaluate the predictive value of CT-derived measurements of the aortic annulus for prosthesis sizing in transcatheter aortic valve implantation (TAVI and to calculate optimal cutoff values for the selection of various prosthesis sizes. METHODS: The local IRB waived approval for this single-center retrospective analysis. Of 441 consecutive TAVI-patients, 90 were excluded (death within 30 days: 13; more than mild aortic regurgitation: 10; other reasons: 67. In the remaining 351 patients, the CoreValve (Medtronic and the Edwards Sapien XT valve (Edwards Lifesciences were implanted in 235 and 116 patients. Optimal prosthesis size was determined during TAVI by inflation of a balloon catheter at the aortic annulus. All patients had undergone CT-angiography of the heart or body trunk prior to TAVI. Using these datasets, the diameter of the long and short axis as well as the circumference and the area of the aortic annulus were measured. Multi-Class Receiver-Operator-Curve analyses were used to determine the predictive value of all variables and to define optimal cutoff-values. RESULTS: Differences between patients who underwent implantation of the small, medium or large prosthesis were significant for all except the large vs. medium CoreValve (all p's<0.05. Furthermore, mean diameter, annulus area and circumference had equally high predictive value for prosthesis size for both manufacturers (multi-class AUC's: 0.80, 0.88, 0.91, 0.88, 0.88, 0.89. Using the calculated optimal cutoff-values, prosthesis size is predicted correctly in 85% of cases. CONCLUSION: CT-based aortic root measurements permit excellent prediction of the prosthesis size considered optimal during TAVI.

  13. Differential effect of walnut oil and safflower oil on the serum cholesterol level and lesion area in the aortic root of apolipoprotein E-deficient mice.

    Science.gov (United States)

    Iwamoto, Masako; Kono, Misaki; Kawamoto, Daisuke; Tomoyori, Hiroko; Sato, Masao; Imaizumi, Katsumi

    2002-01-01

    Walnut oil (WO) is a good source of alpha-linolenic acid. We compared the effects of WO and high-linoleic safflower oil (HLSO) on the serum lipid level and atherosclerosis development in male and female apolipoprotein (apo) E-deficient mice. The WO diet resulted in a higher level of serum cholesterol than with HLSO. Female mice fed on the WO diet had a greater lesion area in the aortic root than did those on the HLSO diet. There was no diet-dependent difference in the level of cholesterol and its oxidation products in the abdominal and thoracic aorta. These results suggest that the unpleasant effects of the WO diet on apo E-deficient mice may be attributable to alpha-linolenic acid.

  14. Aortic valve replacement for aortic stenosis caused by alkaptonuria.

    Science.gov (United States)

    Hiroyoshi, Junko; Saito, Aya; Panthee, Nirmal; Imai, Yasushi; Kawashima, Dai; Motomura, Noboru; Ono, Minoru

    2013-03-01

    We report a case of aortic stenosis associated with ochronosis in a 70-year-old man who underwent biologic aortic valve replacement. Intraoperative findings included ochronosis of a severely calcified pigmented aortic valve along with pigmentation of the intima of the aorta. Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  15. Percutaneous balloon dilation of pulmonary stenosis

    International Nuclear Information System (INIS)

    Hua Yangde; Huang Ming; Li Jinkang; Qian Jinqing; Chen Xiuyu; Yang Siyuan

    2003-01-01

    Objective: Review our experience of balloon dilation of valvular pulmonary stenosis in 32 cases. Methods: Totally 32 cases of pulmonary stenosis admitted from 1995-2001 with age of 1.5-13 yrs mean 6.8. Diagnosis was made by clinical manifestations, EKG, ECHO and angiocardiography. Results: Before dilation, the mean systolic pressure of right ventricle was (93.5 ± 28.5) mmHg, after the procedure it reduced to (42 ± 9.0) mmHg. The pressure gradient between right ventricle and pulmonary artery before dilation was (76 ± 30) mmHg and become (24.5 ± 8.5) mmHg after dilation. The gradient pressure after dilation was less than 25 mmHg in 90.6% cases. A case of Noonan syndrome showed no response to balloon dilation and died during valvulectomy from accompanying left ventricular cardiomyopathy. Conclusions: Balloon dilation of valvular pulmonary stenosis is effective and safe. The selection of proper diameter of pulmonary valvular rings and sized of the balloon are the major factors

  16. Surgery for acute Type I aortic dissection without resection of supra-aortic entry sites leads to unfavourable aortic remodelling.

    Science.gov (United States)

    Heo, Woon; Song, Suk-Won; Lee, Kwang-Hun; Lee, Shin-Young; Kim, Tae-Hoon; Baek, Min-Young; Yoo, Kyung-Jong

    2018-01-29

    This study aimed to evaluate the impact of remnant re-entries in arch branches on postoperative change in the aortic arch and descending aortic diameters and the rate of major adverse aortic events. Between January 2010 and December 2016, 249 patients underwent surgery for acute Type I aortic dissection. Patients who underwent total arch replacement, had Marfan syndrome or had intramural haematoma were excluded. Seventy-two patients with predischarge and follow-up computed tomography scans were enrolled. Patients with and without re-entries in the arch branches after surgery were assigned to the supra-aortic entry (SAE, n = 21) and no supra-aortic entry (n = 51) groups, respectively. Diameters were measured at 7 levels: the innominate artery, left common carotid artery, left subclavian artery, 20 mm distal to the left subclavian artery, pulmonary artery bifurcation, coeliac axis and maximal diameter of the descending thoracic aorta. Growth rates at the levels of the pulmonary artery bifurcation and 20 mm distal to the left subclavian artery were significantly higher in the SAE group than in the no supra-aortic entry group. The rate of freedom from major adverse aortic events (annual growth >5 mm or maximal diameter of the descending thoracic aorta >50 mm) at 5 years was significantly higher in the no supra-aortic entry group than in the SAE group. Remnant SAE leads to unfavourable aortic remodelling after acute Type I aortic dissection repair. © The Author(s) 2018. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

  17. Familial Thoracic Aortic Aneurysm with Dissection Presenting as Flash Pulmonary Edema in a 26-Year-Old Man

    Directory of Open Access Journals (Sweden)

    Sabry Omar

    2014-01-01

    Full Text Available We are reporting a case of familial thoracic aortic aneurysm and dissection in a 26-year-old man with no significant past medical history and a family history of dissecting aortic aneurysm in his mother at the age of 40. The patient presented with cough, shortness of breath, and chest pain. Chest X-ray showed bilateral pulmonary infiltrates. CT scan of the chest showed a dissection of the ascending aorta. The patient underwent aortic dissection repair and three months later he returned to our hospital with new complaints of back pain. CT angiography showed a new aortic dissection extending from the left carotid artery through the bifurcation and into the iliac arteries. The patient underwent replacement of the aortic root, ascending aorta, total aortic arch, and aortic valve. The patient recovered well postoperatively. Genetic studies of the patient and his children revealed no mutations in ACTA2, TGFBR1, TGFBR2, TGFB2, MYH11, MYLK, SMAD3, or FBN1. This case report focuses on a patient with familial TAAD and discusses the associated genetic loci and available screening methods. It is important to recognize potential cases of familial TAAD and understand the available screening methods since early diagnosis allows appropriate management of risk factors and treatment when necessary.

  18. Randomized controlled trial comparing esophageal dilation to no dilation among adults with esophageal eosinophilia and dysphagia.

    Science.gov (United States)

    Kavitt, R T; Ates, F; Slaughter, J C; Higginbotham, T; Shepherd, B D; Sumner, E L; Vaezi, M F

    2016-11-01

    The role of esophageal dilation in patients with esophageal eosinophilia with dysphagia remains unknown. The practice of dilation is currently based on center preferences and expert opinion. The aim of this study is to determine if, and to what extent, dysphagia improves in response to initial esophageal dilation followed by standard medical therapies. We conducted a randomized, blinded, controlled trial evaluating adult patients with dysphagia and newly diagnosed esophageal eosinophilia from 2008 to 2013. Patients were randomized to dilation or no dilation at time of endoscopy and blinded to dilation status. Endoscopic features were graded as major and minor. Subsequent to randomization and endoscopy, all patients received fluticasone and dexlansoprazole for 2 months. The primary study outcome was reduction in overall dysphagia score, assessed at 30 and 60 days post-intervention. Patients with severe strictures (less than 7-mm esophageal diameter) were excluded from the study. Thirty-one patients were randomized and completed the protocol: 17 randomized to dilation and 14 to no dilation. Both groups were similar with regard to gender, age, eosinophil density, endoscopic score, and baseline dysphagia score. The population exhibited moderate to severe dysphagia and moderate esophageal stricturing at baseline. Overall, there was a significant (P dysphagia score at 30 and 60 days post-randomization compared with baseline in both groups. No significant difference in dysphagia scores between treatment groups after 30 (P = 0.93) or 60 (P = 0.21) days post-intervention was observed. Esophageal dilation did not result in additional improvement in dysphagia score compared with treatment with proton pump inhibitor and fluticasone alone. In patients with symptomatic esophageal eosinophilia without severe stricture, dilation does not appear to be a necessary initial treatment strategy. © 2015 International Society for Diseases of the Esophagus.

  19. Acute Aortic Arch Perforation During Transcatheter Aortic Valve Replacement in Bicuspid Aortic Stenosis and a Gothic Aortic Arch

    DEFF Research Database (Denmark)

    Millan-Iturbe, Oscar; Sawaya, Fadi J.; Bieliauskas, Gintautas

    2017-01-01

    AS because of its unique anatomic features. This case report describes an acute aortic perforation during delivery of a transcatheter heart valve to treat a severe bicuspid AS with a “gothic aortic arch”; more careful evaluation of the preprocedural multislice computed tomographic scan would have unveiled...

  20. Benefits and medium-term outcome of the Sorin Pericarbon Freedom stentless aortic prosthesis in cases of acute bacterial endocarditis.

    Science.gov (United States)

    Stefanelli, Guglielmo; Pirro, Fabrizio; Meli, Marco; Trevisan, Davide; Telesca, Mariassunta; Campisano, Barbara; Mussini, Cristina; Barbieri, Andrea

    2016-10-01

    The aim of this study is to evaluate the ease of use and the advantages of Sorin Pericarbon Freedom (SPF) stentless valve in cases of acute bacterial endocarditis and to check the intermediate-term results after the implant of SPF with respect to resistance to infection, valve deterioration and durability. Between June 2003 and February 2015, 26 patients with active aortic valve bacterial endocarditis underwent aortic valve replacement with SPF pericardial stentless aortic prosthesis. The mean age was 57 ± 18 years; 73% of the patients were in preoperative NYHA class III and VI. Mean Logistic EuroSCORE was 14.2 ± 12.7. Endocarditis occurred in 18 patients with native valves, and in 9 patients with prosthetic valves (4 mechanical aortic valve prostheses; 5 aortic bioprostheses). Aortic root abscesses were observed in 16 cases (61.5%). Surgery was emergent in 3 cases (11.5%). Redo surgery was performed in 9 cases (35%). Cumulative follow-up was 126.8 patient-years (mean 4.9 ± 3.3 years). Operative hospital mortality was 0% for all patients. Residual mean prosthetic gradient at discharge was 9.4 ± 3.6 mmHg. Neither residual aortic incompetence nor residual abscess cavity was observed at discharge. Mean ejection fraction at discharge was 54 ± 8% (Min; Max: 35%; 65%). A total of 4 patients died at follow-up, all for non-cardiac causes. One patient was lost to follow-up. Two patients (8%) underwent non-valve-related reoperation with 0% mortality. Residual mean gradient at follow-up was 7.2 ± 2.1 mmHg. Three patients (17%) presented with mild/moderate aortic incompetence and 89% of patients were in NYHA Class I-II at follow-up. At 9 years, actuarial freedom from valve-related reoperation and from structural valve deterioration was 100%. The SPF aortic prosthesis is a true pericardial stentless prosthesis suitable for the treatment of acute bacterial endocarditis. Intermediate-time results in terms of freedom from reoperation, structural valve deterioration and

  1. Hybrid Repair of Complex Thoracic Aortic Arch Pathology: Long-Term Outcomes of Extra-anatomic Bypass Grafting of the Supra-aortic Trunk

    Energy Technology Data Exchange (ETDEWEB)

    Lotfi, S., E-mail: shamim.lotfi@kcl.ac.uk; Clough, R. E.; Ali, T. [Guy' s and St. Thomas' NHS Trust, Vascular Surgery (United Kingdom); Salter, R. [Guy' s and St. Thomas' NHS Trust, Interventional Radiology (United Kingdom); Young, C. P. [Guy' s and St. Thomas' NHS Trust, Cardiac Surgery (United Kingdom); Bell, R.; Modarai, B.; Taylor, P., E-mail: peter.taylor@gstt.nhs.uk [Guy' s and St. Thomas' NHS Trust, Vascular Surgery (United Kingdom)

    2013-02-15

    Hybrid repair constitutes supra-aortic debranching before thoracic endovascular aortic repair (TEVAR). It offers improved short-term outcome compared with open surgery; however, longer-term studies are required to assess patient outcomes and patency of the extra-anatomic bypass grafts. A prospectively maintained database of 380 elective and urgent patients who had undergone TEVAR (1997-2011) was analyzed retrospectively. Fifty-one patients (34 males; 17 females) underwent hybrid repair. Median age was 71 (range, 18-90) years with mean follow-up of 15 (range, 0-61) months. Perioperative complications included death: 10 % (5/51), stroke: 12 % (6/51), paraplegia: 6 % (3/51), endoleak: 16 % (8/51), rupture: 4 % (2/51), upper-limb ischemia: 2 % (1/51), bypass graft occlusion: 4 % (2/51), and cardiopulmonary complications in 14 % (7/51). Three patients (6 %) required emergency intervention for retrograde dissection: (2 aortic root repairs; 2 innominate stents). Early reintervention was performed for type 1 endoleak in two patients (2 proximal cuff extensions). One patient underwent innominate stenting and revision of their bypass for symptomatic restenosis. At 48 months, survival was 73 %. Endoleak was detected in three (6 %) patients (type 1 = 2; type 2 = 1) requiring debranching with proximal stent graft (n = 2) and proximal extension cuff (n = 1). One patient had a fatal rupture of a mycotic aneurysm and two arch aneurysms expanded. No bypass graft occluded after the perioperative period. Hybrid operations to treat aortic arch disease can be performed with results comparable to open surgery. The longer-term outcomes demonstrate low rates of reintervention and high rates of graft patency.

  2. Hybrid Repair of Complex Thoracic Aortic Arch Pathology: Long-Term Outcomes of Extra-anatomic Bypass Grafting of the Supra-aortic Trunk

    International Nuclear Information System (INIS)

    Lotfi, S.; Clough, R. E.; Ali, T.; Salter, R.; Young, C. P.; Bell, R.; Modarai, B.; Taylor, P.

    2013-01-01

    Hybrid repair constitutes supra-aortic debranching before thoracic endovascular aortic repair (TEVAR). It offers improved short-term outcome compared with open surgery; however, longer-term studies are required to assess patient outcomes and patency of the extra-anatomic bypass grafts. A prospectively maintained database of 380 elective and urgent patients who had undergone TEVAR (1997–2011) was analyzed retrospectively. Fifty-one patients (34 males; 17 females) underwent hybrid repair. Median age was 71 (range, 18–90) years with mean follow-up of 15 (range, 0–61) months. Perioperative complications included death: 10 % (5/51), stroke: 12 % (6/51), paraplegia: 6 % (3/51), endoleak: 16 % (8/51), rupture: 4 % (2/51), upper-limb ischemia: 2 % (1/51), bypass graft occlusion: 4 % (2/51), and cardiopulmonary complications in 14 % (7/51). Three patients (6 %) required emergency intervention for retrograde dissection: (2 aortic root repairs; 2 innominate stents). Early reintervention was performed for type 1 endoleak in two patients (2 proximal cuff extensions). One patient underwent innominate stenting and revision of their bypass for symptomatic restenosis. At 48 months, survival was 73 %. Endoleak was detected in three (6 %) patients (type 1 = 2; type 2 = 1) requiring debranching with proximal stent graft (n = 2) and proximal extension cuff (n = 1). One patient had a fatal rupture of a mycotic aneurysm and two arch aneurysms expanded. No bypass graft occluded after the perioperative period. Hybrid operations to treat aortic arch disease can be performed with results comparable to open surgery. The longer-term outcomes demonstrate low rates of reintervention and high rates of graft patency.

  3. Investigation on the Regional Loss Factor and Its Anisotropy for Aortic Aneurysms

    Directory of Open Access Journals (Sweden)

    Nastaran Shahmansouri

    2016-10-01

    Full Text Available An aortic aneurysm is a lethal arterial disease that mainly occurs in the thoracic and abdominal regions of the aorta. Thoracic aortic aneurysms are prevalent in the root/ascending parts of the aorta and can lead to aortic rupture resulting in the sudden death of patients. Understanding the biomechanical and histopathological changes associated with ascending thoracic aortic aneurysms (ATAAs, this study investigates the mechanical properties of the aorta during strip-biaxial tensile cycles. The loss factor—defined as the ratio of dissipated energy to the energy absorbed during a tensile cycle—the incremental modulus, and their anisotropy indexes were compared with the media fiber compositions for aneurysmal (n = 26 and control (n = 4 human ascending aortas. The aneurysmal aortas were categorized into the aortas with bicuspid aortic valves (BAV and tricuspid aortic valves (TAV. The strip-biaxial loss factor correlates well with the diameter of the aortas with BAV and TAV (for the axial direction, respectively, R2 = 0.71, p = 0.0022 and R2 = 0.54, p = 0.0096. The loss factor increases significantly with patients’ age in the BAV group (for the axial direction: R2 = 0.45, p = 0.0164. The loss factor is isotropic for all TAV quadrants, whereas it is on average only isotropic in the anterior and outer curvature regions of the BAV group. The results suggest that loss factor may be a useful surrogate measure to describe the histopathology of aneurysmal tissue and to demonstrate the differences between ATAAs with the BAV and TAV.

  4. Brain Abscess after Esophageal Dilatation

    DEFF Research Database (Denmark)

    Gaïni, S; Grand, M; Michelsen, J

    2007-01-01

    Brain abscess formation is a serious disease often seen as a complication to other diseases and to procedures. A rare predisposing condition is dilatation therapy of esophageal strictures. A case of brain abscess formation after esophageal dilatations is presented. A 59-year-old woman was admitted...... with malaise, progressive lethargy, fever, aphasia and hemiparesis. Six days before she had been treated with esophageal dilatation for a stricture caused by accidental ingestion of caustic soda. The brain abscess was treated with surgery and antibiotics. She recovered completely. This clinical case...... illustrates the possible association between therapeutic esophageal dilatation and the risk of brain abscess formation....

  5. Esophageal achalasia : results of balloon dilation

    Energy Technology Data Exchange (ETDEWEB)

    Ki, Won Woo; Kang, Sung Gwon; Yoon, Kwon Ha; Kim, Nam Hyeon; Lee, Hyo Jeong; Yoon, Hyun Ki; Sung, Kyu Bo; Song, Ho Young [Ulsan Univ. College of Medicine, Seoul (Korea, Republic of)

    1996-08-01

    To evaluate the clinical effectiveness of fluoroscopically guided balloon dilation in the treatment of esophageal achalasia. Under fluoroscopic guidance, 21 balloon dilation procedures were performed in 14 patients with achalasia. A balloon with a diameter of 20 mm was used for the initial attempt.If the patient tolerated this well, the procedure was repeated with a 10-20 mm balloon, placed alongside at the same session. If, however the patient complained of severe chest pain and/or a postprocedural esophagogram showed an improvement,the additional balloon was not used. For patients whose results were unsatisfactory, the dilation procedure was repeated at sessions three to seven days apart. Succesful dilation was achieved in 13 of 14 patients(92.9%), who needed a total of 20 sessions of balloon dilation, ranging from one to three sessions per patient(mean, 1.54 sessions). Esophageal rupture occured in one of 14 patients(7.1%) ; of the 13 patients who underwent a successful dilation procedure, 12(92.3%) were free of recurrent symptoms during the follow-up period of 1-56(mean, 18.5) months. The remaning patient(7.7%) had a recurrence seven months after dilation. Fluoroscopically guided balloon dilation seems to be safe and effective in the treatment of esophageal achalasia.

  6. Esophageal achalasia : results of balloon dilation

    International Nuclear Information System (INIS)

    Ki, Won Woo; Kang, Sung Gwon; Yoon, Kwon Ha; Kim, Nam Hyeon; Lee, Hyo Jeong; Yoon, Hyun Ki; Sung, Kyu Bo; Song, Ho Young

    1996-01-01

    To evaluate the clinical effectiveness of fluoroscopically guided balloon dilation in the treatment of esophageal achalasia. Under fluoroscopic guidance, 21 balloon dilation procedures were performed in 14 patients with achalasia. A balloon with a diameter of 20 mm was used for the initial attempt.If the patient tolerated this well, the procedure was repeated with a 10-20 mm balloon, placed alongside at the same session. If, however the patient complained of severe chest pain and/or a postprocedural esophagogram showed an improvement,the additional balloon was not used. For patients whose results were unsatisfactory, the dilation procedure was repeated at sessions three to seven days apart. Succesful dilation was achieved in 13 of 14 patients(92.9%), who needed a total of 20 sessions of balloon dilation, ranging from one to three sessions per patient(mean, 1.54 sessions). Esophageal rupture occured in one of 14 patients(7.1%) ; of the 13 patients who underwent a successful dilation procedure, 12(92.3%) were free of recurrent symptoms during the follow-up period of 1-56(mean, 18.5) months. The remaning patient(7.7%) had a recurrence seven months after dilation. Fluoroscopically guided balloon dilation seems to be safe and effective in the treatment of esophageal achalasia

  7. Significant intra-valvular pressure loss across EPIC SUPRA and perimount magna supra-annular designed aortic bioprostheses in patients with normal aortic size

    Directory of Open Access Journals (Sweden)

    Jagdish C. Mohan

    2017-01-01

    Full Text Available Doppler-derived trans-prosthetic gradients are higher and the estimated effective valve area is smaller than the catheter-derived and directly measured hemodynamic values, mostly due to pressure recovery phenomenon. Pressure recovery to a varying extent is common to all prosthetic heart valves including bioprostheses. Pressure recovery-related differences are usually small except in patients with bileaflet metallic prosthesis, wherein high-pressure local jets across central orifice have been documented since long back and also in patients with narrow aortic root. We describe two patients with normally functioning stented aortic bioprostheses with supra-annular design (EPIC SUPRA and PERIMOUNT MAGNA, wherein very high trans-prosthetic gradients and critically reduced estimated effective valve orifice areas in presence of normal aortic size were consistently recorded over long periods of follow-up. The valve leaflets, however had normal excursion, were thin, opened with a triangular or oblong shape and had expected geometric valve area (1.7 and 1.6 cm2 respectively measured by 3D trans-oesophageal echocardiographic planimetry. Pressure recovery upstream the valves accounted for 20% and 12% of total pressure gradients respectively. Dominant site for pressure drop was intra-valvular (75–85%. Such a phenomenon has not been reported in vivo for these two valve designs.

  8. Significant intra-valvular pressure loss across EPIC SUPRA and perimount magna supra-annular designed aortic bioprostheses in patients with normal aortic size.

    Science.gov (United States)

    Mohan, Jagdish C; Mohan, Vishwas; Shukla, Madhu; Sethi, Arvind

    Doppler-derived trans-prosthetic gradients are higher and the estimated effective valve area is smaller than the catheter-derived and directly measured hemodynamic values, mostly due to pressure recovery phenomenon. Pressure recovery to a varying extent is common to all prosthetic heart valves including bioprostheses. Pressure recovery-related differences are usually small except in patients with bileaflet metallic prosthesis, wherein high-pressure local jets across central orifice have been documented since long back and also in patients with narrow aortic root. We describe two patients with normally functioning stented aortic bioprostheses with supra-annular design (EPIC SUPRA and PERIMOUNT MAGNA), wherein very high trans-prosthetic gradients and critically reduced estimated effective valve orifice areas in presence of normal aortic size were consistently recorded over long periods of follow-up. The valve leaflets, however had normal excursion, were thin, opened with a triangular or oblong shape and had expected geometric valve area (1.7 and 1.6cm 2 respectively) measured by 3D trans-oesophageal echocardiographic planimetry. Pressure recovery upstream the valves accounted for 20% and 12% of total pressure gradients respectively. Dominant site for pressure drop was intra-valvular (75-85%). Such a phenomenon has not been reported in vivo for these two valve designs. Copyright © 2016. Published by Elsevier B.V.

  9. The use of transcatheter aortic valve replacement vs surgical aortic valve replacement for the treatment of aortic stenosis

    Directory of Open Access Journals (Sweden)

    Jensen HA

    2015-08-01

    Full Text Available Hanna A Jensen, Lillian L Tsai, Vinod H Thourani Division of Cardiothoracic Surgery, Joseph B Whitehead Department of Surgery, Structural Heart and Valve Center, Emory University School of Medicine, Atlanta, GA, USA Abstract: Severe aortic stenosis (AS is associated with considerable morbidity and mortality and is increasing in prevalence as the global population increases. Since AS primarily affects the elderly, many of these patients have comorbidities that make them poor candidates for the gold standard treatment for AS, surgical aortic valve replacement. Transcatheter aortic valve replacement has emerged as a novel technology for the management of AS in higher risk patients over the past decade. Randomized trials have established the safety and efficacy of transcatheter aortic valve replacement, and the medical community has rallied to identify the patients who are most suitable for this transformative treatment. This review focuses on outlining the key procedural differences, describing the unique challenges of both operations, and finally assessing and comparing outcomes both on a general level and in challenging patient subgroups. Keywords: aortic valve replacement, transcatheter aortic valve replacement, surgical aortic valve replacement 

  10. Coexistence of Single Coronary Artery Anomaly and Aortic Arch Anomaly

    Directory of Open Access Journals (Sweden)

    Yilmaz Omur Otlu

    2014-03-01

    Full Text Available A 74-year-old male patient was admitted to our hospital for evaluation of recent onset atypical chest pain. His medical history included hypertension, dislipidemia and smoking. Physical examination was unremarkable. The resting electrocardiogram was demonstrated biphasic T waves on lateral derivations. Transthoracic echocardiography showed normal left and right ventricular dimensions and functions. Coronary angiography was planned for the patient. First, right transradial approach tried; but guidewire could not be advanced to ascendig aorta. Coronary angiography was performed through the right femoral artery. Multiple attempts to cannulate the left coronary ostium were unsuccessful. The right coronary artery cannulated from its normal ostium in the right sinus of Valsalva. After a very short common main stem, the artery divided into a right coronary artery, and separate left anterior descending artery and circumflex artery (Figure A. The coronary arteries were normal without any significant stenosis and any extrinsic compression. An aortic root injection confirmed the absence of left coronary ostium. Also, a retroesophageal right subclavian artery originating from the left aortic arch (arteria lusoria was detected as the last branch of aortic arch on contrast enhanced computerized tomography (Figure B-C. The patient discharged with medical teraphy.

  11. Contemporary insights into the management of type A aortic dissection.

    Science.gov (United States)

    Tolis, George; Sundt, Thoralf M

    2016-10-01

    Acute Type A Dissection remains a surgical emergency with a relatively high operative mortality despite advances in cardiac surgical techniques and medical management over the past thirty years. In this presentation we will discuss the issues surrounding diagnosis, triage, surgical treatment and perioperative medical management as well as long term surveillance of patients suffering from Acute Type A Dissection and present the literature that supports our management strategies. Expert commentary: The ultimate goal of surgical intervention for patients with Type A Acute Aortic Dissection is an alive patient. A more complicated operation which addresses the root and arch and potentially reduces late complications should be approached with caution since it may increase the operative mortality of the procedure itself. With the recent evolution in endovascular techniques, there is hope that later complications can be reduced without increasing the risk of the primary operation. It remains to be seen whether the improved distal aortic remodeling afforded by a combined open/endovascular approach to Acute Type A Dissection will lead to decreased need for aortic reinterventions and overall long term complications of a residual descending thoracic chronic dissection.

  12. In vitro characterization of bicuspid aortic valve hemodynamics using particle image velocimetry.

    Science.gov (United States)

    Saikrishnan, Neelakantan; Yap, Choon-Hwai; Milligan, Nicole C; Vasilyev, Nikolay V; Yoganathan, Ajit P

    2012-08-01

    The congenital bicuspid aortic valve (BAV) is associated with increased leaflet calcification, ascending aortic dilatation, aortic stenosis (AS) and regurgitation (AR). Although underlying genetic factors have been primarily implicated for these complications, the altered mechanical environment of BAVs could potentially accelerate these pathologies. The objective of the current study is to characterize BAV hemodynamics in an in vitro system. Two BAV models of varying stenosis and jet eccentricity and a trileaflet AV (TAV) were constructed from excised porcine AVs. Particle Image Velocimetry (PIV) experiments were conducted at physiological flow and pressure conditions to characterize fluid velocity fields in the aorta and sinus regions, and ensemble averaged Reynolds shear stress and 2D turbulent kinetic energy were calculated for all models. The dynamics of the BAV and TAV models matched the characteristics of these valves which are observed clinically. The eccentric and stenotic BAV showed the strongest systolic jet (V = 4.2 m/s), which impinged on the aortic wall on the non-fused leaflet side, causing a strong vortex in the non-fused leaflet sinus. The magnitudes of TKE and Reynolds stresses in both BAV models were almost twice as large as comparable values for TAV, and these maximum values were primarily concentrated around the central jet through the valve orifice. The in vitro model described here enables detailed characterization of BAV flow characteristics, which is currently challenging in clinical practice. This model can prove to be useful in studying the effects of altered BAV geometry on fluid dynamics in the valve and ascending aorta. These altered flows can be potentially linked to increased calcific responses from the valve endothelium in stenotic and eccentric BAVs, independent of concomitant genetic factors.

  13. Aorto-Right Ventricular Tunnel in Transposition of the Great Arteries

    Directory of Open Access Journals (Sweden)

    Antonio F. Corno

    2018-02-01

    Full Text Available Aorto-ventricular tunnel is an extremely rare congenital heart defect, consisting of failure of attachment of an aortic leaflet along the semilunar hinge. In all published reports the leaflet involved was either the right coronary leaflet, most frequently, or the left coronary leaflet, in most of the cases opening toward the left ventricle, with only one-eighth of the reported cases communicating with the right ventricle. Treatment of the aorto-ventricular tunnel has been anecdotally reported by interventional closure with a device and more frequently with surgical approach, either as an isolated malformation or as associated lesions. To the best of our knowledge, the presence of an aorto-ventricular tunnel of the non-adjacent aortic leaflet in transposition of the great arteries has never been reported. We have observed an aorto-ventricular tunnel involving the non-adjacent leaflet of the aortic root, which after arterial switch became the pulmonary root. The patient presented 18 years after the arterial switch with progressive dilatation of the right ventricle due to severe degree of pulmonary valve regurgitation, confirmed by echocardiography and cardiac MRI. Indication for surgery was given with the plan for a pulmonary valve implantation. Because of the intra-operative finding of disconnection of the anterior leaflet of the pulmonary valve (former aortic valve along the semilunar hinge, the surgical plan was modified and the anterior leaflet was attached to the valve annulus, with subsequent plasty in correspondence with the right and left commissurae to reduce the size of the dilated annulus to normal diameter. The post-operative course was uneventful, with extubation after few hours and discharge 4 days after surgery, with echocardiography showing trivial degree of pulmonary valve regurgitation. The patient remains in good conditions 6 months after surgery.

  14. Characterization of dilation-analytic operators

    Energy Technology Data Exchange (ETDEWEB)

    Balslev, E; Grossmann, A; Paul, T

    1986-01-01

    Dilation analytic vectors and operators are characterized in a new representation of quantum mechanical states through functions analytic on the upper half-plane. In this space H/sub o/-bounded operators are integral operators and criteria for dilation analyticity are given in terms of analytic continuation outside of the half-plane for functions and for kernels. A sufficient condition is given for an integral operator in momentum space to be dilation-analytic.

  15. Long-Term Risk for Aortic Complications After Aortic Valve Replacement in Patients With Bicuspid Aortic Valve Versus Marfan Syndrome.

    Science.gov (United States)

    Itagaki, Shinobu; Chikwe, Joanna P; Chiang, Yuting P; Egorova, Natalia N; Adams, David H

    2015-06-09

    Bicuspid aortic valves are associated with valve dysfunction, ascending aortic aneurysm and dissection. Management of the ascending aorta at the time of aortic valve replacement (AVR) in these patients is controversial and has been extrapolated from experience with Marfan syndrome, despite the absence of comparative long-term outcome data. This study sought to assess whether the natural history of thoracic aortopathy after AVR in patients with bicuspid aortic valve disease is substantially different from that seen in patients with Marfan syndrome. In this retrospective comparison, outcomes of 13,205 adults (2,079 with bicuspid aortic valves, 73 with Marfan syndrome, and 11,053 control patients with acquired aortic valve disease) who underwent primary AVR without replacement of the ascending aorta in New York State between 1995 and 2010 were compared. The median follow-up time was 6.6 years. The long-term incidence of thoracic aortic dissection was significantly higher in patients with Marfan syndrome (5.5 ± 2.7%) compared with those with bicuspid valves (0.55 ± 0.21%) and control group patients (0.41 ± 0.08%, p Marfan syndrome (10.8 ± 4.4%) compared with those with bicuspid valves (4.8 ± 0.8%) and control group patients (1.4 ± 0.2%) (p Marfan syndrome were significantly more likely to undergo thoracic aortic surgery in late follow-up (10.4 ± 4.3%) compared with those with bicuspid valves (2.5 ± 0.6%) and control group patients (0.50 ± 0.09%) (p Marfan syndrome compared with those with bicuspid aortic valves confirm that operative management of patients with bicuspid aortic valves should not be extrapolated from Marfan syndrome and support discrete treatment algorithms for these different clinical entities. Copyright © 2015 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

  16. Percutaneous dilatation of biliary benign strictures

    Energy Technology Data Exchange (ETDEWEB)

    Park, Jae Hyung; Choi, Byung Ihn; Sung, Kyu Bo; Han, Man Chung; Park, Yong Hyun; Yoon, Yong Bum [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    1986-06-15

    Percutaneous biliary dilation was done in 3 patients with benign strictures. The first case was 50-year-old male who had multiple intrahepatic stones with biliary stricture. The second 46-year-old female and the third 25-year-old male suffered from recurrent cholangitis with benign stricture of anastomotic site after choledocho-jejunostomy. In the first case, a 6mm diameter Grunzing dilatation balloon catheter was introduced through the T-tube tract. In the second case, the stricture was dilated with two balloons of 5mm and 8mm in each diameter sequentially through the U-loop tract formed by surgically made jejunostomy and percutaneous transhepatic puncture. In the third case, the dilatation catheter was introduced through the percutaneous transhepatic tract. Dilatation was made with a pressure of 5 to 10 atmospheres for 1 to 3 minutes duration for 3 times. In all 3 cases, the strictures were successfully dilated and in second and third cases internal stent was left across the lesion for prevention of restenosis.

  17. A study of intraluminal flow in patients with aortic disease by cine magnetic resonance imaging

    International Nuclear Information System (INIS)

    Ohnishi, Shusaku; Fukui, Sugao; Kusuoka, Hideo; Kitabatake, Akira; Kamada, Takenobu.

    1990-01-01

    To evaluate the usefulness of cine magnetic resonance (MR) imaging in the diagnosis of aortic disease, we applied cine MR sequences with conventional spin echo (SE) sequence to 15 patients with aortic aneurysm and 5 patients with aortic dissection. ECG gated multi slice SE images (single echo) were obtained in transverse plane and the planes along the long axis of aorta. TE of SE sequence ranged from 15 to 32 msec. Cine MR images were obtained in 17 frames, where TR ranged from 30 to 60 msec and TE from 10 to 22 msec. We got these results as follows. For aortic aneurysm, SE images showed aneurysmal dilatation in thoracic aorta in 9 cases, and in abdominal aorta in 6 cases. Cine MR images showed us the intraluminal flow with high signal intensity. We could differentiate the slow flow image from mural thrombus using SE and cine MR sequences in the same plane. We could evaluate the dynamic flow image within a cavity of aneurysm and in in-flow and out-flow portions of aorta by cine MR imaging. For aortic dissection, SE images delineated the intimal tear and two separated lumens in all cases. We could obtain flow images within true and false lumen by cine MR imaging. The signal intensity within true lumen was higher than that within false lumen. It seemed to be due to the difference of flow velocity and the turbulence within false lumen. Entry to false lumen was seen as the interruption of dissected intima on SE images. Cine MR images clearly depicted the blood flow communication between two lumens through the entry. In 3 of 5 cases the blood flow through the entry was seen in both directions, from true to false lumen in systole and inversely in diastole. Thus, cine MR imaging will add the useful hemodynamic information in the diagnosis of aortic disease by conventional MR imaging. (author)

  18. AP-1 Oligodeoxynucleotides Reduce Aortic Elastolysis in a Murine Model of Marfan Syndrome.

    Science.gov (United States)

    Arif, Rawa; Zaradzki, Marcin; Remes, Anca; Seppelt, Philipp; Kunze, Reiner; Schröder, Hannes; Schwill, Simon; Ensminger, Stephan M; Robinson, Peter N; Karck, Matthias; Müller, Oliver J; Hecker, Markus; Wagner, Andreas H; Kallenbach, Klaus

    2017-12-15

    Marfan syndrome is characterized by high expression of matrix metalloproteinases (MMPs) in aortic smooth muscle cells (AoSMCs) associated with medial elastolysis and aortic root aneurysm. We aimed to reduce aortic elastolysis through decrease of MMP expression with decoy oligodeoxynucleotides (dODNs) neutralizing the transcription factor activating factor-1 (AP-1). AP-1 abundance in nuclear extracts as well as MMP-2 and MMP-9 expression were significantly increased in isolated mAoSMC of mgR/mgR Marfan mice compared to wild-type cells. Exposure to AP-1 neutralizing dODNs resulted in a significant reduction of basal and interleukin-1β-stimulated MMP expression and activity in mAoSMCs. Moreover, increased migration and formation of superoxide radical anions was substantially decreased in mAoSMCs by AP-1 dODN treatment. Aortic grafts from donor Marfan mice were treated with AP-1- dODN ex vivo and implanted as infrarenal aortic interposition grafts in mgR/mgR mice. Pretreatment of aortic grafts with AP-1 dODN led to reduced elastolysis, macrophage infiltration, and MMP activity. Permeability of the endothelial monolayer was increased for dODN in mgR/mgR aortae with observed loss of tight junction proteins ZO-1 and occludin, enabling dODN to reach the tunica media. Targeting AP-1 activity offers a new potential strategy to treat the vascular phenotype associated with Marfan syndrome. Copyright © 2017 The Author(s). Published by Elsevier Inc. All rights reserved.

  19. Traditional Cardiovascular Risk Factors and Their Relation to Future Surgery for Valvular Heart Disease or Ascending Aortic Disease: A Case-Referent Study.

    Science.gov (United States)

    Ljungberg, Johan; Johansson, Bengt; Engström, Karl Gunnar; Albertsson, Elin; Holmer, Paul; Norberg, Margareta; Bergdahl, Ingvar A; Söderberg, Stefan

    2017-05-05

    Risk factors for developing heart valve and ascending aortic disease are based mainly on retrospective data. To elucidate these factors in a prospective manner, we have performed a nested case-referent study using data from large, population-based surveys. A total of 777 patients operated for heart valve disease or disease of the ascending aorta had previously participated in population-based health surveys in Northern Sweden. Median time (interquartile range) from survey to surgery was 10.5 (9.0) years. Primary indications for surgery were aortic stenosis (41%), aortic regurgitation (12%), mitral regurgitation (23%), and dilatation/dissection of the ascending aorta (17%). For each case, referents were allocated, matched for age, sex, and geographical area. In multivariable models, surgery for aortic stenosis was predicted by hypertension, high cholesterol levels, diabetes mellitus, and active smoking. Surgery for aortic regurgitation was associated with a low cholesterol level, whereas a high cholesterol level predicted surgery for mitral regurgitation. Hypertension, blood pressure, and previous smoking predicted surgery for disease of the ascending aorta whereas diabetes mellitus was associated with reduced risk. After exclusion of cases with coronary atherosclerosis, only the inverse associations between cholesterol and aortic regurgitation and between diabetes mellitus and disease of the ascending aorta remained. This is the first truly prospective study of traditional cardiovascular risk factors and their association with valvular heart disease and disease of the ascending aorta. We confirm the strong association between traditional risk factors and aortic stenosis, but only in patients with concomitant coronary artery disease. In isolated valvular heart disease, the impact of traditional risk factors is varying. © 2017 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley.

  20. Clinical implications of pleural effusion in patients with acute type B aortic dissection.

    Science.gov (United States)

    Yamada, Yoshihiro; Tanno, Jun; Nakano, Shintaro; Kasai, Takatoshi; Senbonmatsu, Takaaki; Nishimura, Shigeyuki

    2016-11-01

    Pleural effusion may complicate acute Stanford type B aortic dissection (ABAD). To identify the relationships between the quantity and side of the pleural effusion, biomarkers and outcomes in patients with ABAD. We undertook a retrospective review of 105 patients with ABAD. Their demographics, the data on admission and during hospital stay, the volume of pleural effusion calculated from the area on computed tomography images and clinical outcomes were analysed. The median estimated peak volume (median 6.7 days after onset) was 129 ml (63-192, range 26-514 ml) on the left and 11 ml (6-43, range 2-300 ml) on the right. On univariate analysis, the volume of bilateral effusions was associated with anaemia, hypoalbuminaemia and inflammatory markers, whereas the volume of left-sided effusions was associated with older age, low diastolic blood pressure and maximum aortic diameter. Multivariate analysis revealed that hypoalbuminaemia was independently associated with bilateral effusion volume ( Peffusion volume ( P=0.019). A greater volume of bilateral plural effusion was associated with longer intensive care unit stay. Larger bilateral pleural effusions in patients with ABAD were associated with hypoalbuminaemia and potentially with anaemia and inflammation, and may increase the length of intensive care unit stay. Left-sided effusion volume appears to be influenced by the nature of the aortic dilatation. Multiple mechanisms may underpin the development of pleural effusion in ABAD, and are likely to influence clinical outcomes.

  1. Longitudinal Changes in Segmental Aortic Stiffness Determined by Cardiac Magnetic Resonance in Children and Young Adults With Connective Tissue Disorders (the Marfan, Loeys-Dietz, and Ehlers-Danlos Syndromes, and Nonspecific Connective Tissue Disorders).

    Science.gov (United States)

    Merlocco, Anthony; Lacro, Ronald V; Gauvreau, Kimberlee; Rabideau, Nicole; Singh, Michael N; Prakash, Ashwin

    2017-10-01

    Aortic stiffness measured by cardiac magnetic resonance (CMR) in connective tissue disorder (CTD) patients has been previously shown to be abnormal and to be associated with adverse aortic outcomes. The rate of increase in aortic stiffness with normal aging has been previously described. However, longitudinal changes in aortic stiffness have not been characterized in CTD patients. We examined longitudinal changes in CMR-derived aortic stiffness in children and young adults with CTDs. A retrospective analysis of 50 children and young adults (median age, 20 years; range, 0.2 to 49; 40% age, whereas the β stiffness index increased at all aortic segments. The average rates of decline in distensibility (x10 -3  mm Hg -1 per 10-year increase in age) were 0.7, 1.3, and 1 at the AoR, ascending aorta, and descending aorta, respectively. The rates of decline in distensibility were not associated with the rates of AoR dilation or surgical AoR replacement. In conclusion, on serial CMR measurements in children and young adults with CTDs, aortic stiffness progressively increased with age, with rates of change only slightly higher than those previously reported in healthy adults. Copyright © 2017 Elsevier Inc. All rights reserved.

  2. One-day transhepatic billary stricture dilatation

    International Nuclear Information System (INIS)

    Mueller, P.R.; Saini, S.; Hahn, P.F.; Silverman, S.G.; Ferrucci, J.T.

    1989-01-01

    To overcome technical problems in percutaneous transhepatic dilatation or biliary strictures, the authors performed the entire procedure in a single session with the patient under general anesthesia (N = 7). Six patients had a biliary-enteric anastomosis, and one patient had a traumatic mid common bile duct stricture. All bile ducts were of normal caliber or were minimally dilated by US or transhepatic cholangiography. Dilatation was performed via a fresh transhepatic drainage catheter in six patients and via an in-dwelling T-tube in one patient. High-pressure balloons (10-12 mm) were placed after catheter dilation of the tract, and balloons were inflated for 5-10 minutes three to five times. Dilation was stopped with a waist was no longer present. No strictures have recurred in patients followed up to 24 months

  3. Balloon dilatation of ureteric strictures.

    Directory of Open Access Journals (Sweden)

    Punekar S

    2000-01-01

    Full Text Available AIMS: Evaluation of dilatation as a minimally invasive technique for the treatment of ureteric strictures. MATERIAL AND METHODS: We evaluated this technique in 16 patients with ureteric and secondary pelviureteric junction strictures from June 1998. Of these, 7 were men and 9 were women. The age range was from 14 to 40 years. RESULTS: Balloon dilatation was successful in 69% of patients. Strictures secondary to previous surgery had nearly 100% success. Of the 8 cases diagnosed as genitourinary tuberculosis, success rate was 50%. CONCLUSIONS: Factors affecting success of balloon dilatation are: a age of the stricture b length of the stricture and c etiology of the stricture. In a select group of patients with fresh post-operative or post-inflammatory strictures, balloon dilatation may be an attractive alternative to surgery.

  4. Comparison of the Effect of Aliskiren Versus Negative Controls on Aortic Stiffness in Patients With Marfan Syndrome Under Treatment With Atenolol.

    Science.gov (United States)

    Hwang, Ji-Won; Kim, Eun Kyoung; Jang, Shin Yi; Chung, Tae-Young; Ki, Chang-Seok; Sung, Kiick; Kim, Sung Mok; Ahn, Joonghyun; Carriere, Keumhee; Choe, Yeon Hyeon; Chang, Sung-A; Kim, Duk-Kyung

    2017-11-29

    The aim of this study was to evaluate the effect of aliskiren on aortic stiffness in patients with Marfan syndrome (MS). Twenty-eight MS patients (mean age ± standard deviation: 32.6 ± 10.6 years) were recruited from November 2009 to October 2014. All patients were receiving atenolol as standard beta-blocker therapy. A prospective randomization process was performed to assign participants to either aliskiren treatment (150-300mg orally per day) or no aliskiren treatment (negative control) in an open-label design. Central aortic distensibility and central pulsed wave velocity (PWV) by magnetic resonance imaging (MRI), peripheral PWV, central aortic blood pressure and augmentation index by peripheral tonometry, and aortic dilatation by echocardiography were examined initially and after 24 weeks. The primary endpoint was central aortic distensibility by MRI. In analyses of differences between baseline and 24 weeks for the aliskiren treatment group vs the negative control group, central distensibility (overall; P = .26) and central PWV (0.2 ± 0.9 vs 0.03 ± 0.7 [m/s]; P = .79) by MRI were not significantly different. Central systolic aortic blood pressure tended to be lower by 14mmHg in patients in the aliskiren treatment group than in the control group (P = .09). A significant decrease in peripheral PWV (brachial-ankle PWV) in the aliskiren treatment group (-1.6 m/s) compared with the control group (+0.28 m/s) was noted (P = .005). Among patients with MS, the addition of aliskiren to beta-blocker treatment did not significantly improve central aortic stiffness during a 24-week period. Copyright © 2017 Sociedad Española de Cardiología. Published by Elsevier España, S.L.U. All rights reserved.

  5. Aortic intramural hematoma : assessment of clinical and radiological features in comparison to acute aortic dissection

    International Nuclear Information System (INIS)

    Yoon, Kwon Ha; Hwang, Jae Cheol; Lee, Jin Seong; Kang, Duk Hyun; Song, Jae Kwan; Song, Koun Sik; Lim, Tae Hwan

    1996-01-01

    To compare the clinical and radiological features of aortic intramural hematoma(IMH) to those of acute aortic dissection(AD). We analyzed the clinical and radiological features of 12 patients with aortic IMH and 43 patients with acute AD. In aortic IMH, the diagnoses were made by means of both CT and transesophageal echocardiography(TEE) and included two surgically proven cases. In acute AD, the diagnoses were made by means of CT and TEE and included 21 surgically proven cases. We compared patients ages, etiologies, the extent of the disease, the presence or absence of aortic branch involvement, complications, and outcomes. Aortic IMH tended to develop in older patients (67.8±7.9 vs. 50.4±13.4, P .05). In aortic IMH, there was no involvement of aortic branches, whereas in acute AD, 14(33%) patients showed involvement of one or more aortic branches. Complications of aortic IMH included pericardial effusion (n=2) and pleural effusion (n=4);in acute AD, pericardial effusion (n=7), pleural effusion (n=4), aortic insufficiency (n=8), cerebral infarction (n=3), renal infarction (n=4) and spinal infarction (n=1) were seen. There was one (8%) death due to aortic IMH and ten (23%) deaths due to acute AD (p<.01). Aortic IMH is characterized by its occurrence in older patients with hypertension, a less frequent incidence of complications, and a more favorable outcome than acute AD

  6. Transcatheter aortic valve replacement

    Science.gov (United States)

    ... gov/ency/article/007684.htm Transcatheter aortic valve replacement To use the sharing features on this page, please enable JavaScript. Transcatheter aortic valve replacement (TAVR) is surgery to replace the aortic valve. ...

  7. Severe reversible dilated cardiomyopathy associated with a large left ventricular thrombus in a young child with middle aortic syndrome

    OpenAIRE

    Ponniah, U; Overholt, E

    2014-01-01

    this article reports a case of a seven-year girl who presented with severe dilated cardiomyopathy (DCM) associated with a large thrombus in the left ventricle (LV). She had a long segment stenosis of the lower thoracic descending aorta, possibly due to non-specific aortitis and underwent successful stent angioplasty. The LV thrombus resolved after heparin without sequelae.

  8. Infected aortic aneurysm and inflammatory aortic aneurysm. In search of an optimal differential diagnosis

    International Nuclear Information System (INIS)

    Ishizaka, Nobukazu; Sohmiya, Koichi; Miyamura, Masatoshi; Umeda, Tatsuya; Tsuji, Motomu; Katsumata, Takahiro; Miyata, Tetsuro

    2012-01-01

    Infected aortic aneurysm and inflammatory aortic aneurysm each account for a minor fraction of the total incidence of aortic aneurysm and are associated with periaortic inflammation. Despite the similarity, infected aortic aneurysm generally shows a more rapid change in clinical condition, leading to a fatal outcome; in addition, delayed diagnosis and misuse of corticosteroid or immunosuppressing drugs may lead to uncontrolled growth of microorganisms. Therefore, it is mandatory that detection of aortic aneurysm is followed by accurate differential diagnosis. In general, infected aortic aneurysm appears usually as a saccular form aneurysm with nodularity, irregular configuration; however, the differential diagnosis may not be easy sometimes for the following reasons: symptoms, such as abdominal and/or back pain and fever, and blood test abnormalities, such as elevated C-reactive protein and enhanced erythrocyte sedimentation rate, are common in infected aortic aneurysm, but they are not found infrequently in inflammatory aortic aneurysm; some inflammatory aortic aneurysms are immunoglobulin (Ig) G4-related, but not all of them; the prevalence of IgG4 positivity in infected aortic aneurysm has not been well investigated; enhanced uptake of 18F-fluorodeoxyglucose (FDG) by 18F-FDG-positron emission tomography may not distinguish between inflammation mediated by autoimmunity and that mediated by microorganism infection. Here we discuss the characteristics of these two forms of aortic aneurysm and the points of which we have to be aware before reaching a final diagnosis. (author)

  9. [Value of bedside echocardiography in diagnosis and risk assessment of in-hospital death for patients with Stanford type A aortic dissection].

    Science.gov (United States)

    Wang, H J; Xiao, Z Y; Gu, G R; Xue, Y; Shao, M; Deng, Z; Tao, Z G; Yao, C L; Tong, C Y

    2017-11-24

    Objective: To investigate the value of bedside echocardiography in diagnosis and risk assessment of in-hospital death of patients with Stanford type A aortic dissection. Methods: The clinical data of 229 patients with Stanford type A aortic dissection diagnosed by CT angiography in Zhongshan Hospital affiliated to Fudan University between January 2009 and January 2016 were retrospectively analyzed. The patients were divided into survival group(191 cases)and non-survival group(38 cases)according to presence or absence of in-hospital death. The bedside echocardiography features were analyzed, and influence factors of in-hospital death were determined by multivariate logistic regression analysis. Results: (1) Compared with the survival group, the non-survival group had lower surgery rate (60.52%(23/38) vs. 85.34%(163/191), P 0.05). (2) The bedside echocardiography results showed that prevalence of aortic valve involvement(65.79%(25/38) vs.34.03%(65/191), P 0.05). (3) The multivariate logistic regression analysis showed that aortic valve involvement( OR =3.275, 95% CI 1.290-8.313, P risk factors for in-hospital death in patients with Stanford type A aortic dissection. Conclusions: Bedside echocardiography has significant diagnostic value for Stanford type A aortic dissection. Aortic valve involvement, enlargement of aortic root diameter and without surgery are independent risk factors for patients with Stanford type A aortic dissection.

  10. A new methodological approach to assess cardiac work by pressure-volume and stress-length relations in patients with aortic valve stenosis and dilated cardiomyopathy.

    Science.gov (United States)

    Alter, P; Rupp, H; Rominger, M B; Klose, K J; Maisch, B

    2008-01-01

    In experimental animals, cardiac work is derived from pressure-volume area and analyzed further using stress-length relations. Lack of methods for determining accurately myocardial mass has until now prevented the use of stress-length relations in patients. We hypothesized, therefore, that not only pressure-volume loops but also stress-length diagrams can be derived from cardiac volume and cardiac mass as assessed by cardiac magnetic resonance imaging (CMR) and invasively measured pressure. Left ventricular (LV) volume and myocardial mass were assessed in seven patients with aortic valve stenosis (AS), eight with dilated cardiomyopathy (DCM), and eight controls using electrocardiogram (ECG)-gated CMR. LV pressure was measured invasively. Pressure-volume curves were calculated based on ECG triggering. Stroke work was assessed as area within the pressure-volume loop. LV wall stress was calculated using a thick-wall sphere model. Similarly, stress-length loops were calculated to quantify stress-length-based work. Taking the LV geometry into account, the normalization with regard to ventricular circumference resulted in "myocardial work." Patients with AS (valve area 0.73+/-0.18 cm(2)) exhibited an increased LV myocardial mass when compared with controls (Pwork of AS was unchanged when compared with controls (0.539+/-0.272 vs 0.621+/-0.138 Nm, not significant), whereas DCM exhibited a significant depression (0.367+/-0.157 Nm, Pwork was significantly reduced in both AS and DCM when compared with controls (129.8+/-69.6, 200.6+/-80.1, 332.2+/-89.6 Nm/m(2), Pmethodological approach of using CMR and invasive pressure measurement. Myocardial work was reduced in patients with DCM and noteworthy also in AS, while stroke work was reduced in DCM only. Most likely, deterioration of myocardial work is crucial for the prognosis. It is suggested to include these basic physiological procedures in the clinical assessment of the pump function of the heart.

  11. Basal lamina structural alterations in human asymmetric aneurismatic aorta

    Directory of Open Access Journals (Sweden)

    M Cotrufo

    2009-06-01

    Full Text Available Basal lamina (BL is a crucial mechanical and functional component of blood vessels, constituting a sensor of extracellular microenvironment for endothelial cells and pericytes. Recently, an abnormality in the process of matrix microfibrillar component remodeling has been advocated as a mechanism involved in the development of aortic dilation.We focused our attention on BL composition and organization and studied some of the main components of the Extracellular Matrix such as Tenascin, Laminins, Fibronectin, type I, III and IV Collagens.We used surgical fragments from 27 patients, submitted to operation because of aortic root aneurysm and 5 normal aortic wall specimens from heart donors without any evidence for aneurysmal or atherosclerotic diseases of the aorta. Two samples of aortic wall were harvested from each patient, proximal to the sinotubular junction at the aortic convexity and concavity. Each specimen was processed both for immunohistochemical examination and molecular biology study.We compared the convexity of each aortic sample with the concavity of the same vessel, and both of them with the control samples. The synthesis of mRNA and the levels of each protein were assessed, respectively, by RTPCR and Western Blot analysis. Immunohistochemistry elucidated the organization of BL, whose composition was revealed by molecular biology. All pathological samples showed a wall thinner than normal ones. Basal lamina of the aortic wall evidentiated important changes in the tridimensional arrangement of its major components which lost their regular arrangement in pathological specimens. Collagen I, Laminin a2 chain and Fibronectin amounts decreased in pathological samples, while type IV Collagen and Tenascin synthesis increased. Consistently with the common macroscopic observation that ascending aorta dilations tend to expand asymmetrically, with prevalent involvement of the vessel convexity and relative sparing of the concavity, Collagen type

  12. Balloon dilatation of iatrogenic urethral strictures

    International Nuclear Information System (INIS)

    Acunas, B.; Acunas, G.; Gokmen, E.; Celik, L.

    1988-01-01

    Balloon dilatation of the urethra was performed in five patients with iatrogenic urethral strictures. The urethral strictures were successfully negotiated and dilated in all patients. Redilatation became necessary in a period ranging from 3 to 10 months. The authors believe that balloon dilatation of the urethra can be safely and successfully performed; the procedure produces minimal trauma and immediate relief of symptoms. (orig.)

  13. Vessel dilatation in coronary angiograms

    International Nuclear Information System (INIS)

    Hinterauer, L.; Goebel, N.

    1983-01-01

    Amongst 166 patients with aneurysms, ectasia or megaloarteries shown on coronary angiograms, 86.1% had dilated vessels as part of generalised coronary sclerosis (usually in patients with three-vessel disease). In 9%, dilatation was of iatrogenic origin and in 4.8% it was idiopathic. One patient had Marfan's syndrome. Amongst 9 000 patients, there were eight with megalo-arteries without stenosis; six of these had atypical angina and three suffered an infarct. Patients with definite dilatation of the coronary artery and stagnation of contrast flow required treatment. (orig.) [de

  14. Vessel dilatation in coronary angiograms

    Energy Technology Data Exchange (ETDEWEB)

    Hinterauer, L.; Goebel, N.

    1983-11-01

    Amongst 166 patients with aneurysms, ectasia or megaloarteries shown on coronary angiograms, 86.1% had dilated vessels as part of generalised coronary sclerosis (usually in patients with three-vessel disease). In 9%, dilatation was of iatrogenic origin and in 4.8% it was idiopathic. One patient had Marfan's syndrome. Amongst 9 000 patients, there were eight with megalo-arteries without stenosis; six of these had atypical angina and three suffered an infarct. Patients with definite dilatation of the coronary artery and stagnation of contrast flow required treatment.

  15. Florida Sleeve Procedure Is Durable and Improves Aortic Valve Function in Marfan Syndrome Patients.

    Science.gov (United States)

    Aalaei-Andabili, Seyed Hossein; Martin, Tomas; Hess, Philip; Klodell, Charles; Karimi, Ashkan; Arnaoutakis, George; Lee, Teng; Beaver, Thomas

    2017-09-01

    The Florida sleeve (FS) procedure was developed as a simplified approach for repair of functional type I aortic insufficiency secondary to aortic root aneurysm. We evaluated postoperative aortic valve function, long-term survival, and freedom from reoperation in Marfan syndrome patients who underwent the FS procedure at our center. All Marfan syndrome patients undergoing FS procedure from May 2002 to December 2014 were included. Echocardiography assessment included left ventricular end-diastolic diameter (LVEDD), left ventricular end-systolic diameter (LVESD), ejection fraction, and degree of aortic insufficiency (none = 0, minimal = 1, mild = 2, moderate = 3, severe = 4). Social Security Death Index and primary care physicians' report were used for long-term follow-up. Thirty-seven Marfan syndrome patients, 21 (56.8%) men and 16 (43%) women with mean age of 35.08 ± 13.45 years underwent FL repair at our center. There was no in-hospital or 30-day death or stroke. Two patients required reoperation due to bleeding. Patients' survival rate was 94% at 1 to 8 years. Freedom from reoperation was 100% at 8 years. Twenty-five patients had postoperative follow-up echocardiography at 1 week. Aortic insufficiency grade significantly decreased after the procedure (preoperative mean ± SD: 1.76 ± 1.2 versus 1-week postoperative mean ± SD: 0.48 ± 0.71, p Marfan syndrome patients with immediate improvement in aortic valve function. Long-term survival and freedom from reoperation rates are encouraging. Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  16. Transcatheter valve-in-valve implantation due to severe aortic regurgitation in a degenerated aortic homograft

    DEFF Research Database (Denmark)

    Olsen, Lene Kjaer; Engstrøm, Thomas; Søndergaard, Lars

    2009-01-01

    Transcatheter aortic valve implantation (TAVI) in severe aortic stenosis has proven to be a feasible and effective treatment modality for inoperable patients. Until now, neither aortic regurgitation nor degenerated bioprostheses has been an indication for TAVI. However, this article reports...... a successful valve-in-valve implantation of a CoreValve aortic valve prosthesis through the right subclavian artery in a case of severe aortic regurgitation within a degenerated aortic homograft. The case exemplifies the possibilities of expanding the indications for TAVI, as well as other vascular access...

  17. Eyes wide shut - unusual two stage repair of pectus excavatum and annuloaortic ectasia in a 37 year old marfan patient: case report

    Directory of Open Access Journals (Sweden)

    Barriuso Clemente

    2011-05-01

    Full Text Available Abstract We report about a 37 year old male patient with a pectus excavatum. The patient was in NYHA functional class III. After performed computed tomography the symptoms were thought to be related to the severity of chest deformation. A Ravitch-procedure had been accomplished in a district hospital in 2009. The crack of a metal bar led to a reevaluation 2010, in which surprisingly the presence of an annuloaortic ectasia (root 73 × 74 mm in direct neighborhood of the formerly implanted metal-bars was diagnosed. Echocardiography revealed a severe aortic valve regurgitation, the left ventricle was massively dilated presenting a reduced ejection fraction of 45%. A marfan syndrome was suspected and the patient underwent a valve sparing aortic root replacement (David procedure in our institution with an uneventful postoperative course. A review of the literature in combination with discussion of our case suggests the application of stronger recommendations towards preoperative cardiovascular assessment in patients with pectus excavatum.

  18. Dilation of non-quasifree dissipative evolution

    Energy Technology Data Exchange (ETDEWEB)

    Varilly, J C [Costa Rica Univ., San Jose. Escuela de Matematica

    1981-03-01

    A semigroup evolution for the 1/2-spin which admits a conservative dilation is known to be governed by a Bloch equation in a standard form. Here we construct a conservative dilation directly from the Bloch equation, thus yielding an example of a dilation scheme for an evolution which is not quasifree. Moreover, we show that this conservative evolution is never ergodic in the non-quasifree case.

  19. Three dimensional rotational angiography for assessment of coronary arteries during melody valve implantation: introducing a technique that may improve outcomes.

    Science.gov (United States)

    Pockett, C R; Moore, J W; El-Said, H G

    2017-02-01

    Adverse events from Melody valve implantation may be catastrophic. To date a role for three dimensional rotational angiography of the aortic root (3DRAA) during Melody valve implantation has not been established. To describe the role of 3DRAA in the assessment of Melody valve candidacy and to demonstrate that it may improve outcomes. All patients who underwent cardiac catheterisation for Melody valve implantation and 3DRAA between August 2013 and February 2015 were reviewed. 31 patients had 3DRAA with balloon sizing. Ten were deemed not Melody candidates (5 coronary compression, 2 aortic root distortion with cusp flattening, 2 RVOT was too large, and 1 had complex branch stenosis and a short landing zone). Of the 21 patients who were Melody candidates, 12 had conduits, 6 prosthetic valves and 3 native RVOTs. In patients with conduits, the technique of stenting the conduit prior to dilation was used after measuring the distance between the conduit and the coronary arteries on 3DRAA. In the Melody patients, we had 100% procedural success and no serious adverse events (coronary compression, tears, stent fracture or endocarditis). As a tool for case selection, 3DRAA may facilitate higher procedural success and decreased risk of serious adverse events. Furthermore, 3D rotational angiography allows stenting of the conduit prior to dilation, which may prevent tears and possibly endocarditis.

  20. Aortic arch/elephant trunk procedure with Sienna(TM) graft and endovascular stenting of thoraco-abdominal aorta for treatment of complex chronic dissection.

    Science.gov (United States)

    Wong, Randolph H L; Baghai, Max; Yu, Simon C H; Underwood, Malcolm J

    2013-05-01

    Aneurismal dilatation of the remaining thoracic aorta after ascending aortic interposition grafting for type 'A' aortic dissection is not uncommon. For such complex cases, one treatment option is total arch replacement and elephant trunk procedure with the Sienna(TM) collared graft (Vascutek, Inchinnan, UK) technique followed by a staged thoracic endovascular aortic repair (TEVAR). The video illustrates our technique in a 56-year-old man with an extensive aortic arch and descending thoracic aortic dissecting aneurysm. For the 'open' procedure femoral arterial and venous cannulation was used along with systemic cooling and circulatory arrest at 22 °C. Upon circulatory arrest, the aortic arch was incised and antegrade cerebral perfusion achieved via selective cannulation to the right brachiocephalic and left common carotid artery, keeping flow rates at 10-15 mL/kg/min and perfusion pressure at 50-60 mmHg. Arch replacement with an elephant trunk component was then performed and after completion of the distal aortic anastomosis antegrade perfusion via a side-arm in the graft was started and the operation completed using a variation of the 'sequential' clamping technique to maximize cerebral perfusion. The second endovascular stage was performed two weeks after discharge. Two covered stents were landing from the elephant trunk to the distal descending thoracic aorta, to secure the distal landing a bare stent of was placed to cover the aorta just distal to the origin of the celiac axis. The left subclavian artery was embolised with fibre coils. Post TEVAR angiogram showed no endoleak Although re-operative total arch replacement and elephant trunk procedure and subsequent TEVAR remained a challenging procedure, we believe excellent surgical outcome can be achieved with carefully planned operative strategy.

  1. A characterization of dilation-analytic operators

    International Nuclear Information System (INIS)

    Balslev, E.; Grossmann, A.; Paul, T.

    1986-01-01

    Dilation analytic vectors and operators are characterized in a new representation of quantum mechanical states through functions analytic on the upper half-plane. In this space H o -bounded operators are integral operators and criteria for dilation analyticity are given in terms of analytic continuation outside of the half-plane for functions and for kernels. A sufficient condition is given for an integral operator in momentum space to be dilation-analytic

  2. Segmental dilatation of the ileum

    Directory of Open Access Journals (Sweden)

    Tune-Yie Shih

    2017-01-01

    Full Text Available A 2-year-old boy was sent to the emergency department with the chief problem of abdominal pain for 1 day. He was just discharged from the pediatric ward with the diagnosis of mycoplasmal pneumonia and paralytic ileus. After initial examinations and radiographic investigations, midgut volvulus was impressed. An emergency laparotomy was performed. Segmental dilatation of the ileum with volvulus was found. The operative procedure was resection of the dilated ileal segment with anastomosis. The postoperative recovery was uneventful. The unique abnormality of gastrointestinal tract – segmental dilatation of the ileum, is described in details and the literature is reviewed.

  3. ω-3 Polyunsaturated fatty acids prevent pressure overload-induced ventricular dilation and decrease in mitochondrial enzymes despite no change in adiponectin

    Directory of Open Access Journals (Sweden)

    O'Shea Karen M

    2010-09-01

    Full Text Available Abstract Background Pathological left ventricular (LV hypertrophy frequently progresses to dilated heart failure with suppressed mitochondrial oxidative capacity. Dietary marine ω-3 polyunsaturated fatty acids (ω-3 PUFA up-regulate adiponectin and prevent LV dilation in rats subjected to pressure overload. This study 1 assessed the effects of ω-3 PUFA on LV dilation and down-regulation of mitochondrial enzymes in response to pressure overload; and 2 evaluated the role of adiponectin in mediating the effects of ω-3 PUFA in heart. Methods Wild type (WT and adiponectin-/- mice underwent transverse aortic constriction (TAC and were fed standard chow ± ω-3 PUFA for 6 weeks. At 6 weeks, echocardiography was performed to assess LV function, mice were terminated, and mitochondrial enzyme activities were evaluated. Results TAC induced similar pathological LV hypertrophy compared to sham mice in both strains on both diets. In WT mice TAC increased LV systolic and diastolic volumes and reduced mitochondrial enzyme activities, which were attenuated by ω-3 PUFA without increasing adiponectin. In contrast, adiponectin-/- mice displayed no increase in LV end diastolic and systolic volumes or decrease in mitochondrial enzymes with TAC, and did not respond to ω-3 PUFA. Conclusion These findings suggest ω-3 PUFA attenuates cardiac pathology in response to pressure overload independent of an elevation in adiponectin.

  4. Blood flow dynamic improvement with aneurysm repair detected by a patient-specific model of multiple aortic aneurysms.

    Science.gov (United States)

    Sughimoto, Koichi; Takahara, Yoshiharu; Mogi, Kenji; Yamazaki, Kenji; Tsubota, Ken'ichi; Liang, Fuyou; Liu, Hao

    2014-05-01

    Aortic aneurysms may cause the turbulence of blood flow and result in the energy loss of the blood flow, while grafting of the dilated aorta may ameliorate these hemodynamic disturbances, contributing to the alleviation of the energy efficiency of blood flow delivery. However, evaluating of the energy efficiency of blood flow in an aortic aneurysm has been technically difficult to estimate and not comprehensively understood yet. We devised a multiscale computational biomechanical model, introducing novel flow indices, to investigate a single male patient with multiple aortic aneurysms. Preoperative levels of wall shear stress and oscillatory shear index (OSI) were elevated but declined after staged grafting procedures: OSI decreased from 0.280 to 0.257 (first operation) and 0.221 (second operation). Graftings may strategically counter the loss of efficient blood delivery to improve hemodynamics of the aorta. The energy efficiency of blood flow also improved postoperatively. Novel indices of pulsatile pressure index (PPI) and pulsatile energy loss index (PELI) were evaluated to characterize and quantify energy loss of pulsatile blood flow. Mean PPI decreased from 0.445 to 0.423 (first operation) and 0.359 (second operation), respectively; while the preoperative PELI of 0.986 dropped to 0.820 and 0.831. Graftings contributed not only to ameliorate wall shear stress or oscillatory shear index but also to improve efficient blood flow. This patient-specific modeling will help in analyzing the mechanism of aortic aneurysm formation and may play an important role in quantifying the energy efficiency or loss in blood delivery.

  5. Reverse extra-anatomic aortic arch debranching procedure allowing thoracic endovascular aortic repair of a chronic ascending aortic aneurysm

    Directory of Open Access Journals (Sweden)

    Ludovic Canaud, MD, PhD

    2018-06-01

    Full Text Available A 79-year-old woman was admitted with a large chronic dissecting ascending aortic aneurysm starting 5 mm distal to the ostia of the left coronary artery and ending immediately proximal to the innominate artery. A reverse extra-anatomic aortic arch debranching procedure was performed. During the same operative time, through a transapical approach, a thoracic stent graft was deployed with the proximal landing zone just distal to the coronary ostia and the distal landing zone excluding the origin of the left common carotid artery. The postoperative course was uneventful. Computed tomography at 12 months documented patent extra-anatomic aortic arch debranching and no evidence of endoleak. Keywords: Ascending aorta, Thoracic aorta, Aortic dissection, Stent graft

  6. Retrograde ascending aortic dissection during or after thoracic aortic stent graft placement: insight from the European registry on endovascular aortic repair complications

    DEFF Research Database (Denmark)

    Eggebrecht, Holger; Thompson, Matt; Rousseau, Hervé

    2009-01-01

    BACKGROUND: Single-center reports have identified retrograde ascending aortic dissection (rAAD) as a potentially lethal complication of thoracic endovascular aortic repair (TEVAR). METHODS AND RESULTS: Between 1995 and 2008, 28 centers participating in the European Registry on Endovascular Aortic...

  7. Application of Electrocautery Needle Knife Combined with Balloon Dilatation versus Balloon Dilatation in the Treatment of Tracheal Fibrotic Scar Stenosis.

    Science.gov (United States)

    Bo, Liyan; Li, Congcong; Chen, Min; Mu, Deguang; Jin, Faguang

    Electrocautery needle knives can largely reduce scar and granulation tissue hyperplasia and play an important role in treating patients with benign stricture. The aim of this retrospective study was to evaluate the efficacy and safety of electrocautery needle knife combined with balloon dilatation versus balloon dilatation alone in the treatment of tracheal stenosis caused by tracheal intubation or tracheotomy. We retrospectively analysed the clinical data of 43 patients with tracheal stenosis caused by tracheotomy or tracheal intubation in our department from January 2013 to January 2016. Among these 43 patients, 23 had simple web-like stenosis and 20 had complex steno sis. All patients were treated under general anaesthesia, and the treatment methods were (1) balloon dilatation alone, (2) needle knife excision of fibrotic tissue combined with balloon dilatation, and (3) needle knife radial incision of fibrotic tissue combined with balloon dilatation. After treatment the symptoms, such as shortness of breath, were markedly improved immediately in all cases. The stenosis degree of patients who were treated with the elec-trocautery needle knife combined with balloon dilatation had better improvement compared with that of those treated with balloon dilatation treatment alone after 3 months (0.45 ± 0.04 vs. 0.67 ± 0.05, p knife combined with balloon dilatation is an effective and safe treatment for tracheal fibrotic stenosis compared with balloon dilatation alone. © 2017 S. Karger AG, Basel.

  8. Magnetic resonance measurement of turbulent kinetic energy for the estimation of irreversible pressure loss in aortic stenosis.

    Science.gov (United States)

    Dyverfeldt, Petter; Hope, Michael D; Tseng, Elaine E; Saloner, David

    2013-01-01

    The authors sought to measure the turbulent kinetic energy (TKE) in the ascending aorta of patients with aortic stenosis and to assess its relationship to irreversible pressure loss. Irreversible pressure loss caused by energy dissipation in post-stenotic flow is an important determinant of the hemodynamic significance of aortic stenosis. The simplified Bernoulli equation used to estimate pressure gradients often misclassifies the ventricular overload caused by aortic stenosis. The current gold standard for estimation of irreversible pressure loss is catheterization, but this method is rarely used due to its invasiveness. Post-stenotic pressure loss is largely caused by dissipation of turbulent kinetic energy into heat. Recent developments in magnetic resonance flow imaging permit noninvasive estimation of TKE. The study was approved by the local ethics review board and all subjects gave written informed consent. Three-dimensional cine magnetic resonance flow imaging was used to measure TKE in 18 subjects (4 normal volunteers, 14 patients with aortic stenosis with and without dilation). For each subject, the peak total TKE in the ascending aorta was compared with a pressure loss index. The pressure loss index was based on a previously validated theory relating pressure loss to measures obtainable by echocardiography. The total TKE did not appear to be related to global flow patterns visualized based on magnetic resonance-measured velocity fields. The TKE was significantly higher in patients with aortic stenosis than in normal volunteers (p < 0.001). The peak total TKE in the ascending aorta was strongly correlated to index pressure loss (R(2) = 0.91). Peak total TKE in the ascending aorta correlated strongly with irreversible pressure loss estimated by a well-established method. Direct measurement of TKE by magnetic resonance flow imaging may, with further validation, be used to estimate irreversible pressure loss in aortic stenosis. Copyright © 2013 American

  9. Comparison of aortic root dimensions and geometries before and after transcatheter aortic valve implantation by 2- and 3-dimensional transesophageal echocardiography and multislice computed tomography.

    NARCIS (Netherlands)

    Ng, A.C.; Delgado, V.; Kley, F. van der; Shanks, M.; Veire, N.R. van de; Bertini, M.; Nucifora, G.; Bommel, R.J. van; Tops, L.F.; Weger, A. de; Tavilla, G.; Roos, A. de; Kroft, L.J.; Leung, D.Y.; Schuijf, J.; Schalij, M.J.; Bax, J.J.

    2010-01-01

    BACKGROUND: 3D transesophageal echocardiography (TEE) may provide more accurate aortic annular and left ventricular outflow tract (LVOT) dimensions and geometries compared with 2D TEE. We assessed agreements between 2D and 3D TEE measurements with multislice computed tomography (MSCT) and changes in

  10. Comparison of Pneumatic Dilation with Pneumatic Dilation Plus Botulinum Toxin for Treatment of Achalasia

    Directory of Open Access Journals (Sweden)

    Alireza Bakhshipour

    2010-03-01

    Full Text Available Among the therapeutic options for achalasia are pneumatic dilatation (PD, an appropriate long-term therapy, and botulinum toxin injection (BT that is a relatively short-term therapy. This study aimed to compare therapeutic effect of repetitive pneumatic dilation with a combined method (botulinum toxin injection and pneumatic dilation in a group of achalasia patients who are low responder to two initial pneumatic dilations. Thirty- four patients with documented primary achalasia that had low response to two times PD (<50% decrease in symptom score and barium height at 5 minute in timed esophagogram after 3month of late PD were randomized to receive pneumatic dilation (n=18 or botulinum toxin injection and pneumatic dilation by four weeks interval (n=16, PD and BT+PD groups respectively. Symptom scores were evaluated before and at 1, 6 and 12 months after treatment. Clinical remission was defined as a decrease in symptom score ≥ 50% of baseline. There were no significant differences between the two groups in gender, age and achalasia type. Remission rate of patients in BT-PD group in comparison with PD group were 87.5% vs. 67.1% (P = 0.7, 87.5% vs. 61.1% (P = 0.59 and 87.5% vs. 55.5% (P = 0.53 at 1, 6 and 12 months respectively .There were no major complications in either group. The mean symptom score decreased by 62.71% in the BT-PD group (P < 0.002 and 50.77% in the PD group (P < 0.01 at the end of the first year. Despite a better response rate in BT+PD group, a difference was not statistically significant. A difference may be meaningful if a large numbers of patients are included in the study.

  11. A insuficiência da valva aórtica na dissecção crônica da aorta proximal: troca ou reconstrução valvar? Aortic valve insufficiency in chronic aortic dissection: valve replacement or reconstruction?

    Directory of Open Access Journals (Sweden)

    Ricardo Beyruti

    1993-06-01

    Full Text Available Com o objetivo de avaliar os resultados clínicos e ecocardiográficos tardios obtidos com a correção da insuficiência aórtica decorrente da dissecção crônica da aorta proximal, foram estudados 48 pacientes consecutivos operados entre janeiro de 1980 e dezembro de 1989, separados em 2 grupos de 24 pacientes cada. Grupo A - pacientes nos quais a valva aórtica foi preservada pela "resuspensão comissural"; Grupo B - pacientes nos quais a valva aórtica foi substituída. Na avaliação ecocardiográfica pré-operatória, os pacientes do Grupo B apresentavam grau de insuficiência aórtica, diâmetros ventriculares (sistólico e diastólico e da aorta ascendente significativamente maiores do que os do Grupo A (p=0,03, sendo comparáveis nos demais parâmetros. A mortalidade hospitalar foi 12,5% no Grupo A e de 4,17% no Grupo B e a sobrevida aos 7 anos, respectivamente, 75,75% ± 9,82% e 82,72% ± 7,87% (NS. A avaliação clínica mostrou que, no pós-operatório, houve melhora significativa (pFrom January 1980 to December 1989, 48 consecutive patients with chronic aortic dissection and aortic valve insufficiency underwent operation. They were analyzed in 2 groups (24 patients each to evaluate the late clinical and echocardiography outcome of those in whom the aortic valve was preserved (Group A compared with those having valve replacement (Group B. The overall preoperative characteristics of the two groups were similar except for the age (Group A 47.9 ± 10.8 versus Group B 40.2 ± 9.5 years, p=0.03. In the preoperative echocardiographic evaluation, Group B patients had significantly higher aortic root and left ventricle systolic and diastolic diameters (p<0.03, and aortic insufficiency grade (p=0.02. The hospital mortality was 12.5% in Group A and 4.2% in Group B and the seven years actuarial survival rate was respectively 75.7% ± 9.8% and 82.7% ± 7.9%. Postoperatively there was, in both groups, a significant improvement in all clinical

  12. Vaginal dilator therapy for women receiving pelvic radiotherapy.

    Science.gov (United States)

    Miles, Tracie; Johnson, Nick

    2014-09-08

    Vaginal dilation therapy is advocated after pelvic radiotherapy to prevent stenosis (abnormal narrowing of the vagina), but can be uncomfortable and psychologically distressing. To assess the benefits and harms of different types of vaginal dilation methods offered to women treated by pelvic radiotherapy for cancer. Searches included the Cochrane Central Register of Controlled Trials (CENTRAL 2013, Issue 5), MEDLINE (1950 to June week 2, 2013), EMBASE (1980 to 2013 week 24) and CINAHL (1982 to 2013). Comparative data of any type, which evaluated dilation or penetration of the vagina after pelvic radiotherapy treatment for cancer. Two review authors independently assessed whether potentially relevant studies met the inclusion criteria. We found no trials and therefore analysed no data. We identified no studies for inclusion in the original review or for this update. However, we felt that some studies that were excluded warranted discussion. These included one randomised trial (RCT), which showed no improvement in sexual scores associated with encouraging women to practise dilation therapy; a recent small RCT that did not show any advantage to dilation over vibration therapy during radiotherapy; two non-randomised comparative studies; and five correlation studies. One of these showed that objective measurements of vaginal elasticity and length were not linked to dilation during radiotherapy, but the study lacked power. One study showed that women who dilated tolerated a larger dilator, but the risk of objectivity and bias with historical controls was high. Another study showed that the vaginal measurements increased in length by a mean of 3 cm after dilation was introduced 6 to 10 weeks after radiotherapy, but there was no control group; another case series showed the opposite. Three recent studies showed less stenosis associated with prophylactic dilation after radiotherapy. One small case series suggested that dilation years after radiotherapy might restore the

  13. Geometric Deformations of the Thoracic Aorta and Supra-Aortic Arch Branch Vessels Following Thoracic Endovascular Aortic Repair.

    Science.gov (United States)

    Ullery, Brant W; Suh, Ga-Young; Hirotsu, Kelsey; Zhu, David; Lee, Jason T; Dake, Michael D; Fleischmann, Dominik; Cheng, Christopher P

    2018-04-01

    To utilize 3-D modeling techniques to better characterize geometric deformations of the supra-aortic arch branch vessels and descending thoracic aorta after thoracic endovascular aortic repair. Eighteen patients underwent endovascular repair of either type B aortic dissection (n = 10) or thoracic aortic aneurysm (n = 8). Computed tomography angiography was obtained pre- and postprocedure, and 3-D geometric models of the aorta and supra-aortic branch vessels were constructed. Branch angle of the supra-aortic branch vessels and curvature metrics of the ascending aorta, aortic arch, and stented thoracic aortic lumen were calculated both at pre- and postintervention. The left common carotid artery branch angle was lower than the left subclavian artery angles preintervention ( P Supra-aortic branch vessel angulation remains relatively static when proximal landing zones are distal to the left common carotid artery.

  14. Aortic obstruction: anatomy and echocardiography

    Directory of Open Access Journals (Sweden)

    Keirns Candace

    2006-09-01

    Full Text Available Abstract Echocardiography is a valuable non-invasive technique for identifying the site and type of aortic obstruction. Knowledge of the morphological details of each type of obstruction is the basis for correct interpretation of the diagnostic images and clinical decisions. This study was undertaken to correlate the echocardiographic images with anatomic specimens of equivalent valvular and supravalvular aortic obstruction. Specimens were part of the collection of the Department of Embryology. Fifty six patients were studied, and forty specimens with aortic obstruction were analyzed. Echocardiographic characteristics: Thirty one (55.3% patients were women and twenty five (44.7% men. Valvular aortic obstruction was found in Thirty six patients (64.3 % and supravalvular aortic obstruction in twenty (35.7%. Anatomic characteristics: Of the forty specimens examined, twenty one (52.5% had valvular aortic obstruction and nineteen (47.5% supravalvular aortic obstruction. The anatomoechocardiographic correlation clearly showed that the anatomic findings of the specimen hearts and aortas corresponded to echocardiographic images of valvular and supravalvular aortic obstruction and provided solid corroboration of echocardiographic diagnoses.

  15. Unusual Case of Overt Aortic Dissection Mimicking Aortic Intramural Hematoma

    Directory of Open Access Journals (Sweden)

    Kushtrim Disha

    2016-04-01

    Full Text Available We report an interesting case in which overt aortic dissection mimicked two episodes of aortic intramural hematoma (IMH (Stanford A, DeBakey I. This took place over the course of four days and had a major influence on the surgical treatment strategy. The first episode of IMH regressed completely within 15 hours after it was clinically diagnosed and verified using imaging techniques. The recurrence of IMH was detected three days thereafter, resulting in an urgent surgical intervention. Overt aortic dissection with evidence of an intimal tear was diagnosed intraoperatively.

  16. Supra-aortic interventions for endovascular exclusion of the entire aortic arch.

    Science.gov (United States)

    Andrási, Terézia B; Grossmann, Marius; Zenker, Dieter; Danner, Bernhard C; Schöndube, Friedrich A

    2017-07-01

    Our aim was to analyze the outcomes of endovascular exclusion of the entire aortic arch (proximal landing in zone 0, distal landing in zone III or beyond, after Ishimaru) in which complete surgical debranching of the supra-aortic vessels (I), endovascular supra-aortic revascularization (chimney, fenestrated, or branched grafts) with partial surgical debranching (II), or total endovascular supra-aortic revascularization (III) was additionally performed. Publications describing endovascular repair of the aortic arch (2000-2016) were systematically searched and reviewed. From a total of 53 relevant studies including 1853 patients, only 1021 patients undergoing 35 different total aortic arch procedures were found eligible for further evaluation and included in group I, II, or III (429, 190, and 402 patients, respectively). Overall early mortality was higher in group I vs groups II and III (P = .001; 1 - β = 95.6%) but exceeded in group III (18.6%) and group II (14.0%) vs group I (8.0%; P = .044; 1 - β = 57.4%) for diseases involving zone 0. Mortality was higher in all subgroups treated for zone 0 disease compared with corresponding subgroups treated for zone I to zone III disease. The incidence of cerebral ischemic events was increased in groups I and II vs group III (7.5% and 11% vs 1.7%; P = .0001) and correlated with early mortality (R 2  = .20; P = .033). The incidence of type II endoleaks and endovascular reintervention was similar between groups and correlated with each other (R 2  = .37; P = .004). Type Ia endoleak occurred more often in groups II and III than in group I (7.1% and 12.1% vs 5.8%; P = .023) and correlated with midterm mortality (R 2  = .53; P = .005). Retrograde type A dissection was low in all groups, whereas aneurysm growth was higher in group III (2.6%, 4.2%, 10.7%; P = .002), correlating with midterm mortality (R 2  = .311; P = .009). Surgical revision slightly correlated with surgical complications (R 2  = .18; P = .044

  17. Contemporary results of aortic valve repair for congenital disease: lessons for management and staged strategy.

    Science.gov (United States)

    Vergnat, Mathieu; Asfour, Boulos; Arenz, Claudia; Suchowerskyj, Philipp; Bierbach, Benjamin; Schindler, Ehrenfried; Schneider, Martin; Hraska, Victor

    2017-09-01

    Any aortic valve (AoV) operation in children (repair, Ross or mechanical replacement) is a palliation and reinterventions are frequent. AoV repair is a temporary solution primarily aimed at allowing the patient to grow to an age when more definitive solutions are available. We retrospectively analysed AoV repair effectiveness across the whole age spectrum of children, excluding neonates and AoV disease secondary to congenital heart disease. From 2003 to 2015, 193 consecutive patients were included. The mean age was 9.2 ± 6.9 years (22% disease. The procedures performed were commissurotomy shaving (n = 74; 38%), leaflet replacement (n = 78; 40%), leaflet extension (n = 21; 11%) and neocommissure creation (n = 21; 11%). Post-repair geometry was tricuspid in 137 (71%) patients. The 10-year survival rate was 97.1%. Freedom from reoperation and replacement at 7 years was, respectively, 57% (95% confidence interval, 47-66) and 68% (95% confidence interval, 59-76). In multivariate analysis, balloon dilatation before 6 months, the absence of a developed commissure, a non-tricuspid post-repair geometry and cross-clamp duration were predictors for reoperation and replacement. After a mean follow-up period of 5.1 ± 3.0 years, 145 (75%) patients had a preserved native valve, with undisturbed valve function (peak gradient <40 mmHg, regurgitation ≤mild) in 113 (58%). Aortic valve repair in children is safe and effective in delaying the timing for more definitive solution. Surgical strategy should be individualized according to the age of the patient. Avoidance of early balloon dilatation and aiming for a tricuspid post-repair arrangement may improve outcomes. © The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

  18. Early and mid-term results with the Growth Stent--a possible concept for transcatheter treatment of aortic coarctation from infancy to adulthood by stent implantation?

    Science.gov (United States)

    Ewert, Peter; Peters, Bjoern; Nagdyman, Nicole; Miera, Oliver; Kühne, Titus; Berger, Felix

    2008-01-01

    Evaluation of the Growth Stent-a stent consisting of two stent halves connected by reabsorbable sutures-for the treatment of aortic coarctation in infants. Surveillance study of 13 Growth Stents implanted in 12 patients aged 1-15 months (median 5 months). Body weight ranged from 3.4-12.8 kg (median 5.4 kg). Eight patients suffered from aortic (re-)coarctation, four of them from stenosis of the aortic anastomosis after a Norwood I procedure. The follow-up period was 24 months (11-51 months). Pressure gradients immediately after stent implantation decreased from 30 mm Hg (range 20-50 mm Hg) to 8 mm Hg (range 0-15 mm Hg). Five patients had one (3 pts) or two (2 pts) balloon dilations 3-28 months (median 12 months) after Growth Stent implantation. The median pressure gradient decreased from 25 mm Hg (range 15-30 mm Hg) to 15 mm Hg (range 5-25 mm Hg). Six patients received a large stent after 19-34 months. Median body weight was 11.8 kg (9.4-15 kg). The Growth Stent is suitable for the acute treatment of aortic coarctation in infants and can be overstented later on--if necessary--with a larger stent without causing restriction. Copyright 2008 Wiley-Liss, Inc.

  19. Clinical Efficacy of Transthoracic Echocardiography for Screening Abdominal Aortic Aneurysm in Turkish Patients.

    Science.gov (United States)

    Kilic, Salih; Saracoglu, Erhan; Cekici, Yusuf

    2018-03-01

    The objective of this study was to investigate the prevalence of abdominal aortic aneurysm (AAA) in Turkish patients aged ≥ 65 years, and to demonstrate the applicability of echocardiography to AAA screening. Transthoracic echocardiography (TTE) was performed in all consecutive patients aged ≥ 65 years who were referred to cardiology clinics or were referred from other outpatient clinics. The abdominal aorta (AA) of each patient was scanned using the same probe, and the time spent was recorded. Demographic and clinic characteristics of the patients were recorded at the end of the echocardiography. Among 1948 patients (mean age 70.9 ± 6 years; 49.8% male), the AA was visualized in 96.3%. AAA was identified in 3.7% (69/1878) of the patients, of whom AAA was previously known in 20.3% (n = 14). The prevalence of unknown AAA was 2.93%. The average time needed to scan and measure the AA was 1 minute and 3 seconds (±23 seconds). Aortic root diameters were significantly higher in the patients with AAA than in those without AAA (34.7 ± 4.2 vs. 29.8 ± 4.7; p < 0.001). Age (per 1 year increase) [odds ratio (OR), 1.245; p < 0.001], male gender (OR, 5.382; p < 0.001), smoking (OR, 2.118; p = 0.037), and aortic root diameter (per 1 mm increase) (OR, 1.299; p < 0.001) were independent predictors of AAA. This study is important in that it showed a high prevalence of AAA in Turkish patients aged ≥ 65 years, and demonstrated that AAA can be visualized in the majority of patients in as little as 1 minute during TTE.

  20. Endoluminal Treatment of Ruptured Abdominal Aortic Aneurysm with Small Intestinal Submucosa Sandwich Endografts: A Pilot Study in Sheep

    International Nuclear Information System (INIS)

    Yamada, Katsuyuki; Pavcnik, Dusan; Uchida, Barry T.; Timmermans, Hans A.; Corless, Christopher L.; Yin, Qiang; Yamakado, Koichiro; Wha Park, Joong; Roesch, Josef; Keller, Frederick S.; Sato, Morio; Yamada, Ryusaku

    2001-01-01

    Purpose: To evaluate efficacy of small intestinal submucosa (SIS) Sandwich endografts for the treatment of acute rupture of abdominal aortic aneurysms (AAA) and to explore the short-term reaction of the aorta to this material.Methods: In eight adult sheep, an infrarenal AAA was created transluminally by dilation of a short Palmaz stent. In six sheep, the aneurysm was then ruptured by overdilation of the stent with a large angioplasty balloon. Two sheep with AAAs that were not ruptured served as controls. A SIS Sandwich endograft, consisting of a Z stent frame with 5 bodies and covered inside and out with SIS, was used to exclude the ruptured and non-ruptured AAAs. Follow-up aortography was done immediately after the procedure and before sacrifice at 4, 8, or 12 weeks. Autopsy and histologic studies followed.Results: Endograft placement was successful in all eight sheep. Both ruptured and non-ruptured AAAs were successfully excluded. Three animals with AAA rupture developed hind leg paralysis due to compromise of the arterial supply to the lower spinal cord and were sacrificed 1 day after the procedure. In five animals, three with rupture and two controls, follow-up aortograms revealed no aortic stenoses and no perigraft leaks. Gross and histologic studies revealed incorporation of the endografts into the aortic wall with replacement of SIS by dense neointima that was completely endothelialized in areas where the endograft was in direct contact with the aortic wall. In central portions of the endograft, in contact with the thrombosed aneurysm, endothelialization was incomplete even at 12 weeks.Conclusion: The SIS Sandwich endografts effectively excluded simple AAAs and ruptured AAAs. They were rapidly incorporated into the aortic wall. A detailed long-term study is warranted