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Sample records for aortic arch syndromes

  1. Inflammatory aortic arch syndrome: contrast-enhanced, three-dimensional MR - angiography in stenotic lesions

    International Nuclear Information System (INIS)

    Both, M.; Mueller-Huelsbeck, S.; Biederer, J.; Heller, M.; Reuter, M.

    2004-01-01

    Purpose: To determine the value of contrast-enhanced, three-dimensional MR angiography for the evaluation of stenotic and occlusive vascular lesions in inflammatory aortic arch syndrome. Materials and Methods: 14 patients with inflammatory aortic arch syndrome (giant cell arteritis: n = 8, Takayasu arteritis: n = 4, ankylosing spondylitis: n = 1 sarcoidosis: n = 1) underwent MR angiography of the aortic arch and the supra-aortic vessels (n = 15,2 patients were examined twice) and of the abdominal aorta (n = 2). MRA was performed using a 3D-FLASH sequence (TR/TE 4.6/1.8 ms, flip angle 30 ) on a 1.5T system. MRA imaging was compared with the findings of DSA, which served as gold standard. Results: In a total of 467 examined vascular territories, DSA revealed 50 stenoses and 35 occlusions. All lesions were detected by MRA. In 23 segments, the degree of stenosis was overestimated by MRA. Sensitivity and specificity of MRA were 100% and 94,3%, positive and negative predictive values were 73.6 and 100%, and the accuracy was 95,1%. Conclusions: Despite a tendency to overestimate stenoses, contrast-enhanced three-dimensional MR angiography is a valid, non-invasive technique in the assessment of inflammatory aortic arch syndrome. (orig.) [de

  2. Aortic arch malformations

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    Kellenberger, Christian J. [University Children' s Hospital, Department of Diagnostic Imaging, Zuerich (Switzerland)

    2010-06-15

    Although anomalies of the aortic arch and its branches are relatively uncommon malformations, they are often associated with congenital heart disease. Isolated lesions may be clinically significant when the airways are compromised by a vascular ring. In this article, the development and imaging appearance of the aortic arch system and its various malformations are reviewed. (orig.)

  3. Aortic arch malformations

    International Nuclear Information System (INIS)

    Kellenberger, Christian J.

    2010-01-01

    Although anomalies of the aortic arch and its branches are relatively uncommon malformations, they are often associated with congenital heart disease. Isolated lesions may be clinically significant when the airways are compromised by a vascular ring. In this article, the development and imaging appearance of the aortic arch system and its various malformations are reviewed. (orig.)

  4. Double aortic arch

    Science.gov (United States)

    Surgery can be done to fix double aortic arch. The surgeon ties off the smaller branch and separates it from the larger branch. Then the surgeon closes the ends of the aorta with stitches. This relieves pressure on the esophagus and windpipe.

  5. [A re-operative case of bentall operation and aortic arch replacement using a stent graft for a Marfan syndrome, post sternum turnover and post mitral valve replacement].

    Science.gov (United States)

    Sasaki, H; Aomi, S; Noji, S; Uwabe, K; Kihara, S; Kurihara, H; Koyanagi, H

    2001-09-01

    A 36-year-old male with Marfan syndrome succesfully underwent Bentall operation and aortic arch replacement using a stent graft as an elephant trunk. He had received MVR with sternum turn over 14 years previously. Median sternotomy was performed. Under circulatory arrest with rertograde cerebral perfusion we performed Bentall operation and aortic arch replacement using a stent graft. The sternum was cured well. Retractive breathing was not detected. This surgical procedure was effective for cardiovascular disease with Marfan syndrome.

  6. Bovine aortic arch with supravalvular aortic stenosis.

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    Idhrees, Mohammed; Cherian, Vijay Thomas; Menon, Sabarinath; Mathew, Thomas; Dharan, Baiju S; Jayakumar, K

    2016-09-01

    A 5-year-old boy was diagnosed to have supravalvular aortic stenosis (SVAS). On evaluation of CT angiogram, there was associated bovine aortic arch (BAA). Association of BAA with SVAS has not been previously reported in literature, and to best of our knowledge, this is the first case report of SVAS with BAA. Recent studies show BAA as a marker for aortopathy. SVAS is also an arteriopathy. In light of this, SVAS can also possibly be a manifestation of aortopathy associated with BAA. Copyright © 2015 Cardiological Society of India. Published by Elsevier B.V. All rights reserved.

  7. Bovine aortic arch with supravalvular aortic stenosis

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    Mohammed Idhrees

    2016-09-01

    Full Text Available A 5-year-old boy was diagnosed to have supravalvular aortic stenosis (SVAS. On evaluation of CT angiogram, there was associated bovine aortic arch (BAA. Association of BAA with SVAS has not been previously reported in literature, and to best of our knowledge, this is the first case report of SVAS with BAA. Recent studies show BAA as a marker for aortopathy. SVAS is also an arteriopathy. In light of this, SVAS can also possibly be a manifestation of aortopathy associated with BAA.

  8. Ortner’s syndrome: Cardiovocal syndrome caused by aortic arch ps

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    Adil H. Al Kindi

    2016-10-01

    Ortner’s syndrome describes vocal changes caused by cardiovascular pathology. It should be included in the differential diagnosis of patients with cardiovascular risk factors presenting with hoarseness. This case demonstrates the use of endovascular stents to treat the causative pathology with resolution of symptoms. In expert hands, it represents low risk, minimally invasive therapeutic strategy with excellent early results in patients who are high risk for open procedure.

  9. Mycotic Aneurysm of the Aortic Arch

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    Ji Hye Seo

    2014-08-01

    Full Text Available A mycotic aneurysm of the thoracic aorta is rare. We report a case of mycotic aneurysm that developed in the aortic arch. An 86-year-old man was admitted with fever and general weakness. Blood culture yielded methicillin-resistant Staphylococcus aureus. Chest X-ray showed an enlarged aortic arch, and computed tomography scan revealed an aneurysm in the aortic arch. The patient was treated only with antibiotics and not surgically. The size of the aneurysm increased rapidly, resulting in bronchial obstruction and superimposed pneumonia. The patient died of respiratory failure.

  10. Separate origins of the left internal and external carotid arteries from the aortic arch and cervical internal carotid artery aneurysm in a patient with Noonan syndrome.

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    Tahir, Rizwan Ahmad; Asmaro, Karam; Pabaney, Aqueel; Kole, Max; Nypaver, Timothy; Marin, Horia

    2017-04-01

    Distinct origins of the external carotid artery and the internal carotid artery (ICA) from the aortic arch have been rarely described, and represent an aberrant development of the aortic arches during fetal life. This anatomical variation is usually discovered incidentally; infrequently, an aneurysm of the cervical ICA might accompany this rare configuration. We describe one such case in a patient with Noonan syndrome who presented with pulsatile neck mass. The diagnostic features and management of the aneurysm and a review of the literature are presented. 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/.

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

    International Nuclear Information System (INIS)

    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.

  12. Acute Aortic Arch Perforation During Transcatheter Aortic Valve Replacement in Bicuspid Aortic Stenosis and a Gothic Aortic Arch.

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    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.

  13. Arch reconstruction with autologous pulmonary artery patch in interrupted aortic arch.

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    Lee, Won-Young; Park, Jeong-Jun

    2014-04-01

    Various surgical techniques have been developed for the repair of an interrupted aortic arch. However, tension and Gothic arch formation at the anastomotic site have remained major problems for these techniques: Excessive tension causes arch stenosis and left main bronchus compression, and Gothic arch configuration is related to cardiovascular complications. To resolve these problems, we adopted a modified surgical technique of distal aortic arch augmentation using an autologous main pulmonary artery patch. The descending aorta was then anastomosed to the augmented aortic arch in an end-to-side manner. Here, we report two cases of interrupted aortic arch that were repaired using this technique.

  14. Hybrid treatment of aortic arch disease

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    Metzger, Patrick Bastos; Rossi, Fabio Henrique; Moreira, Samuel Martins; Issa, Mario; Izukawa, Nilo Mitsuru; Dinkhuysen, Jarbas J.; Spina Neto, Domingos; Kambara, Antônio Massamitsu

    2014-01-01

    Introduction The management of thoracic aortic disease involving the ascending aorta, aortic arch and descending thoracic aorta are technically challenging and is an area in constant development and innovation. Objective To analyze early and midterm results of hybrid treatment of arch aortic disease. Methods Retrospective study of procedures performed from January 2010 to December 2012. The end points were the technical success, therapeutic success, morbidity and mortality, neurologic outcomes, the rate of endoleaks and reinterventions. Results A total of 95 patients treated for thoracic aortic diseases in this period, 18 underwent hybrid treatment and entered in this study. The average ages were 62.3 years. The male was present in 66.7%. The technical and therapeutic success was 94.5% e 83.3%. The perioperative mortality rate of 11.1%. There is any death during one-year follow- up. The reoperation rates were 16.6% due 2 cases of endoleak Ia and one case of endoleak II. There is any occlusion of anatomic or extra anatomic bypass during follow up. Conclusion In our study, the hybrid treatment of aortic arch disease proved to be a feasible alternative of conventional surgery. The therapeutic success rates and re- interventions obtained demonstrate the necessity of thorough clinical follow-up of these patients in a long time. PMID:25714205

  15. A bovine aortic arch in humans

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    María Elena Arnáiz-García

    2014-05-01

    Full Text Available We describe a curious congenital variation of human aortic arch (AA branching pattern termed the “bovine aortic arch”. Rather than arising directly from the AA as a separate branch as occurs in the most common AA branching pattern, the left common carotid artery moves to the right and merges from the brachiocephalic trunk. It is the normal AA branching pattern presented in a number of animals (canines, felines or Macaque monkeys but it has nothing to do with anatomy of AA in ruminant animals, including cattle and buffalo. That is why it is one of the most widely misnomers used in medical literature whose origin is nowadays unknown.

  16. Recurrent Pneumonia due to Double Aortic Arch

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    I. Sedighi

    2012-04-01

    Full Text Available Introduction: Pneumonia is one of the most common infections during childhood. In children with recurrent bacterial pneumonia complete evaluation for underlying factors is necessary. The most common underlying diseases include: antibody deficiencies , cystic fibrosis , tracheoesophageal fistula and increased pulmonary blood flow. Vascular ring and its pressure effect is a less common cause of stridor and recurrent pneumonia. Congenital abnormalities in aortic arch and main branches which form vascular ring around esophagus and trachea with variable pressure effect cause respiratory symptoms such as stridor , wheezing and recurrent pneumoniaCase Report: A 2 year old boy was admitted in our hospital with respiratory distress and cough . Chest x-Ray demonstrated right lobar pneumonia. He had history of stridor and wheezing from neonatal period and hospitalization due to pneumonia for four times. The patient received appropriate antibiotics. Despite fever and respiratory distress improvement, wheezing continued. Review of his medical documents showed fixed pressure effect on posterior aspect of esophagus in barium swallow. In CT angiography we confirmed double aortic arch.Conclusion: Double aortic arch is one of the causes of persistant respiratory symptom and recurrent pneumonia in children for which fluoroscopic barium swallow is the first non-invasive diagnostic method.(Sci J Hamadan Univ Med Sci 2012;19(1:70-74

  17. Is there a prospect for hybrid aortic arch surgery?

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    Bashir, Mohamad; Harky, Amer; Bilal, Haris

    2018-05-16

    The surge of endovascular repair of aortic aneurysm in current modern aortic surgery practice has been the key for surgical management of elective cases of thoracic aortic aneurysms. This has paved way for the combined hybrid approach to be amongst the armamentarium for the management of aortic arch disease. The pivotal understanding of the aortic arch natural history coupled with device technology advancement allowed surgeons insight into delivery of hybrid surgery with acceptable morbidity and mortality results. This review article provides current insights into hybrid technique of aortic arch aneurysm repair and the evidences behind its applicability to arch surgery. It is aimed to highlight the challenges encountered for this innovative approach and correlate its challenges to those that are met by the conventional open aortic arch repair.

  18. [Single coronary artery and right aortic arch].

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    Martínez-Quintana, Efrén; Rodríguez-González, Fayna

    2015-01-01

    Coronary anomalies are mostly asymptomatic and diagnosed incidentally during coronary angiography or echocardiography. However, they must be taken into account in the differential diagnosis of angina, dyspnea, syncope, acute myocardial infarction or sudden death in young patients. The case is presented of two rare anomalies, single coronary artery originating from right sinus of Valsalva and right aortic arch, in a 65 year-old patient with atherosclerotic coronary artery disease treated percutaneously. Copyright © 2014 Sociedad Española de Arteriosclerosis. Published by Elsevier España. All rights reserved.

  19. [Right lung cancer with right aortic arch].

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    Kawaguchi, Yasuo; Noriyuki, T; Kuroda, Y; Kuranishi, F; Nakahara, M; Fukuda, T; Ishizaki, Y; Hotta, R; Akimoto, E; Mori, H

    2008-02-01

    An abnormal shadow was detected on chest X-ray mass screening in an asymptomatic 63-year-old man. The further examinations revealed the shadow to be primary lung cancer (Rt. S6. adenocarcinoma, cT2N0M0, c-stage IB) with right aortic arch. We used 3 dimentional-computed tomography (3D-CT) to assess an anatomical feature of vessels in detail. The right lower lobectomy and the dissection of medi astinal lymph nodes was performed. We confirmed no abnormal anatomy of pulmonary artery and vein at surgery, and it was possible to perform right lower lobectomy with the common procedure. Since lymph node was found by intraopetrative pathological examination, since no metastasis from interlobar to subcarinal lymph node was found, we did not perform dissection of upper mediastinal dissection, which was equivalent to ND2a lymph nodes dissection of the left lung cancer in General Rule for Clinical and Pathological Record of Lung Cancer. The patient with right aortic arch is known to have variant anatomy of other intrathoracic vessels occasionally. 3D-CT was quite useful in assessing anatomical feature, and enabled us to perform safe operation.

  20. Comparison of Total Arch and Partial Arch Transposition During Hybrid Endovascular Repair for Aortic Arch Disease.

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    Kang, W C; Ko, Y-G; Oh, P C; Shin, E K; Park, C-H; Choi, D; Youn, Y N; Lee, D Y

    2016-08-01

    Total arch transposition (TAT) during hybrid endovascular repair for aortic arch disease is believed to allow a better landing zone, but also to be associated with higher peri-operative mortality than partial arch transposition (PAT). Information on this issue is limited. This study was a retrospective analysis. All 53 consecutive patients with aortic arch disease (41 males, mean age 65.0 years) who underwent hybrid endovascular repair with TAT (zone 0, n=20) or PAT (zone 1 or 2, n=33) from 2008 to 2014 were analyzed retrospectively. The peri-operative and late outcomes of these two groups were compared. Baseline characteristics, including EuroSCORE II results, were similar in the two groups. After procedures, peri-operative mortalities and stroke rates were similar in the two groups (5.0% vs. 9.1%, p=1.000, and 10.0% vs. 6.1%, p=.627). Interestingly, all four strokes occurred in patients with a type III aortic arch irrespective of transposition type. Primary success rates (80.0% vs. 69.7%, p=.527) and type I endoleak incidences (20.0% vs. 27.3%, p=.744) were not significantly different. During follow up (mean duration 36.9 months), overall survival (89.7% vs. 87.4% at 1 year and 89.7% vs. 79.3% at 3 years; p=.375) and re-intervention free survival rates (78.6% vs. 92.0% at 1 year; 72.0% vs. 62.2% at 3 years, p=.872) were similar in the two groups. Morbidity and mortality were high within the first year of hybrid endovascular therapy for aortic arch disease, implying that candidates for hybrid procedures need to be selected carefully. Hybrid endovascular repair with TAT was found to have peri-operative mortality, stroke, and long-term survival rates comparable with PAT, so hybrid endovascular repair may be considered, irrespective of type of arch reconstruction, when clinically indicated. Copyright © 2016 European Society for Vascular Surgery. Published by Elsevier Ltd. All rights reserved.

  1. Selective cerebro-myocardial perfusion in complex congenital aortic arch pathology: a novel technique.

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    De Rita, Fabrizio; Lucchese, Gianluca; Barozzi, Luca; Menon, Tiziano; Faggian, Giuseppe; Mazzucco, Alessandro; Luciani, Giovanni Battista

    2011-11-01

    Simultaneous cerebro-myocardial perfusion has been described in neonatal and infant arch surgery, suggesting a reduction in cardiac morbidity. Here reported is a novel technique for selective cerebral perfusion combined with controlled and independent myocardial perfusion during surgery for complex or recurrent aortic arch lesions. From April 2008 to April 2011, 10 patients with arch pathology underwent surgery (two hypoplastic left heart syndrome [HLHS], four recurrent arch obstruction, two aortic arch hypoplasia + ventricular septal defect [VSD], one single ventricle + transposition of the great arteries + arch hypoplasia, one interrupted aortic arch type B + VSD). Median age was 63 days (6 days-36 years) and median weight 4.0 kg (1.6-52). Via midline sternotomy, an arterial cannula (6 or 8 Fr for infants) was directly inserted into the innominate artery or through a polytetrafluoroethylene (PTFE) graft (for neonates cerebro-myocardial perfusion was 39 ± 18 min (17-69). Weaning from cardiopulmonary bypass was achieved without inotropic support in three and with low dose in seven patients. One patient required veno-arterial extracorporeal membrane oxygenation. Four patients, body weight cerebro-myocardial perfusion is feasible in patients with complex or recurrent aortic arch disease, starting from premature newborn less than 2.0 kg of body weight to adults. The technique is as safe as previously reported methods of cerebro-myocardial perfusion and possibly more versatile. © 2011, Copyright the Authors. Artificial Organs © 2011, International Center for Artificial Organs and Transplantation and Wiley Periodicals, Inc.

  2. Right-ventricular global longitudinal strain may predict neo-aortic arch obstruction after Norwood/Sano procedure in children with hypoplastic left heart syndrome.

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    Raucci, Frank J; Seckeler, Michael D; Saunders, Christine; Gangemi, James J; Peeler, Benjamin B; Jayakumar, K Anitha

    2013-01-01

    Neo-aortic arch obstruction (NAAO) is a common complication following the Norwood/Sano procedure (NP) for hypoplastic left heart syndrome (HLHS) and is associated with increased morbidity and mortality. However, there is currently no objective method for predicting which patients will develop NAAO. This study was designed to test the hypothesis that hemodynamic changes from development of NAAO after NP in patients with HLHS will lead to changes in myocardial dynamics that could be detected before clinical symptoms develop with strain analysis using velocity vector imaging. Patients with HLHS who had at least one cardiac catheterization after NP were identified retrospectively. Strain analysis was performed on all echocardiograms preceding the first catheterization and any subsequent catheterization performed for intervention on NAAO. Twelve patients developed NAAO and 30 patients never developed NAAO. Right ventricular strain was worse in the group that developed NAAO (-6.2 vs. -8.6 %, p = 0.040) at a median of 59 days prior to diagnosis of NAAO. Those patients that developed NAAO following NP were significantly younger at the time of first catheterization than those that did not develop NAAO (92 ± 50 vs. 140 ± 36 days, p = 0.001). This study demonstrates that right ventricular GLS is abnormal in HLHS patients following NP and worsening right ventricular strain may be predictive of the future development of NAAO.

  3. 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.)

  4. Persistent truncus arteriosis associated with interruption of the aortic arch.

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    Nath, P H; Zollikofer, C; Castaneda-Zuniga, W; Formanek, A; Amplatz, K

    1980-09-01

    Five patients with a combination of truncus arteriosus and interruption of the aortic arch are reported. The combination of those defects significantly increases the surgical risk. This rare cardiac malformation can only be diagnosed radiographically. An aberrant right subclavian artery is present in 25% of patients. It is helpful in suspecting the diagnosis from plain films of the chest. The diagnosis can be made from a ventriculogram, but usually a truncogram is necessary to define the anatomy of the aortic arch.

  5. Anatomical Study of Healthy Aortic Arches.

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    Girsowicz, Elie; Georg, Yannick; Lefebvre, François; Lejay, Anne; Thaveau, Fabien; Roy, Catherine; Ohana, Mickael; Chakfe, Nabil

    2017-10-01

    With the steady increase of endovascular procedures involving the aortic arch (AA), an actual depiction of its anatomy has become mandatory. It has also become necessary to evaluate the natural evolution of the AA morphology as part of the evaluation of endovascular devices durability. The objective of this study was to perform a morphological and anatomical study of the AA and of the supra aortic trunks (SAT) in healthy patients, with an evaluation of their evolution with time, with a specific orientation applied to endovascular therapies of the AA. Sixty-one patients (31 men, mean age 50.8 [18-82]) with a normal anatomy were included in the study. Measurements included the diameters of the AA and SAT in 17 locations, their distance and angulation based on computed tomography angiography data. Statistical analysis focused on descriptive statistics, differences between genders, as well as correlations with age. Aortic diameters (mean ± SD) were 29.5 ± 3.9 mm at the ascending aorta, 28.6 ± 3.9 mm at the innominate artery (IA), 27.1 ± 3.2 mm at the left common carotid artery (LCCA), 25.3 ± 3.0 mm at the left subclavian artery (LSCA), 23.9 ± 3.3 mm at the descending aorta. Mean angulation of the AA was 82° (95% confidence interval [CI]: 78.95-85.19°), mean angulation between LSCA/LCCA was -5.7° (95% CI: -0.9 to 18.7°) and -1.8° (95% CI: 5.4-26.4°) between LCCA/IA. Mean distance between the LSCA and the LCCA was 14.3 mm (95% CI: 13-15.6 mm) and 21.8 mm (95% CI: 20.3-23.4 mm) between LCCA and IA. All diameters of the AA increased with age (P Men had diameters statistically (P women except at the LCCA ostium level. A statistically significant increase of the distances between the LSCA and the LCCA, between the LSCA and the IA and between the IA and the LCCA was found with age, P = 0.027, better understanding of the three-dimensional aspects of the AA, confirmed the variability and heterogeneity of the SAT disposition, and discussed the principles of vascular

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

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    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.

  7. Contemporary results of surgical repair of recurrent aortic arch obstruction.

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    Mery, Carlos M; Khan, Muhammad S; Guzmán-Pruneda, Francisco A; Verm, Raymond; Umakanthan, Ramanan; Watrin, Carmen H; Adachi, Iki; Heinle, Jeffrey S; McKenzie, E Dean; Fraser, Charles D

    2014-07-01

    There is a paucity of data on the current outcomes of surgical intervention for recurrent aortic arch obstruction (RAAO) after initial aortic arch repair in children. The goal of this study is to report the long-term results in these patients. All patients undergoing surgical intervention for RAAO at Texas Children's Hospital from 1995 to 2012 were included. The cohort was divided into four groups based on initial procedure: (1) simple coarctation repair, (2) Norwood procedure, (3) complex congenital heart disease, and (4) interrupted aortic arch. A total of 48 patients age 9 months (range, 22 days to 36 years) underwent 49 procedures for RAAO. All patients had an anatomic repair consisting of either patch aortoplasty (n=27, 55%), aortic arch advancement (n=8, 16%), sliding arch aortoplasty (n=6, 12%), placement of an interposition graft (n=2, 17%), reconstruction with donor allograft (n=4, 8%), extended end-to-end anastomosis (n=1, 2%), or redo Norwood-type reconstruction (n=1, 2%). Most procedures (n=46, 94%) were performed through a median sternotomy using cardiopulmonary bypass. At a median follow-up of 6.1 years (range, 9 days to 17 years), only 2 patients required surgical or catheter-based intervention for RAAO. Hypertension was present in 10% of patients at last follow-up. There were no neurologic or renal complications. There was 1 perioperative death after an aortic arch advancement in group 1. Four other patients have died during follow-up, none of the deaths related to RAAO. Anatomic repair of RAAO is a safe procedure associated with low morbidity and mortality, and low long-term reintervention rates. Copyright © 2014 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  8. Imaging findings in the right aortic arch with mirror image branching of arch vessels: An unusual cause of dysphagia

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    Guneet Singh

    2015-01-01

    Full Text Available We report a case of a 72-year-old female with a right aortic arch with mirror-image branching of arch vessels presenting with dysphagia, and characteristic images on barium esophagogram, contrast-enhanced computed tomography scan, and magnetic resonance aortography. Right-sided thoracic aortic arch with mirror-image branching of the brachiocephalic vessels causing dysphagia without associated congenital cardiac anomalies is extremely uncommon. Right-sided aortic arch is a rare congenital abnormality with incidence of 0.05-0.1% in the normal population. Anomalies of great vessels are usually incidental findings, because they are asymptomatic. Right aortic arch infrequently presents with a vascular ring that can cause complete or partial obstruction of the trachea and/or esophagus. The understanding of this arch anomaly is based on Edward′s hypothesis about the double arch system during embryonic developmental.

  9. Right-sided aortic arch with Kommerell′s aneurysm

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    Sanjay Orathi Patangi

    2014-01-01

    Full Text Available We present a case report of a 55-year-old lady who presented with progressive dysphagia and was diagnosed with a Kommerell′s aneurysm and a right-sided aortic arch. This case report outlines our management strategy and the challenges encountered during the perioperative period in a patient with this rare anomaly.

  10. A case of complete double aortic arch visualized by transthoracic echocardiography.

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    Saito, Naka; Kato, Shingo; Saito, Noritaka; Nakachi, Tatsuya; Fukui, Kazuki; Iwasawa, Tae; Kosuge, Masami; Kimura, Kazuo

    2017-08-01

    A case of double aortic arch that was well visualized using transthoracic echocardiography is reported. A 38-year-old man underwent transthoracic echocardiography for the evaluation of dyspnea. A suprasternal view of transthoracic echocardiography showed the ascending aorta bifurcate to left and right aortic arches, with blood flow from the ascending aorta to bilateral aortic arches. The diagnosis of right side-dominant double aortic arch was made, and the patient's symptom was conceivably related to compression of the trachea due to a vascular ring. This report indicates the potential usefulness of transthoracic echocardiography for noninvasive detection of double aortic arch in adults. © 2017, Wiley Periodicals, Inc.

  11. Neonatal aortic arch reconstruction avoiding circulatory arrest and direct arch vessel cannulation.

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    Tchervenkov, C I; Korkola, S J; Shum-Tim, D; Calaritis, C; Laliberté, E; Reyes, T U; Lavoie, J

    2001-11-01

    Aortic arch reconstruction in neonates routinely requires deep hypothermic circulatory arrest. We reviewed our experience with techniques of continuous low-flow cerebral perfusion (LFCP) avoiding direct arch vessel cannulation. Eighteen patients, with a median age of 11 days (range 1 to 85 days) and a mean weight of 3.2 +/- 0.8 kg, underwent aortic arch reconstruction with LFCP. Seven had biventricular repairs with arch reconstruction, 9 underwent the Norwood operation and 2 had isolated arch repairs. In 1 Norwood and 7 biventricular repair patients, LFCP was maintained by advancing the cannula from the distal ascending aorta into the innominate artery. In 8 of 9 Norwood patients, LFCP was maintained by directing the arterial cannula into the pulmonary artery confluence and perfusing the innominate artery through the right modified Blalock-Taussig shunt fully constructed before cannulation for cardiopulmonary bypass. In 2 patients requiring isolated arch reconstruction, the ascending aorta was cannulated and the cross-clamp was applied just distal to the innominate artery. LFCP was maintained at 0.6 +/- 0.2 L x min(-1) x m(-2) for 41.0 +/- 13.9 minutes at 18.5 degrees C +/- 1.1 degrees C. In 10 of the 18 patients, blood pressure during LFCP was 15 +/- 8 mm Hg remote from the innominate artery (left radial, umbilical or femoral arteries). In 8 of the 18 patients, right radial pressure during LFCP was 24 +/- 10 mm Hg. The mean mixed-venous saturation was 79.8% +/- 10% during LFCP. Two patients had preoperative seizures, whereas none had seizures postoperatively. One patient died. Neonatal aortic arch reconstruction is possible without circulatory arrest or direct arch vessel cannulation. These techniques maintained adequate mixed-venous oxygen saturations with no associated adverse neurologic outcomes.

  12. Total Endovascular Aortic Repair in a Patient with Marfan Syndrome.

    Science.gov (United States)

    Amako, Mau; Spear, Rafaëlle; Clough, Rachel E; Hertault, Adrien; Azzaoui, Richard; Martin-Gonzalez, Teresa; Sobocinski, Jonathan; Haulon, Stéphan

    2017-02-01

    The aim of this study is to describe a total endovascular aortic repair with branched and fenestrated endografts in a young patient with Marfan syndrome and a chronic aortic dissection. Open surgery is the gold standard to treat aortic dissections in patients with aortic disease and Marfan syndrome. In 2000, a 38-year-old man with Marfan syndrome underwent open ascending aorta repair for an acute type A aortic dissection. One year later, a redo sternotomy was performed for aortic valve replacement. In 2013, the patient presented with endocarditis and pulmonary infection, which necessitated tracheostomy and temporary dialysis. In 2014, the first stage of the endovascular repair was performed using an inner branched endograft to exclude a 77-mm distal arch and descending thoracic aortic aneurysm. In 2015, a 63-mm thoracoabdominal aortic aneurysm was excluded by implantation of a 4-fenestrated endograft. Follow-up after both endovascular repairs was uneventful. Total aortic endovascular repair was successfully performed to treat a patient with arch and thoraco-abdominal aortic aneurysm associated with chronic aortic dissection and Marfan syndrome. The postoperative images confirmed patency of the endograft and its branches, and complete exclusion of the aortic false lumen. Endovascular repair is a treatment option in patients with connective tissue disease who are not candidates for open surgery. Long-term follow-up is required to confirm these favorable early outcomes. Copyright © 2016 Elsevier Inc. All rights reserved.

  13. A geometric reappraisal of proximal landing zones for thoracic endovascular aortic repair according to aortic arch types

    NARCIS (Netherlands)

    Marrocco-Trischitta, Massimiliano M.; de Beaufort, Hector W.; Secchi, Francesco; van Bakel, Theodorus M.; Ranucci, Marco; van Herwaarden, Joost A.; Moll, Frans L.; Trimarchi, Santi

    Objective: This study assessed whether the additional use of the aortic arch classification in type I, II, and III may complement Ishimaru's aortic arch map and provide valuable information on the geometry and suitability of proximal landing zones for thoracic endovascular aortic repair. Methods:

  14. 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...

  15. Effect of aging on the configurational change of the aortic arch

    Directory of Open Access Journals (Sweden)

    Atsuhiro Kojima

    2016-04-01

    Full Text Available We explored the relationship between aging and the configuration of the aortic arch using computed tomography angiography (CTA. We retrospectively reviewed CTA obtained in 140 cases. The configuration of the aortic arch was categorized into three types based on the criteria mentioned by Madhwal et al., and the relationships between each configuration and patient characteristics were analyzed. Anomalies of the aortic arch were also explored. Twenty patients had a type-1 aortic arch (mean age, 56.1 years, 30 patients had a type-2 aortic arch (mean age, 66.3 years, and 89 patients had a type-3 aortic arch (mean age, 71.7 years. The mean age of patients with a type-3 aortic arch was significantly higher than that of patients with a type-1 aortic arch. No significant correlations between the type of aortic arch and other factors, such as smoking habit, were seen. The configuration of the aortic arch in our study appears to be significantly affected by the age of the patients.

  16. Right cervical aortic arch with aberrant left subclavian artery.

    Science.gov (United States)

    Tjang, Yanto S; Aramendi, José I; Crespo, Alejandro; Hamzeh, Gadah; Voces, Roberto; Rodríguez, Miguel A

    2008-08-01

    The combination of right cervical aortic arch, aberrant retroesophageal left subclavian artery originating from a Kommerell's diverticulum, and a ligamentum arteriosum, constitutes a rare form of vascular ring. Two patients aged 21 days and 54 years, who were diagnosed by multislice 3-dimensional computed tomography and magnetic resonance imaging, underwent surgical division of a vascular ring. The adult required resection of a Kommerell's aneurysm and subclavian artery reimplantation.

  17. Complex atheromatosis of the aortic arch in cerebral infarction.

    Science.gov (United States)

    Capmany, Ramón Pujadas; Ibañez, Montserrat Oliveras; Pesquer, Xavier Jané

    2010-08-01

    In many stroke patients it is not possible to establish the etiology of stroke. However, in the last two decades, the use of transesophageal echocardiography in patients with stroke of uncertain etiology reveals atherosclerotic plaques in the aortic arch, which often protrude into the lumen and have mobile components in a high percentage of cases. Several autopsy series and retrospective studies of cases and controls have shown an association between aortic arch atheroma and arterial embolism, which was later confirmed by prospectively designed studies. The association with ischemic stroke was particularly strong when atheromas were located proximal to the ostium of the left subclavian artery, when the plaque was ≥ 4 mm thick and particularly when mobile components are present. In these cases, aspirin might not prevent adequately new arterial ischemic events especially stroke. Here we review the evidence of aortic arch atheroma as an independent risk factor for stroke and arterial embolism, including clinical and pathological data on atherosclerosis of the thoracic aorta as an embolic source. In addition, the impact of complex plaques (≥ 4 mm thick, or with mobile components) on increasing the risk of stroke is also reviewed. In non-randomized retrospective studies anticoagulation was superior to antiplatelet therapy in patients with stroke and aortic arch plaques with mobile components. In a retrospective case-control study, statins significantly reduced the relative risk of new vascular events. However, given the limited data available and its retrospective nature, randomized prospective studies are needed to establish the optimal secondary prevention therapeutic regimens in these high risk patients.

  18. Dissection of Retroesophageal Aortic Diverticulum and Descending Aorta in a Patient with Right Aortic Arch: Magnetic Resonance Demonstration

    International Nuclear Information System (INIS)

    Ko, S.-F.; Ng, S.-H.; Fu, Morgan; Lo, P.-H.; Cheng, Y.-F.; Lee, T.-Y.

    1996-01-01

    An acute aortic dissection involved the retroesophageal aortic diverticulum (RAD) and descending thoracic aorta in a patient with right aortic arch. The RAD, which was separated into false and true lumens by an intimal flap-the classic diagnostic sign of aortic dissection-was overlooked on transesophageal echocardiography and computed tomography but was clearly depicted on magnetic resonance imaging (MRI). It was found that MRI can delineate the anatomy of a congenital arch anomaly complicated by great vessels disease

  19. [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.

  20. [Thymic carcinoma involving aortic arch; report of a case].

    Science.gov (United States)

    Noriyuki, Toshio; Hamamoto, M; Takazawa, Y; Katoh, K; Hashimoto, M; Kuranishi, F; Nakahara, M; Fukuda, T; Ishizaki, Y; Okuda, H; Akimoto, E; Yonehara, S

    2009-05-01

    Adenocarcinoma of the thymus is a very rare malignant tumor. The standard treatment for advanced thymic carcinoma has not yet been established, and the prognosis is poor. We report a case of thymic carcinoma that involving the aortic arch and the innominate vein. A 78-year-old woman was admitted to our hospital complaining of hoarseness in April 2007. The computed tomography (CT) scan showed an anterior mediastinal tumor contiguous to the aortic arch and the innominate vein with swelling lymphnodes. Microspcopic examinations of specimens obtained by CT-guided needle biopsy revealed poorly differenciated adenocarcinoma. The carcinoembryonic antigen (CEA) level of serum elevated at 54.9 ng/ml. Thymic carcinoma was diagnosed. The chemoradiotherapy [concurrent, carboplatin (CBDCA) + paclitaxel(TXL)-->vinorelbine (NVB), 60 Gy] was performed, but the effect of the therapy was limited. The resection of the tumor with a part of aortic arch and other peripheral tissues was performed in Augast 2007. The postoperative course was uneventful and the CEA level of serum lowered to the normal. She was discharged 30 days after surgery.

  1. [Outcomes of endovascular repairing aortic arch disease hybrid with supra-arch debranching procedures].

    Science.gov (United States)

    Wang, Mian; Chang, Guangqi; Yin, Henghui; Yao, Chen; Wang, Jinsong; Wang, Shenming

    2015-11-01

    To summarize the experience of endovascular repairing aortic arch disease hybrid with supra-arch debranching procedures. It was a retrospective study. From January 2002 to December 2014, 42 high risk patients with aortic arch disease were treated by supra-arch debranching hybrid with subsequent endovascular repair in the First Affiliated Hospital of Sun Yat-sen University. There were 39 male and 3 female patients with a mean age of (53±13) years (ranging from 34 to 80 years). Of the 42 patients, 7 were thoracic aortic aneurysm, 20 were Stanford type B aortic dissection and 15 were Stanford type A aortic dissection. After the supra-aortic debranching technique, simultaneous (n=16) or staged (n=26, mean interval (7±3) days) endovascular repair were performed. Fisher exact test was used to compare the in-hospital mortality of ascending aorta based debranching and non-ascending aorta based debranching. Technical success rate was 81.0% (34/42). The overall 30-day complication rate was 31.0% (13/42), including 3 cerebral stroke (7.1%), 8 endoleak (19.0%, including 6 type I endoleak and 2 type II endoleak), 1 circulatory failure, 1 aorto-tracheal fistula. The 30-day mortality was 9.5% (4/42), 2 died of cerebral stroke, 1 died of circulatory failure, 1 died of aorto-tracheal fistula. The in-hospital mortality of ascending aorta based debranching group was obviously higher than that of the non-ascending aorta based debranching group (4/16 vs. 0, P=0.02). The median time of follow-up was 64.8 (2 to 156.9) months. CT scanning was performed at 1, 3 months after surgery and annually thereafter. The overall survival rate was 76.6%. During the follow-up period, there was 4 deaths, and 2 of them were aortic artery related (5.3%). There were 4 de novo complications during the follow-up period, 1 stroke attributed to bypass occlusion was cured by medical treatment, 2 pseudoaneurysm was successfully treated with open surgery, 1 stent-graft induced new distal entry tear was

  2. Tracheal Compression Caused by a Mediastinal Hematoma After Interrupted Aortic Arch Surgery.

    Science.gov (United States)

    Hua, Qingwang; Lin, Zhiyong; Hu, Xingti; Zhao, Qifeng

    2017-08-03

    Congenital abnormalities of the aortic arch include interrupted aortic arch (IAA), coarctation of the aorta (CoA), and double aortic arch (DAA). Aortic arch repair is difficult and postoperative complications are common. However, postoperative tracheobronchial stenosis with respiratory insufficiency is an uncommon complication and is usually caused by increased aortic anastomotic tension. We report here a case of tracheal compression by a mediastinal hematoma following IAA surgery. The patient underwent a repeat operation to remove the hematoma and was successfully weaned off the ventilator.In cases of tracheobronchial stenosis after aortic arch surgery, airway compression by increased aortic anastomotic tension is usually the first diagnosis considered by clinicians. Other causes, such as mediastinal hematomas, are often ignored. However, the severity of symptoms with mediastinal hematomas makes this an important entity.

  3. Contrast-enhanced magnetic resonance angiography of persistent fifth aortic arch in children

    International Nuclear Information System (INIS)

    Zhong, Yumin; Zhu, Ming; Sun, Aimin; Li, Yuhua; Jaffe, Richard B.; Gao, Wei

    2007-01-01

    Cine angiography and echocardiography have been utilized to diagnose congenital aortic arch anomalies. However, the visualization of great vessels by echocardiography is limited, while cine angiography requires cardiac catheterization with ionizing radiation. Contrast-enhanced magnetic resonance angiography (MRA) is a noninvasive modality suitable for visualization of congenital aortic arch anomalies. To evaluate the utility of contrast-enhanced MRA in the diagnosis of persistent fifth aortic arch, a rare congenital aortic arch anomaly, and to compare the diagnostic accuracy of MRA with that of echocardiography and cine angiography. In four pediatric patients, contrast-enhanced MRA studies were performed for diagnosing persistent fifth aortic arch. The findings of MRA were compared with echocardiographic findings and confirmed by cine angiography and operation. Transthoracic surface echocardiography noted an aberrant vessel arising from the ascending aorta in two of four patients; the etiology of this vessel was uncertain. In the other two patients a diagnosis of coarctation was made. Of the four patients, only one was diagnosed with interruption of the aortic arch. Contrast-enhanced MRA clarified uncertain echocardiographic findings, enabling the correct diagnosis of persistent fifth aortic arch with fourth aortic arch interruption in all four patients. Contrast-enhanced MRA is a safe, accurate, and fast imaging technique for the evaluation of persistent fifth aortic arch and may obviate the need for conventional cine angiography. Cardiac catheterization may be reserved for some types of complicated congenital heart disease and for obtaining hemodynamic information. (orig.)

  4. 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.

  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. 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.

  7. Detecting atheromatous plaques in the aortic arch or supra-aortic arteries for more accurate stroke subtype classification.

    Science.gov (United States)

    Cui, Xiaoyang; Wu, Simiao; Zeng, Quantao; Xiao, Jiahe; Liu, Ming

    2015-02-01

    To investigate the correlations of atheromatous plaques in the aortic arch or supra-aortic arteries with intracranial arterial stenosis and carotid plaques in stroke patients, and to determine whether taking these plaques into account will reduce the proportion of patients in the undetermined etiology group. We prospectively enrolled 308 ischemic stroke patients, whose clinical characteristics and A-S-C-O classifications were compared with analyses of intracranial arteries, carotid arteries, aortic arch, and supra-aortic arteries. 125(40.6%) patients had plaques in the aortic arch or supra-aortic arteries, of which 106 (84.8%) had complex plaques. No correlations were observed between these plaques and carotid plaques ( p = 0.283) or intracranial arterial stenosis ( p = 0.097). After detecting the mobile thrombi in the aortic arch and supra-aortic arteries, the proportion of patients in the atherothrombosis group was increased from 33.8% to 55.5% ( p = 0.00), whereas the proportion of patients in stroke of undetermined etiology group was decreased from 19.2% to 11.0% ( p = 0.00). Examining only the carotid and intracranial arteries may not provide adequate information about large arteries in stroke patients. Therefore, it would be better to include a search for relevant plaques in the aortic arch or supra-aortic arteries in modern stroke workup, for it may lead to more accurate stroke subtype classification and guide secondary prevention.

  8. 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

  9. RIGHT-SIDED AORTIC ARCH WITH ABERRANT LEFT SUBCLAVIAN ARTERY AND DUPLICATION OF SUPERIOR VENA CAVA

    Directory of Open Access Journals (Sweden)

    Parikhita Hazarika

    2017-08-01

    Full Text Available Right-sided aortic arch is a rare anatomical variant present in about 0.1% of the adult population.1,2 Half of the cases are associated with an aberrant left subclavian artery (0.05%-0.1%. Right-sided aortic arch with aberrant left subclavian artery is less common than left-sided aortic arch with aberrant right subclavian artery (0.5-2.0%.3,4 A rightsided aortic arch is an anatomic variant resulting from persistence of the right fourth aortic arch and involution of the left. It can be associated with an aberrant left subclavian artery arises from Kommerell’s diverticulum. It is usually asymptomatic and diagnosed incidentally during adult age. A 40-year-old male presented with cough and a hump in the back. The patient was evaluated for scoliosis and plain CT thorax was done.

  10. Aortic Arch Aneurysms: Treatment with Extra anatomical Bypass and Endovascular Stent-Grafting

    International Nuclear Information System (INIS)

    Kato, Noriyuki; Shimono, Takatsugu; Hirano, Tadanori; Mizumoto, Toru; Ishida, Masaki; Fujii, Hideki; Yada, Isao; Takeda, Kan

    2002-01-01

    Endovascular repair of thoracic aortic aneurysms is emerging as an attractive alternative to surgical graft replacement. However,patients with aortic arch aneurysms are often excluded from the target of endovascular repair because of lack of suitable landing zones, especially at the proximal ones. In this paper we describe our method for treating patients with aortic arch aneurysms using a combination of extra anatomical bypass surgery and endovascular stent-grafting

  11. 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.

  12. Abnormalities of aortic arch shape, central aortic flow dynamics, and distensibility predispose to hypertension after successful repair of aortic coarctation.

    Science.gov (United States)

    Donazzan, Luca; Crepaz, Robert; Stuefer, Josef; Stellin, Giovanni

    2014-10-01

    Systemic hypertension (HT) is a major long-term complication even after successful repair of aortic coarctation (CoA), and many factors are involved in this pathophysiology. To investigate the role of abnormalities in the aortic arch shape, central aortic flow dynamics, and distensibility in developing HT after successful repair of CoA. We selected a group of 26 normotensive patients (mean age 16.9±7.3 years, range 9-32 years) with anatomically successful repair of CoA among 140 patients regularly followed after repair of CoA and analyzed their last clinical and echocardiographic data. Bicycle exercise test and ambulatory blood pressure monitoring (ABPM) were also obtained. Mean age at surgical repair was 3.2±3.9 years (range 10 days-15 years); 12 patients underwent surgical correction during the first year of life. Repair of CoA was performed by end-to-end anastomosis (TT) in 23 patients (extended TT in 6 patients with arch hypoplasia), patch aortoplasty in 2, and subcalvian flap aortoplasty in 1. The postsurgical follow-up was 13.8±7.2 years (range 3.5-29.4 years). The shape of the aortic arch was defined by magnetic resonance imaging (MRI) on this global geometry (normal-gothic-crenel), ratio of the height-transverse diameter (A/T), percentage of residual stenosis, and growth index of the transverse arch segments. Flow mapping by phase-contrast imaging in the ascending and descending aorta was performed in order to measure the systolic waveforms and central aortic distensibility. Twenty normal age-matched patients submitted to the same MRI protocol were used as controls. Six patients were found to have a gothic and 20 a normal aortic arch shape. Patients with gothic aortic arch shape had an increased A/T ratio (0.80±0.07 vs 0.58±0.05, P135 mm Hg on ABPM were higher in the gothic than in the normal arch group. There was a correlation between nocturnal SBP, 24 hours pulse pressure on ABPM in the whole group, and different MRI variables (A/T, distensibility of

  13. Anomalous Origin of the Left Vertebral Artery from the Aortic Arch.

    Science.gov (United States)

    Einstein, Evan H; Song, Linda H; Villela, Natalia L A; Fasani-Feldberg, Gregory B; Jacobs, Jonathan L; Kim, Dolly O; Nathawat, Akshay; Patel, Devika; Bender, Roger B; Peters, Daniel F

    2016-04-01

    Anatomic anomalies of the aortic arch have implications for clinical practice if their significance is understood. Our case study involves a cadaveric finding of the left vertebral artery originating directly from the aortic arch. Although this anatomical variation has been documented, the prevalence of this anomaly may be generally underestimated. After noting this anomaly, we analyzed 27 cases and found that four female cadavers had the left vertebral artery originating from the aortic arch rather than the left subclavian artery. With a prevalence rate of 14.8%, it would seem that this anomaly is more significant than previously thought, which could have implications for surgical practice.

  14. 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...

  15. 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...

  16. [Bentall operation combined with total arch replacement and stented elephant trunk implantation for serious Debakey I aortic dissecting aneurysm].

    Science.gov (United States)

    Gu, Tian-Xiang; Wang, Chun; Zhang, Yu-Hai

    2008-12-01

    To summarize the clinical experience of Bentall operation combined with total arch replacement and stented elephant trunk implantation for serious Debakey I aortic dissecting aneurysm. Twelve patients with serious Debakey I aortic dissecting aneurysm underwent surgical treatment from January 2005 to December 2007. There were 10 male and 2 female with the mean age of (40.1 +/- 9.5) years old. There were acute aortic dissection in 9 cases, chronic aortic dissection in 3 cases. The inner diameter of aorta was (5.3 +/- 1.8) cm. There were Marfan syndrome in 4 cases, aortic regurgitation in all cases, severely persistent chest pain in 9 cases, acute left heart failure in 8 cases, and cardiac tamponade in 4 cases. Bentall operations combined with total arch replacement and stented elephant trunk implantation were performed by using deep hypothermic circulatory arrest and antegrade selective cerebral perfusion in all cases. Urgent surgery underwent in 9 cases. The mean interval between the onset of aortic dissection and the accomplishment of surgery was (41.0 +/- 15.9) hours. Cardiopulmonary bypass time was (191 +/- 26) min, average cross clamp time was (134 +/- 31) min, and average deep hypothermic circulatory arrest time was (50.0 +/- 14.5) min. One patient died in hospital. The time stayed in ICU was 3 to 27 d. Mental disorder in 6 cases, hemi-paralysis in 1 case, amputation in 1 case, hemorrhage of anastomosis in 1 case, hemorrhage of alimentary tract in 1 case, and pleural effusion in 4 cases were recorded. Eleven cases were followed-up for 8 weeks to 36 months. There were no bending of the stents and no obstruction in the vascular prosthesis.No re-operation was needed. One case died 6 months postoperatively. Bentall operation combined with total arch replacement and stented elephant trunk implantation is safe and effective for serious Debakey I aortic dissecting aneurysm, while good organs protection and consummate cardiopulmonary bypass were taken.

  17. Variations of the aortic arch - a study on the most common branching patterns

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    Muller, Marguerite; Schmitz, Bernd L; Schick, Melanie; Schloetzer, Wiebke (Dept. of Diagnostic and Interventional Radiology, Section Neuroradiology, Univ. Ulm (Germany)), email: marguerite.mueller@uni-ulm.de; Pauls, Sandra (Dept. of Diagnostic and Interventional Radiology, Univ. Ulm (Germany)); Roehrer, Stefan; Kapapa, Thomas (Dept. of Neurosurgery, Univ. Ulm (Germany))

    2011-09-15

    Background: Anatomical variants of the aortic arch and its branching patterns often appear as an incidental finding during routine computed tomography (CT) scanning. These variations can be of relevance when performing angiography or endovascular interventions and may cause symptoms such as dysphagia. Purpose: To analyze common anatomical variations found within the arteries originating from the aortic arch in patients using contrast CT imaging techniques. Material and Methods: A total of 2033 contrast CT scans were analyzed. To obtain a truly representative sample, cases were chosen from different hospital departments without previous knowledge of the patient history. Results: The total percentage of variations within the analyzed patients was 13.3%. In 8.0% a truncus bicaroticus was found. 4.2% of the patients showed a left vertebral artery originating directly from the aortic arch, mostly proximal, and in 1 case distal to the left subclavian artery. In 1.0% we found an aberrant right subclavian artery. We also found a single case of a right descending aortic arch. Conclusion: Variations of the aortic arch and its branching are frequently found, mostly as an incidental finding during routine diagnostic scanning. A contrast-enhanced CT scan is a good method with which to study the aortic arch and its associated branching pattern

  18. Ductal Stent Implantation in Tetralogy of Fallot with Aortic Arch Abnormality

    Science.gov (United States)

    Ergul, Yakup; Saygi, Murat; Ozyilmaz, Isa; Guzeltas, Alper; Odemis, Ender

    2015-01-01

    Stenting of patent ductus arteriosus is an alternative to palliative cardiac surgery in newborns with duct-dependent or decreased pulmonary circulation; however, the use of this technique in patients with an aortic arch abnormality presents a challenge. Tetralogy of Fallot is a congenital heart defect that is frequently associated with anomalies of the aortic arch and its branches. The association is even more common in patients with chromosome 22q11 deletion. We present the case of an 18-day-old male infant who had cyanosis and a heart murmur. After an initial echocardiographic evaluation, the patient was diagnosed with tetralogy of Fallot and right-sided aortic arch. The pulmonary annulus and the main pulmonary artery and its branches were slightly hypoplastic; the ductus arteriosus was small. Conventional and computed tomographic angiograms revealed a double aortic arch and an aberrant left subclavian artery. The right aortic arch branched into the subclavian arteries and continued into the descending aorta, whereas the left aortic arch branched into the common carotid arteries and ended with the patent ductus arteriosus. After evaluation of the ductal anatomy, we implanted a 3.5 × 15-mm coronary stent in the duct. Follow-up injections showed augmented pulmonary flow and an increase in oxygen saturation from 65% to 94%. The patient was also found to have chromosome 22q11 deletion. PMID:26175649

  19. Variations of the aortic arch - a study on the most common branching patterns

    International Nuclear Information System (INIS)

    Muller, Marguerite; Schmitz, Bernd L; Schick, Melanie; Schloetzer, Wiebke; Pauls, Sandra; Roehrer, Stefan; Kapapa, Thomas

    2011-01-01

    Background: Anatomical variants of the aortic arch and its branching patterns often appear as an incidental finding during routine computed tomography (CT) scanning. These variations can be of relevance when performing angiography or endovascular interventions and may cause symptoms such as dysphagia. Purpose: To analyze common anatomical variations found within the arteries originating from the aortic arch in patients using contrast CT imaging techniques. Material and Methods: A total of 2033 contrast CT scans were analyzed. To obtain a truly representative sample, cases were chosen from different hospital departments without previous knowledge of the patient history. Results: The total percentage of variations within the analyzed patients was 13.3%. In 8.0% a truncus bicaroticus was found. 4.2% of the patients showed a left vertebral artery originating directly from the aortic arch, mostly proximal, and in 1 case distal to the left subclavian artery. In 1.0% we found an aberrant right subclavian artery. We also found a single case of a right descending aortic arch. Conclusion: Variations of the aortic arch and its branching are frequently found, mostly as an incidental finding during routine diagnostic scanning. A contrast-enhanced CT scan is a good method with which to study the aortic arch and its associated branching pattern

  20. Early Results of Chimney Technique for Type B Aortic Dissections Extending to the Aortic Arch

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    Huang, Chen [Affiliated Hospital of Nantong University, Department of General Surgery (China); Tang, Hanfei; Qiao, Tong; Liu, Changjian; Zhou, Min, E-mail: 813477618@qq.com [The Affiliated Hospital of Nanjing University Medical School, Department of Vascular Surgery, Nanjing Drum Tower Hospital (China)

    2016-01-15

    ObjectiveTo summarize our early experience gained from the chimney technique for type B aortic dissection (TBAD) extending to the aortic arch and to evaluate the aortic remodeling in the follow-up period.MethodsFrom September 2011 to July 2014, 27 consecutive TBAD patients without adequate proximal landing zones were retrograde analyzed. Chimney stent-grafts were deployed parallel to the main endografts to reserve flow to branch vessels while extending the landing zones. In the follow-up period, aortic remodeling was observed with computed tomography angiography.ResultsThe technical success rate was 100 %, and endografts were deployed in zone 0 (n = 3, 11.1 %), zone 1 (n = 18, 66.7 %), and zone 2 (n = 6, 22.2 %). Immediately, proximal endoleaks were detected in 5 patients (18.5 %). During a mean follow-up period of 17.6 months, computed tomography angiography showed all the aortic stent-grafts and chimney grafts to be patent. Favorable remodeling was observed at the level of maximum descending aorta and left subclavian artery with expansion of true lumen (from 18.4 ± 4.8 to 25 ± 0.86 mm, p < 0.001 and 27.1 ± 0.62 to 28.5 ± 0.37 mm, p < 0.001) and depressurization of false lumen (from 23.7 ± 2.7 to 8.7 ± 3.8 mm, p < 0.001, from 5.3 ± 1.2 to 2.1 ± 2.1 mm, p < 0.001). While at the level of maximum abdominal aorta, suboptimal remodeling of the total aorta (from 24.1 ± 0.4 to 23.6 ± 1.5 mm, p = 0.06) and true lumen (from 13.8 ± 0.6 to 14.5 ± 0.4 mm, p = 0.08) was observed.ConclusionBased on our limited experience, the chimney technique with thoracic endovascular repair is demonstrated to be promising for TBAD extending to the arch with favorable aortic remodeling.

  1. Thoracoabdominal aortic aneurysm repair in patients with marfan syndrome.

    Science.gov (United States)

    Mommertz, G; Sigala, F; Langer, S; Koeppel, T A; Mess, W H; Schurink, G W H; Jacobs, M J

    2008-02-01

    We assessed the surgical outcome of descending thoracic aortic aneurysm repair (DTAA) and thoracoabdominal aortic aneurym (TAAA) repair in patients with Marfan syndrome. During a six year period, 206 patients underwent DTAA and TAAA repair. In 22 patients, Marfan syndrome was confirmed. The median age was 40 years with a range between 18 and 57 years. The extend of the aneurysms included 6 DTAA (1 with total arch, 2 with distal hemi-arch), 11 type II TAAA (2 with total arch, 3 with distal hemi-arch), 4 type III and one type IV TAAA. All patients suffered from previous type A (n=6) or type B (n=16) aortic dissection and 15 already underwent aortic procedures like Bentall (n=7) and ascending aortic replacement (n=8). All patients were operated on according to the standard protocol with cerebrospinal fluid drainage, distal aortic and selective organ perfusion and monitoring motor evoked potentials. In patients undergoing simultaneous arch replacement (via left thoracotomy), transcranial Doppler and EEG assessed cerebral physiology during antegrade brain perfusion. In four patients circulatory arrest under moderate hypothermia was required. In-hospital mortality did not occur. Major postoperative complications like paraplegia, renal failure, stroke and myocardial infarction were not encountered. Mean pre-operative creatinine level was 125mmol/L, which peaked to a mean maximal level of 130 and returned to 92mmol/L at discharge. Median intubation time was 1.5 days (range 0.33-30 days). Other complications included bleeding requiring surgical intervention (n=1), arrhythmia (n=2), pneumonia (n=2) and respiratory distress syndrome (n=1). At a median follow-up of 38 months all patients were alive. Using CT surveillance, new or false aneurysms were not detected, except in one patient who developed a visceral patch aneurysm six years after open type II repair. Surgical repair of descending and thoracoabdominal aortic aneurysms provides excellent short- and mid-term results in

  2. Repair of an aneurysm of the ascending aorta and arch in an infant with Loeys-Dietz syndrome.

    Science.gov (United States)

    Jaiswal, Pratiksha; Shetty, Varun; Patel, Ebrahim; Shetty, Deviprasad

    2018-05-01

    Aortic aneurysms in childhood are rare disease entities and are usually seen in patients with genetic connective tissue disorders such as Marfans, Ehler-Danlos, and Loeys-Dietz syndrome (LDS). Patients affected with LDS present early in life and have a rapid disease progression. We report a case of repair of an ascending and aortic arch aneurysm in an infant with Loeys-Dietz syndrome. © 2018 Wiley Periodicals, Inc.

  3. Goal-directed-perfusion in neonatal aortic arch surgery.

    Science.gov (United States)

    Cesnjevar, Robert Anton; Purbojo, Ariawan; Muench, Frank; Juengert, Joerg; Rueffer, André

    2016-07-01

    Reduction of mortality and morbidity in congenital cardiac surgery has always been and remains a major target for the complete team involved. As operative techniques are more and more standardized and refined, surgical risk and associated complication rates have constantly been reduced to an acceptable level but are both still present. Aortic arch surgery in neonates seems to be of particular interest, because perfusion techniques differ widely among institutions and an ideal form of a so called "total body perfusion (TBP)" is somewhat difficult to achieve. Thus concepts of deep hypothermic circulatory arrest (DHCA), regional cerebral perfusion (RCP/with cardioplegic cardiac arrest or on the perfused beating heart) and TBP exist in parallel and all carry an individual risk for organ damage related to perfusion management, chosen core temperature and time on bypass. Patient safety relies more and more on adequate end organ perfusion on cardiopulmonary bypass, especially sensitive organs like the brain, heart, kidney, liver and the gut, whereby on adequate tissue protection, temperature management and oxygen delivery should be visualized and monitored.

  4. Regional cerebral perfusion for surgical correction of neonatal aortic arch obstruction.

    Science.gov (United States)

    Zhang, Hui; Cheng, Pei; Hou, Jia; Li, Lei; Liu, Hu; Liu, Ruifang; Ji, Bingyang; Luo, Yi

    2009-05-01

    One-stage repair of aortic arch obstruction and associated cardiac anomalies is a surgical challenge in infants.The purpose of the present study is to review the current outcome using regional cerebral perfusion (RCP) during a procedure correcting interrupted aortic arch (IAA) and also isolated aortic coarctation (CoA) and CoA combined with hypoplastic aortic arch (CoA-HyAA) in our center. Between January 2007 and July 2008, 24 infant patients with interrupted aortic arch (IAA) (n=3), isolated aortic coarctation (iCoA) (n=9) and aortic coarctation with hypoplastic aortic arch (CoA-HyAA) (n=12) underwent one-stage surgical correction in our hospital. End-to-end anastomosis was employed in 12 infants (IAA n=3 and iCoA n=9); for the other 12 patients with CoA-HyAA, an end-to-end extended anastomosis was used in 8 cases, end-to-side anastomosis in 2 cases, and composite heterologous pericardial patch in 2 cases. RCP with 40 mL/kg/min through the innominate artery during aortic arch reconstruction was employed for all pediatric patients. One single-dose histidine-ketoglutarate-tryptophan (HTK) solution was used for myocardial protection during CPB. Cardiopulmonary bypass time and aortic cross-clamp time were 165.6+/-32.4 min and 81.7+/-30.0 min, respectively. The mean regional cerebral perfusion time was 31.0+/-10.6 min; lowest nasopharyngeal temperature was 19.1+/-1.1 degrees C. Operative mortality rate in both groups was 8.3%. Mean follow-up was 10.5+/-4.8 months. There was no late mortality or postoperative neurologic, renal or hepatic complications. All patients are asymptomatic and are developing normally. One-stage total arch repair using the RCP technique is an excellent method that may minimize neurologic and renal complications. Our surgical strategy for arch anomaly has a low rate of residual and recurrent coarctation when performed in these infants.

  5. Bovine aortic arch and idiopathic pulmonary artery aneurysm associated with bronchial compression

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    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.

  6. Extended aortic repair using frozen elephant trunk technique for Marfan syndrome with acute aortic dissection.

    Science.gov (United States)

    Uchida, Naomichi; Katayama, Akira; Kuraoka, Masatsugu; Katayama, Keijiro; Takahashi, Shinya; Takasaki, Taiichi; Sueda, Taijiro

    2013-01-01

    The aim of this study was to analyze midterm results of frozen elephant trunk technique for Marfan syndrome with acute aortic dissection. Between February 1999 and August 2011 we performed arch replacement uisng frozen elephant trunk technique for acute aortic dissection in 8 patients with Marfan syndrome containing two complicated type B dissections and six type A dissections.Five patients compromised annulo-aortic ectasia who performed Bentall operation. No patients died in the initial operation. Fate of false lumen on the stent graft border was expressed by CT scan follow-up that were patent in 0, thrombosis in 5 and absorption in 3 patients. One patient who had new aortic dissection 8 years after initial surgery required the Crawford V operation. Ten-years-survival rate was 100% and ten years-event free rate was 67%. Frozen elephant trunk technique was feasible for Marfan syndrome with acute aortic dissection and might become alternative prophylactic treatment to the downstream aorta for acute aortic dissection.

  7. Oesophageal foreign body and a double aortic arch: rare dual pathology.

    Science.gov (United States)

    O'Connor, T E; Cooney, T

    2009-12-01

    We report the rare case of an oesophageal foreign body which lodged above the site of oesophageal compression by a double aortic arch. Case report and a review of the literature surrounding the classification, embryology, diagnosis and management of vascular rings and slings. An eight-month-old male infant presented with symptoms of tracheal compression following ingestion of an oesophageal foreign body. Following removal of the oesophageal foreign body, the infant's symptoms improved initially. However, subsequent recurrence of respiratory symptoms lead to a repeat bronchoscopy and the diagnosis of a coexisting double aortic arch, causing tracheal and oesophageal compression. To our knowledge, this is only the second reported case of a double aortic arch being diagnosed in a patient following removal of an oesophageal foreign body.

  8. Acute Type A Aortic Dissection Successfully Managed with One-stage Surgery of Total Aortic Arch Replacement with Supra-aortic Transposition Plus Frozen Elephant Trunk Technique

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    Meng-Lin Lee

    2014-09-01

    Full Text Available Acute type A aortic dissection has long been a challenging issue. The surgical techniques traditionally vary with the anatomic extent of the aortic dissection. Simple ascending aortic grafting can be lifesaving, but the lesions beyond the aorta, which include the arch vessels and descending aorta, remain potential hazards. In this paper, we present a patient in which acute type A aortic dissection with lesions extending into descending thoracic aorta was successfully managed by total arch replacement with supra-aortic transposition plus the frozen elephant trunk technique to the descending aorta. A 67-year-old gentleman presented with severe tearing pain from the anterior to posterior chest. Computed tomography confirmed the diagnosis of acute type A dissection extending to the level of the right common iliac artery. An emergent operation was performed as in the aforementioned technique. The surgery went well and the patient was discharged without comorbidities on postoperative day 25. The patient had regular outpatient clinical follow-up. The follow-up computed tomography images showed adequate results with the obliteration of the false lumen. In conclusion, total aortic arch replacement with supra-aortic transposition plus frozen elephant trunk technique is a safe and feasible operative method for patients with detrimental acute type A aortic dissection.

  9. Management of an elderly patient with respiratory failure due to double aortic arch

    Directory of Open Access Journals (Sweden)

    Changwan Ryu

    2016-01-01

    Full Text Available Vascular rings are congenital malformations of the aortic arch. A double aortic arch (DAA, the most common type of vascular ring, results from the failure of the fourth embryonic branchial arch to regress, leading to an ascending aorta that divides into a left and right arch that fuse together to completely encircle the trachea and esophagus. The subsequent DAA causes compressive effects on the trachea and esophagus that typically manifests in infancy or early childhood. Adult presentations, particularly in the elderly, are exceedingly rare. Historically such patients have a long-standing history of dyspnea on exertion and dysphagia, with many assumed to have obstructive lung or intrinsic cardiac disease. We describe a case of an elderly woman who presented with respiratory failure due to DAA. In her case, surgery was not feasible and we describe our experience with airway stenting.

  10. Anatomic variations of the branches of the aortic arch in a Peruvian population.

    Science.gov (United States)

    Huapaya, Julio Arturo; Chávez-Trujillo, Kristhy; Trelles, Miguel; Dueñas Carbajal, Roy; Ferrandiz Espadin, Renato

    2015-07-31

    Previous publications from two countries in South America found one anatomical variation not previously reported in the rest of the world, which in turn give some clues with regard to a racial difference. The objective of the present study is to describe variations in the anatomical distribution of the branches of the aortic arch in a Peruvian population. To describe variations in the anatomical distribution of the branches of the aortic arch in a Peruvian population. A descriptive study of patients who underwent a tomography angiography of the aorta was performed. We analyzed the reports that showed the description of the variations of the branches of the aortic arch based on the eight types currently described in the literature. From 361 analyzed reports, 282 patients (78.12%) had a normal aortic arch configuration (type I; aortic arch gives rise to the brachiocephalic trunk, left common carotid and left subclavian arteries); followed by type II (left common carotid artery as a branch of the aorta) with 41 patients (11.36%); and type IX (common ostium for the brachiocephalic trunk and the left common carotid artery) with 25 patients (6.93%). The latter and two other types are new variations. Aortic Arch Type I, Type II and Type IX were the most frequent variations in this Peruvian study. Additionally, we also found two more new types that have not been previously described in the literature. Further investigation regarding these variations is needed in order to assess a racial factor in South America and possible relationships with clinical or surgical events.

  11. Tracheal compression due to an elongated aortic arch in patients with congenital heart disease: evaluation using multidetector-row CT

    Energy Technology Data Exchange (ETDEWEB)

    Watanabe, Noriko; Hayabuchi, Yasunobu; Inoue, Miki; Sakata, Miho; Nabo, Manal Mohamed Helmy; Nakagawa, Ryuji; Saijo, Takahiko; Kagami, Shoji [University of Tokushima, Department of Pediatrics, Tokushima (Japan)

    2009-10-15

    The airway can become obstructed as a result of compression by an elongated aortic arch. In this study we evaluated tracheal compression using multidetector-row CT in patients with congenital heart disease and an elongated aortic arch. The trachea was measured at the level of the aortic arch in 205 children and young adults and then the severity of tracheal compression was determined by measuring the tracheal diameter ratio (short axis diameter/long axis diameter). Patients were divided as follows: group I (normal aortic arch; n=166), group II (transversely running aortic arch; n=22), and group III (elongated aortic arch; n=17). From the viewpoint of the relationship of the great arteries, group II had D-malposition, and group III had L-malposition. Age, height, weight and body surface area were significantly correlated with the short and long axis diameter in group I. There was a negative correlation between tracheal diameter ratio and the physical size parameters. The tracheal diameter ratio in group III was 0.50{+-}0.13, which was significantly lower than in groups I and II (P<0.01 and 0.05, respectively). Even apparently asymptomatic patients with an elongated aortic arch can have tracheal compression. An elongated aortic arch may be a useful predictor of tracheal compression. (orig.)

  12. GROWTH OF THE HYPOPLASTIC AORTIC-ARCH AFTER SIMPLE COARCTATION RESECTION AND END-TO-END ANASTOMOSIS

    NARCIS (Netherlands)

    BROUWER, MHJ; CROMMEDIJKHUIS, AH; EBELS, T; EIJGELAAR, A

    Surgical treatment of a hypoplastic aortic arch associated with an aortic coarctation is controversial. The controversy concerns the claimed need to surgically enlarge the diameter of the hypoplastic arch, in addition to resection and end-to-end anastomosis. The purpose of this prospective study is

  13. Cardiogenic shock due to coronary artery disease associated with interrupted aortic arch

    Directory of Open Access Journals (Sweden)

    Luís Alberto Oliveira Dallan

    2013-06-01

    Full Text Available Acute pulmonary edema is a serious event. Its occurrence in association with interrupted aortic arch and coronary heart disease is rare. Recently, an old patient developed cardiogenic shock and acute pulmonary edema due to acute coronary insufficiency, associated with interrupted aortic arch. The coronary angiography revealed occlusion of the right coronary artery and 95% obstruction in the left main coronary artery, associated with interruption of the descending aorta. Coronary artery bypass graft was performed, without extracorporeal circulation, to the anterior descending coronary artery. We discuss the initial management, given the seriousness of the case.

  14. 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.

  15. Invasive aspergillosis in the aortic arch with infectious Aspergillus lesions in pulmonary bullae

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    Isao Watanabe

    2015-03-01

    Full Text Available A patient with pulmonary bullae died of massive hemoptysis. At autopsy a hole was observed in the aortic wall. A microscopic examination indicated small Aspergillus lesions in pulmonary bullae and extensive necrotic lesions with Aspergillus hyphae in the media of the thoracic aorta. These findings led to a diagnosis of invasive aspergillosis in the aortic arch. This is a rare case in which Aspergillus invaded the aorta in a patient without hematologic neoplasms or neutropenia.

  16. 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

  17. Vascular geometry as a risk factor for non-penetrating traumatic injuries of the aortic arch.

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    Andreas Schicho

    Full Text Available To assess biomechanical factors in aortic arch geometry contributing to the development of non-penetrating aortic arch injury (NAAI in multiply injured patients with an Injury Severity Score (ISS ≥ 16.230 consecutive multiply injured trauma patients with an ISS ≥ 16 admitted to our Level-I trauma center during a consecutive 24-month period were prospectively included of whom 13 presented with NAAI (5.7%. Standardized whole-body CT in a 2x128-detector-row scanner included a head-and-neck CTA. Aortic arch diameters, width, height, angles and thoracic width and height were measured in individuals with NAAI and ISS-, sex-, age-, and trauma mechanism-matched controls.There was no difference between groups regarding sex, age, ISS, and aortic diameters. The aortic arch angle in individuals with NAAI (71.3° ± 14.9° was larger than in healthy control (60.7° ± 8.6°; p*<0.05. In patients with NAAI, the distance between ascendent and descendent aorta was larger (5.2 cm ± 1.9 cm than in control (2.8 ± 0.5 cm; ***p<0.001. The aortic arch is higher above tracheal bifurcation in NAAI (3.6 cm ± 0.6 cm than in matched control (2.4 cm ± 0.3 cm; ***p<0.001. Accordingly, the area under the aortic arch, calculated as half of an eliptic shape, is significantly larger in patients with NAAI (15.0 cm2 ± 6.5 cm2 when compared to age- and sex-matched controls without NAAI (5.5 cm2 ± 1.3 cm2; ***p<0.001.Besides the magnitude of deceleration and direction of impact, width and height of the aortic arch are the 3rd and 4th factor directly contributing to the risk of developing traumatic NAAI in severely injured patients.

  18. 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.

  19. Three-dimensional printing in surgical planning: A case of aortopulmonary window with interrupted aortic arch

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    Ryan A Moore

    2018-01-01

    Full Text Available Better anatomical understanding and conceptualization of complex congenital heart defects using three-dimensional (3D printing may improve surgical planning, especially in rare defects. In this report, we utilized 3D printing to delineate the exact cardiac anatomy of a neonate with an aortopulmonary window associated with interrupted aortic arch to devise a novel approach to the repair.

  20. Neurodevelopmental Outcomes Following Regional Cerebral Perfusion with Neuromonitoring for Neonatal Aortic Arch Reconstruction

    Science.gov (United States)

    Andropoulos, Dean B.; Easley, R. Blaine; Brady, Ken; McKenzie, E. Dean; Heinle, Jeffrey S.; Dickerson, Heather A.; Shekerdemian, Lara S.; Meador, Marcie; Eisenman, Carol; Hunter, Jill V.; Turcich, Marie; Voigt, Robert G.; Fraser, Charles D.

    2013-01-01

    Background In this study we report magnetic resonance imaging (MRI) brain injury, and 12 month neurodevelopmental outcomes, when regional cerebral perfusion (RCP) is utilized for neonatal aortic arch reconstruction. Methods Fifty seven neonates receiving RCP during aortic arch reconstruction were enrolled in a prospective outcome study. RCP flows were determined by near-infrared spectroscopy and transcranial Doppler monitoring. Brain MRI were performed preoperatively and 7 days postoperatively. Bayley Scales of Infant Development III was performed at 12 months. Results Mean RCP time was 71 ± 28 minutes (range 5–121), mean flow 56.6 ± 10.6 ml/kg/min. New postoperative MRI brain injury was seen in 40% of patients. For 35 RCP patients at age 12 months, mean Bayley III composite standard scores were: Cognitive = 100.1 ± 14.6,(range 75–125); Language = 87.2 ± 15.0, (range 62–132); Motor = 87.9 ± 16.8, (range 58–121).Increasing duration of RCP was not associated with adverse neurodevelopmental outcomes. Conclusions Neonatal aortic arch repair with RCP utilizing a neuromonitoring strategy results in 12-month cognitive outcomes that are at reference population norms; language and motor outcomes are lower than the reference population norms by 0.8–0.9 standard deviation. This largest RCP group with neurodevelopmental outcomes published to date demonstrates that this technique is effective and safe in supporting the brain during neonatal aortic arch reconstruction. PMID:22766302

  1. Traumatic partial avulsion of a single right subclavian artery from the aortic arch and definitive repair.

    Science.gov (United States)

    Kapetanakis, Emmanouil I; Sears-Rogan, Pamela; Young, Richard S; Kanda, Louis T; Ellis, Jennifer L

    2006-01-01

    Blunt injury to the right subclavian artery is a rare complication of severe deceleration trauma often associated with significant morbidity and mortality. We describe an atypical presentation in a patient who sustained a traumatic avulsion of his right subclavian artery arising off the aortic arch. An interposition graft was used to restore the continuity of the artery to the ascending thoracic aorta.

  2. MRI-based multiscale models for the hemodynamic and structural evaluation of surgically reconstructed aortic arches

    DEFF Research Database (Denmark)

    Pittaccio, S; Migliavacca, F; Balossino, R

    2007-01-01

    ) geometries of a porcine aortic arch were derived from magnetic resonance imaging (MRI) images. Inlet conditions were derived from MRI velocimetry. A multiscale approach was used for the imposition of outlet conditions, wherein a lumped parameter net provided an active afterload. Evidence was found that ring...

  3. Abnormal pulmonary vein drainage in upper right lobe associated with double aortic arch : magnetic resonance angiography

    International Nuclear Information System (INIS)

    Busto, M.; Dolz, J.L.; Capdevilla, A.; Castanon, M.; Mulet, J.

    1997-01-01

    We present the magnetic resonance (MR) and magnetic resonance angiography (MRA) findings in a case of abnormal pulmonary vein drainage from upper right lobe to superior vena cava, associated with double aortic arch, in a six-month-old boy. (Author) 9 refs

  4. Right-sided aortic arch with anomalous origin of the left subclavian artery: Case report

    Directory of Open Access Journals (Sweden)

    Vučurević Goran

    2011-01-01

    Full Text Available Introduction. A right-sided aortic arch is a rare congenital defect of the aorta with incidence of 0.05% to 0.1% reported in published series. Usually it is associated with congenital heart anomalies and esophageal and tracheal compression symptoms. We present a case of a right-sided aortic arch of anomalous left subclavian artery origin, accidentally revealed during multislice CT (MSCT supraaortic branches angiography. Case Outline. A 53-year-old female patient was examined at the Outpatients’ Unit of the Vascular Surgery University Clinic for vertigo, occasional dizziness and difficulty with swallowing. Physical examination revealed a murmur of the left supraclavicular space, with 15 mmHg lower rate of arterial tension on the left arm. Ultrasound of carotid arteries revealed 60% stenosis of the left subclavian artery and bilateral internal carotid artery elongation. MSCT angiography revealed a right-sided aortic arch with aberrant separation of the left subclavian artery that was narrowed 50%, while internal carotid arteries were marginally elongated. There was no need for surgical treatment or percutaneous interventions, so that conservative treatment was indicated. Conclusion. A right-sided aortic arch is a very rare anomaly of the location and branching of the aorta. Multislice CT angiography is of great importance in the diagnostics of this rare disease.

  5. A Right-sided Aortic Arch with Kommerell's Diverticulum of the Aberrant Left Subclavian Artery Presenting with Syncope

    Directory of Open Access Journals (Sweden)

    Ming-Hsun Yang

    2009-05-01

    Full Text Available A right-sided aortic arch with an aneurysm of the aberrant subclavian artery is a rare disease. We report a case of Kommerell's diverticulum of an aberrant left subclavian artery in a patient with a right-sided aortic arch. Fewer than 50 cases have been reported in the literature. A number of operative strategies are described. Right thoracotomy provides good exposure and avoids the morbidity associated with bilateral thoracotomy or sternotomy and thoracotomy. In our patient with symptoms of dysphagia, syncope, and left subclavian steal syndrome, a left thoracotomy was used. The repair was accomplished by division of a left ligamentum arteriosum, obliteration of the Kommerell's aneurysm, and an aorto-subclavian bypass. Postoperative complications included left vocal cord palsy and Horner's syndrome. Hoarseness and left ptosis recovered spontaneously 3 months after surgery, and the patient remained symptom-free at the 1-year follow-up. We believe a left thoracotomy for direct repair of Kommerell's diverticulum is a simple and safe method without the increased morbidity found in other procedures.

  6. Angular (Gothic) aortic arch leads to enhanced systolic wave reflection, central aortic stiffness, and increased left ventricular mass late after aortic coarctation repair: evaluation with magnetic resonance flow mapping.

    Science.gov (United States)

    Ou, Phalla; Celermajer, David S; Raisky, Olivier; Jolivet, Odile; Buyens, Fanny; Herment, Alain; Sidi, Daniel; Bonnet, Damien; Mousseaux, Elie

    2008-01-01

    We sought to investigate the mechanism whereby a particular deformity of the aortic arch, an angulated Gothic shape, might lead to hypertension late after anatomically successful repair of aortic coarctation. Fifty-five normotensive patients with anatomically successful repair of aortic coarctation and either a Gothic (angulated) or a Romanesque (smooth and rounded) arch were studied with magnetic resonance angiography and flow mapping in both the ascending and descending aortas. Systolic waveforms, central aortic stiffness, and pulse velocity were measured. We hypothesized that arch angulation would result in enhanced systolic wave reflection with loss of energy across the aortic arch, as well as increased central aortic stiffness. Twenty patients were found to have a Gothic, and 35 a Romanesque, arch. Patients with a Gothic arch showed markedly augmented systolic wave reflection (12 +/- 6 vs 5 +/- 0.3 mL, P Gothic arch (5.6 +/- 1.1 vs 4.1 +/- 1 m/s, P Gothic aortic arch is associated with increased systolic wave reflection, as well as increased central aortic stiffness and left ventricular mass index. These findings explain (at least in part) the association between this pattern of arch geometry and late hypertension at rest and on exercise in subjects after coarctation repair.

  7. Morphometric changes in the aortic arch with advancing age in fetal to mature thoroughbred horses.

    Science.gov (United States)

    Endoh, Chihiro; Matsuda, Kazuya; Okamoto, Minoru; Tsunoda, Nobuo; Taniyama, Hiroyuki

    2017-03-28

    Aortic rupture is a well recognized cause of sudden death in thoroughbred horses. Some microscopic lesions, such as those caused by cystic medial necrosis and medionecrosis, can lead to aortic rupture. However, these microscopic lesions are also observed in normal horses. On the other hand, a previous study of aortic rupture suggested that underlying elastin and collagen deposition disorders might be associated with aortic rupture. Therefore, the purpose of this study was to compare the structural components of the tunica media of the aortic arch, which is composed of elastin, collagen, smooth muscle cells and mucopolysaccharides (MPS), in fetal to mature thoroughbred horses. The percentage area of elastin was greatest in the young horses and subsequently decreased with aging. The percentage area of collagen increased with aging, and the elderly horses (aged ≥20) exhibited significantly higher percentage areas of collagen than the young horses. The percentage area of smooth muscle cells did not change with age. The percentage area of MPS was inversely proportional to the percentage area of elastin. The fetuses exhibited a markedly larger percentage area of MPS than the mature horses. We concluded that the medial changes seen in the aortic arch, which included a reduction in the amount of elastin and increases in the amounts of collagen and MPS, were age-related variations.

  8. Selective Cerebro-Myocardial Perfusion in Complex Neonatal Aortic Arch Pathology: Midterm Results.

    Science.gov (United States)

    Hoxha, Stiljan; Abbasciano, Riccardo Giuseppe; Sandrini, Camilla; Rossetti, Lucia; Menon, Tiziano; Barozzi, Luca; Linardi, Daniele; Rungatscher, Alessio; Faggian, Giuseppe; Luciani, Giovanni Battista

    2018-04-01

    Aortic arch repair in newborns and infants has traditionally been accomplished using a period of deep hypothermic circulatory arrest. To reduce neurologic and cardiac dysfunction related to circulatory arrest and myocardial ischemia during complex aortic arch surgery, an alternative and novel strategy for cerebro-myocardial protection was recently developed, where regional low-flow perfusion is combined with controlled and independent coronary perfusion. The aim of the present retrospective study was to assess short-term and mid-term results of selective and independent cerebro-myocardial perfusion in neonatal aortic arch surgery. From April 2008 to August 2015, 28 consecutive neonates underwent aortic arch surgery under cerebro-myocardial perfusion. There were 17 male and 11 female, with median age of 15 days (3-30 days) and median body weight of 3 kg (1.6-4.2 kg), 9 (32%) of whom with low body weight (cerebro-myocardial perfusion was 30 ± 11 min (15-69 min). Renal dysfunction, requiring a period of peritoneal dialysis was observed in 10 (36%) patients, while liver dysfunction was noted only in 3 (11%). There were three (11%) early and two late deaths during a median follow-up of 2.9 years (range 6 months-7.7 years), with an actuarial survival of 82% at 7 years. At latest follow-up, no patient showed signs of cardiac or neurologic dysfunction. The present experience shows that a strategy of selective and independent cerebro-myocardial perfusion is safe, versatile, and feasible in high-risk neonates with complex congenital arch pathology. Encouraging outcomes were noted in terms of cardiac and neurological function, with limited end-organ morbidity. © 2018 International Center for Artificial Organs and Transplantation and Wiley Periodicals, Inc.

  9. Retrosternal friction-induced late disruption of the anastomotic site between Bentall's valved conduit and an aortic arch graft: report of a case.

    Science.gov (United States)

    Fukada, Johji; Morishita, Kiyofumi; Kawaharada, Nobuyoshi; Kurimoto, Yoshihiko; Muraki, Satoshi; Satsu, Takuma; Abe, Tomio

    2003-01-01

    We report a case of late mediastinal false aneurysm originating from disruption of the suture line between synthetic vascular grafts for aortic root and total aortic arch replacements. This aneurysm developed without any infection in a patient with Marfan's syndrome. To our knowledge, this event has never been reported before. The only possible cause of this disruption was that the monofilament suture was broken by continuous friction between the pointed anastomotic line and the sternum since the operation. The treatment options for this unusual event after extended synthetic graft replacement are discussed.

  10. 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

  11. Video-assisted thoracoscopic left lower lobectomy in a patient with lung cancer and a right aortic arch

    Directory of Open Access Journals (Sweden)

    Wada Hideyuki

    2012-11-01

    Full Text Available Abstract A right aortic arch is a rare congenital anomaly, with a reported incidence of around 0.1%. A patient with a right aortic arch underwent video-assisted thoracic surgery left lower lobectomy and mediastinal lymph node dissection for squamous cell carcinoma. There was no aortic arch or descending aorta in the left thoracic cavity, but the esophagus. There was no anomaly in the location or branching of the pulmonary vessels, the bronchi, and the lobulation of the lungs. The vagus nerve was found at the level of the left pulmonary artery. The arterial ligament was found between the left subclavian artery and the left pulmonary artery. The recurrent laryngeal nerve was recurrent around the left subclavian artery. A Kommerell diverticulum was found at the origin of the left subclavian artery. The patient experienced no complications. We conclude that video-assisted thoracoscopic lobectomy with mediastinal dissection is feasible for treating lung cancer with a right aortic arch.

  12. Endovascular Retrieval of Entrapped Elephant Trunk Graft During Complex Hybrid Aortic Arch Repair

    Energy Technology Data Exchange (ETDEWEB)

    Damodharan, Karthikeyan, E-mail: drdkarthik@hotmail.com [Singapore General Hospital, Department of Diagnostic Radiology (Singapore); Chao, Victor T. T., E-mail: victor.chao.t.t@singhealth.com.sg [National Heart Centre, Department of Cardiothoracic Surgery (Singapore); Tay, Kiang Hiong, E-mail: tay.kiang.hiong@singhealth.com.sg [Singapore General Hospital, Department of Diagnostic Radiology (Singapore)

    2016-12-15

    Entrapment of the elephant trunk graft within the false lumen is a rare complication of surgical repair of an aortic dissection. This is normally retrieved by emergent open surgery. We describe a technique of endovascular retrieval of the dislodged graft, during hybrid aortic arch repair. The elephant trunk was cannulated through and through from a femoral access and the free end of the wire was snared and retrieved from a brachial access. The wire was externalised from both accesses and was used to reposition the graft into the true lumen using a body flossing technique.

  13. Pattern-based approach to fetal congenital cardiovascular anomalies using the transverse aortic arch view on prenatal cardiac MRI

    Energy Technology Data Exchange (ETDEWEB)

    Dong, Su-Zhen; Zhu, Ming [Shanghai Jiaotong University School of Medicine, Department of Radiology, Shanghai Children' s Medical Center, Shanghai (China)

    2015-05-01

    Fetal echocardiography is the imaging modality of choice for prenatal diagnosis of congenital cardiovascular anomalies. However, echocardiography has limitations. Fetal cardiac magnetic resonance imaging (MRI) has the potential to complement US in detecting congenital cardiovascular anomalies. This article draws on our experience; it describes the transverse aortic arch view on fetal cardiac MRI and important clues on an abnormal transverse view at the level of the aortic arch to the diagnosis of fetal congenital cardiovascular anomalies. (orig.)

  14. Pattern-based approach to fetal congenital cardiovascular anomalies using the transverse aortic arch view on prenatal cardiac MRI

    International Nuclear Information System (INIS)

    Dong, Su-Zhen; Zhu, Ming

    2015-01-01

    Fetal echocardiography is the imaging modality of choice for prenatal diagnosis of congenital cardiovascular anomalies. However, echocardiography has limitations. Fetal cardiac magnetic resonance imaging (MRI) has the potential to complement US in detecting congenital cardiovascular anomalies. This article draws on our experience; it describes the transverse aortic arch view on fetal cardiac MRI and important clues on an abnormal transverse view at the level of the aortic arch to the diagnosis of fetal congenital cardiovascular anomalies. (orig.)

  15. [Metabolic syndrome and aortic stiffness].

    Science.gov (United States)

    Simková, A; Bulas, J; Murín, J; Kozlíková, K; Janiga, I

    2010-09-01

    The metabolic syndrome (MS) is a cluster of risk factors that move the patient into higher level of risk category of cardiovascular disease and the probability of type 2 diabetes mellitus manifestation. Definition of MS is s based on the presence of selected risk factors as: abdominal obesity (lager waist circumpherence), atherogenic dyslipidemia (low value of HDL-cholesterol and increased level of triglycerides), increased fasting blood glucose (or type 2 DM diagnosis), higher blood pressure or antihypertensive therapy. In 2009 there were created harmonizing criteria for MS definition; the condition for assignment of MS is the presence of any 3 criteria of 5 mentioned above. The underlying disorder of MS is an insulin resistance or prediabetes. The patients with MS more frequently have subclinical (preclinical) target organ disease (TOD) which is the early sings of atherosclerosis. Increased aortic stiffness is one of the preclinical diseases and is defined by pathologically increased carotidofemoral pulse wave velocity in aorta (PWV Ao). With the aim to assess the influence of MS on aortic stiffness we examined the group of women with arterial hypertension and MS and compare them with the group of women without MS. The aortic stiffness was examined by Arteriograph--Tensiomed, the equipment working on the oscillometric principle in detection of pulsations of brachial artery. This method determines the global aortic stiffness based on the analysis of the shape of pulse curve of brachial artery. From the cohort of 49 pts 31 had MS, the subgroups did not differ in age or blood pressure level. The mean number of risk factors per person in MS was 3.7 comparing with 1.7 in those without MS. In the MS group there was more frequently abdominal obesity present (87% vs 44%), increased fasting blood glucose (81% vs 22%) and low HDL-cholesterol level. The pulse wave velocity in aorta, PWV Ao, was significantly higher in patients with MS (mean value 10,19 m/s vs 8,96 m

  16. Kabuki syndrome in a girl with mosaic 45,X/47,XXX and aortic coarctation.

    Science.gov (United States)

    Chen, Chih-Ping; Lin, Shuan-Pei; Tsai, Fuu-Jen; Chern, Schu-Rern; Wang, Wayseen

    2008-06-01

    To describe the clinical findings of a patient with mosaic 45,X/47,XXX and aortic coarctation. Descriptive case study. Tertiary medical center. A 6-year-old girl with stigmata of Turner syndrome, aortic coarctation, patent ductus arteriosus, and a peculiar facial appearance. None. Cytogenetic analysis. The patient manifested a characteristic Kabuki syndrome facial appearance with long palpebral fissures, everted lateral third of lower eyelids, arched eyebrows, a depressed nasal tip, large dysplastic ears and epicanthic folds. She had undergone cardiac surgery for treatment of aortic coarctation and patent ductus arteriosus. Cytogenetic analysis of the blood lymphocytes revealed a karyotype of mos 45,X,9ph [35 cells]/47,XXX,9ph [5 cells]. This is the first report of mosaic 45,X/47,XXX associated with Kabuki syndrome. We emphasize that Kabuki syndrome, a peculiar facial appearance and aortic coarctation, should be considered in girls with sex chromosome abnormalities.

  17. [Right patent ductus arteriosus with an ipsilateral aortic arch: percutaneous closure with amplatzer devices].

    Science.gov (United States)

    Santiago, Justo; Acuña, Manuel; Arispe, Elizabeth; Camargo, Ronaldo; Neves, Juliana; Arnoni, Daniel; Fontes, Valmir F; Pedra, Carlos A

    2007-03-01

    The association of a right aortic arch with an ipsilateral patent ductus arteriosus is rare, especially when there are no other intracardiac anomalies. We report three female patients aged 26, 35 and 9 years with this combination in whom previous attempts at surgical closure by thoracotomy and sternotomy were unsuccessful and who subsequently underwent successful percutaneous closure of the defects using Amplatzer devices. In two patients, although angiography demonstrated the presence of type-A patent ductus arteriosus, it was not possible to determine the minimum diameter accurately and it was necessary to measure it using a sizing balloon. An Amplatzer duct occluder was used in two patients and an Amplatzer muscular ventricular septal defect occluder, in the other. In all patients, full closure was confirmed in the catheterization laboratory and the patients were discharged on the same day with no complications. Percutaneous closure of a right patent ductus arteriosus associated with a right aortic arch is feasible, safe and effective.

  18. A current systematic evaluation and meta-analysis of chimney graft technology in aortic arch diseases.

    Science.gov (United States)

    Ahmad, Wael; Mylonas, Spyridon; Majd, Payman; Brunkwall, Jan Sigge

    2017-11-01

    The aim of this study was to provide a review of the literature on the use of chimney graft (CG) technique in treating arterial diseases of the aortic arch and to extrapolate conclusions by summarizing the reported outcomes in a meta-analysis. An extensive electronic search was made using PubMed/MEDLINE, Science Direct Databases, and the Cochrane Library. Included in this meta-analysis were all papers published up to February 2016 on endovascular chimney technique in the arch vessels with or without adjunct extra-anatomic debranching, in any language, providing data about at least one of the essential outcomes: early and late type I endoleak, 30-day mortality rate, development of perioperative stroke, patency, and retrograde aortic dissection. Of the 478 reports yielded by the electronic search, a total of 11 publications (on 373 patients and 387 CGs) fulfilled the inclusion criteria and were included in this study. The overall estimated proportion of technical success was 91.3% (95% confidence interval [CI], 87.4%-94.0%). Of the 373 patients, 26 (7%) experienced a type Ia endoleak in the perioperative period. The overall estimated proportion of early type Ia endoleak was 9.4% (95% CI, 6.5%-13.4%). Among the 10 studies that provided data, a retrograde type A dissection was observed in 2 of 351 patients, resulting in an overall estimated proportion of 1.8% (95% CI, 0.8%-4.0%). The pooled 30-day mortality rate was 7.9% (95% CI, 4.6%-13.2%). The pooled estimation for reintervention was 10.6% (95% CI, 5%-21%); for major stroke, 2.6% (95% CI, 1.3%-5.0%); for early patency, 97.9% (95% CI, 95.8%-99%); and for late patency, 92.9% (95% CI, 87.3%-96%). Treatment of aortic diseases involving the aortic arch poses a great challenge. The CG technique has been applied as an alternative treatment option. This meta-analysis shows that endovascular repair of aortic arch disease using a CG technique in the aortic arch vessels is technically feasible and effective but not without

  19. Left-Sided Patent Ductus Arteriosus in a Right-Sided Aortic Arch

    Directory of Open Access Journals (Sweden)

    Ming-Yen Ng

    2014-01-01

    Full Text Available We present a 31-year-old female with repaired tetralogy of Fallot (TOF and right-sided aortic arch (RAA with left-sided patent ductus arteriosus (PDA originating from the left brachiocephalic artery. This is a rare finding but most common site for a PDA in TOF and a RAA. To the best of our knowledge, this is the first demonstration of this rare finding on MRI in the literature.

  20. Interrupted Aortic Arch Associated with Absence of Left Common Carotid Artery: Imaging with MDCT

    International Nuclear Information System (INIS)

    Onbas, Omer; Olgun, Hasim; Ceviz, Naci; Ors, Rahmi; Okur, Adnan

    2006-01-01

    Interrupted aortic arch (IAA) is a rare severe congenital heart defect defined as complete luminal and anatomic discontinuity between ascending and descending aorta. Although its association with various congenital heart defects has been reported, absence of left common carotid artery (CCA) in patients with IAA has not been reported previously. We report a case of IAA associated with the absence of left CCA which was clearly shown on multidetector-row spiral CT

  1. Neurodevelopmental outcomes after regional cerebral perfusion with neuromonitoring for neonatal aortic arch reconstruction.

    Science.gov (United States)

    Andropoulos, Dean B; Easley, R Blaine; Brady, Ken; McKenzie, E Dean; Heinle, Jeffrey S; Dickerson, Heather A; Shekerdemian, Lara S; Meador, Marcie; Eisenman, Carol; Hunter, Jill V; Turcich, Marie; Voigt, Robert G; Fraser, Charles D

    2013-02-01

    In this study we report magnetic resonance imaging (MRI) brain injury and 12-month neurodevelopmental outcomes when regional cerebral perfusion (RCP) is used for neonatal aortic arch reconstruction. Fifty-seven neonates receiving RCP during aortic arch reconstruction were enrolled in a prospective outcome study. RCP flows were determined by near-infrared spectroscopy and transcranial Doppler monitoring. Brain MRI was performed preoperatively and 7 days postoperatively. Bayley Scales of Infant Development III was performed at 12 months. Mean RCP time was 71 ± 28 minutes (range, 5 to 121 minutes) and mean flow was 56.6 ± 10.6 mL/kg/min. New postoperative MRI brain injury was seen in 40% of patients. For 35 RCP patients at age 12 months, mean Bayley Scales III Composite standard scores were: Cognitive, 100.1 ± 14.6 (range, 75 to 125); Language, 87.2 ± 15.0 (range, 62 to 132); and Motor, 87.9 ± 16.8 (range, 58 to 121). Increasing duration of RCP was not associated with adverse neurodevelopmental outcomes. Neonatal aortic arch repair with RCP using a neuromonitoring strategy results in 12-month cognitive outcomes that are at reference population norms. Language and motor outcomes are lower than the reference population norms by 0.8 to 0.9 standard deviations. The neurodevelopmental outcomes in this RCP cohort demonstrate that this technique is effective and safe in supporting the brain during neonatal aortic arch reconstruction. Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  2. Abnormal innominate vein and right aortic arch, an uncommon association that should be taken into account

    International Nuclear Information System (INIS)

    Catala, J.; Martin, C.

    2000-01-01

    A case of abnormal innominate vein associated with right aortic arch and aberrant left subclavian artery is presented. It was an incidental finding during the radiological study of an 8-year old boy suspected of having tuberculosis. The authors review the different etiological theories, radiological features and diagnostic impact of this uncommon venous malformation, as well as its relationship to other cardiovascular anomalies. (Author) 9 refs

  3. Aortic arch reconstruction: deep and moderate hypothermic circulatory arrest with selective antegrade cerebral perfusion.

    Science.gov (United States)

    Wu, YanWen; Xiao, LiQiong; Yang, Ting; Wang, Lei; Chen, Xin

    2017-07-01

    To compare the effects of moderate and deep hypothermic circulatory arrest (DHCA) with selective antegrade cerebral perfusion (SACP) during aortic arch surgery in adult patients and to offer the evidence for the detection of the temperature which provides best brain protection in the subjects who accept aortic arch reconstruction surgery. A total of 109 patients undergoing surgery of the aortic arch were divided into the moderate hypothermic circulatory arrest group (Group I) and the deep hypothermic circulatory arrest group (Group II). We recorded the data of the patients and their cardiopulmonary bypass (CPB) time, aortic clamping time, SACP time and postoperative anesthetized recovery time, tracheal intubation time, time in the intensive care unit (ICU) and postoperative neurologic dysfunction. Patient characteristics were similar in the two groups. There were four patients who died in Group II and 1 patient in Group I. There were no significant differences in aortic clamping time of each group (111.4±58.4 vs. 115.9±16.2) min; SACP time (27.4±5.9 vs. 23.5±6.1) min of the moderate hypothermic circulatory arrest group and the deep hypothermic circulatory arrest group; there were significant differences in cardiopulmonary bypass time (207.4±20.9 vs. 263.8±22.6) min, postoperative anesthetized recovery time (19.0±11.1 vs. 36.8±25.3) hours, extubation time (46.4±15.1 vs. 64.4±6.0) hours; length of stay in the intensive care unit (ICU) (4.7±1.7 vs. 8±2.3) days and postoperative neurologic dysfunction in the two groups. Compared to deep hypothermic circulatory arrest, moderate hypothermic circulatory arrest can provide better brain protection and achieve good clinical results.

  4. 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.

  5. Persistent fifth arch anomalies - broadening the spectrum to include a variation of double aortic arch vascular ring

    Energy Technology Data Exchange (ETDEWEB)

    Newman, Beverley; Chan, Frandics [Stanford Children' s Hospital and Stanford University, Department of Radiology, Stanford, CA (United States); Hanneman, Kate [University of Toronto, Department of Medical Imaging, Toronto, ON (Canada)

    2016-12-15

    Fifth arch anomalies are rare and complex and frequently misdiagnosed or mistaken for other entities. We report a double arch vascular ring that is thought to consist of right fourth arch and left fifth arch components, a previously undescribed persistent fifth arch variant. The currently recognized spectrum and classification of fifth arch vascular anomalies are expanded along with illustrative images to justify the proposed changes. Reviewing and expanding the classification of fifth arch anomalies to include a double arch ring variant will promote recognition, correct diagnosis and appropriate management of these anomalies. (orig.)

  6. Importance of stent-graft design for aortic arch aneurysm repair

    Directory of Open Access Journals (Sweden)

    C Singh

    2017-02-01

    Full Text Available Aneurysm of the aorta is currently treated by open surgical repair or endovascular repair. However, when the aneurysm occurs in regions between the aortic arch and proximal descending aorta, it can be a complex pathology to treat due to its intricate geometry. When complex aortic aneurysms are treated with the conventional procedures, some of the patients present with significant post-operative complications and high mortality rate. Consequently, a clinically driven hybrid innovation known as the frozen elephant trunk procedure was introduced to treat complex aortic aneurysms. Although this procedure significantly reduces mortality rate and operating time, it is still associated with complications such as endoleaks, spinal cord ischemia, renal failure and stroke. Some of these complications are consequences of a mismatch in the biomechanical behaviour of the stent-graft device and the aorta. Research on complex aneurysm repair tended to focus more on the surgical procedure than the stent-graft design. Current stent-graft devices are suitable for straight vessels. However, when used to treat aortic aneurysm with complex geometry, these devices are ineffective in restoring the normal biological and biomechanical function of the aorta. A stent-graft device with mechanical properties that are comparable with the aorta and aortic arch could possibly lead to fewer post-operative complications, thus, better outcome for patients with complex aneurysm conditions. This review highlights the influence stent-graft design has on the biomechanical properties of the aorta which in turn can contribute to complications of complex aneurysm repair. Design attributes critical for minimising postoperative biomechanical mismatch are also discussed.

  7. Repair of aortic arch aneurysm under cardiopulmonary bypass and deep hypothermia with low flow: A case report

    Directory of Open Access Journals (Sweden)

    Md. Rezwanul Hoque

    2016-07-01

    Full Text Available Aortic arch surgery is the challenging and most difficult surgery among the cardiovascular operations. Cerebral and spinal complications are the most feared and common complications of aortic arch surgery. With best available techniques for cerebral and spinal protection, anesthetic management and good post-operative care; aortic arch surgery is considerably safer nowadays and satisfactory results can be achieved in most patients. Also, selecting the sites for arterial cannulation to maintain whole body circulation, during isolation of the aortic arch to operate on it, need proper anatomical description of the extent of the aneurysm. This is also achievable by the availability of the imaging techniques like Computed Tomog­raphy (CT with or without contrast, CT Angiography (CTA and Magnetic Resonance Imaging (MRI. We are reporting a case of aneurysm of aortic arch in a young adult, who had undergone repair under cardiopulmonary bypass and deep hypothermia with low flow and had normal convalescence without any cerebral or spinal complications.

  8. Hoarseness —As a presenting feature of aortic arch aneurysm

    OpenAIRE

    Mathai, John; Swapna, U. P.

    2006-01-01

    Hoarseness due to left recurrent laryngeal nerve paralysis (LRLN) caused by identifiable cardiovascular disease is described as Ortner’s syndrome or Cardiovocal syndrome. This was first described by Ortner in 1897 to describe left recurrent laryngeal nerve palsy secondary to Mitral Stenosis. The common conditions which gave rise to Ortners’ syndrome include mitral stenosis, mitral valve prolapse, aortic aneurysm, septal defect, following cardiothoracic surgery, high altitude pulmonary hyperte...

  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. Multi-detector row computed tomographic evaluation of a rare type of complete vascular ring: Double aortic arch with atretic left arch distal to the origin of left subclavian artery

    Energy Technology Data Exchange (ETDEWEB)

    Hung, Ying Ying; Fu, Ching Yun; Wei, Hao Ji; Tsai, I Chen; Chen, Clayton Chi Chang [Taichung Veterans General Hospital, Taichung (China)

    2013-10-15

    Double aortic arch with an atretic left arch distal to the origin of left subclavian artery was diagnosed with multi-detector row computed tomography (MDCT) in two children with dysphagia. This rare type of complete vascular ring is clinically important because it may be confused with right aortic arch in mirror imaging. Anatomic details of this rare type of complete vascular ring demonstrated on MDCT facilitated appropriate surgical treatment.

  11. Selective Heart, Brain and Body Perfusion in Open Aortic Arch Replacement.

    Science.gov (United States)

    Maier, Sven; Kari, Fabian; Rylski, Bartosz; Siepe, Matthias; Benk, Christoph; Beyersdorf, Friedhelm

    2016-09-01

    Open aortic arch replacement is a complex and challenging procedure, especially in post dissection aneurysms and in redo procedures after previous surgery of the ascending aorta or aortic root. We report our experience with the simultaneous selective perfusion of heart, brain, and remaining body to ensure optimal perfusion and to minimize perfusion-related risks during these procedures. We used a specially configured heart-lung machine with a centrifugal pump as arterial pump and an additional roller pump for the selective cerebral perfusion. Initial arterial cannulation is achieved via femoral artery or right axillary artery. After lower body circulatory arrest and selective antegrade cerebral perfusion for the distal arch anastomosis, we started selective lower body perfusion simultaneously to the selective antegrade cerebral perfusion and heart perfusion. Eighteen patients were successfully treated with this perfusion strategy from October 2012 to November 2015. No complications related to the heart-lung machine and the cannulation occurred during the procedures. Mean cardiopulmonary bypass time was 239 ± 33 minutes, the simultaneous selective perfusion of brain, heart, and remaining body lasted 55 ± 23 minutes. One patient suffered temporary neurological deficit that resolved completely during intensive care unit stay. No patient experienced a permanent neurological deficit or end-organ dysfunction. These high-risk procedures require a concept with a special setup of the heart-lung machine. Our perfusion strategy for aortic arch replacement ensures a selective perfusion of heart, brain, and lower body during this complex procedure and we observed excellent outcomes in this small series. This perfusion strategy is also applicable for redo procedures.

  12. Early and midterm outcomes of open stent-graft treatment for distal aortic arch aneurysm

    International Nuclear Information System (INIS)

    Yamada, Kazunori; Mochizuki, Takaaki; Tsubota, Hideki; Funamoto, Masaki

    2008-01-01

    The aim of this study was to investigate early and late outcomes for open stent-graft treatment, which was introduced as a less-invasive technique for thoracic aortic aneurysm of the distal arch, and to clarify the validity of and indications for this treatment. We retrospectively investigated 38 patients with thoracic aortic aneurysm of the distal arch who underwent open stent-graft placement at our hospital between June 2000 and September 2006. Five patients died in hospital (hospital mortality 13.2%). Age at the time of surgery and onset of postoperative paraplegia were identified as risk factors. Four patients (10.5%) had postoperative paraplegia, but no significant risk factors were seen. The size of the aneurysm was clearly reduced in 18 of the 25 patients (75.8%), in whom computed tomography was performed after discharge, and late outcomes were good. Mural thrombus thickness on the stent landing zone of <4 mm was a predictor for aneurysm shrinkage. Seven patients died during the late period, and the 5-year survival rate among hospital survivors was 80.1%. Early outcomes for open stent-graft are not necessarily good, and late survival is also not excellent. Open stent-graft thus cannot be regarded as an ideal technique for all patients with distal aortic arch aneurysm. However, after aneurysm shrinkage was confirmed during the early period, late outcomes were good. Absence of thick mural thrombus on the stent landing zone may represent a good indication for open stent-graft surgery. (author)

  13. Pregnancy with aortic dissection in Ehler-Danlos syndrome. Staged replacement of the total aorta (10-year follow-up).

    Science.gov (United States)

    Babatasi, G; Massetti, M; Bhoyroo, S; Khayat, A

    1997-10-01

    Pregnancy complicated by aortic dissection in patients with hereditary disorder of connective tissue presents interesting considerations including management of caesarean section with the unexpected need for cardiac surgery in emergency. Generalizations can be made on management principles with long-term follow-up requiring an aggressive individualized approach by a multidisciplinary team. A 33-year-old parturient presenting an aortic dissection at 37 weeks gestation required prompt diagnosis of Ehlers-Danlos syndrome in combination with correct surgical therapy resulted in the survival of both the mother and infant. During the 10-year follow-up, multiple complex dissection required transverse aortic arch and thoracoabdominal aortic replacement.

  14. Analysis of autologous platelet-rich plasma during ascending and transverse aortic arch surgery.

    Science.gov (United States)

    Zhou, Shao-Feng; Estrera, Anthony L; Miller, Charles C; Ignacio, Craig; Panthayi, Sreelatha; Loubser, Paul; Sagun, Dean L; Sheinbaum, Roy; Safi, Hazim J

    2013-05-01

    Coagulopathy is a common complication after ascending and transverse arch aortic surgery with profound hypothermic circuit arrest (PHCA). Blood conservation strategies to reduce transfusion have been ongoing and involve multiple treatment modalities in modern cardiac surgery. The purpose of this study is to evaluate the effectiveness of autologous platelet-rich plasma (aPRP) as a blood conservation technique to reduce blood transfusion in ascending and arch aortic surgery. Between 2003 and 2009, we retrospectively reviewed 685 cases of ascending aorta and transverse arch repair using PHCA. A total of 287 patients in which aPRP was used (aPRP group) were compared with 398 patients who did have aPRP (non-aPRP group). Perioperative transfusion requirements and clinical outcomes that included early mortality, postoperative stroke, renal dysfunction, prolonged ventilation, coagulopathy, and length of postoperative intensive care unit stay were analyzed. The data were analyzed by mean and frequency for continuous variables and qualitative variables. To account for potential selection bias, 2 types of propensity analysis were performed. In both unadjusted and adjusted analysis, perioperative transfusions were fewer in the aPRP group compared with the non-aPRP group: (3.9 units fewer packed red blood cells, 4.5 units fewer fresh frozen plasma, 7.9 units fewer platelets, and 6.8 units fewer cryoprecipitate). In all analyses, postoperative morbidity (stroke, duration of mechanical ventilation, and intensive care unit stay) were significantly improved. Hospital mortality rate was not significantly decreased. The utilization of aPRP was associated with a reduction in allogeneic blood transfusions as well as a decrease in early postoperative morbidity during repairs of the ascending and transverse arch aorta using PHCA. Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  15. Total aortic arch replacement with the elephant trunk technique: single-centre 30-year results.

    Science.gov (United States)

    Shrestha, Malakh; Martens, Andreas; Krüger, Heike; Maeding, Illona; Ius, Fabio; Fleissner, Felix; Haverich, Axel

    2014-02-01

    The combined disease of the aortic arch and the descending aorta (aneurysms and dissection) remains a surgical challenge. Various approaches have been used to treat this complex pathology. In the two-stage operation, at the first-stage operation, the aortic arch is replaced through a median sternotomy. Later, at the second-stage operation, the descending thoracic aorta is replaced through a lateral thoracotomy. The elephant trunk (ET) technique was introduced by H.G. Borst at our centre in March 1982, greatly simplifying the second-phase operation. We present our 30-year experience. From March 1982 to March 2012, 179 patients (112 males, age 56.4±12.6 years) received an ET procedure for the combined disease of the aortic arch and the descending aorta (91 aneurysms, 88 dissections (47 acute)). Fifty-six of these patients had undergone previous cardiac operations. Concomitant procedures were performed if necessary. The cerebral protection was done either by deep (till 1999) or moderate hypothermic circulatory arrest and selective antegrade cerebral perfusion (SACP, after 1999). Cardiopulmonary bypass (CPB) and X-clamp times were 208.5±76.5 min and 123.7±54.8 min, respectively. The intraoperative mortality and 30-day mortality during the first-stage operation were 1.7% (3/179) and 17.3% (31/179, 15 with AADA), respectively. Perioperative stroke was 7.9% (n=14/176). Postoperative recurrent nerve palsy was present in 18.2% (32/176) and paraplegia in 5.6% (10/176). The second-stage completion operation was performed as early as possible. Fifty-seven second-stage completion procedures were performed, either surgically (n=50) or through interventional techniques (n=7). The intraoperative and 30-day mortality after the second-stage completion procedures were 5.2% (3/57) and 7.0% (4/57), respectively. The stroke, recurrent nerve palsy and paraplegia rates were 0, 0 and 7% (4/54), respectively. The ET technique has greatly facilitated the two-stage approach to the surgical

  16. Comparison of hybrid endovascular and open surgical repair for proximal aortic arch diseases.

    Science.gov (United States)

    Kang, Woong Chol; Ko, Young-Guk; Shin, Eak Kyun; Park, Chul-Hyun; Choi, Donghoon; Youn, Young Nam; Lee, Do Yun

    2016-01-15

    To compare the outcomes of hybrid endovascular and open surgical repair for proximal aortic arch diseases. A total of 55 consecutive patients with aortic arch aneurysm or aortic dissection involving any of zone 0 to 1 (39 male, age 63.4 ± 14.3 years) underwent a hybrid endovascular repair (n=35) or open surgical repair (n=20) from 2006 to 2014 were analyzed retrospectively. Perioperative and late outcomes were compared. Baseline characteristics were similar between the two groups, except age and EuroSCORE II, which were higher in the hybrid group. Perioperative mortality or stroke was not significantly different between the two groups, however, tended to be lower in the hybrid repair group than in the open repair group (11.4% vs. 30.0%, p=0.144). Incidences of other morbidities did not differ. During follow-up, over-all survival was similar between the hybrid and the open repair was similar (87.3% vs. 79.7% at 1 year and 83.8% vs. 72.4% at 3 years; p=0.319). However, reintervention-free survival was significantly lower for hybrid repair compared with open repair (83.8% vs. 100% at 1 year and 65.7% vs. 100% at 3 years; p=0.022). Hybrid repair of proximal aortic disease showed comparable perioperative and late outcomes compared with open surgical repair despite a higher reintervention rate during follow-up. Therefore, hybrid repair may be considered as an acceptable treatment alternative to surgery especially in patients at high surgical risk. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

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

    Science.gov (United States)

    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.

  18. Isolation of the Left Innominate Artery with a Right Aortic Arch in a 76 year-old Man: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Young Tong; Jou, Sung Shick; Bae, Won Kyung [Soonchunhyang University Cheonan Hospital, Cheonan (Korea, Republic of)

    2009-05-15

    Isolation of the left innominate artery is a rare anomaly and is usually combined with a right side aortic arch. Most patients are neonates or children with congenital heart disease. We report the MDCT findings of a right aortic arch and isolation of the left innominate artery in a 76-year-old man without congenital heart disease

  19. Interrupção do arco aórtico tipo B em uma paciente com síndrome de olho de gato Interrupción del arco aórtico tipo B en una paciente con síndrome del ojo de gato Interrupted aortic arch type B in A patient with cat eye syndrome

    Directory of Open Access Journals (Sweden)

    Sintia Iole Nogueira Belangero

    2009-05-01

    Full Text Available Relatamos um caso de paciente com Síndrome do Olho de Gato (Cat Eye Syndrome-CES e interrupção do arco aórtico tipo B, um achado típico na síndrome da deleção 22q11.2. A análise cromossômica e a técnica de hibridização fluorescente in situ (FISH mostraram um cromossomo marcador isodicêntrico supranumerário com bi-satélite derivado do cromossomo 22. O segmento de 22pter a 22q11.2 no cromossomo supranumerário encontrado em nosso paciente não estava em sobreposição com a região deletada em pacientes com a síndrome da deleção 22q11.2. Entretanto, o achado de interrupção do arco aórtico tipo B não é usual na CES, mas é um defeito cardíaco freqüente na síndrome da deleção 22q11.Informamos un caso de paciente con Síndrome de Ojo de Gato (Cat Eye Syndrome-CES e Interrupción del Arco Aórtico tipo B, un hallazgo típico en el síndrome de la deleción 22q11.2. El análisis cromosómico y la técnica de hibridación in situ fluorescente (FISH mostraron un cromosoma marcador isodicéntrico supernumerario bisatelitado derivado del cromosoma 22. El segmento de 22pter a 22q11.2 en el cromosoma supernumerario encontrado en nuestro paciente no estaba en sobreposición con la región deletada en pacientes con el síndrome de la deleción 22q11.2. Con todo, el hallazgo de interrupción del arco aórtico tipo B no es usual en el CES, sino que es un defecto cardíaco frecuente en el síndrome de deleción 22q11.We report a patient with cat eye syndrome and interrupted aortic arch type B, a typical finding in the 22q11.2 deletion syndrome. Chromosomal analysis and fluorescent in situ hybridization (FISH showed a supernumerary bisatellited isodicentric marker chromosome derived from chromosome 22. The segment from 22pter to 22q11.2 in the supernumerary chromosome found in our patient does not overlap with the region deleted in patients with the 22q11.2 deletion syndrome. However, the finding of an interrupted aortic arch type B is

  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. Medical image of the week: atherosclerotic aneurysm of aortic arch and descecnding thoracic aorta

    Directory of Open Access Journals (Sweden)

    Parasram M

    2016-02-01

    Full Text Available No abstract available. Article truncated after 150 words. A 94-year-old Spanish-speaking woman presented to the hospital with intermittent episodes of dyspnea and abdominal pain for one week. Her past medical history was notable for 30 pack-year smoking history and hypertension, which was reportedly controlled with medical therapy. Physical exam showed trace peripheral edema bilaterally, intact peripheral pulses, and a mild abdominal bruit. Work up at the emergency department revealed a non-ST elevation myocardial infarction with troponin T of 0.34 ng/mL but no ST-wave abnormality on electrocardiography. Chest x-ray displayed an incidental thoracic aneurysm (Figure 1. Chest computed tomography with contrast demonstrated a continuous aneurysm of the aortic arch and descending thoracic aorta with diameters measuring 6.8 cm and 6 cm, respectively (Figure 2A and 2B. Eccentric thrombi are noted in the aortic arch and the descending aorta. Interestingly, the distal descending thoracic aorta curves as it transitions to the abdominal aorta, which is evidence of a tortuous descending ...

  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. 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.

  4. 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.

  5. 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.

  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. Surgical modification for preventing a gothic arch after aortic arch repair without the use of foreign material.

    Science.gov (United States)

    Seo, Dong-Man; Park, Jiyoung; Goo, Hyun Woo; Kim, Young Hwue; Ko, Jae-Kon; Jhang, Won Kyoung

    2015-04-01

    Systemic hypertension is the main late complication after arch reconstruction in patients with arch obstruction. Gothic arch geometry is suspected to be one of its possible causes. Accordingly, we evaluated here if a modified arch repair technique using an autologous pulmonary patch is effective in preventing gothic arch development. Fifty infants who underwent arch repair with either a modified (n = 17) or conventional (n = 33) technique between January 2006 and August 2012 by a single surgeon were retrospectively reviewed. Arch geometry was compared using three categories (gothic, crenel or roman), classified by the height/width (H/W) ratio and the arch angle measured in computed tomography. No gothic arch geometry was observed in the modified group, whereas it was observed in 9 cases in the conventional group (P = 0.005). Moreover, reintervention for arch restenosis was performed only in the conventional group (n = 4; P = 0.29). No associated complications were observed, although the selective cerebral perfusion time was longer in the modified group than in the conventional group (28.5 ± 6.2 vs 17.1 ± 9.9 min; P gothic arch geometry, but also as equally safe in terms of early clinical outcomes as conventional arch reconstruction techniques. © The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

  8. Hemiarch versus total aortic arch replacement in acute type A dissection: a systematic review and meta-analysis.

    Science.gov (United States)

    Poon, Shi Sum; Theologou, Thomas; Harrington, Deborah; Kuduvalli, Manoj; Oo, Aung; Field, Mark

    2016-05-01

    Despite recent advances in aortic surgery, acute type A aortic dissection remains a surgical emergency associated with high mortality and morbidity. Appropriate management is crucial to achieve satisfactory outcomes but the optimal surgical approach is controversial. The present systematic review and meta-analysis sought to access cumulative data from comparative studies between hemiarch and total aortic arch replacement in patients with acute type A aortic dissection. A systematic review of the literature using six databases. Eligible studies include comparative studies on hemiarch versus total arch replacement reporting short, medium and long term outcomes. A meta-analysis was performed on eligible studies reporting outcome of interest to quantify the effects of hemiarch replacement on mortality and morbidity risk compared to total arch replacement. Fourteen retrospective studies met the inclusion criteria and 2,221 patients were included in the final analysis. Pooled analysis showed that hemiarch replacement was associated with a lower risk of post-operative renal dialysis [risk ratio (RR) =0.72; 95% confidence interval (CI): 0.56-0.94; P=0.02; I(2)=0%]. There was no significant difference in terms of in-hospital mortality between the two groups (RR =0.84; 95% CI: 0.65-1.09; P=0.20; I(2)=0%). Cardiopulmonary bypass, aortic cross clamp and circulatory arrest times were significantly longer in total arch replacement. During follow up, no significant difference was reported from current studies between the two operative approaches in terms of aortic re-intervention and freedom from aortic reoperation. Within the context of publication bias by high volume aortic centres and non-randomized data sets, there was no difference in mortality outcomes between the two groups. This analysis serves to demonstrate that for those centers doing sufficient total aortic arch activity to allow for publication, excellent and equivalent outcomes are achievable. Conclusions on

  9. First and second branchial arch syndromes: multimodality approach

    International Nuclear Information System (INIS)

    Senggen, Elodie; Laswed, Tarek; Meuwly, Jean-Yves; Maestre, Leonor Alamo; Meuli, Reto; Gudinchet, Francois; Jaques, Bertrand

    2011-01-01

    First and second branchial arch syndromes (BAS) manifest as combined tissue deficiencies and hypoplasias of the face, external ear, middle ear and maxillary and mandibular arches. They represent the second most common craniofacial malformation after cleft lip and palate. Extended knowledge of the embryology and anatomy of each branchial arch derivative is mandatory for the diagnosis and grading of different BAS lesions and in the follow-up of postoperative patients. In recent years, many new complex surgical approaches and procedures have been designed by maxillofacial surgeons to treat extensive maxillary, mandibular and external and internal ear deformations. The purpose of this review is to evaluate the role of different imaging modalities (orthopantomogram (OPG), lateral and posteroanterior cephalometric radiographs, CT and MRI) in the diagnosis of a wide spectrum of first and second BAS, including hemifacial microsomia, mandibulofacial dysostosis, branchio-oto-renal syndrome, Pierre Robin sequence and Nager acrofacial dysostosis. Additionally, we aim to emphasize the importance of the systematic use of a multimodality imaging approach to facilitate the precise grading of these syndromes, as well as the preoperative planning of different reconstructive surgical procedures and their follow-up during treatment. (orig.)

  10. First and second branchial arch syndromes: multimodality approach

    Energy Technology Data Exchange (ETDEWEB)

    Senggen, Elodie; Laswed, Tarek; Meuwly, Jean-Yves; Maestre, Leonor Alamo; Meuli, Reto; Gudinchet, Francois [University Hospital of Lausanne, Radiology Department, Lausanne (Switzerland); Jaques, Bertrand [University Hospital of Lausanne, Department of Otorhinolaryngology, Lausanne (Switzerland)

    2011-05-15

    First and second branchial arch syndromes (BAS) manifest as combined tissue deficiencies and hypoplasias of the face, external ear, middle ear and maxillary and mandibular arches. They represent the second most common craniofacial malformation after cleft lip and palate. Extended knowledge of the embryology and anatomy of each branchial arch derivative is mandatory for the diagnosis and grading of different BAS lesions and in the follow-up of postoperative patients. In recent years, many new complex surgical approaches and procedures have been designed by maxillofacial surgeons to treat extensive maxillary, mandibular and external and internal ear deformations. The purpose of this review is to evaluate the role of different imaging modalities (orthopantomogram (OPG), lateral and posteroanterior cephalometric radiographs, CT and MRI) in the diagnosis of a wide spectrum of first and second BAS, including hemifacial microsomia, mandibulofacial dysostosis, branchio-oto-renal syndrome, Pierre Robin sequence and Nager acrofacial dysostosis. Additionally, we aim to emphasize the importance of the systematic use of a multimodality imaging approach to facilitate the precise grading of these syndromes, as well as the preoperative planning of different reconstructive surgical procedures and their follow-up during treatment. (orig.)

  11. 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.

  12. 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.

  13. 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.

  14. Spontaneous perforation of a syphilitic aneurysm of the aortic arch into the pulmonary artery

    International Nuclear Information System (INIS)

    Stampfel, G.

    1983-01-01

    An aortopulmonary fistula in case of a bag-shaped aneurysm of the aortic arch is demonstrated via angiography. A diagnosis to the effect that the existence of this phenomenon is suspected, can already be arrived at when examining the plain radiography of the thorax. We can say that a sign of an aortopulmonary fistula is represented, in case of an aneurysm of the aorta ascendens and of the arcus aortae, by the existence of a left-right shunt with hypercirculation in the pulmonary circulation, in most cases also signs of right ventricular insufficiency. This is often combined with a typical previous history of sudden dyspnoeas and thoracic pain. The article points out the theoretical possibility of balloon catheter occlusion of the fistula to relieve the acute right ventricular load. (orig.) [de

  15. Duplo arco aórtico: a quebra do silêncio Double aortic arch: the break of silence

    Directory of Open Access Journals (Sweden)

    Ana Rita Abrão

    2011-03-01

    Full Text Available Anéis vasculares representam 1-2% dos casos das cardiopatias congênitas. Relatamos um caso raro de duplo arco aórtico. Mulher, 60 anos, procurou atendimento na clínica médica apresentando 1 ano de história de disfagia, 6 meses de dispneia e 2 meses de dor torácica esporádica. Raio X de tórax revelou: hiperinsuflação pulmonar difusa, alargamento mediastinal, coração com volume e configurações normais, arco aórtico à direita e alterações degenerativas vertebrais. Tomografia computadorizada do tórax: arco aórtico duplo circundando e comprimindo a traqueia e o esôfago. Arco direito mais calibroso, emergindo dele o tronco braquiocefálico. Do arco esquerdo emergem a artéria carótida comum e a subclávia esquerda. Diagnóstico: anel vascular traqueoesofagiano decorrente do duplo arco aórtico, sendo o arco direito dominante. No presente caso, optou-se por seguimento clínico da paciente, levando-se em conta a intensidade dos sintomas apresentados.Vascular rings represent 1 to 2% of cases of congenital heart disease. We report a rare case of double aortic arch. A 60-year-old woman was admitted to the hospital presenting a one-year history of dysphagia, six months of dyspnea and two months of sporadic chest pain. Radiograph of the chest revealed diffuse pulmonary hyper inflation, widening of the mediastinum, heart of normal size and shape, a right-sized aortic arch, and degenerative changes of the thoracic spine. Computed tomography of the chest showed a double aortic arch encircling and compressing the trachea and the esophagus. The right aortic arch had a larger caliber, with brachiocephalic trunk arising from it. The left common carotid artery and the left subclavian artery arose from the left aortic arch. Diagnosis: tracheoesophageal vascular ring due to double aortic arch, with dominant right arch. In this case, we chose to follow the patient medically, taking into consideration the mildness of the symptoms.

  16. External carotid compression: a novel technique to improve cerebral perfusion during selective antegrade cerebral perfusion for aortic arch surgery.

    Science.gov (United States)

    Grocott, Hilary P; Ambrose, Emma; Moon, Mike

    2016-10-01

    Selective antegrade cerebral perfusion (SACP) involving cannulation of either the axillary or innominate artery is a commonly used technique for maintaining cerebral blood flow (CBF) during the use of hypothermic cardiac arrest (HCA) for operations on the aortic arch. Nevertheless, asymmetrical CBF with hypoperfusion of the left cerebral hemisphere is a common occurrence during SACP. The purpose of this report is to describe an adjunctive maneuver to improve left hemispheric CBF during SACP by applying extrinsic compression to the left carotid artery. A 77-yr-old male patient with a history of aortic valve replacement presented for emergent surgical repair of an acute type A aortic dissection of a previously known ascending aortic aneurysm. His intraoperative course included cannulation of the right axillary artery, which was used as the aortic inflow during cardiopulmonary bypass and also allowed for subsequent SACP during HCA. After the onset of HCA, the innominate artery was clamped at its origin to allow for SACP. Shortly thereafter, however, the left-sided cerebral oxygen saturation (SrO2) began to decrease. Augmenting the PaO2, PaCO2 and both SACP pressure and flow failed to increase left hemispheric SrO2. Following the use of ultrasound guidance to confirm the absence of atherosclerotic disease in the carotid artery, external pressure was applied partially compressing the artery. With the carotid compression, the left cerebral saturation abruptly increased, suggesting pressurization of the left cerebral hemispheric circulation and augmentation of CBF. Direct ultrasound visualization and cautious partial compression of the left carotid artery may address asymmetrical CBF that occurs with SACP during HCA for aortic arch surgery. This strategy may lead to improved symmetry of CBF and corresponding cerebral oximetry measurements during aortic arch surgery.

  17. [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.

  18. Blood conservation strategies reduce the need for transfusions in ascending and aortic arch surgery.

    Science.gov (United States)

    Chu, M W A; Losenno, K L; Moore, K; Berta, D; Hewitt, J; Ralley, F

    2013-07-01

    Ascending and aortic arch surgery is associated with higher levels of blood loss and subsequent need for allogeneic blood transfusions. We hypothesized that aggressive, comprehensive blood conservation strategies may limit the need for transfusions and, subsequently, improve postoperative outcomes. Over a five-year period, 189 patients underwent proximal aortic surgery at our institution. Fifty-one patients underwent surgery using a comprehensive blood conservation strategy (BCS), including preoperative hemoglobin optimization, antifibrinolytic therapy, intraoperative acute normovolemic hemodilution, cell salvage and meticulous surgical technique. The remaining 138 patients underwent surgery using conventional techniques (CONV). Patients in the BCS group required fewer transfusions during their hospital stay compared to the conventional group (56.9% vs. 72.5%, p=0.041). When examining elective cases, this trend widens, with 40.0% of BCS patients requiring any transfusions compared to 72.9% patients in the conventional group (p=0.001). Red cell (47.1% vs. 62.3%, p=0.06), plasma (43.1% vs. 61.6%, p=0.02) and platelets (27.5% vs. 47.8%, p=0.01) were also less frequently required in the BCS group than the conventional group, respectively. When a transfusion was required, patients in the BCS group received significantly fewer units of red blood cells (2.8 ± 7.0 units) than the conventional group (5.81 ± 9.5 units; p=0.039). Mortality was similar in both groups (BCS 7.8%, conventional 10.9%, p=0.54); however, there was significantly less morbidity in the BCS group, using a composite of any of 10 major postoperative complications (23.5% vs. 39.1%; p=0.046). Median intensive care unit (ICU) and hospital lengths of stay were 2 and 7 days in the BCS group and 2 and 8 days in the CONV group (p=0.15), respectively. The aggressive use of a comprehensive blood conservation strategy in ascending and aortic arch surgery can significantly reduce the need for blood transfusions

  19. Thoracic type Ia endoleak: direct percutaneous coil embolization of the aortic arch at the blood entry site after TEVAR and double-chimney stent-grafts

    Energy Technology Data Exchange (ETDEWEB)

    Bangard, Christopher; Franke, Mareike; Maintz, David; Chang, De-Hua [University Hospital, University of Cologne, Department of Radiology, Cologne (Germany); Pfister, Roman [University Hospital, University of Cologne, Department of Internal Medicine III, Cologne (Germany); Deppe, Antje-Christin [University Hospital, University of Cologne, Department of Cardiothoracic Surgery, Cologne (Germany); Matoussevitch, Vladimir [University Hospital, University of Cologne, Department of Vascular Surgery, Cologne (Germany)

    2014-06-15

    To introduce a novel percutaneous technique to stop blood entry at the lesser aortic arch curvature by coil embolisation in type Ia endoleak after TEVAR. A 61-year-old Marfan patient presented with type Ia endoleak of the aortic arch and a growing aortic arch pseudoaneurysm after TEVAR. Multiple preceding operations and interventions made an endovascular approach unsuccessful. Direct percutaneous puncture of the aneurysmal sac would have cured the sign, but not the cause of blood entry at the lesser curvature of the aortic arch. Direct CT-guided percutaneous puncture of the blood entry site in the aortic arch with fluoroscopically guided coil embolisation using detachable extra-long coils was successfully performed. Three weeks after the intervention, the patient developed fever because of superinfection of the pseudoaneurysm. The blood cultures and CT-guided mediastinal aspirate were sterile. After intravenous administration of antibiotics, the fever disappeared and the patient recovered. Six-month follow-up showed permanent closure of the endoleak and a shrinking aneurysmal sac. Direct percutaneous puncture of the aortic arch at the blood entry site of a thoracic type Ia endoleak after TEVAR and double-chimney stent-grafts with coil embolisation of the wedge-shaped space between the lesser aortic curvature and the stent-graft is possible. (orig.)

  20. Thoracic type Ia endoleak: direct percutaneous coil embolization of the aortic arch at the blood entry site after TEVAR and double-chimney stent-grafts

    International Nuclear Information System (INIS)

    Bangard, Christopher; Franke, Mareike; Maintz, David; Chang, De-Hua; Pfister, Roman; Deppe, Antje-Christin; Matoussevitch, Vladimir

    2014-01-01

    To introduce a novel percutaneous technique to stop blood entry at the lesser aortic arch curvature by coil embolisation in type Ia endoleak after TEVAR. A 61-year-old Marfan patient presented with type Ia endoleak of the aortic arch and a growing aortic arch pseudoaneurysm after TEVAR. Multiple preceding operations and interventions made an endovascular approach unsuccessful. Direct percutaneous puncture of the aneurysmal sac would have cured the sign, but not the cause of blood entry at the lesser curvature of the aortic arch. Direct CT-guided percutaneous puncture of the blood entry site in the aortic arch with fluoroscopically guided coil embolisation using detachable extra-long coils was successfully performed. Three weeks after the intervention, the patient developed fever because of superinfection of the pseudoaneurysm. The blood cultures and CT-guided mediastinal aspirate were sterile. After intravenous administration of antibiotics, the fever disappeared and the patient recovered. Six-month follow-up showed permanent closure of the endoleak and a shrinking aneurysmal sac. Direct percutaneous puncture of the aortic arch at the blood entry site of a thoracic type Ia endoleak after TEVAR and double-chimney stent-grafts with coil embolisation of the wedge-shaped space between the lesser aortic curvature and the stent-graft is possible. (orig.)

  1. Presentation of an uncommon form of aortic dissection and rupture in Marifoan syndrome; Presentacion de una forma infecuente de diseccion y rotura aortica en el sindrome de Marfan

    Energy Technology Data Exchange (ETDEWEB)

    Delgado, I; Ruiz, R; Villanueva, J M; Fernandez Cueto, J L [Servicio de Radiodiagnostico, Complejo Hospitalario, Ciudad Real (Spain)

    1995-11-01

    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.

  2. Aortic microcalcification is associated with elastin fragmentation in Marfan syndrome

    NARCIS (Netherlands)

    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

    2017-01-01

    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 ( <5 cm) may unexpectedly experience an aortic dissection or rupture, despite yearly monitoring. Hence,

  3. Histological aspects of arterial abnormalities produced by X-irradiation of the aortic and branchial arches in the chick embryo

    International Nuclear Information System (INIS)

    Fischer, J.-L.

    1976-01-01

    Low dose X irradiations centred on one or two aortic and branchial arches of 3-day chick embryos result in teratogenic effects on the arteries (21-92%) and in low mortality (4-12%). Histological studies of abnormal arteries show different structural malformations of the arterial wall: inorganized arterial fibres, abnormal intima, newly formed arterial tissue in arterial lumen, coalescence of two arteries. It is suggested that a disturbance at the aortic differentiation level results under experimental conditions in the observed arterial abnormalities

  4. Outflow tract septation and the aortic arch system in reptiles: lessons for understanding the mammalian heart

    Directory of Open Access Journals (Sweden)

    Robert E. Poelmann

    2017-05-01

    Full Text Available Abstract Background Cardiac outflow tract patterning and cell contribution are studied using an evo-devo approach to reveal insight into the development of aorto-pulmonary septation. Results We studied embryonic stages of reptile hearts (lizard, turtle and crocodile and compared these to avian and mammalian development. Immunohistochemistry allowed us to indicate where the essential cell components in the outflow tract and aortic sac were deployed, more specifically endocardial, neural crest and second heart field cells. The neural crest-derived aorto-pulmonary septum separates the pulmonary trunk from both aortae in reptiles, presenting with a left visceral and a right systemic aorta arising from the unseptated ventricle. Second heart field-derived cells function as flow dividers between both aortae and between the two pulmonary arteries. In birds, the left visceral aorta disappears early in development, while the right systemic aorta persists. This leads to a fusion of the aorto-pulmonary septum and the aortic flow divider (second heart field population forming an avian aorto-pulmonary septal complex. In mammals, there is also a second heart field-derived aortic flow divider, albeit at a more distal site, while the aorto-pulmonary septum separates the aortic trunk from the pulmonary trunk. As in birds there is fusion with second heart field-derived cells albeit from the pulmonary flow divider as the right 6th pharyngeal arch artery disappears, resulting in a mammalian aorto-pulmonary septal complex. In crocodiles, birds and mammals, the main septal and parietal endocardial cushions receive neural crest cells that are functional in fusion and myocardialization of the outflow tract septum. Longer-lasting septation in crocodiles demonstrates a heterochrony in development. In other reptiles with no indication of incursion of neural crest cells, there is either no myocardialized outflow tract septum (lizard or it is vestigial (turtle. Crocodiles

  5. Outflow tract septation and the aortic arch system in reptiles: lessons for understanding the mammalian heart.

    Science.gov (United States)

    Poelmann, Robert E; Gittenberger-de Groot, Adriana C; Biermans, Marcel W M; Dolfing, Anne I; Jagessar, Armand; van Hattum, Sam; Hoogenboom, Amanda; Wisse, Lambertus J; Vicente-Steijn, Rebecca; de Bakker, Merijn A G; Vonk, Freek J; Hirasawa, Tatsuya; Kuratani, Shigeru; Richardson, Michael K

    2017-01-01

    Cardiac outflow tract patterning and cell contribution are studied using an evo-devo approach to reveal insight into the development of aorto-pulmonary septation. We studied embryonic stages of reptile hearts (lizard, turtle and crocodile) and compared these to avian and mammalian development. Immunohistochemistry allowed us to indicate where the essential cell components in the outflow tract and aortic sac were deployed, more specifically endocardial, neural crest and second heart field cells. The neural crest-derived aorto-pulmonary septum separates the pulmonary trunk from both aortae in reptiles, presenting with a left visceral and a right systemic aorta arising from the unseptated ventricle. Second heart field-derived cells function as flow dividers between both aortae and between the two pulmonary arteries. In birds, the left visceral aorta disappears early in development, while the right systemic aorta persists. This leads to a fusion of the aorto-pulmonary septum and the aortic flow divider (second heart field population) forming an avian aorto-pulmonary septal complex. In mammals, there is also a second heart field-derived aortic flow divider, albeit at a more distal site, while the aorto-pulmonary septum separates the aortic trunk from the pulmonary trunk. As in birds there is fusion with second heart field-derived cells albeit from the pulmonary flow divider as the right 6th pharyngeal arch artery disappears, resulting in a mammalian aorto-pulmonary septal complex. In crocodiles, birds and mammals, the main septal and parietal endocardial cushions receive neural crest cells that are functional in fusion and myocardialization of the outflow tract septum. Longer-lasting septation in crocodiles demonstrates a heterochrony in development. In other reptiles with no indication of incursion of neural crest cells, there is either no myocardialized outflow tract septum (lizard) or it is vestigial (turtle). Crocodiles are unique in bearing a central shunt, the

  6. [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.

  7. [Mycotic aneurism in aortic arch by Aspergillus fumigatus: contribution of a case and review of the literature].

    Science.gov (United States)

    Burón Fernández, M R; Oruezábal Moreno, M J

    2005-09-01

    The micotic aneurisms by Aspergillus are rare and usually appear in the context of an invasive pulmonary aspergilosis, or by septicum embolism or direct extension from the lungs, for that reason the location the more frequents is in aortic arch and the ascending aorta.8 cases of micotic aneurisms by Aspergillus spp. have been described in literature between 1966 and 2000, being the most frequent location the ascending aorta or the aortic arch. The Aspergillus fumigatus is the isolated species with more frequency, affecting mainly to patients undergoing inmunosupression. The diagnosis of a micotic aneurism requires a high clinical suspicion, given to its peculiarity and the presence of inespecific symptoms, being frequently an accidental finding in an invasive pulmonary aspergilosis.The case of a patient with a micotic aneurism by A. fumigatus appears and we reviewed the similar cases previously disclosed.

  8. Correlation of atherosclerotic changes in peripheral arteries with pathological involvement of aortic arch in coronary bypass patients

    Directory of Open Access Journals (Sweden)

    Eshraghi N

    2010-10-01

    Full Text Available "nBackground: A correlation between coronary artery disease (CAD and atherosclerosis of peripheral arteries and the determination of noninvasive indexes for its existence and extent have been sought by many researchers. Some studies report that the intima-media thickness (IMT of peripheral arteries could play this role. This study evaluated the correlation between the IMTs of common carotid and common femoral arteries and the degree of atherosclerosis in aortic arch and to evaluate the severity of CAD in candidates of coronary artery bypass grafting (CABG."n "nMethods: In a cross-sectional analytic-descriptive study, The severity of CAD, the grade of atherosclerosis of the aortic arch, and the IMTs of the common carotid and common femoral arteries were determined."n "nResults: There was a significant weak positive correlation between the IMT of common carotid artery (ρ = 0.193, p = 0.039 and common femoral artery (ρ = 0.206, p = 0.028 with the number of involved carotid vessels; the mean of these two parameters was not significantly different between the three CAD groups. There was not any significant relation between the IMTs of common carotid and common femoral arteries with the severity of atherosclerosis in the aortic arch too. There was not any significant relation between the presences of atherosclerotic plaque in the common carotid or the common femoral arteries with the severity of CAD. The severe atherosclerosis of the aortic arch was significantly higher in patients with three vessel disease."n "nConclusion: According to our results, the IMTs of common carotid and/or common femoral arteries may increase with the severity of CAD; however, these parameters are not a surrogate for predicting the CAD severity.

  9. Aberrant right vertebral artery originating from the aortic arch distal to the left subclavian artery: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Baek, Soo Heui; Baek, Hye Jin [Dept. of Radiology, Haeundae Paik Hospital, Inje University College of Medicine, Busan (Korea, Republic of)

    2014-03-15

    We present a rare case of an aberrant right vertebral artery originated from the distal aortic arch. This issue has been incidentally detected on a preoperative CT angiography after a stabbing injury of the cervical spinal cord. Normally, the right vertebral artery originates from the right subclavian artery. Therefore, in this case report we will review the incidence and the embryological mechanism of this aberrant course of the right vertebral artery and we will discuss as well the clinical importance of this variation.

  10. Whole body perfusion for hybrid aortic arch repair: evolution of selective regional perfusion with a modified extracorporeal circuit.

    Science.gov (United States)

    Fernandes, Philip; Walsh, Graham; Walsh, Stephanie; O'Neil, Michael; Gelinas, Jill; Chu, Michael W A

    2017-04-01

    Patients undergoing hybrid aortic arch reconstruction require careful protection of vital organs. We believe that whole body perfusion with tailored dual circuitry may help to achieve optimal patient outcomes. Our circuit has evolved from a secondary circuit utilizing a cardioplegia delivery device for lower body perfusion to a dual-oxygenator circuit. This allows individually controlled regional perfusion with ease of switching from secondary to primary circuit for total body flow. The re-design allows for separate flow and temperature regulation with two oxygenators in parallel. All patients underwent a single-stage operation for simultaneous treatment of arch and descending aortic pathology via a sternotomy, using a hybrid frozen elephant trunk technique. We report six consecutive patients undergoing hybrid arch and frozen elephant trunk reconstruction using a dual-oxygenator circuit. Five patients underwent elective surgery and one was emergent. One patient had an acute dissection while three underwent concomitant procedures, including a Ross procedure and two valve-sparing root reconstructions. Three cases were redo sternotomies. The mean pump time was 358 ± 131 min, the aortic cross clamp time 243 ± 135 min, the cardioplegia volume of 33,208 ml ± 16,173, cerebral ischemia 0 min, lower body ischemia 76 ± 34 min and the average lower body perfusion time was 142 min. Two patients did not require any donor blood products. The median intensive care unit (ICU) and hospital lengths of stay (LOS) were two days and 10 days, respectively. The average peak serum lactate on CPB was 7.47 mmol/L and, at admission to the ICU, it was 3.37 mmol/L. Renal and respiratory failure developed in the salvage acute type A dissection patient. No other complications occurred in this series. Whole body perfusion as delivered through individually controlled dual-oxygenator circuitry allows maximum flexibility for hybrid aortic arch reconstruction. A modified circuit perfusion

  11. 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.

  12. Factors influencing prognosis in patients with marfan syndrome after aortic surgery.

    Science.gov (United States)

    Gao, Linggen; Zhou, Xianliang; Zhang, Lin; Wen, Dan; Chang, Qian; Wu, Yongbo; Sun, Lizhong; Hui, Rutai

    2011-08-01

    Aortic aneurysm formation leading eventually to aortic rupture or dissection in early adult life is a fatal outcome of Marfan syndrome (MFS). Advances in the treatment of the syndrome have improved prognosis, but the long-term reoperation rate is still high. It remains unknown which factors influence the long-term prognosis, including the reoperation and mortality rates, in surgically treated Chinese patients with MFS. The authors studied 125 such patients to investigate factors influencing prognosis after aortic surgery. A retrospective clinical investigation. An academic medical center. One hundred twenty-five Marfan patients who had undergone aortic surgery. None. The indications for aortic surgery were aortic aneurysm and/or dissection in the 125 Marfan patients. The most commonly performed procedure was the Bentall in 92 patients. Sixteen patients underwent total arch replacement combined with stented elephant trunk implantation. Ten patients underwent the David procedure. Overall in-hospital and 30-day mortality rate was 1.6%. The survival rate was 97.5%, 91.4%, and 74.2% at 1, 5, and 10 years after surgery, respectively. The reoperation rate was 2.5%, 12.9%, and 32.9% at 1, 5, and 10 years after surgery, respectively. Multivariate analysis revealed that increased systolic blood pressure (Sys BP) was the predictor of death (p < 0.05), and body mass index and smoking were significant predictors of reoperation (p < 0.05). The present findings report the factors influencing the prognosis of Chinese patients with MFS after aortic surgical procedures. Managing these risk factors may enable health care professionals to improve the prognosis of MFS patients after aortic surgical procedures. Copyright © 2011 Elsevier Inc. All rights reserved.

  13. 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.

  14. Automated measurement of uptake in cerebellum, liver, and aortic arch in full-body FDG PET/CT scans.

    Science.gov (United States)

    Bauer, Christian; Sun, Shanhui; Sun, Wenqing; Otis, Justin; Wallace, Audrey; Smith, Brian J; Sunderland, John J; Graham, Michael M; Sonka, Milan; Buatti, John M; Beichel, Reinhard R

    2012-06-01

    The purpose of this work was to develop and validate fully automated methods for uptake measurement of cerebellum, liver, and aortic arch in full-body PET/CT scans. Such measurements are of interest in the context of uptake normalization for quantitative assessment of metabolic activity and/or automated image quality control. Cerebellum, liver, and aortic arch regions were segmented with different automated approaches. Cerebella were segmented in PET volumes by means of a robust active shape model (ASM) based method. For liver segmentation, a largest possible hyperellipsoid was fitted to the liver in PET scans. The aortic arch was first segmented in CT images of a PET/CT scan by a tubular structure analysis approach, and the segmented result was then mapped to the corresponding PET scan. For each of the segmented structures, the average standardized uptake value (SUV) was calculated. To generate an independent reference standard for method validation, expert image analysts were asked to segment several cross sections of each of the three structures in 134 F-18 fluorodeoxyglucose (FDG) PET/CT scans. For each case, the true average SUV was estimated by utilizing statistical models and served as the independent reference standard. For automated aorta and liver SUV measurements, no statistically significant scale or shift differences were observed between automated results and the independent standard. In the case of the cerebellum, the scale and shift were not significantly different, if measured in the same cross sections that were utilized for generating the reference. In contrast, automated results were scaled 5% lower on average although not shifted, if FDG uptake was calculated from the whole segmented cerebellum volume. The estimated reduction in total SUV measurement error ranged between 54.7% and 99.2%, and the reduction was found to be statistically significant for cerebellum and aortic arch. With the proposed methods, the authors have demonstrated that

  15. Pulmonary artery stenosis caused by a large aortic arch pseudoaneurysm detected 10 years after a minor trauma

    Directory of Open Access Journals (Sweden)

    Jalal Zamani

    2016-03-01

    Full Text Available Pseudoaneurysm of aorta is a rare condition usually seen after aortic surgeries or serious accidents. Here we report a 60 years old man without any previous medical condition who presented with non-specific symptoms and underwent different investigations for more than 1 year, until the presence of a continuous murmur raised suspicion toward his cardiovascular system. In echocardiographic and computed tomography (CT angiographic studies a large pseudoaneurysm of aortic arch with compression effect on pulmonary artery was detected. At this stage he remembered having suffered a minor trauma 10 years ago. He finally underwent operation and his aortic wall was repaired successfully with a patch. This case highlights the importance of thorough history taking and physical examination in patients irrespective of symptoms and high index of suspicion to detect this life-threatening condition.

  16. The Infant with Aortic Arch Hypoplasia and Small Left Heart Structures: Echocardiographic Indices of Mitral and Aortic Hypoplasia Predicting Successful Biventricular Repair.

    Science.gov (United States)

    Plymale, Jennifer M; Frommelt, Peter C; Nugent, Melodee; Simpson, Pippa; Tweddell, James S; Shillingford, Amanda J

    2017-08-01

    In infants with aortic arch hypoplasia and small left-sided cardiac structures, successful biventricular repair is dependent on the adequacy of the left-sided structures. Defining accurate thresholds of echocardiographic indices predictive of successful biventricular repair is paramount to achieving optimal outcomes. We sought to identify pre-operative echocardiographic indices of left heart size that predict intervention-free survival in infants with small left heart structures undergoing primary aortic arch repair to establish biventricular circulation (BVC). Infants ≤2 months undergoing aortic arch repair from 1999 to 2010 with aortic and/or mitral valve hypoplasia, (Z-score ≤-2) were included. Pre-operative and follow-up echocardiograms were reviewed. Primary outcome was successful biventricular circulation (BVC), defined as freedom from death, transplant, or single ventricular conversion at 1 year. Need for catheter based or surgical re-intervention (RI), valve annular growth, and significant late aortic or mitral valve obstruction were additional outcomes. Fifty one of 73 subjects (79%) had successful BVC and were free of RI at 1 year. Seven subjects failed BVC; four of those died. The overall 1 year survival for the cohort was 95%. Fifteen subjects underwent a RI but maintained BVC. In univariate analysis, larger transverse aorta (p = 0.006) and aortic valve (p = 0.02) predicted successful BVC without RI. In CART analysis, the combination of mitral valve (MV) to tricuspid valve (TV) ratio ≤0.66 with an aortic valve (AV) annulus Z-score ≤-3 had the greatest power to predict BVC failure (sensitivity 71%, specificity 94%). In those with successful BVC, the combination of both AV and MV Z-score ≤-2.5 increased the odds of RI (OR 3.8; CI 1.3-11.4). Follow-up of non-RI subjects revealed improvement in AV and MV Z-score (median AV annulus changed over time from -2.34 to 0.04 (p indices. In this complex population, 1 year survival is high, but

  17. 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.

  18. INTERRUPTION OF THE AORTIC-ARCH, DISTAL AORTOPULMONARY WINDOW, ARTERIAL DUCT AND AORTIC ORIGIN OF THE RIGHT PULMONARY-ARTERY IN A NEONATE - REPORT OF A CASE SUCCESSFULLY REPAIRED IN A ONE-STAGE OPERATION

    NARCIS (Netherlands)

    BOONSTRA, PW; EBELS, T

    The case of a 4-day-old male neonate with the rare combination of interruption of the aortic arch beyond the left subclavian artery, distal aortopulmonary window, persistent arterial duct and aortic origin of the right pulmonary artery is presented. Review of the literature reveals this patient to

  19. Total aortic arch replacement with the frozen elephant trunk technique: 10-year follow-up single-centre experience.

    Science.gov (United States)

    Ius, Fabio; Fleissner, Felix; Pichlmaier, Maximilian; Karck, Matthias; Martens, Andreas; Haverich, Axel; Shrestha, Malakh

    2013-11-01

    Since August 2001, the frozen elephant trunk (FET) technique has been used at our institution to treat degenerative or dissecting aneurysms involving the aortic arch and descending aorta as a potential 'single-stage' procedure. The aim of this study was to review our FET experience and to present the 10-year results. Between August 2001 and January 2012, 131 patients underwent FET implant with three different prostheses: the custom-made Chavan-Haverich (n = 66), the Jotec E-vita (n = 30) and the Vascutek Thoraflex (n = 35) prostheses. Concomitant procedures included aortic valve-sparing operations (David, n = 17) and aortic root replacement (Bentall, n = 25). Patient records and the first postoperative and last available computer tomography (CT) were retrospectively reviewed. Incidence of rethoracotomy for bleeding, stroke, spinal cord injury, prolonged ventilatory support (>96 h) and acute renal failure requiring dialysis were 18, 11, 1, 41 and 16%, respectively. In-hospital mortality was 15%. The mean follow-up was 42 ± 37 (range 1-134 months). At 1, 5 and 10 years, survivals were 82 ± 3, 72 ± 5 and 58 ± 8%, respectively. Freedoms from distal aortic operation were 81 ± 4, 67 ± 5 and 43 ± 13%, respectively. Thirty-six patients underwent 40 distal aortic operations, either open surgical (n = 22, 55%) or endovascular (n = 18, 45%). Chronic aortic dissection was identified as an independent risk factor for distal aortic operation (odds ratio = 3.8; 95% confidence interval 1.5-9.3; P = 0.004). At last CT control, false-lumen thrombosis rates up to 93% were achieved around the stent graft. An FET concept adds to the armament of the surgeon in the treatment of complex and diverse aortic arch pathologies. The preoperative patient risk profile explains the postoperative morbidity and in-hospital mortality. The FET can potentially be still a 'one-stage' procedure in selected patients. However, the extension of FET to patients with extensive aortic aneurysms has led

  20. 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.

  1. Combined Repair of Ascending Aortic Pseudoaneurysm and Abdominal Aortic Aneurysm in a Patient with Marfan Syndrome

    OpenAIRE

    Kokotsakis, John N.; Lioulias, Achilleas G.; Foroulis, Christophoros N.; Skouteli, Eleni Anna T.; Milonakis, Michael K.; Bastounis, Elias A.; Boulafendis, Dimitrios G.

    2003-01-01

    Pseudoaneurysms of the ascending aorta after the original inclusion/wrap technique of the Bentall procedure present a difficult surgical management problem and are associated with substantial morbidity and mortality. Patients with Marfan syndrome frequently develop aneurysms and dissections that involve multiple aortic segments. We present the case of a Marfan patient who successfully underwent repair of a giant ascending aortic pseudoaneurysm and concomitant repair of an abdominal aortic ane...

  2. The role of aortic wall CT attenuation measurements for the diagnosis of acute aortic syndromes

    International Nuclear Information System (INIS)

    Knollmann, Friedrich D.; Lacomis, Joan M.; Ocak, Iclal; Gleason, Thomas

    2013-01-01

    Objectives: To determine if measurements of aortic wall attenuation can improve the CT diagnosis of acute aortic syndromes. Methods: CT reports from a ten year period were searched for acute aortic syndromes (AAS). Studies with both an unenhanced and a contrast enhanced (CTA) series that had resulted in the diagnosis of intramural hematoma (IMH) were reviewed. Diagnoses were confirmed by medical records. The attenuation of aortic wall abnormalities was measured. The observed attenuation threshold was validated using studies from 39 new subjects with a variety of aortic conditions. Results: The term “aortic dissection” was identified in 1206, and IMH in 124 patients’ reports. IMH was confirmed in 31 patients, 21 of whom had both unenhanced and contrast enhanced images. All 21 had pathologic CTA findings, and no CTA with IMH was normal. Attenuation of the aortic wall was greater than 45 HUs on the CTA images in all patients with IMH. When this threshold was applied to the new group, sensitivity for diagnosing AAS was 100% (19/19), and specificity 94% (16/17). Addition of unenhanced images did not improve accuracy. Conclusions: Measurements of aortic wall attenuation in CTA have a high negative predictive value for the diagnosis of acute aortic syndromes

  3. The role of aortic wall CT attenuation measurements for the diagnosis of acute aortic syndromes

    Energy Technology Data Exchange (ETDEWEB)

    Knollmann, Friedrich D., E-mail: friedrich.knollmann@ucdmc.ucdavis.edu [Department of Radiology, University of California, Davis, 4860 Y Street, Sacramento, CA 95817 (United States); Departments of Radiology and Cardiothoracic Surgery, University of Pittsburgh, 200 Lothrop Street, Pittsburgh, PA 15213 (United States); Lacomis, Joan M.; Ocak, Iclal; Gleason, Thomas [Department of Radiology, University of California, Davis, 4860 Y Street, Sacramento, CA 95817 (United States); Departments of Radiology and Cardiothoracic Surgery, University of Pittsburgh, 200 Lothrop Street, Pittsburgh, PA 15213 (United States)

    2013-12-01

    Objectives: To determine if measurements of aortic wall attenuation can improve the CT diagnosis of acute aortic syndromes. Methods: CT reports from a ten year period were searched for acute aortic syndromes (AAS). Studies with both an unenhanced and a contrast enhanced (CTA) series that had resulted in the diagnosis of intramural hematoma (IMH) were reviewed. Diagnoses were confirmed by medical records. The attenuation of aortic wall abnormalities was measured. The observed attenuation threshold was validated using studies from 39 new subjects with a variety of aortic conditions. Results: The term “aortic dissection” was identified in 1206, and IMH in 124 patients’ reports. IMH was confirmed in 31 patients, 21 of whom had both unenhanced and contrast enhanced images. All 21 had pathologic CTA findings, and no CTA with IMH was normal. Attenuation of the aortic wall was greater than 45 HUs on the CTA images in all patients with IMH. When this threshold was applied to the new group, sensitivity for diagnosing AAS was 100% (19/19), and specificity 94% (16/17). Addition of unenhanced images did not improve accuracy. Conclusions: Measurements of aortic wall attenuation in CTA have a high negative predictive value for the diagnosis of acute aortic syndromes.

  4. 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

  5. Early- and Mid-term Results of the Chimney Technique in the Repair of Aortic Arch Pathologies

    Energy Technology Data Exchange (ETDEWEB)

    Zou, Junjie; Jiao, Yuanyong, E-mail: wishlucky@163.com, E-mail: johemail@163.com; Zhang, Xiwei; Jiang, Jun; Yang, Hongyu; Ma, Hao [First Affiliated Hospital of Nanjing Medical University, Division of Vascular Surgery, Department of General Surgery (China)

    2016-11-15

    PurposeTo examine the safety, feasibility, and mid-term efficacy of the chimney technique for aortic arch pathologies.MethodsFrom February 2011 to December 2014, a total of 35 patients (30 men; mean age 54.3 ± 14.1 years) with aortic arch pathologies underwent thoracic endovascular aortic repair combined with chimney stents. The indication was a proximal landing zone <1.5 cm. Follow-up was performed at 3, 6, and 12 months and then yearly thereafter.ResultsA total of 36 chimney stents were deployed (innominate artery, n = 1; left common carotid artery, n = 9; right subclavian artery, n = 1; left subclavian artery, n = 25). The technical success rate was 94.3 % (33/35). Immediate type Ia endoleaks (ELIa) were observed in two patients (8.6 %, 2/35). Twenty-five patients were successfully followed-up for a median period of 29.3 months (range, 6–48 months). One patient died due to aortic dissection aneurysm rupture at 36 months (mortality rate of 4 %, 1/25). Three late ELIa were observed and no reinterventions were performed. The overall incidence of ELIa was 20 % (5/25). During follow-up, the patency rate for chimney stents was 92 % (23/25).ConclusionOur limited experience demonstrates that the chimney technique is a viable and relatively safe treatment for patients with challenging thoracic aortic pathologies at least in the mid-term follow-up period.

  6. Early- and Mid-term Results of the Chimney Technique in the Repair of Aortic Arch Pathologies

    International Nuclear Information System (INIS)

    Zou, Junjie; Jiao, Yuanyong; Zhang, Xiwei; Jiang, Jun; Yang, Hongyu; Ma, Hao

    2016-01-01

    PurposeTo examine the safety, feasibility, and mid-term efficacy of the chimney technique for aortic arch pathologies.MethodsFrom February 2011 to December 2014, a total of 35 patients (30 men; mean age 54.3 ± 14.1 years) with aortic arch pathologies underwent thoracic endovascular aortic repair combined with chimney stents. The indication was a proximal landing zone <1.5 cm. Follow-up was performed at 3, 6, and 12 months and then yearly thereafter.ResultsA total of 36 chimney stents were deployed (innominate artery, n = 1; left common carotid artery, n = 9; right subclavian artery, n = 1; left subclavian artery, n = 25). The technical success rate was 94.3 % (33/35). Immediate type Ia endoleaks (ELIa) were observed in two patients (8.6 %, 2/35). Twenty-five patients were successfully followed-up for a median period of 29.3 months (range, 6–48 months). One patient died due to aortic dissection aneurysm rupture at 36 months (mortality rate of 4 %, 1/25). Three late ELIa were observed and no reinterventions were performed. The overall incidence of ELIa was 20 % (5/25). During follow-up, the patency rate for chimney stents was 92 % (23/25).ConclusionOur limited experience demonstrates that the chimney technique is a viable and relatively safe treatment for patients with challenging thoracic aortic pathologies at least in the mid-term follow-up period.

  7. Periodontal Disease Associated with Aortic Arch Atheroma in Patients with Stroke or Transient Ischemic Attack.

    Science.gov (United States)

    Sen, Souvik; Chung, Matthew; Duda, Viktoriya; Giamberardino, Lauren; Hinderliter, Alan; Offenbacher, Steven

    2017-10-01

    Periodontal disease (PD) is associated with recurrent vascular event in stroke or transient ischemic attack (TIA). In this study, we investigated whether PD is independently associated with aortic arch atheroma (AA). We also explored the relationship PD has with AA plaque thickness and other characteristics associated with atheroembolic risk among patients with stroke or TIA. Finally, we confirmed the association between AA and recurrent vascular event in patients with stroke or TIA. In this prospective longitudinal hospital-based cohort study, PD was assessed in patients with stroke and TIA. Patients with confirmed stroke and TIA (n = 106) were assessed by calibrated dental examiners to determine periodontal status and were followed over a median of 24 months for recurrent vascular events (stroke, myocardial infarction, and death). The extent of AA and other plaque characteristics was assessed by transesophageal echocardiography. Within our patient cohort, 27 of the 106 participants had recurrent vascular events (including 16 with stroke or TIA) over the median of 24-month follow-up. Severe PD was associated with increased AA plaque thickness and calcification. The results suggest that PD may be a risk factor for AA. In this cohort, we confirm the association of severe AA with recurrent vascular events. In patients with stroke or TIA, severe PD is associated with increased AA plaque thickness, a risk factor for recurrent events. Further studies are needed to confirm this finding and to determine whether treatment of PD can reduce the rate of AA plaque progression and recurrent vascular events. Copyright © 2017 National Stroke Association. Published by Elsevier Inc. All rights reserved.

  8. Characterizing saccular aortic arch aneurysms from the geometry-flow dynamics relationship.

    Science.gov (United States)

    Natsume, Kayoko; Shiiya, Norihiko; Takehara, Yasuo; Sugiyama, Masataka; Satoh, Hiroshi; Yamashita, Katsushi; Washiyama, Naoki

    2017-06-01

    Low wall shear stress (WSS) has been reported to be associated with accelerated atherosclerosis, aneurysm growth, or rupture. We evaluated the geometry of aortic arch aneurysms and their relationship with WSS by using the 4-dimensional flow magnetic resonance imaging to better characterize the saccular aneurysms. We analyzed the geometry in 100 patients using multiplanar reconstruction of computed tomography. We evaluated WSS and vortex flow using 4-dimensional flow magnetic resonance imaging in 16 of them, which were compared with 8 age-matched control subjects and eight healthy young volunteers. Eighty-two patients had a saccular aneurysm, and 18 had a fusiform aneurysm. External diameter/aneurysm length ratio and sac depth/neck width ratio of the fusiform aneurysms were constant at 0.76 ± 0.18 and 0.23 ± 0.09, whereas those of saccular aneurysms, especially those involving the outer curvature, were higher and more variable. Vortex flow was always present in the aneurysms, resulting in low WSS. When the sac depth/neck width ratio was less than 0.8, peak WSS correlated inversely with luminal diameter even in the saccular aneurysms. When this ratio exceeded 0.8, which was the case only with the saccular aneurysms, such correlation no longer existed and WSS was invariably low. Fusiform aneurysms elongate as they dilate, and WSS is lower as the diameter is larger. Saccular aneurysms dilate without proportionate elongation, and they, especially those occupying the inner curvature, have higher and variable sac depth/neck width ratio. When this ratio exceeds 0.8, WSS is low regardless of diameter, which may explain their malignant clinical behavior. Copyright © 2016 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.

  9. 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.

  10. 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.

  11. 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.

  12. Fabrication of a compliant phantom of the human aortic arch for use in Particle Image Velocimetry (PIV experimentation

    Directory of Open Access Journals (Sweden)

    Hütter Larissa

    2016-09-01

    Full Text Available Compliant phantoms of the human aortic arch can mimic patient specific cardiovascular dysfunctions in vitro. Hence, phantoms may enable elucidation of haemodynamic disturbances caused by aortic dysfunction. This paper describes the fabrication of a thin-walled silicone phantom of the human ascending aorta and brachiocephalic artery. The model geometry was determined via a meta-analysis and modelled in SolidWorks before 3D printing. The solid model surface was smoothed and scanned with a 3D scanner. An offset outer mould was milled from Ebalta S-Model board. The final phantom indicated that ABS was a suitable material for the internal model, the Ebalta S-Model board yielded a rough external surface. Co-location of the moulds during silicone pour was insufficient to enable consistent wall thickness. The resulting phantom was free of air bubbles but did not have the desired wall thickness consistency.

  13. Dynamics of the aortic arch submitted to a shock loading: Parametric study with fluid-structure models.

    Science.gov (United States)

    El Baroudi, A; Razafimahery, F; Rakotomanana, L

    2012-01-01

    This work aims to present some fluid-structure models for analyzing the dynamics of the aorta during a brusque loading. Indeed, various lesions may appear at the aortic arch during car crash or other accident such as brusque falling. Aortic stresses evolution are simulated during the shock at the cross section and along the aorta. One hot question was that if a brusque deceleration can generate tissue tearing, or a shock is necessary to provoke such a damage. Different constitutive laws of blood are then tested whereas the aorta is assumed linear and elastic. The overall shock model is inspired from an experimental jig. We show that the viscosity has strong influence on the stress and parietal moments and forces. The nonlinear viscosity has no significant additional effects for healthy aorta, but modifies the stress and parietal loadings for the stenotic aorta.

  14. 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.

  15. 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.

  16. 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.

  17. 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.

  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. Utility of fetal cardiac magnetic resonance imaging to assess fetuses with right aortic arch and right ductus arteriosus.

    Science.gov (United States)

    Dong, Su-Zhen; Zhu, Ming

    2018-06-01

    To evaluate the utility of fetal cardiac magnetic resonance imaging (MRI) to diagnose right aortic arch (RAA) with right ductus arteriosus. This retrospective study included six fetuses with right aortic arch and right ductus arteriosus. The six fetal cases were examined using a 1.5-T magnetic resonance unit. The steady-state free precession (SSFP) and single-shot turbo spin echo (SSTSE) sequences were used to evaluate the fetal heart and airway. The gestational age of the six fetuses ranged from 22 to 35 weeks (mean, 26.5 weeks). The age of the pregnant women ranged from 23 to 40 years (mean 31 years). Fetal cardiac MRI diagnosed the six fetal cases with RAA with right ductus arteriosus correctly. Among the six fetuses, four were associated with other congenital heart defects. In three of six cases, the diagnoses established using prenatal echocardiography (echo) was correct when compared with postnatal diagnosis. Fetal cardiac MRI is a useful complementary tool to assess fetuses with RAA and right ductus arteriosus.

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

    OpenAIRE

    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 ...

  1. Acute aortic syndromes: definition, prognosis and treatment options.

    Science.gov (United States)

    Carpenter, S W; Kodolitsch, Y V; Debus, E S; Wipper, S; Tsilimparis, N; Larena-Avellaneda, A; Diener, H; Kölbel, T

    2014-04-01

    Acute aortic syndromes (AAS) are life-threatening vascular conditions of the thoracic aorta presenting with acute pain as the leading symptom in most cases. The incidence is approximately 3-5/100,000 in western countries with increase during the past decades. Clinical suspicion for AAS requires immediate confirmation with advanced imaging modalities. Initial management of AAS addresses avoidance of progression by immediate medical therapy to reduce aortic shear stress. Proximal symptomatic lesions with involvement of the ascending aorta are surgically treated in the acute setting, whereas acute uncomplicated distal dissection should be treated by medical therapy in the acute period, followed by surveillance and repeated imaging studies. Acute complicated distal dissection requires urgent invasive treatment and thoracic endovascular aortic repair has become the treatment modality of choice because of favorable outcomes compared to open surgical repair. Intramural hematoma, penetrating aortic ulcers, and traumatic aortic injuries of the descending aorta harbor specific challenges compared to aortic dissection and treatment strategies are not as uniformly defined as in aortic dissection. Moreover these lesions have a different prognosis. Once the acute period of aortic syndrome has been survived, a lifelong medical treatment and close surveillance with repeated imaging studies is essential to detect impending complications which might need invasive treatment within the short-, mid- or long-term.

  2. 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.

  3. Combined Repair of Ascending Aortic Pseudoaneurysm and Abdominal Aortic Aneurysm in a Patient with Marfan Syndrome

    Science.gov (United States)

    Kokotsakis, John N.; Lioulias, Achilleas G.; Foroulis, Christophoros N.; Skouteli, Eleni Anna T.; Milonakis, Michael K.; Bastounis, Elias A.; Boulafendis, Dimitrios G.

    2003-01-01

    Pseudoaneurysms of the ascending aorta after the original inclusion/wrap technique of the Bentall procedure present a difficult surgical management problem and are associated with substantial morbidity and mortality. Patients with Marfan syndrome frequently develop aneurysms and dissections that involve multiple aortic segments. We present the case of a Marfan patient who successfully underwent repair of a giant ascending aortic pseudoaneurysm and concomitant repair of an abdominal aortic aneurysm. An aggressive surgical strategy followed by life-long cardiovascular monitoring is warranted in order to prolong the survival of these patients. (Tex Heart Inst J 2003;30:233–5) PMID:12959210

  4. 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

  5. A systematic review and meta-analysis of variations in branching patterns of the adult aortic arch.

    Science.gov (United States)

    Popieluszko, Patrick; Henry, Brandon Michael; Sanna, Beatrice; Hsieh, Wan Chin; Saganiak, Karolina; Pękala, Przemysław A; Walocha, Jerzy A; Tomaszewski, Krzysztof A

    2018-07-01

    The aortic arch (AA) is the main conduit of the left side of the heart, providing a blood supply to the head, neck, and upper limbs. As it travels through the thorax, the pattern in which it gives off the branches to supply these structures can vary. Variations of these branching patterns have been studied; however, a study providing a comprehensive incidence of these variations has not yet been conducted. The objective of this study was to perform a meta-analysis of all the studies that report prevalence data on AA variants and to provide incidence data on the most common variants. A systematic search of online databases including PubMed, Embase, Scopus, ScienceDirect, Web of Science, SciELO, BIOSIS, and CNKI was performed for literature describing incidence of AA variations in adults. Studies including prevalence data on adult patients or cadavers were collected and their data analyzed. A total of 51 articles were included (N = 23,882 arches). Seven of the most common variants were analyzed. The most common variants found included the classic branching pattern, defined as a brachiocephalic trunk, a left common carotid, and a left subclavian artery (80.9%); the bovine arch variant (13.6%); and the left vertebral artery variant (2.8%). Compared by geographic data, bovine arch variants were noted to have a prevalence as high as 26.8% in African populations. Although patients who have an AA variant are often asymptomatic, they compose a significant portion of the population of patients and pose a greater risk of hemorrhage and ischemia during surgery in the thorax. Because of the possibility of encountering such variants, it is prudent for surgeons to consider potential variations in planning procedures, especially of an endovascular nature, in the thorax. Copyright © 2017 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.

  6. Discrete subvalvular aortic stenosis in the Beckwith-Wiedemann syndrome.

    Science.gov (United States)

    Shirani, J; Natarajan, K; Varga, P; Vitullo, D A

    1993-07-01

    Various congenital cardiac malformations have been described in patients with Beckwith-Wiedemann (BW) syndrome, including reversible obstructive subaortic stenosis in one patient. We herein present a case of a 2.5-year-old black boy with BW syndrome and discrete subvalvular aortic stenosis of the membraneous type. Such association of these two entities has previously not been documented.

  7. Autologous platelet-rich plasma reduces transfusions during ascending aortic arch repair: a prospective, randomized, controlled trial.

    Science.gov (United States)

    Zhou, Shao Feng; Estrera, Anthony L; Loubser, Paul; Ignacio, Craig; Panthayi, Sreelatha; Miller, Charles; Sheinbaum, Roy; Safi, Hazim J

    2015-04-01

    Blood conservation using autologous platelet-rich plasma (aPRP), a technique of whole blood harvest that separates red blood cells from plasma and platelets before cardiopulmonary bypass with retransfusion of the preserved platelets after completion of cardiopulmonary bypass, has not been studied extensively. We sought to prospectively determine whether aPRP reduces blood transfusions during ascending and transverse aortic arch repair. We randomly assigned 80 patients undergoing elective ascending and transverse aortic arch repair using deep hypothermic circulatory arrest to receive either aPRP (n = 38) or no aPRP (n = 42). Volume of aPRP retransfused was 726 ± 124 mL. The primary end point was transfusion amount. Secondary end points were death, stroke, renal failure, pulmonary failure, and transfusion costs. Perioperative transfusion rate was defined as blood transfusions given during surgery and up to 72 hours afterward. The surgeon and intensivist were blinded to the treatment arm. Because an anesthesiologist initiated the protocol, the surgeon was not aware of aPRP collection, as this occurred only after the sterile drape was in place. In addition, because cell salvage was performed on all cases, differentiation in perfusionist activities (during spinning of aPRP) was not evident. Platelet, fresh frozen plasma, and cryoprecipitate intraoperative transfusions were performed only after heparin was reversed and the patient was judged as coagulopathic on the basis of associated criteria: cryoprecipitate transfusion for fibrinogen level less than 150 μg/dL, platelet transfusion for platelet count less than 80,000, and fresh frozen plasma when thromboelastogram test was suggestive or a partial thromboplastin time was greater than 55 seconds, and prothrombin time was greater than 1.6 seconds. Early mortality, stroke, and respiratory complications were similar between groups. Only acute renal failure was reduced in the aPRP group, 7% versus 0% (p platelets by 56

  8. A Meta-Analysis of Total Arch Replacement With Frozen Elephant Trunk in Acute Type A Aortic Dissection.

    Science.gov (United States)

    Takagi, Hisato; Umemoto, Takuya

    2016-01-01

    To assess the safety and efficacy, we performed a meta-analysis of total arch replacement with frozen elephant trunk in exclusive acute type A (neither chronic nor type B) aortic dissection. Databases including MEDLINE and EMBASE were searched through March 2015 using Web-based search engines (PubMed and OVID). Eligible studies were case series of frozen elephant trunk enrolling patients with acute type A (neither chronic nor type B) aortic dissection reporting at least early (in-hospital or 30-day) all-cause mortality. Study-specific estimates were combined in both fixed- and random-effect models. Fifteen studies enrolling 1279 patients were identified and included. Pooled analyses demonstrated the cardiopulmonary bypass time of 207.1 (95% confidence interval [CI], 186.1-228.1) minutes, aortic cross-clamp time of 123.3 (95% CI, 113.1-133.5) minutes, selective antegrade cerebral perfusion time of 49.3 (95% CI, 37.6-61.0) minutes, hypothermic circulatory arrest time of 39.0 (95% CI, 30.7-47.2) minutes, early mortality of 9.2% (95% CI, 7.7-11.0%), stroke of 4.8% (95% CI, 2.5-9.0%), spinal cord injury of 3.5% (95% CI, 1.9-6.6%), mid- to long-term (≥1-year) overall mortality of 13.0% (95% CI, 10.4-16.0%), reintervention of 9.6% (95% CI, 5.6-15.8%), and false lumen thrombosis of 96.8% (95% CI, 90.7-98.9%). Total arch replacement with frozen elephant trunk provides a safe alternative to that with conventional elephant trunk in patients with acute type A aortic dissection, with acceptable early mortality and morbidity. The rates of mid- to long-term reintervention and false lumen non-thrombosis may be lower in patients undergoing the frozen than conventional elephant trunk procedure. © The Author(s) 2016.

  9. [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.

  10. Silent Aortic Dissection Presenting as Transient Locked-In Syndrome

    Science.gov (United States)

    Nadour, Wadih; Goldwasser, Brian; Biederman, Robert W.; Taffe, Kevin

    2008-01-01

    Acute aortic dissection is a medical emergency. Without prompt recognition and treatment, the mortality rate is high. An atypical presentation makes timely diagnosis difficult, especially if the patient is experiencing no characteristic pain. Many patients with aortic dissection are reported to have presented with various neurologic manifestations, but none with only a presentation of transient locked-in syndrome. Herein, we report a case of completely painless aortic dissection in a woman who presented with a transient episode of anarthria, quadriplegia, and preserved consciousness. On physical examination, she had a 40-point difference in blood pressure between her left and right arms, and a loud diastolic murmur. The diagnosis of acute aortic dissection was reached via a combination of radiography, computed tomography, echocardiography, and a high index of clinical suspicion. The patient underwent emergency surgery and ultimately experienced a successful outcome. To our knowledge, this is the 1st report of aortic dissection that presented solely as locked-in syndrome. We suggest that silent aortic dissection be added to the differential diagnosis for transient locked-in syndrome. PMID:18941610

  11. A Case of an Upper Gastrointestinal Bleeding Due to a Ruptured Dissection of a Right Aortic Arch

    International Nuclear Information System (INIS)

    Born, Christine; Forster, Andreas; Rock, Clemens; Pfeifer, Klaus-Juergen; Rieger, Johannes; Reiser, Maximilian

    2003-01-01

    We report a case of severe upper gastrointestinal hemorrhage with a rare underlying cause. The patient was unconscious when he was admitted to the hospital. No chest radiogram was performed. Routine diagnostic measures, including endoscopy, failed to reveal the origin of the bleeding, which was believed to originate from the esophagus secondary to a peptic ulcer or varices. Exploratory laparotomy added no further information, but contrast-enhanced multislice computed tomography (MSCT) of the chest showed dextroposition of the widened aortic arch with a ruptured type-B dissection and a consecutive aorto-esophageal fistula (AEF). The patient died on the day of admission. Noninvasive MSCT angiography gives rapid diagnostic information on patients with occult upper gastrointestinal bleeding and should be considered before more invasive conventional angiography or surgery

  12. Variation in Perfusion Strategies for Neonatal and Infant Aortic Arch Repair: Contemporary Practice in the STS Congenital Heart Surgery Database.

    Science.gov (United States)

    Meyer, David B; Jacobs, Jeffrey P; Hill, Kevin; Wallace, Amelia S; Bateson, Brian; Jacobs, Marshall L

    2016-09-01

    Regional cerebral perfusion (RCP) is used as an adjunct or alternative to deep hypothermic circulatory arrest (DHCA) for neonates and infants undergoing aortic arch repair. Clinical studies have not demonstrated clear superiority of either strategy, and multicenter data regarding current use of these strategies are lacking. We sought to describe the variability in contemporary practice patterns for use of these techniques. The Society of Thoracic Surgeons Congenital Heart Surgery Database (2010-2013) was queried to identify neonates and infants whose index operation involved aortic arch repair with cardiopulmonary bypass. Perfusion strategy was classified as isolated DHCA, RCP (with less than or equal to ten minutes of DHCA), or mixed (RCP with more than ten minutes of DHCA). Data were analyzed for the entire cohort and stratified by operation subgroups. Overall, 4,523 patients (105 centers) were identified; median age seven days (interquartile range: 5.0-13.0). The most prevalent perfusion strategy was RCP (43%). Deep hypothermic circulatory arrest and mixed perfusion accounted for 32% and 16% of cases, respectively. In all, 59% of operations involved some period of RCP. Regional cerebral perfusion was the most prevalent perfusion strategy for each operation subgroup. Neither age nor weight was associated with perfusion strategy, but reoperations were less likely to use RCP (31% vs 45%, P RCP and DHCA in the RCP group was longer than the DHCA time in the DHCA group (45 vs 36 minutes, P neonates and infants. In contemporary practice, RCP is the most prevalent perfusion strategy for these procedures. Use of DHCA is also common. Further investigation is warranted to ascertain possible relative merits of the various perfusion techniques. © The Author(s) 2016.

  13. Aortic Disease in the Young: Genetic Aneurysm Syndromes, Connective Tissue Disorders, and Familial Aortic Aneurysms and Dissections

    Directory of Open Access Journals (Sweden)

    Marcelo Cury

    2013-01-01

    Full Text Available There are many genetic syndromes associated with the aortic aneurysmal disease which include Marfan syndrome (MFS, Ehlers-Danlos syndrome (EDS, Loeys-Dietz syndrome (LDS, familial thoracic aortic aneurysms and dissections (TAAD, bicuspid aortic valve disease (BAV, and autosomal dominant polycystic kidney disease (ADPKD. In the absence of familial history and other clinical findings, the proportion of thoracic and abdominal aortic aneurysms and dissections resulting from a genetic predisposition is still unknown. In this study, we propose the review of the current genetic knowledge in the aortic disease, observing, in the results that the causative genes and molecular pathways involved in the pathophysiology of aortic aneurysm disease remain undiscovered and continue to be an area of intensive research.

  14. 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.

  15. The Transcranial Doppler Sonography for Optimal Monitoring and Optimization of Cerebral Perfusion in Aortic Arch Surgery: A Case Series.

    Science.gov (United States)

    Ghazy, Tamer; Darwisch, Ayham; Schmidt, Torsten; Nguyen, Phong; Elmihy, Sohaila; Fajfrova, Zuzana; Zickmüller, Claudia; Matschke, Klaus; Kappert, Utz

    2017-06-16

    To analyze the feasibility and advantages of transcranial doppler sonography (TCD) for monitoring and optimization of selective cerebral perfusion (SCP) in aortic arch surgery. From April 2013 to April 2014, nine patients with extensive aortic pathology underwent surgery under moderate hypothermic cardiac arrest with unilateral antegrade SCP under TCD monitoring in our institution. Adequate sonographic window and visualization of circle of Willis were to be confirmed. Intraoperatively, a cerebral cross-filling of the contralateral cerebral arteries on the unilateral SCP was to be confirmed with TCD. If no cross-filling was confirmed, an optimization of the SCP was performed via increasing cerebral flow and increasing PCO2. If not successful, the SCP was to be switched to bilateral perfusion. Air bubble hits were recorded at the termination of SCP. A sonographic window was confirmed in all patients. Procedural success was 100%. The mean operative time was 298 ± 89 minutes. Adequate cross-filling was confirmed in 8 patients. In 1 patient, inadequate cross-filling was detected by TCD and an optimization of cerebral flow was necessary, which was successfully confirmed by TCD. There was no conversion to bilateral perfusion. Extensive air bubble hits were confirmed in 1 patient, who suffered a postoperative stroke. The 30-day mortality rate was 0. Conclusion: The TCD is feasible for cerebral perfusion monitoring in aortic surgery. It enables a confirmation of adequacy of cerebral perfusion strategy or the need for its optimization. Documentation of calcific or air-bubble hits might add insight into patients suffering postoperative neurological deficits.

  16. Interdependencies of aortic arch secondary flow patterns, geometry, and age analysed by 4-dimensional phase contrast magnetic resonance imaging at 3 Tesla

    Energy Technology Data Exchange (ETDEWEB)

    Frydrychowicz, Alex [University Hospital Schleswig-Holstein, Clinic for Radiology and Nuclear Medicine, Luebeck (Germany); Berger, Alexander; Russe, Maximilian F.; Bock, Jelena [University Hospital Freiburg, Department of Radiology, Medical Physics, Freiburg (Germany); Munoz del Rio, Alejandro [University of Wisconsin - Madison, Departments of Radiology and Medical Physics, Madison, WI (United States); Harloff, Andreas [University Hospital Freiburg, Department of Neurology and Clinical Neurophysiology, Freiburg (Germany); Markl, Michael [University Hospital Freiburg, Department of Radiology, Medical Physics, Freiburg (Germany); Northwestern University, Departments of Radiology and Biomedical Engineering, Chicago, IL (United States)

    2012-05-15

    It was the aim to analyse the impact of age, aortic arch geometry, and size on secondary flow patterns such as helix and vortex flow derived from flow-sensitive magnetic resonance imaging (4D PC-MRI). 62 subjects (age range = 20-80 years) without circumscribed pathologies of the thoracic aorta (ascending aortic (AAo) diameter: 3.2 {+-} 0.6 cm [range 2.2-5.1]) were examined by 4D PC-MRI after IRB-approval and written informed consent. Blood flow visualisation based on streamlines and time-resolved 3D particle traces was performed. Aortic diameter, shape (gothic, crook-shaped, cubic), angle, and age were correlated with existence and extent of secondary flow patterns (helicity, vortices); statistical modelling was performed. Helical flow was the typical pattern in standard crook-shaped aortic arches. With altered shapes and increasing age, helicity was less common. AAo diameter and age had the highest correlation (r = 0.69 and 0.68, respectively) with number of detected vortices. None of the other arch geometric or demographic variables (for all, P {>=} 0.177) improved statistical modelling. Substantially different secondary flow patterns can be observed in the normal thoracic aorta. Age and the AAo diameter were the parameters correlating best with presence and amount of vortices. Findings underline the importance of age- and geometry-matched control groups for haemodynamic studies. (orig.)

  17. Interdependencies of aortic arch secondary flow patterns, geometry, and age analysed by 4-dimensional phase contrast magnetic resonance imaging at 3 Tesla

    International Nuclear Information System (INIS)

    Frydrychowicz, Alex; Berger, Alexander; Russe, Maximilian F.; Bock, Jelena; Munoz del Rio, Alejandro; Harloff, Andreas; Markl, Michael

    2012-01-01

    It was the aim to analyse the impact of age, aortic arch geometry, and size on secondary flow patterns such as helix and vortex flow derived from flow-sensitive magnetic resonance imaging (4D PC-MRI). 62 subjects (age range = 20-80 years) without circumscribed pathologies of the thoracic aorta (ascending aortic (AAo) diameter: 3.2 ± 0.6 cm [range 2.2-5.1]) were examined by 4D PC-MRI after IRB-approval and written informed consent. Blood flow visualisation based on streamlines and time-resolved 3D particle traces was performed. Aortic diameter, shape (gothic, crook-shaped, cubic), angle, and age were correlated with existence and extent of secondary flow patterns (helicity, vortices); statistical modelling was performed. Helical flow was the typical pattern in standard crook-shaped aortic arches. With altered shapes and increasing age, helicity was less common. AAo diameter and age had the highest correlation (r = 0.69 and 0.68, respectively) with number of detected vortices. None of the other arch geometric or demographic variables (for all, P ≥ 0.177) improved statistical modelling. Substantially different secondary flow patterns can be observed in the normal thoracic aorta. Age and the AAo diameter were the parameters correlating best with presence and amount of vortices. Findings underline the importance of age- and geometry-matched control groups for haemodynamic studies. (orig.)

  18. 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.

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

    Science.gov (United States)

    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. [Bacteremia associated with mycotic aneurysm of the transversal aortic arch and myocarditis caused by Salmonella enteritidis].

    Science.gov (United States)

    Martínez-Martínez, L; Mesa, E; Rodríguez, J E; Sánchez, M P; Ugarte, J; Algora Weber, A; Dámaso, D; Daza, R M; Mendaza, P

    1989-02-01

    A 60-year-old male with diabetes mellitus had Salmonella enteritidis bacteremia associated with mycotic aneurysm of the transverse aortic arc and myocarditis. Antibiotic therapy with ampicillin and chloramphenicol was ineffective despite the fact that the microorganism was sensitive in vitro to those antimicrobials, and the patient had a progressive clinical deterioration which culminated in death.

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

    Science.gov (United States)

    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.

  2. Perfect and least invasive sealing technique on the lesser curvature of the aortic arch: application of a novel stent graft to an aneurysm developing on a postoperative ductus arteriosus.

    Science.gov (United States)

    Soeda, Takeshi; Yokoi, Yoshihiko; Yuri, Koichi; Saito, Yuuhei; Setozaki, Shuji; Harada, Hisao

    2013-01-01

    A 78-year-old woman who underwent an operation for a patent ductus arteriosus (PDA) about thirty years ago developed an aneurysm on the aortic side of the remnant ductal tissue. To avoid risky, open surgery, we performed endovascular aortic therapy using a novel stent graft (SG), which was pre-curved, fenestrated and custom-made type. This graft was designed to configure to the patient's whole aortic arch anatomy, and was capable of accurately adjusting its fenestrations to the arch branch orifices during the procedure. The operation was successful, and the patient was discharged uneventfully on 16th postoperative day. The advantage of this fenestrated SG is close sealing, especially over the lesser curvature of the arch. This device could be a simple and effective option to deal with an otherwise normal aortic arch with such a ductus-related localized lesion.

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

    OpenAIRE

    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 ...

  4. Marfan's syndrome presenting with abdominal aortic aneurysm: A ...

    African Journals Online (AJOL)

    We present the case of a 16-year old student with Marfan's syndrome and abdominal aortic aneurysm who presented with a diagnostic conundrum. He presented with a three months history of progressive painful left upper abdominal mass and back pain. It became severe in the last two weeks before presentation and was ...

  5. Acute aortic dissection in pregnancy with the marfan syndrome.

    Science.gov (United States)

    Kim, Si Wook; Kim, Dohun; Hong, Jong-Myeon

    2014-06-01

    Acute aortic dissection (AAD) during pregnancy can be fatal to both the pregnant mother and the baby, particularly in patients with the Marfan syndrome. We report a case of the modified Bentall procedure in surgery for AAD in a 31-year-old pregnant woman at 24 weeks of gestation with the Marfan syndrome. The patient recovered well after the operation, but unfortunately, the fetus could not be saved.

  6. Acute Aortic Dissection in Pregnancy with the Marfan Syndrome

    OpenAIRE

    Kim, Si Wook; Kim, Dohun; Hong, Jong-Myeon

    2014-01-01

    Acute aortic dissection (AAD) during pregnancy can be fatal to both the pregnant mother and the baby, particularly in patients with the Marfan syndrome. We report a case of the modified Bentall procedure in surgery for AAD in a 31-year-old pregnant woman at 24 weeks of gestation with the Marfan syndrome. The patient recovered well after the operation, but unfortunately, the fetus could not be saved.

  7. Expanding arch aneurysm causing a "kink" in a Bentall graft and heart failure.

    Science.gov (United States)

    Al-Mohaissen, Maha A; Skarsgard, Peter; Khoo, Clarence; Virani, Sean A; Munt, Brad; Leipsic, Jonathon; Ignaszewski, Andrew

    2012-07-01

    Marfan syndrome is associated with a high incidence of aortic root aneurysm and life-threatening aortic dissection. With the successful use of surgical aortic root replacement, dissection-related mortality has been significantly reduced. We present the case of a patient with Marfan syndrome who presented with heart failure secondary to an unusual graft-related complication 14 years after a Bentall procedure. Investigations revealed a supra-aortic stenosis resulting from a kink in the Bentall graft caused by pressure from an expanding aortic arch aneurysm. The patient underwent surgery with improvement in his ejection fraction and heart failure symptoms.

  8. A case of hypoplasia of left lung with very rare associations with congenital absence of left pulmonary artery and right-sided aortic arch

    Directory of Open Access Journals (Sweden)

    Trilok Chand

    2017-01-01

    Full Text Available The absence of one of the pulmonary artery with associated hypoplasia of lung and great vessel abnormality is a rare finding. The incidence of this rare congenital abnormality is around 1 in 200,000 live birth. The absence of the left side pulmonary artery is again uncommon, and associated cardiac malformations are usually tetralogy of fallot or septal defects rather than an aortic arch defect. Our case is a unique case in It’s associated congenital anomalies. He was presented with recurrent pneumothorax and hemoptysis, and on thorough workup, he was diagnosed to have an absence of left pulmonary artery with hypoplasia of the left lung and associated right-sided aortic arch. The patient’s family has declined the surgical option, and he was managed conservatively and kept in close follow-up.

  9. 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.

  10. 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

  11. Foot medial longitudinal-arch deformation during quiet standing and gait in subjects with medial tibial stress syndrome

    DEFF Research Database (Denmark)

    Bandholm, Thomas Quaade; Boysen, Lisbeth; Haugaard, Stine

    2008-01-01

    The objective of this study was to investigate (1) if subjects with medial tibial stress syndrome demonstrate increased navicular drop and medial longitudinal-arch deformation during quiet standing and gait compared with healthy subjects, and (2) the relationship between medial longitudinal-arch ...

  12. Hybrid treatment of penetrating aortic ulcer

    International Nuclear Information System (INIS)

    Lara, Juan Antonio Herrero; Martins-Romeo, Daniela de Araujo; Escudero, Carlos Caparros; Falcon, Maria del Carmen Prieto; Batista, Vinicius Bianchi; Vazquez, Rosa Maria Lepe

    2015-01-01

    Penetrating atherosclerotic aortic ulcer is a rare entity with poor prognosis in the setting of acute aortic syndrome. In the literature, cases like the present one, located in the aortic arch, starting with chest pain and evolving with dysphonia, are even rarer. The present report emphasizes the role played by computed tomography in the diagnosis of penetrating atherosclerotic ulcer as well as in the differentiation of this condition from other acute aortic syndromes. Additionally, the authors describe a new therapeutic approach represented by a hybrid endovascular surgical procedure for treatment of the disease. (author)

  13. Hybrid treatment of penetrating aortic ulcer

    Energy Technology Data Exchange (ETDEWEB)

    Lara, Juan Antonio Herrero; Martins-Romeo, Daniela de Araujo; Escudero, Carlos Caparros; Falcon, Maria del Carmen Prieto; Batista, Vinicius Bianchi, E-mail: jaherrero5@hotmail.com [Unidade de Gestao Clinica (UGC) de Diagnostico por Imagem - Hosppital Universitario Virgen Macarena, Sevilha (Spain); Vazquez, Rosa Maria Lepe [Unit of Radiodiagnosis - Hospital Nuestra Senora de la Merced, Osuna, Sevilha (Spain)

    2015-05-15

    Penetrating atherosclerotic aortic ulcer is a rare entity with poor prognosis in the setting of acute aortic syndrome. In the literature, cases like the present one, located in the aortic arch, starting with chest pain and evolving with dysphonia, are even rarer. The present report emphasizes the role played by computed tomography in the diagnosis of penetrating atherosclerotic ulcer as well as in the differentiation of this condition from other acute aortic syndromes. Additionally, the authors describe a new therapeutic approach represented by a hybrid endovascular surgical procedure for treatment of the disease. (author)

  14. In vitro flow investigations in the aortic arch during cardiopulmonary bypass with stereo-PIV.

    Science.gov (United States)

    Büsen, Martin; Kaufmann, Tim A S; Neidlin, Michael; Steinseifer, Ulrich; Sonntag, Simon J

    2015-07-16

    The cardiopulmonary bypass is related to complications like stroke or hypoxia. The cannula jet is suspected to be one reason for these complications, due to the sandblast effect on the vessel wall. Several in silico and in vitro studies investigated the underlying mechanisms, but the applied experimental flow measurement techniques were not able to address the highly three-dimensional flow character with a satisfying resolution. In this work in vitro flow measurements in a cannulated and a non-cannulated aortic silicone model are presented. Stereo particle image velocimetry measurements in multiple planes were carried out. By assembling the data of the different measurement planes, quasi 3D velocity fields with a resolution of~1.5×1.5×2.5 mm(3) were obtained. The resulting velocity fields have been compared regarding magnitude, streamlines and vorticity. The presented method shows to be a suitable in vitro technique to measure and address the three-dimensional aortic CPB cannula flow with a high temporal and spatial resolution. Copyright © 2015 Elsevier Ltd. All rights reserved.

  15. Takayasu arteritis with middle aortic syndrome and mesenteric ischemia treated by aortic stenting

    Directory of Open Access Journals (Sweden)

    Sarah Morrissey, MD, MA, FRCSC

    2017-09-01

    Full Text Available A 48-year-old woman suffering from Takayasu arteritis presented with middle aortic syndrome consisting of abdominal pain, refractory hypertension with pulmonary edema and pleural effusions, and lower limb ischemia. She failed to improve with high-dose steroid therapy and underwent endovascular stenting of two severe stenoses in the supraceliac and infrarenal aorta. Her symptoms resolved and hypertension improved after the procedure.

  16. Secondary hypertension due to isolated interrupted aortic arch in a 45-year-old person: A case report.

    Science.gov (United States)

    Zhou, Jian Mei; Liu, Xin Wen; Yang, Yi; Wang, Bo Zhong; Wang, Jian An

    2017-12-01

    Though it is rare, isolated interrupted aortic arch (IAA) could lead to hypertension. Surgical repair is the only effective curative method to treat IAA conditions and patients with IAA can hardly survive to adulthood with medication alone. We report an IAA case that of a 45-year-old male patient who survived for 45 years without surgical treatment. A 45-year-old man was referred to the hospital presenting with abnormal blood pressure level. Both computed tomography angiogram (CTA) and angiography revealed IAA. The patient was diagnosed as IAA based on computed tomography angiogram (CTA) and angiography. The patient's blood pressure was severely high and refractory. He refused surgical treatment and accepted antihypertensive medication for 10 days. The patient's office blood pressure level was abnormal, fluctuating between 140/90 and 160/100 mm Hg, but 24-hour ambulatory blood pressure monitoring showed normal level. Hypertension due to IAA could be controlled with medications, even surgery is not performed. The discrepancy between ambulatory and office blood pressure levels may be due to the white coat effect.

  17. Endovascular Embolization of Bronchial Artery Originating from the Upper Portion of Aortic Arch in Patients with Massive Hemoptysis

    International Nuclear Information System (INIS)

    Jiang, Sen; Sun, Xi-Wen; Yu, Dong; Jie, Bing

    2014-01-01

    PurposeOur experience with endovascular embolization (EVE) of the bronchial artery (BA) originating from the upper portion of the aortic arch (AA) in six patients is described.MethodsAltogether, 818 patients with hemoptysis underwent multidetector row computed tomography angiography (MDCTA) before EVE or AA angiography during EVE. Aberrant BAs originating from the upper portion of the AA were the source of massive hemoptysis in six patients (0.73 %). MDCT angiograms and/or Digital subtraction angiograms were retrospectively reviewed. Selective catheterization and embolization were performed.ResultsThe ostia of the BAs were located on the superior surface of the AA between the brachiocephalic trunk and left common carotid artery in three patients, the junction of the aorta and medial surface of the left subclavian artery in two, and the posterior wall of the upper portion of the AA in one. The six BAs comprised two common trunks, three single right sides, and one single left side. The targeted vessels were successfully catheterized and embolized by a coaxial microcatheter system using polyvinyl alcohol particles. Other pathologic BAs and nonbronchial systemic arteries also were embolized. Bleeding was immediately controlled in all patients with no recurrence of hemoptysis. No procedure-related complications occurred.ConclusionsApplication of EVE of anomalous origin of BAs in patients with hemoptysis is important, as demonstrated in the six reported patients. MDCTA before EVE or AA angiography during EVE is critical to avoid missing a rare aberrant BA originating from the upper portion of the AA

  18. 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

  19. 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.

  20. 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

  1. 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.

  2. Spontaneous Thrombosis of a Bicuspid Aortic valve due to Primary Antiphospholipid Syndrome

    Directory of Open Access Journals (Sweden)

    Sarah Farrell

    2010-08-01

    Full Text Available We present the case of a 51-year-old man who was admitted as an emergency with spontaneous thrombosis of the aortic valve and ascending aorta. At operation he was found to have a congenitally bicuspid aortic valve and subsequent investigation revealed primary antiphospholipid syndrome. He underwent successful removal of the thrombus combined with mechanical replacement of the aortic valve.

  3. 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.

  4. [Use of Airwayscope with pediatric intlock in a patient with first and second branchial arch syndrome].

    Science.gov (United States)

    Kubota, Aiko; Takeda, Akiko; Arai, Toshimi; Murozono, Michihiro

    2013-12-01

    First and second branchial arch syndrome is a congenital anomaly of craniofacial dysplasia involving organs derived from the second branchial arch. The main characteristics are microtia and mandibular hypoplasia. A 6-year-old boy was scheduled for adenoidectomy and bilateral myringotomy and tube placement. Slow induction was performed with oxygen, nitrous oxide, and sevoflurane. No difficulties were encountered during mask ventilation, and rocuronium was administered intravenously. His epiglottis was not visible during laryngoscopy. Therefore, we used the Airwayscope (AWS). His glottis was visible after application of cricold pressure from the left side. However, we could not closely conform his epiglottis to the mark on the AWS. Therefore, we passed a fiberoptic bronchoscope through a tracheal tube and placed it in the AWS. We attempted to intubate the trachea, but could not guide the bronchoscope to his glottis. We then attempted to pull the tracheal tube to improve the mobility of the bronchoscope. Control of the bronchoscope consequently became easy We successfully guided it to his glottis and performed tracheal intubation. His condition was stable during the procedure. In conclusion, we safely performed tracheal intubation in a patient with first and second branchial arch syndrome using the AWS and a fiberoptic bronchoscope.

  5. 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.

  6. Clinical and genetic aspects of Marfan syndrome and familial thoracic aortic aneurysms and dissections

    NARCIS (Netherlands)

    Hilhorst-Hofstee, Yvonne

    2013-01-01

    This thesis concerns the clinical and genetic aspects of familial thoracic aortic aneurysms and dissections, in particular in Marfan syndrome. It includes the Dutch multidisciplinary guidelines for diagnosis and management of Marfan syndrome. These guidelines contain practical directions for

  7. Aortic anomalies in an adolescent with the Williams' elfin facies syndrome

    International Nuclear Information System (INIS)

    Williams, R.L.; Azouz, E.M.

    1984-01-01

    An adolescent with classical Williams' syndrome who presented with hypertensive encephalopathy is described. He had the unusual combination of supravalvular aortic stenosis, long segment coarctation of the aorta, aortic hypoplasia and a high bifurcation of the abdominal aorta. Surgical resection of the coarctation was required; however, the patient has remained moderately hypertensive. The aortic anomalies in this syndrome are reviewed and their frequency and importance examined. (orig.)

  8. Comparison and usefulness of cardiac magnetic resonance versus computed tomography in infants six months of age or younger with aortic arch anomalies without deep sedation or anesthesia.

    Science.gov (United States)

    Fogel, Mark A; Pawlowski, Thomas W; Harris, Matthew A; Whitehead, Kevin K; Keller, Marc S; Wilson, Justine; Tipton, Deanna; Harris, Christine

    2011-07-01

    The present project investigated whether cardiac magnetic resonance (CMR) of aortic arch anomalies can be performed successfully in infants <6 months of age without the use of cardiac anesthesia or deep sedation. We performed a retrospective review of infants ≤6 months old from 2005 to 2009 who underwent either CMR or computed tomography angiography to investigate aortic arch abnormalities. The CMR procedure used a "feed and swaddle" protocol without deep sedation or cardiac anesthesia. Of the 52 infants referred for CMR, 24 underwent the feed and swaddle protocol (aged 2.6 ± 1.4 months). One patient awoke during the study, and examination of the remaining 23 yielded a definitive diagnosis (success rate 96%). The scanning time was 6.2 ± 3.1 minutes, with the large airways evaluation accounting for 1/2 the time. Single-shot axial steady-state free precession, in which the definitive diagnosis was made, accounted for 0.59 ± 0.3 minutes. Fifteen infants were diagnosed with a vascular ring. Of the 8 infants who underwent surgery, the diagnostic accuracy was 100%. During the same period, 19 patients, who had undergone computed tomography angiography (aged 1.67 ± 1.20 months), were referred for aortic arch evaluation. Of these 19 patients, 6 (32%) underwent sedation or anesthesia. The imaging time was 0.08 ± 0.06 minutes, significantly different from the CMR times (p <0.01). However, the overall room times (31.3 ± 22.3 and 35.8 ± 3.86 minutes, respectively) were not different between the CMR and angiographic groups. The radiation dose was 1.41 ± 1.03 mSv. In conclusion, CMR evaluation of aortic arch anomalies in children <6 months old can be successfully completed quickly using a feed and swaddle approach with high diagnostic accuracy. This protocol avoids the risks of sedation, as well as the radiation associated with computed tomography angiography. Copyright © 2011 Elsevier Inc. All rights reserved.

  9. 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

  10. 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

  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. Rupture of an abdominal aortic aneurysm in a young man with Marfan Syndrome

    DEFF Research Database (Denmark)

    Pedersen, Maria Weinkouff; Huynh, Khiem Dinh; Baandrup, Ulrik Thorngren

    2018-01-01

    Abdominal aortic aneurysms are very rare in Marfan syndrome. We present a case with a young non-smoking and normotensive male with Marfan syndrome, who developed an infrarenal abdominal aortic aneurysm that presented with rupture to the retroperitoneal cavity causing life-threatening bleeding shock...

  13. Progressive Supranuclear Palsy-like Syndrome After Aortic Aneurysm Repair: A Case Series

    Directory of Open Access Journals (Sweden)

    Sirisha Nandipati

    2013-12-01

    Full Text Available The syndrome of progressive supranuclear palsy‐like syndrome is a rare complication of ascending aortic aneurysm repair. We report two patients with videos and present a table of prior reported cases. To our knowledge there is no previously published video of this syndrome. The suspected mechanism is brainstem injury though neuroimaging is often negative for an associated infarct. We hope our report will increase recognition of this syndrome after aortic surgery, especially in patients with visual complaints.

  14. 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

  15. Idiopathic subvalvular aortic aneurysm masquerading as acute coronary syndrome.

    Science.gov (United States)

    Natarajan, Balaji; Ramanathan, Sundar; Subramaniam, Natarajan; Janardhanan, Rajesh

    2016-09-02

    Subvalvular aneurysms are the least common type of left ventricular (LV) aneurysms and can be fatal. Subaortic LV aneurysms are much rarer than submitral LV aneurysms and mostly reported in infancy. They can be congenital or acquired secondary to infections, cardiac surgery or trauma. Here, we report a unique presentation of a large, idiopathic subaortic aneurysm in an adult masquerading as an acute coronary syndrome. Diagnosis was made with the help of a CT aortography. Aneurysm was surgically resected with good results. This case highlights the clinical presentation and management of subaortic aneurysms, an important differential for congenital aortic malformations. 2016 BMJ Publishing Group Ltd.

  16. 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.

  17. Pathogenetic Basis of Aortopathy and Aortic Valve Disease

    Science.gov (United States)

    2018-02-19

    Aortopathies; Thoracic Aortic Aneurysm; Aortic Valve Disease; Thoracic Aortic Disease; Thoracic Aortic Dissection; Thoracic Aortic Rupture; Ascending Aortic Disease; Descending Aortic Disease; Ascending Aortic Aneurysm; Descending Aortic Aneurysm; Marfan Syndrome; Loeys-Dietz Syndrome; Ehlers-Danlos Syndrome; Shprintzen-Goldberg Syndrome; Turner Syndrome; PHACE Syndrome; Autosomal Recessive Cutis Laxa; Congenital Contractural Arachnodactyly; Arterial Tortuosity Syndrome

  18. Decreased aortic growth and middle aortic syndrome in patients with neuroblastoma after radiation therapy

    International Nuclear Information System (INIS)

    Sutton, Elizabeth J.; Tong, Ricky T.; Gillis, Amy M.; Haas-Kogan, Daphne A.; Henning, Tobias D.; Boddington, Sophie; Sha, Vinil; Gooding, Charles; Coakley, Fergus V.; Daldrup-Link, Heike; Weinberg, Vivian A.; Matthay, Katherine

    2009-01-01

    Long-term CT follow-up studies are required in pediatric patients who have received intraoperative radiation therapy (IORT) and external beam radiation therapy (EBRT) to assess vascular toxicities and to determine the exact complication rate. To analyze with CT the effects of radiation therapy (RT) on the growth of the aorta in neuroblastoma patients. Abdominal CT scans of 31 patients with intraabdominal neuroblastoma (stage II-IV), treated with RT (20 IORT±EBRT, 11 EBRT alone), were analyzed retrospectively. The diameter of the abdominal aorta was measured before and after RT. These data were compared to normal and predicted normal aortic diameters of children, according to the model of Fitzgerald, Donaldson and Poznanski (aortic diameter in centimeters = 0.844+0.0599 x age in years), and to the diameters of a control group of children who had not undergone RT. Statistical analyses for the primary aims were performed using the chi-squared test, t-test, Mann-Whitney test, nonparametric Wilcoxon matched-pairs test and analysis of variance for repeated measures. Clinical files and imaging studies were evaluated for signs of late vascular complications of neuroblastoma patients who had received RT. The mean diameter before and after RT and the growth of the aorta were significantly lower than expected in patients with neuroblastoma (P<0.05 for each) and when compared to the growth in a control group with normal and nonirradiated aortas. Among the patients who had received RT, there was no difference due to the type of RT. Seven patients from the IORT±EBRT group developed vascular complications, which included hypertension (five), middle aortic syndrome (two), death due to mesenteric ischemia (one) and critical aortic stenosis, which required aortic bypass surgery (two). Patients with neuroblastoma who had received RT showed impaired growth of the abdominal aorta. Significant long-term vascular complications occurred in seven patients who received IORT±EBRT. Thus

  19. 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.

  20. Platypnea-Orthodeoxia Syndrome after Transcatheter Aortic Valve Implantation

    Directory of Open Access Journals (Sweden)

    Andrew K. Roy

    2016-01-01

    Full Text Available Progressive dyspnea and hypoxaemia in the subacute phase after transcatheter aortic valve implantation (TAVI are uncommon and warrant immediate assessment of valve and prosthesis leaflet function to exclude thrombosis, as well as investigation for other causes related to the procedure, such as left ventricular dysfunction, pulmonary embolism, and respiratory sepsis. In this case, we report the observation of a patient presenting two weeks after TAVI with arterial hypoxaemia in an upright position, relieved by lying flat, and coupled with an intracardiac shunt detected on echocardiography in the absence of pulmonary hypertension, raising the suspicion of Platypnea-Orthodeoxia Syndrome (POS. Invasive intracardiac haemodynamic assessment showed a significant right-to-left shunt (Qp/Qs = 0.74, which confirmed the diagnosis, with subsequent closure of the intracardiac defect resulting in immediate relief of symptoms and hypoxaemia. To our knowledge, this is the first reported case of an interatrial defect and shunt causing Platypnea-Orthodeoxia Syndrome after transcatheter aortic valve implantation, resolved by percutaneous device closure.

  1. The impact of the metabolic syndrome on the outcome after aortic valve replacement.

    Science.gov (United States)

    Tadic, Marijana; Vukadinovic, Davor; Cvijanovic, Dane; Celic, Vera; Kocica, Mladen; Putnik, Svetozar; Ivanovic, Branislava

    2014-10-01

    The aim of this study was to examine the influence of the metabolic syndrome on the left ventricular geometry as well as on the early and mid-time outcome in patients with aortic stenosis who underwent aortic valve replacement. The study included 182 patients who underwent aortic valve replacement due to aortic stenosis. The metabolic syndrome was defined by the presence of at least three AHA-NHLB (American Heart Association/National Heart, Lung and Blood Institute) criteria. All the patients were followed for at least 2 years after the surgery. The metabolic syndrome did not influence the severity of aortic stenosis (mean gradient and aortic valve area). However, the metabolic syndrome was associated with the reduced prevalence of the normal left ventricular geometry and the increased risk of concentric left ventricular hypertrophy in patients with aortic stenosis. Among the metabolic syndrome criteria, only increased blood pressure was simultaneously associated with the short-term and mid-term outcome, independently of other risk factors. Increased fasting glucose level was an independent predictor of the only 30-day outcome after the valve replacement. The metabolic syndrome and left ventricular hypertrophy were, independently of hypertension and diabetes, associated with the 30-day outcome, as well as incidence of major cerebrovascular and cardiovascular events in the 2-year postoperative period. The metabolic syndrome does not change severity of the aortic stenosis, but significantly impacts the left ventricular remodeling in these patients. The metabolic syndrome and left ventricular hypertrophy, irrespective of hypertension and diabetes, are predictors of the short-term and mid-term outcome of patients with aortic stenosis who underwent aortic valve replacement.

  2. Congenital defects of C1 arches and odontoid process in a child with Down′s syndrome: A case presentation

    Directory of Open Access Journals (Sweden)

    Catherine Hatzantonis

    2016-01-01

    Full Text Available We present the case of a 2-year-old child with Down′s syndrome who presented to our unit with torticollis. Imaging studies revealed the rare occurrence of anterior and posterior C1 arch defects, absent odontoid process, and atlantoaxial subluxation. We managed her conservatively for 3 years without neurological deficits or worsening of atlantoaxial subluxation. We discuss the rare occurrences of anterior and posterior arch defects of the atlas, the radiological presentations of axis defects in patients, and the occurrence of atlantoaxial instability in patients with Down′s syndrome. Management options with consideration to surgery in asymptomatic and symptomatic patients are also discussed.

  3. 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

  4. 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.

  5. 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.

  6. Acute aortic dissection mimics acute inferoposterior wall myocardial infarction in a Marfan syndrome patient

    OpenAIRE

    Phowthongkum, Prasit

    2010-01-01

    A 30-year old man with acute chest pain was diagnosed with acute inferoposterior wall myocardial infarction following electrocardiography. After a failed coronary angiography, an echocardiogram revealed an aortic intimal flap after which acute aortic dissection was diagnosed. The patient received a successful Bentall operation without immediate complication. Retrospective examination then confirmed the diagnosis of Marfan syndrome. This case demonstrates acute aortic dissection may mimic acut...

  7. Acute aortic dissection mimics acute inferoposterior wall myocardial infarction in a Marfan syndrome patient.

    Science.gov (United States)

    Phowthongkum, Prasit

    2010-01-01

    A 30-year old man with acute chest pain was diagnosed with acute inferoposterior wall myocardial infarction following electrocardiography. After a failed coronary angiography, an echocardiogram revealed an aortic intimal flap after which acute aortic dissection was diagnosed. The patient received a successful Bentall operation without immediate complication. Retrospective examination then confirmed the diagnosis of Marfan syndrome. This case demonstrates acute aortic dissection may mimic acute myocardial infarction.

  8. Increased frequency of FBN1 truncating and splicing variants in Marfan syndrome patients with aortic events.

    Science.gov (United States)

    Baudhuin, Linnea M; Kotzer, Katrina E; Lagerstedt, Susan A

    2015-03-01

    Marfan syndrome is a systemic disorder that typically involves FBN1 mutations and cardiovascular manifestations. We investigated FBN1 genotype-phenotype correlations with aortic events (aortic dissection and prophylactic aortic surgery) in patients with Marfan syndrome. Genotype and phenotype information from probands (n = 179) with an FBN1 pathogenic or likely pathogenic variant were assessed. A higher frequency of truncating or splicing FBN1 variants was observed in Ghent criteria-positive patients with an aortic event (n = 34) as compared with all other probands (n = 145) without a reported aortic event (79 vs. 39%; P Marfan syndrome patients with FBN1 truncating and splicing variants.Genet Med 17 3, 177-187.

  9. [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.

  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)

    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

  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)

    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

  12. Prenatal Diagnosis of Fetal Interrupted Aortic Arch Type A by Two-Dimensional Echocardiography and Four-Dimensional Echocardiography with B-Flow Imaging and Spatiotemporal Image Correlation.

    Science.gov (United States)

    Zhang, Dongyu; Zhang, Ying; Ren, Weidong; Sun, Feifei; Guo, Yajun; Sun, Wei; Wang, Yu; Huang, Liping; Cai, Ailu

    2016-01-01

    Fetal interrupted aortic arch (IAA) is a rare cardiac anomaly and its prenatal diagnosis is challenging. The purpose of our report is to evaluate the use of two-dimensional echocardiography (2DE) and 4D echocardiography with B-flow imaging and spatiotemporal image correlation (4D BF-STIC) in detecting IAA type A (IAA-A). Twenty-three cases of confirmed IAA-A identified by fetal echocardiography were involved in the study. The fetal echocardiography image data were reviewed to analyze the ratio of right ventricle to left ventricle (RV/LV) diameter, the ratio of main pulmonary artery to ascending aorta (MPA/AAO) diameter, and the correlation of RV/LV diameter ratio and size of ventricular septal defect (VSD). 4D BF-STIC was performed in 21 fetuses using the sagittal view (4D BF-STIC-sagittal) and the four-chamber view (4D BF-STIC-4CV) as initial planes of view. An additional 183 normal fetuses were also included in our study. RV/LV and MPA/AAO ratios were calculated and compared with that of IAA-A fetuses. Fetal 2DE, 4D BF-STIC-sagittal, and 4D BF-STIC-4CV were used to visualize the aortic arch and its associated neck vessels. Six subgroups were evaluated according to gestational age. Fetal 2DE, 4D BF-STIC-sagittal, and 4D BF-STIC-4CV made the correct prenatal diagnosis of IAA-A in 19/23 (82.6%), 14/21 (66.7%), and 19/21 (90.5%) of patients, respectively. A significantly enlarged MPA combined with symmetric ventricles was found in the IAA-A fetuses, while the size of the VSD was negatively correlated with RV/LV ratio. 4D BF-STIC-sagittal and 4D BF-STIC-4CV were better than traditional 2D ultrasound in detecting the aortic arch and neck vessels between 17 and 28 gestational weeks and 29 to 40 gestational weeks in normal fetuses. It is demonstrated that IAA-A could be diagnosed by traditional fetal echocardiography, while 4D technique could better display the anatomic structure and the spatial relationships of the great arteries. Use of volume reconstruction may

  13. Bicuspid aortic valve morphology and associated cardiovascular abnormalities in fetal Turner syndrome: a pathomorphological study

    NARCIS (Netherlands)

    van Engelen, Klaartje; Bartelings, Margot M.; Gittenberger-de Groot, Adriana C.; Baars, Marieke J. H.; Postma, Alex V.; Bijlsma, Emilia K.; Mulder, Barbara J. M.; Jongbloed, Monique R. M.

    2014-01-01

    Bicuspid aortic valve (BAV) is common in Turner syndrome (TS). In adult TS, 82-95% of BAVs have fusion of the right and left coronary leaflets. Data in fetal stages are scarce. The purpose of this study was to gain insight into aortic valve morphology and associated cardiovascular abnormalities in a

  14. Upper gastrointestinal obstruction secondary to aortoduodenal syndrome owing to a noninflammatory abdominal aortic aneurysm.

    LENUS (Irish Health Repository)

    Cahill, Kevin

    2012-01-31

    Aortoduodenal syndrome is a rare complication of an abdominal aortic aneurysm wherein the aneurysm sac obstructs the patient\\'s duodenum. It presents with the symptoms of an upper gastrointestinal tract obstruction and requires surgical intervention to relieve it. Previously, gastric bypass surgery was advocated, but now aortic replacement is the mainstay of treatment. We report a case of a 67-year-old woman whose aortoduodenal syndrome was successfully managed and review the literature on this topic.

  15. Massive Thoracoabdominal Aortic Thrombosis in a Patient with Iatrogenic Cushing Syndrome

    International Nuclear Information System (INIS)

    Kim, Dong Hun; Choi, Dong Hyun; Lee, Young Min; Kim, Bo Bae; Ki, Young Jae; Kim, Jin Hwa; Chung, Joong Wha; Koh, Young Youp; Kang, Joon Tae; Chae, Seung Seok

    2014-01-01

    Massive thoracoabdominal aortic thrombosis is a rare finding in patients with iatrogenic Cushing syndrome in the absence of any coagulation abnormality. It frequently represents an urgent surgical situation. We report the case of an 82-year-old woman with massive aortic thrombosis secondary to iatrogenic Cushing syndrome. A follow-up computed tomography scan showed a decreased amount of thrombus in the aorta after anticoagulation therapy alone.

  16. Aortic dissection in patients with Marfan syndrome based on the IRAD data.

    Science.gov (United States)

    de Beaufort, Hector W L; Trimarchi, Santi; Korach, Amit; Di Eusanio, Marco; Gilon, Dan; Montgomery, Daniel G; Evangelista, Arturo; Braverman, Alan C; Chen, Edward P; Isselbacher, Eric M; Gleason, Thomas G; De Vincentiis, Carlo; Sundt, Thoralf M; Patel, Himanshu J; Eagle, Kim A

    2017-11-01

    Between January 1996 and May 2017, the International Registry on Acute Aortic Dissections has collected information on a total of 6,424 consecutive patients with acute aortic dissection, including 258 individuals with a diagnosis of Marfan syndrome. Patients with Marfan syndrome presented at a significantly younger age compared to patients without Marfan syndrome (38.2±13.2 vs . 63.0±14.0 years; PMarfan syndrome cohort, but no larger diameters more distally. The in-hospital mortality in type A dissection was not significantly different in patients with or without Marfan syndrome, despite the differences in age and comorbidities and the lower incidence of aortic rupture in the Marfan syndrome cohort. In contrast, the in-hospital mortality of Marfan syndrome patients with type B dissection appears to be lower than that of patients without Marfan syndrome. The Marfan syndrome cohort that was treated with open surgery for type B dissection seemed to do especially well, with a 0% mortality rate (n=27). Follow-up data for type A and B dissections combined show an estimated five-year survival rate of 80.1% and an estimated reintervention rate of 55.3% in patients with Marfan syndrome. Such a high rate of reinterventions highlights the need for careful surveillance and treatment for patients with Marfan syndrome surviving the acute phase of aortic dissection.

  17. 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.

  18. 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.)

  19. 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.)

  20. 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.

  1. 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.

  2. Moderate versus deep hypothermic circulatory arrest for ascending aorta and aortic arch surgeries using open distal anastomosis technique

    Directory of Open Access Journals (Sweden)

    Ahmed Abdelgawad

    2017-12-01

    Conclusions: MHCA with ACP achieved very good and comparable results to DHCA with RCP for ascending and aortic reconstruction. Furthermore, MHCA significantly shortened total operative, cardiopulmonary bypass and ischaemic times and, basically, decreased transfusion requirements compared with the former strategy and consequently may lead to better patient's outcome.

  3. Acute coronary syndrome in a patient with Marfan syndrome following emergent surgical repair of aortic dissection.

    Science.gov (United States)

    Bovolato, Francesca Elisa; Isabella, Giambattista; Rampazzo, Debora; Guglielmi, Cosimo; Gerosa, Gino; Iliceto, Sabino; Bilato, Claudio

    2008-06-01

    We report a case of acute coronary syndrome in a patient with suspect Marfan syndrome, 25 days after emergent modified Bentall-De Bono intervention for acute type I peripartum aortic dissection. She was admitted to our intensive care unit because of unstable angina, caused by critical blood flow reduction in a large portion of the myocardium, according to the severity of the symptoms and the electrocardiographic alterations. Coronary angiography showed a sub-occlusive stenosis of the left main coronary artery as a result of the dissection extension to the coronary ostium. Because of the high risk related to heart surgery, the patient was successfully treated by unprotected angioplasty and drug-eluting stent positioning. Short- and mid-term outcomes were favourable. Subsequent tests confirmed the diagnosis of Marfan syndrome. After 2 years of follow-up, the patient remains asymptomatic and in good health. To our knowledge, this is the first report of a successful percutaneous intervention of the left main coronary artery in a patient with Marfan syndrome who had already undergone ascending aortic root and valve replacement by the Bentall-De Bono procedure for acute dissection.

  4. 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.

  5. Aortic calcification and renal cysts demonstrated by CT in a teenager with Alagille syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Pombo, F. [Dept. of Radiology, Hospital Juan Canalejo, La Coruna (Spain); Isla, C. [Dept. of Radiology, Hospital Juan Canalejo, La Coruna (Spain); Gayol, A. [Dept. of Radiology, Hospital Juan Canalejo, La Coruna (Spain); Bargiela, A. [Dept. of Radiology, Hospital Juan Canalejo, La Coruna (Spain)

    1995-06-01

    Alagille syndrome, or arteriohepatic dysplasia, is a disorder characterized by paucity of intrahepatic bile ducts, peculiar facies and skeletal anomalies. We report a typical case of this syndrome in an 18-year-old girl, in whom abdominal CT showed bilateral renal cysts and aortic wall calcification, findings unreported in the radiological literature. (orig.)

  6. 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.

  7. Plasma Lactate Dehydrogenase Levels Predict Mortality in Acute Aortic Syndromes

    Science.gov (United States)

    Morello, Fulvio; Ravetti, Anna; Nazerian, Peiman; Liedl, Giovanni; Veglio, Maria Grazia; Battista, Stefania; Vanni, Simone; Pivetta, Emanuele; Montrucchio, Giuseppe; Mengozzi, Giulio; Rinaldi, Mauro; Moiraghi, Corrado; Lupia, Enrico

    2016-01-01

    Abstract In acute aortic syndromes (AAS), organ malperfusion represents a key event impacting both on diagnosis and outcome. Increased levels of plasma lactate dehydrogenase (LDH), a biomarker of malperfusion, have been reported in AAS, but the performance of LDH for the diagnosis of AAS and the relation of LDH with outcome in AAS have not been evaluated so far. This was a bi-centric prospective diagnostic accuracy study and a cohort outcome study. From 2008 to 2014, patients from 2 Emergency Departments suspected of having AAS underwent LDH assay at presentation. A final diagnosis was obtained by aortic imaging. Patients diagnosed with AAS were followed-up for in-hospital mortality. One thousand five hundred seventy-eight consecutive patients were clinically eligible, and 999 patients were included in the study. The final diagnosis was AAS in 201 (20.1%) patients. Median LDH was 424 U/L (interquartile range [IQR] 367–557) in patients with AAS and 383 U/L (IQR 331–460) in patients with alternative diagnoses (P < 0.001). Using a cutoff of 450 U/L, the sensitivity of LDH for AAS was 44% (95% confidence interval [CI] 37–51) and the specificity was 73% (95% CI 69–76). Overall in-hospital mortality for AAS was 23.8%. Mortality was 32.6% in patients with LDH ≥ 450 U/L and 16.8% in patients with LDH < 450 U/L (P = 0.006). Following stratification according to LDH quartiles, in-hospital mortality was 12% in the first (lowest) quartile, 18.4% in the second quartile, 23.5% in the third quartile, and 38% in the fourth (highest) quartile (P = 0.01). LDH ≥ 450 U/L was further identified as an independent predictor of death in AAS both in univariate and in stepwise logistic regression analyses (odds ratio 2.28, 95% CI 1.11–4.66; P = 0.025), in addition to well-established risk markers such as advanced age and hypotension. Subgroup analysis showed excess mortality in association with LDH ≥ 450 U/L in elderly, hemodynamically stable

  8. Complete Resolution of a Large Bicuspid Aortic Valve Thrombus with Anticoagulation in Primary Antiphospholipid Syndrome

    Directory of Open Access Journals (Sweden)

    Rayan Jo Rachwan

    2017-09-01

    Full Text Available Native aortic valve thrombosis in primary antiphospholipid syndrome (APLS is a rare entity. We describe a 38-year-old man who presented with neurological symptoms and a cardiac murmur. Transthoracic echocardiography detected a large bicuspid aortic valve thrombus. Laboratory evaluation showed the presence of antiphospholipid antibodies. Anticoagulation was started, and serial echocardiographic studies showed complete resolution of the aortic valve vegetation after 4 months. The patient improved clinically and had no residual symptoms. This report and review of the literature suggests that vegetations in APLS can be treated successfully with conservative treatment, regardless of their size.

  9. The Acute aortic syndrome – what do we know and what should we know?

    DEFF Research Database (Denmark)

    Houlind, Kim Christian

    . Intramural hematomas (IMH) are often found on CT-scans in patients with typical aortic pain. They are characterized by the presence of a hematoma in the media , but the absence of flow in the false lumen and the absence of a primary intimal tear. An intimal tear may, however, occur secondary to the IMH...... al. International heterogeneity in diagnostic frequency and clinical outcomes of ascending aortic intramural hematoma. J Am Soc Echocardiogr 2007;20:1260-8 4.Nienaber CA, Powell J. Management of Acute Aortic Syndromes. European Heart Journal 2012; 33:26-35...

  10. [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.

  11. Prospective comparative study of brain protection in total aortic arch replacement: deep hypothermic circulatory arrest with retrograde cerebral perfusion or selective antegrade cerebral perfusion.

    Science.gov (United States)

    Okita, Y; Minatoya, K; Tagusari, O; Ando, M; Nagatsuka, K; Kitamura, S

    2001-07-01

    The purpose of this study was to compare the results of total aortic arch replacement using two different methods of brain protection, particularly with respect to neurologic outcome. From June 1997, 60 consecutive patients who underwent total arch replacement through a midsternotomy were alternately allocated to one of two methods of brain protection: deep hypothermic circulatory arrest with retrograde cerebral perfusion (RCP: 30 patients) or with selective antegrade cerebral perfusion (SCP: 30 patients). Preoperative and postoperative (3 weeks) brain CT scan, neurological examination, and cognitive function tests were performed. Serum 100b protein was assayed before and after the cardiopulmonary bypass, as well as 24 hours and 48 hours after the operation. Hospital mortality occurred in 2 patients in the RCP group (6.6%) and 2 in the SCP group (6.6%). New strokes occurred in 1 (3.3%) of the RCP group and in 2 (6.6%) of the SCP group (p = 0.6). The incidence of transient brain dysfunction was significantly higher in the RCP group than in the SCP group (10, 33.3% vs 4, 13.3%, p = 0.05). Except in patients with strokes, S-100b values showed no significant differences in the two groups (RCP: SCP, prebypass 0.01+/-0.04: 0.05+/-0.16, postbypass 2.17+/-0.94: 1.97+/-1.00, 24 hours 0.61+/-0.36: 0.60+/-0.37, 48 hours 0.36+/-0.45: 0.46+/-0.40 microg/L, p = 0.7). There were no intergroup differences in the scores of memory decline (RCP 0.74+/-0.99; SCP 0.55+/-1.19, p = 0.6), orientation (RCP 1.11+/-1.29; SCP 0.50+/-0.76, p = 0.08), or intellectual function (RCP 1.21+/-1.27; SCP 1.05+/-1.15, p = 0.7). Both methods of brain protection for patients undergoing total arch replacement resulted in acceptable levels of mortality and morbidity. However, the prevalence of transient brain dysfunction was significantly higher in patients with the RCP.

  12. Aortic aneurysm and non-Hodgkin’s lymphoma in Marfan syndrome

    Directory of Open Access Journals (Sweden)

    Sujoy Ghosh

    2009-03-01

    Full Text Available The combination of Marfan syndrome with lymphoma is extremely rare. This report describes a case of Marfan syndrome who presented with chest discomfort and was diagnosed to have an aortic aneurysm and an additional incidental mediastinal mass that on further investigation turned out to be a diffuse large B cell lymphoma. We have suggested a hypothesis which can explain the occurrence of lymphoma in Marfan syndrome.

  13. 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

  14. 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/

  15. Personal resources and satisfaction with life in Marfan syndrome patients with aortic pathology and in abdominal aortic aneurysm patients.

    Science.gov (United States)

    Stanišić, Michał-Goran; Rzepa, Teresa; Gawrońska, Alicja; Kubaszewski, Przemysław; Putowski, Maciej; Stefaniak, Sebastian; Perek, Bartłomiej

    2018-03-01

    Whether or not the source of aortic pathology is Marfan syndrome (MFS) or other processes leading to development of abdominal aorta aneurysms (AAA), the awareness of pathology may lead to an emotional upset and low assessment of satisfaction with life. To assess, in regard to MFS patients with aortic pathology and to abdominal aortic aneurysm patients: 1) whether or not self-efficacy (SE) and health locus of control (HLoC) affect the patients' satisfaction with life; 2) whether the two groups of patients differ in terms of mental dispositions. The study population consisted of 16 MFS patients with aortic pathology and 16 AAA patients, 9 men and 7 women in each group. The mean age of the MFS patients was 28.5 ±8.214, and of the AAA patients 64.25 ±7.019. The following scales were applied: Generalized Self-Efficacy Scale, Satisfaction With Life Scale, Multidimensional Health Locus of Control Scale. Abdominal aorta aneurysms patients compared to MFS patients gave a higher rating for SE ( MD = 33.94 and MD = 29.56), internal health locus of control ( MD = 25.00 and MD = 21.13), external personal HL o C ( MD = 24.50 and MD = 19.25), external impersonal HLoC ( MD = 23.06 and MD = 18.25), and satisfaction with life ( M = 22.06 and M = 20.13). Internal and external HL o C were significantly lower in MFS patients compared to AAA patients. In patients with aortic diseases, special attention must be paid to the state of personal resources (PR). Interactions made by medical professionals should focus on enhancing PR supporting the patients' self-knowledge on their SE. This will help to improve their satisfaction with life and form a positive attitude to the illness.

  16. Management strategies for thoracic stent-graft repair of distal aortic arch lesions: is intentional subclavian artery occlusion a safe procedure?

    Energy Technology Data Exchange (ETDEWEB)

    Fanelli, Fabrizio; Salvatori, Filippo Maria; Pucci, Armando; Lucatelli, Pierleone; Rossi, Plinio; Passariello, Roberto [University of Rome La Sapienza, Department of Radiological Sciences, Rome (Italy); Dake, Michael D. [Stanford University School of Medicine, Department of Cardiothoracic Surgery Radiology, Stanford, CA (United States); Mazzesi, Giuseppe [University of Rome La Sapienza, Institute of Heart and Great Vessels, Rome (Italy)

    2009-10-15

    The aim of this retrospective analysis was to assess the clinical consequences after intentional left subclavian artery (LSA) occlusion. Thirty-seven patients, 27 type B dissection and 10 thoracic aneurysm, with short proximal neck (less than 2 cm) underwent endovascular treatment with intentional exclusion of LSA origin. No immediate complications occurred. Mean arterial pressure gradient, between right and left arms, ranged from 15 to 45 mmHg. After a mean follow-up of 43.70{+-}24.01 months, mild left arm symptoms secondary to flow reduction occurred in eight cases (21.6%) but only one required LSA transposition, after 8 months, for visual impairment. Type II endoleaks from excluded LSA occurred in 10 cases (27.0%): in seven patients, leaks were treated with coils and/or glue embolization; in one case, leak sealed spontaneously; one patient died before leak embolization could occur; one patient refused any further treatment. Intentional exclusion of the LSA may be justified when a longer proximal landing zone in the aortic arch is required. (orig.)

  17. 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.

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

    Science.gov (United States)

    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.

  19. 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.)

  20. 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.)

  1. 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.

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

    Science.gov (United States)

    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.

  3. Modified Sleeve Technique in Aortic Valve-Sparing Operation for Marfan Syndrome.

    Science.gov (United States)

    Wu, Yung-Szu; Hsieh, Shih-Rong; Wang, Chung-Chi; Tsai, Chung-Lin

    2018-03-22

    We devised a simple modification of the Florida Sleeve procedure to perform aortic valve-sparing surgery. This technique is simple, quick, effective, and safe. We used this technique in operations performed on two young patients with Marfan syndrome. The initial and short-term results were satisfactory.

  4. Aortic dimensions in girls and young women with turner syndrome: a magnetic resonance imaging study

    DEFF Research Database (Denmark)

    Cleemann, Line; Mortensen, Kristian H; Holm, Kirsten

    2010-01-01

    This study aimed to determine the dimensions of the thoracic aorta and the predictors of aortic dimensions in girls and young women with Turner syndrome (TS). A cross-sectional study was performed at a secondary care center. The study compared 41 TS patients with 50 healthy age-matched control...

  5. Middle aortic syndrome as a cause of heart failure in children and its management.

    OpenAIRE

    Gupta, S; Goswami, B; Ghosh, D C; Sen Gupta, A N

    1981-01-01

    Two cases of middle aortic syndrome in children are described along with two other cases reported earlier. In childhood, this disease may present as incipient or overt cardiac failure. Surgical treatment should be undertaken based on an objective assessment of the severity of the stricture and after taking into account the future growth of the child.

  6. Chronic type B aortic dissection in association with Hemolyticuremic syndrome in a child

    OpenAIRE

    Gera, D. N.; Ghuge, P. P.; Gandhi, S.; Vanikar, A. V.; Shrimali, J. D.; Kute, V. B.; Trivedi, H. L.

    2013-01-01

    Aortic dissection (AD) is a potentially life-threatening medical emergency usually encountered in the elderly. Here, we report a 9-year-old child who was incidentally detected to have asymptomatic chronic type B dissecting aneurysm of aorta when he presented with relapse of Hemolytic uremic syndrome (HUS) without any genetic abnormalities like Marfan or Ehler-Danlos syndrome. To the best of our knowledge, this is the first case of AD associated with HUS in a child without any known associated...

  7. Ascending aortic aneurysm and diaphragmatic hernia in a case of Marfan syndrome.

    Science.gov (United States)

    Kothari, Jignesh; Hinduja, Manish; Baria, Kinnaresh; Pandya, Himani

    2017-06-01

    Marfan syndrome commonly affects the skeletal, ocular, and cardiovascular systems. Involvement of the gastrointestinal system is known but uncommon. Intervention depends upon the system involved and the severity of symptoms. Special awareness is required for the diagnosis and management of gastrointestinal involvement in these patients. We report a rare case of simultaneous surgical repair of an ascending aortic aneurysm and a type IV hiatal hernia in a 35-year-old man with Marfan syndrome.

  8. Surgical treatment of interrupted aortic arch associated with ventricular septal defect and patent ductus arteriosus in patients over one year of age.

    Science.gov (United States)

    Li, Zhiqiang; Li, Bin; Fan, Xiangming; Su, Junwu; Zhang, Jing; He, Yan; Liu, Yinglong

    2014-01-01

    Interrupted aortic arch (IAA) is a rare congenital anomaly affecting 1.5% of infants with congenital heart disease. Neonatal repair of IAA is required to avoid irreversible pulmonary vascular lesion. However, in China, patients with IAA associated with ventricular septal defect (VSD) and patent ductus arteriosus (PDA) over one year of age are common. So we investigated the outcome of surgical treatment of IAA with VSD and PDA in patients over one year of age. From January 2009 to December 2012, 19 patients with IAA have undergone complete single-stage repair. The patients' mean age was 4.4 years, ranging 1 to 15 years; and their mean weight was 12.8 kg, ranging 4.2 to 36.0 kg. Fifteen IAA were type A, four were type B. Preoperative cardiac catheterization data were available from all patients. Mean pulmonary arterial pressure (mPAP) and pulmonary vascular resistance (PVR) were measured. The measurements of postoperative pulmonary artery pressure were taken in the operating room at the end of the case. All patients underwent echocardiographic examinations before discharged from the hospital. In addition, cardiac catheterization and echocardiographic examinations were performed during follow-up. Selective brain perfusion through the innominate artery during aortic arch reconstruction was used in all patients. Mean follow-up was (1.6±0.8) years. There were two hospital deaths (2/19, 11%). One patient died of pulmonary hypertension crisis, and another died of postoperative low cardiac output. Five cases had other main postoperative complications but no postoperative neurologic complications. Seventeen survivors were followed up, and there were no late deaths or reoperation. Mean cross-clamp duration was (85±22) minutes and selective brain perfusion duration was (34±11) minutes. Two patients required delayed sternal closure at two days postoperatively. Intensive care unit and hospital stays were (9±8) days and (47±24) days, respectively. Pressure gradients across

  9. 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.

  10. 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

  11. 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.

  12. [Images in cardiology after clinical observation - aortic dissection in Marfan syndrome].

    Science.gov (United States)

    Cabanelas, Nuno; Nobre, Angelo; Guerra, Nuno; Gallego, Javier; Ferreira, Ricardo; Carvalheiro, Catarina; Roque, João; Peres, Marisa; Siopa, Luís; Martins, Vítor Paulo; Silva, Graça; Cravino, João

    2011-09-01

    Stanford type A aortic dissection is a rare phenomenon with high short-term mortality and clinical manifestations that can make differential diagnosis a lengthy process requiring several diagnostic examinations. Based on a case report, the aim is to highlight the importance of physical examination in the initial management of these patients and of rapid access to a surgical center. A brief review follows on the diagnosis and treatment of ascending aortic dissection, and its specific nature in Marfan syndrome. A 33-year-old man was admitted to the emergency department of a district hospital with chest and back pain associated with vomiting, 20 hours after symptom onset. Initial physical examination revealed an aortic systolic murmur and musculoskeletal morphological abnormalities compatible with Marfan syndrome. Given suspected aortic dissection, a transthoracic echocardiogram was immediately performed, which showed an extensive intimal flap originating at the sinotubular junction. He was transferred to the cardiothoracic surgery department of a referral hospital where he was treated by a Bentall procedure. In this case, careful physical examination during initial assessment raised the suspicion that this patient was in a high-risk group for aortic dissection, thus avoiding unnecessary and lengthy exams. This diagnosis requires emergent surgical treatment, and so direct contact in real time between those making in the diagnosis and the surgeon is essential, as well as protocols governing immediate access to a surgical center. Copyright © 2011 Sociedade Portuguesa de Cardiologia. Published by Elsevier España. All rights reserved.

  13. Allometric considerations when assessing aortic aneurysms in Turner syndrome: Implications for activity recommendations and medical decision-making.

    Science.gov (United States)

    Corbitt, Holly; Maslen, Cheryl; Prakash, Siddharth; Morris, Shaine A; Silberbach, Michael

    2018-02-01

    In Turner syndrome, the potential to form thoracic aortic aneurysms requires routine patient monitoring. However, the short stature that typically occurs complicates the assessment of severity and risk because the relationship of body size to aortic dimensions is different in Turner syndrome compared to the general population. Three allometric formula have been proposed to adjust aortic dimensions, all employing body surface area: aortic size index, Turner syndrome-specific Z-scores, and Z-scores based on a general pediatric and young adult population. In order to understand the differences between these formula we evaluated the relationship between age and aortic size index and compared Turner syndrome-specific Z-scores and pediatric/young adult based Z-scores in a group of girls and women with Turner syndrome. Our results suggest that the aortic size index is highly age-dependent for those under 15 years; and that Turner-specific Z-scores are significantly lower than Z-scores referenced to the general population. Higher Z-scores derived from the general reference population could result in stigmatization, inappropriate restriction from sports, and increasing the risk of unneeded medical or operative treatments. We propose that when estimating aortic dissection risk clinicians use Turner syndrome-specific Z-score for those under fifteen years of age. © 2017 Wiley Periodicals, Inc.

  14. 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.

  15. Tratamento cirúrgico dos aneurismas e dissecções do arco aórtico Surgical treatment of aneurysms and dissections of the aortic arch

    Directory of Open Access Journals (Sweden)

    Bayard GONTIJO FILHO

    1999-10-01

    Full Text Available No período de jan/1990 a mar/1999, foram realizados 354 operações para tratamento de lesões da aorta torácica em nosso Serviço. Destas, 47 foram relacionadas a procedimentos no arco aórtico; 31 (66,0% pacientes eram do sexo masculino e a idade variou de 26 a 74 anos (m = 54,9 ± 10,9. A dissecção aórtica foi responsável pela indicação cirúrgica em 33 (70,2% pacientes e os aneurismas fusiformes ou saculares em 14 (29,8%. A operação foi indicada em caráter de urgência em 10 (21,3% pacientes, havendo 4 casos com sinais de rotura; 11 (23,4% pacientes já haviam sido submetidos a operação cardíaca prévia. O acesso cirúrgico foi a esternotomia mediana (42 casos ou a toracotomia esquerda ou bilateral (5 casos. Hipotermia profunda com parada circulatória (PC foi empregada em 97,8% dos casos com o tempo PC variando de 15 a 60 minutos (m = 30,6 ± 12,6. A técnica do tipo hemiarco anterior foi empregada em 19 (40,4% pacientes, o hemiarco posterior em 5 (10,6%, a substituição total em 18 (38,3%, plastia em 4 (8,5% e derivação extra-anatômica em 1 (2,1%. Foram realizados os seguintes procedimentos concomitantes: substituição da aorta ascendente por conduto valvulado (15, revascularização miocárdica (9, tromba de elefante (5 e troca valvar aórtica (3. A mortalidade hospitalar foi de 12,3% (6 pacientes sendo a complicação não fatal mais freqüente a insuficiência respiratória (7 casos. A análise dos fatores determinantes de mortalidade hospitalar demonstraram tendência estatística para idade acima de 60 anos (p = 0,17 e para portadores de dissecção aguda (p = 0,07. Dos 41 pacientes que receberam alta, 39 foram seguidos por um período de 1 mês a 9 anos. Houve 3 óbitos, sendo 1 por AVC e 2 em reoperações, necessárias em 5 pacientes. A sobrevida de 9 anos é de 80,85%.From Jan/1990 to Mar/1999 we performed 354 operations on the thoracic aorta in our hospital. Among these, 47 procedures involved the aortic arch

  16. Three-year-old child with middle aortic syndrome treated by endovascular stent implantation.

    Science.gov (United States)

    Moszura, Tomasz; Goreczny, Sebastian; Dryzek, Pawel; Niwald, Marek

    2013-04-01

    Middle aortic syndrome (MAS) is an extremely rare anomaly and represents both a diagnostic and therapeutic challenge, particularly in young children. A case of a 3.5 year-old child with MAS and arterial hypertension is reported, where owing to the patient's young age and the length of the hypoplastic aortic segment, surgical correction with end-to-end anastomosis was not feasible. Instead of palliative bypass grafting between the thoracic and abdominal aorta, successful percutaneous balloon angioplasty and stenting of the lesion was performed with the assistance of three-dimensional rotational angiography.

  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. 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. 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.

  20. 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

  1. Increasing the number of inter-arch contacts improves mastication in adults with Down syndrome: a prospective controlled trial.

    Science.gov (United States)

    Hennequin, Martine; Mazille, Marie-Noëlle; Cousson, Pierre-Yves; Nicolas, Emmanuel

    2015-06-01

    Feeding difficulties due to their condition have been widely described for babies, children and adults with Down syndrome (DS). A previous study demonstrated that, compared with wearing a placebo appliance, wearing an occlusal appliance increased inter-arch dental contacts, improved the oral health status of adults with DS and normalised their mandibular rest position. This longitudinal prospective controlled trial aimed to evaluate whether increasing inter-arch contacts in adults with DS would lead to improved masticatory efficiency. Fourteen subjects with DS (mean age±SD: 28.5±9.3years) and twelve controls without DS (24.6±1.0years) were video recorded while chewing samples of carrot and peanuts with and without an oral appliance that was designed to equalise the number of posterior functional units (PFUs) in both groups. Three parameters were collected during mastication for 15cycles and until swallowing: food refusals, food bolus granulometry (D50) and kinematic parameters of the chewing process (number of cycles, chewing duration and cycle frequency within the chewing sequence). In the DS group, increasing the number of PFUs led to a decrease in bolus particle size, to fewer masticatory cycles needed to produce a bolus ready for swallowing and to a decrease in the occurrence of food refusal, while mean chewing frequency did not vary. In the control group, bolus granulometry and chewing time increased with appliance wear while mean chewing frequency decreased. These changes clearly indicate a functional improvement in subjects with DS. This study also demonstrated a causal relationship between the number of functional pairs of posterior teeth and improved mastication. Any evaluation of feeding behaviour in persons with DS should consider inter-arch dental contacts as an explicative variable for feeding problems and their nutritional and respiratory consequences. Copyright © 2015 Elsevier Inc. All rights reserved.

  2. 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...

  3. 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.

  4. Long-term outcomes of frozen elephant trunk for type A aortic dissection in patients with Marfan syndrome.

    Science.gov (United States)

    Ma, Wei-Guo; Zhang, Wei; Zhu, Jun-Ming; Ziganshin, Bulat A; Zhi, Ai-Hua; Zheng, Jun; Liu, Yong-Min; Elefteriades, John A; Sun, Li-Zhong

    2017-10-01

    The use of the frozen elephant trunk (FET) technique for repair of type A aortic dissection (TAAD) in Marfan syndrome (MFS) is controversial. We seek to evaluate the efficacy of FET and total arch replacement (TAR) for TAAD in patients with MFS. The early and long-term outcomes were analyzed for 106 patients with MFS (mean age, 34.5 ± 9.7 years) undergoing FET + TAR for TAAD. Operative mortality was 6.6% (7 of 106). Spinal cord injury and stroke occurred in 1 patient each (0.9%), and reexploration for bleeding occurred in 6 patients (5.7%). Extra-anatomic bypass was the sole risk factor for operative mortality and morbidity (odds ratio [OR], 7.120; 95% confidence interval [CI], 1.018-49.790; P = .048). Follow-up was complete in 97.0% (96 of 99), averaging 6.3 ± 2.8 years. Late death occurred in 17 patients. Patients with acute TAAD were less prone to late death than those with chronic TAAD (OR, 0.112; 95% CI, 0.021-0.587; P = .048). Twelve patients required late reoperation, including thoracoabdominal aortic repair in 8, thoracic endovascular aortic repair for distal new entry in 3, and coronary anastomotic repair in 1. At 5 years, survival was 86.6% (95% CI, 77.9%-92.0%) and freedom from reoperation was 88.8% (95% CI, 80.1%-93.4%), and at 8 years, survival was 74.1% (95% CI, 61.9%-83.0%) and freedom from reoperation was 84.2% (95% CI, 72.4%-91.2%). In competing risks analysis, mortality was 4% at 5 years, 18% at 8 years, and 25% at 10 years; the respective rates of reoperation were 10%, 15%, and 15%; and the respective rates of survival without reoperation were 86%, 67%, and 60%. Survival was significantly higher in patients who underwent root procedures during FET + TAR (P = .047). Risk factors for reoperation were days from diagnosis to surgery (OR, 1.160; 95% CI, 1.043-1.289; P = .006) and Bentall procedure (OR, 12.012; 95% CI, 1.041-138.606; P = .046). The frozen elephant trunk and total arch replacement procedure can be safely performed

  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 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.

  7. Metabolic syndrome and its effect on aortic stiffness in premenopausal women.

    Science.gov (United States)

    Simkova, A; Bulas, J; Balogova, S; Reptova, A; Kisa, B; Luha, J; Kinova, S

    2013-01-01

    Metabolic syndrome (MS) is a cluster of proatherogenic risk factors (RF) (abdominal obesity, atherogenic dyslipidemia, impaired fasting glucose or type 2 diabetes mellitus, higher blood pressure or antihypertensive therapy) that move patients into a higher risk for development of cardiovascular disease (CVD) and type 2 diabetes. The preclinical (subclinical) target organ diseases (SOD) are early signs of atherosclerosis. An increased aortic stiffness characterised by an increased pulse wave velocity in aorta (PWV Ao) is one of SOD.The aim of the present study was to assess the impact of metabolic syndrome (MS) on aortic wall stiffness and the risk profile in premenopausal women. The aortic stiffness was measured using Arteriograph-Tensiomed, based on oscillometric measurement and analysis of the shape of brachial pulse wave, giving the PWV Ao. The results of measurements characterise a global aortic stiffness. We examined 81 premenopausal women (without history of CVD). The MS (according to the 2009 "harmonizing" definition) was present in 31 women (mean age 41.5 y), in the control group, there were 50 women (39 y). The most frequent components of MS were abdominal obesity (93 % vs 42%), arterial hypertension (68 % vs 10 %) and dyslipidemia (29 % vs 8 %). The PWV Ao was significantly higher in women with MS (9.26 m/s) compared to the control group (7.44 m/s). The aortic stiffness in women with MS compared to controls was significantly higher despite a presumed general protective hormonal effect on cardiovascular system in women with child-bearing potential (Tab. 4, Ref. 25).

  8. Defining acute aortic syndrome after trauma: Are Abbreviated Injury Scale codes a useful surrogate descriptor?

    Science.gov (United States)

    Leach, R; McNally, Donal; Bashir, Mohamad; Sastry, Priya; Cuerden, Richard; Richens, David; Field, Mark

    2012-10-01

    The severity and location of injuries resulting from vehicular collisions are normally recorded in Abbreviated Injury Scale (AIS) code; we propose a system to link AIS code to a description of acute aortic syndrome (AAS), thus allowing the hypothesis that aortic injury is progressive with collision kinematics to be tested. Standard AIS codes were matched with a clinical description of AAS. A total of 199 collisions that resulted in aortic injury were extracted from a national automotive collision database and the outcomes mapped onto AAS descriptions. The severity of aortic injury (AIS severity score) and stage of AAS progression were compared with collision kinematics and occupant demographics. Post hoc power analyses were used to estimate maximum effect size. The general demographic distribution of the sample represented that of the UK population in regard to sex and age. No significant relationship was observed between estimated test speed, collision direction, occupant location or seat belt use and clinical progression of aortic injury (once initiated). Power analysis confirmed that a suitable sample size was used to observe a medium effect in most of the cases. Similarly, no association was observed between injury severity and collision kinematics. There is sufficient information on AIS severity and location codes to map onto the clinical AAS spectrum. It was not possible, with this data set, to consider the influence of collision kinematics on aortic injury initiation. However, it was demonstrated that after initiation, further progression along the AAS pathway was not influenced by collision kinematics. This might be because the injury is not progressive, because the vehicle kinematics studied do not fully represent the kinematics of the occupants, or because an unknown factor, such as stage of cardiac cycle, dominates. Epidemiologic/prognostic study, level IV.

  9. Chronic type B aortic dissection in association with Hemolyticuremic syndrome in a child.

    Science.gov (United States)

    Gera, D N; Ghuge, P P; Gandhi, S; Vanikar, A V; Shrimali, J D; Kute, V B; Trivedi, H L

    2013-11-01

    Aortic dissection (AD) is a potentially life-threatening medical emergency usually encountered in the elderly. Here, we report a 9-year-old child who was incidentally detected to have asymptomatic chronic type B dissecting aneurysm of aorta when he presented with relapse of Hemolytic uremic syndrome (HUS) without any genetic abnormalities like Marfan or Ehler-Danlos syndrome. To the best of our knowledge, this is the first case of AD associated with HUS in a child without any known associated genetic or inherited risk factors.

  10. Chronic type B aortic dissection in association with Hemolyticuremic syndrome in a child

    Directory of Open Access Journals (Sweden)

    D N Gera

    2013-01-01

    Full Text Available Aortic dissection (AD is a potentially life-threatening medical emergency usually encountered in the elderly. Here, we report a 9-year-old child who was incidentally detected to have asymptomatic chronic type B dissecting aneurysm of aorta when he presented with relapse of Hemolytic uremic syndrome (HUS without any genetic abnormalities like Marfan or Ehler-Danlos syndrome. To the best of our knowledge, this is the first case of AD associated with HUS in a child without any known associated genetic or inherited risk factors.

  11. Mutations of the RTEL1 Helicase in a Hoyeraal-Hreidarsson Syndrome Patient Highlight the Importance of the ARCH Domain.

    Science.gov (United States)

    Jullien, Laurent; Kannengiesser, Caroline; Kermasson, Laetitia; Cormier-Daire, Valérie; Leblanc, Thierry; Soulier, Jean; Londono-Vallejo, Arturo; de Villartay, Jean-Pierre; Callebaut, Isabelle; Revy, Patrick

    2016-05-01

    The DNA helicase RTEL1 participates in telomere maintenance and genome stability. Biallelic mutations in the RTEL1 gene account for the severe telomere biology disorder characteristic of the Hoyeraal-Hreidarsson syndrome (HH). Here, we report a HH patient (P4) carrying two novel compound heterozygous mutations in RTEL1: a premature stop codon (c.949A>T, p.Lys317*) and an intronic deletion leading to an exon skipping and an in-frame deletion of 25 amino-acids (p.Ile398_Lys422). P4's cells exhibit short and dysfunctional telomeres similarly to other RTEL1-deficient patients. 3D structure predictions indicated that the p.Ile398_Lys422 deletion affects a part of the helicase ARCH domain, which lines the pore formed with the core HD and the iron-sulfur cluster domains and is highly specific of sequences from the eukaryotic XPD family members. © 2016 WILEY PERIODICALS, INC.

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

    Directory of Open Access Journals (Sweden)

    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

  13. Induction of macrophage chemotaxis by aortic extracts from patients with Marfan syndrome is related to elastin binding protein.

    Directory of Open Access Journals (Sweden)

    Gao Guo

    Full Text Available Marfan syndrome is an autosomal dominantly inherited disorder of connective tissue with prominent skeletal, ocular, and cardiovascular manifestations. Aortic aneurysm and dissection are the major determinants of premature death in untreated patients. In previous work, we showed that extracts of aortic tissues from the mgR mouse model of Marfan syndrome showed increased chemotactic stimulatory activity related to the elastin-binding protein. Aortic samples were collected from 6 patients with Marfan syndrome and 8 with isolated aneurysms of the ascending aorta. Control samples were obtained from 11 organ donors without known vascular or connective tissue diseases. Soluble proteins extracted from the aortic samples of the two patient groups were compared against buffer controls and against the aortic samples from controls with respect to the ability to induce macrophage chemotaxis as measured using a modified Boyden chamber, as well as the reactivity to a monoclonal antibody BA4 against bioactive elastin peptides using ELISA. Samples from Marfan patients displayed a statistically significant increase in chemotactic inductive activity compared to control samples. Additionally, reactivity to BA4 was significantly increased. Similar statistically significant increases were identified for the samples from patients with idiopathic thoracic aortic aneurysm. There was a significant correlation between the chemotactic index and BA4 reactivity, and the increases in chemotactic activity of extracts from Marfan patients could be inhibited by pretreatment with lactose, VGVAPG peptides, or BA4, which indicates the involvement of EBP in mediating the effects. Our results demonstrate that aortic extracts of patients with Marfan syndrome can elicit macrophage chemotaxis, similar to our previous study on aortic extracts of the mgR mouse model of Marfan syndrome (Guo et al., Circulation 2006; 114:1855-62.

  14. Induction of Macrophage Chemotaxis by Aortic Extracts from Patients with Marfan Syndrome Is Related to Elastin Binding Protein

    Science.gov (United States)

    Guo, Gao; Gehle, Petra; Doelken, Sandra; Martin-Ventura, José Luis; von Kodolitsch, Yskert; Hetzer, Roland; Robinson, Peter N.

    2011-01-01

    Marfan syndrome is an autosomal dominantly inherited disorder of connective tissue with prominent skeletal, ocular, and cardiovascular manifestations. Aortic aneurysm and dissection are the major determinants of premature death in untreated patients. In previous work, we showed that extracts of aortic tissues from the mgR mouse model of Marfan syndrome showed increased chemotactic stimulatory activity related to the elastin-binding protein. Aortic samples were collected from 6 patients with Marfan syndrome and 8 with isolated aneurysms of the ascending aorta. Control samples were obtained from 11 organ donors without known vascular or connective tissue diseases. Soluble proteins extracted from the aortic samples of the two patient groups were compared against buffer controls and against the aortic samples from controls with respect to the ability to induce macrophage chemotaxis as measured using a modified Boyden chamber, as well as the reactivity to a monoclonal antibody BA4 against bioactive elastin peptides using ELISA. Samples from Marfan patients displayed a statistically significant increase in chemotactic inductive activity compared to control samples. Additionally, reactivity to BA4 was significantly increased. Similar statistically significant increases were identified for the samples from patients with idiopathic thoracic aortic aneurysm. There was a significant correlation between the chemotactic index and BA4 reactivity, and the increases in chemotactic activity of extracts from Marfan patients could be inhibited by pretreatment with lactose, VGVAPG peptides, or BA4, which indicates the involvement of EBP in mediating the effects. Our results demonstrate that aortic extracts of patients with Marfan syndrome can elicit macrophage chemotaxis, similar to our previous study on aortic extracts of the mgR mouse model of Marfan syndrome (Guo et al., Circulation 2006; 114:1855-62). PMID:21647416

  15. 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.

  16. 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.

  17. Quantification of aortic and cutaneous elastin and collagen morphology in Marfan syndrome by multiphoton microscopy.

    Science.gov (United States)

    Cui, Jason Z; Tehrani, Arash Y; Jett, Kimberly A; Bernatchez, Pascal; van Breemen, Cornelis; Esfandiarei, Mitra

    2014-09-01

    In a mouse model of Marfan syndrome, conventional Verhoeff-Van Gieson staining displays severe fragmentation, disorganization and loss of the aortic elastic fiber integrity. However, this method involves chemical fixatives and staining, which may alter the native morphology of elastin and collagen. Thus far, quantitative analysis of fiber damage in aorta and skin in Marfan syndrome has not yet been explored. In this study, we have used an advanced noninvasive and label-free imaging technique, multiphoton microscopy to quantify fiber fragmentation, disorganization, and total volumetric density of aortic and cutaneous elastin and collagen in a mouse model of Marfan syndrome. Aorta and skin samples were harvested from Marfan and control mice aged 3-, 6- and 9-month. Elastin and collagen were identified based on two-photon excitation fluorescence and second-harmonic-generation signals, respectively, without exogenous label. Measurement of fiber length indicated significant fragmentation in Marfan vs. control. Fast Fourier transform algorithm analysis demonstrated markedly lower fiber organization in Marfan mice. Significantly reduced volumetric density of elastin and collagen and thinner skin dermis were observed in Marfan mice. Cutaneous content of elastic fibers and thickness of dermis in 3-month Marfan resembled those in the oldest control mice. Our findings of early signs of fiber degradation and thinning of skin dermis support the potential development of a novel non-invasive approach for early diagnosis of Marfan syndrome. Copyright © 2014 Elsevier Inc. All rights reserved.

  18. Osteoprotegerin in Turner syndrome - relationship to aortic diameter

    DEFF Research Database (Denmark)

    Trolle, Christian; Mortensen, Kristian Havmand; Bjerre, Mette

    2015-01-01

    BACKGROUND: Cardiovascular disease is a cardinal trait of Turner syndrome (TS), causing half of the 3-fold excess mortality. Since osteoprotegerin (OPG) is as a potential biomarker of cardiovascular disease, this cross-sectional and prospective study aimed at elucidating OPG levels in TS and its...

  19. Preventing the aortic complications of Marfan syndrome: a case-example of translational genomic medicine

    Science.gov (United States)

    Li-Wan-Po, Alain; Loeys, Bart; Farndon, Peter; Latham, David; Bradley, Caroline

    2011-01-01

    The translational path from pharmacological insight to effective therapy can be a long one. We aim to describe the management of Marfan syndrome as a case-example of how pharmacological and genomic insights can contribute to improved therapy. We undertook a literature search for studies of Marfan syndrome, to identify milestones in description, understanding and therapy of the syndrome. From the studies retrieved we then weaved an evidence-based description of progress. Marfan syndrome shows considerable heterogeneity in clinical presentation. It relies on defined clinical criteria with confirmation based on FBN1 mutation testing. Surgical advances have prolonged life in Marfan syndrome. First-line prophylaxis of complications with β-adrenoceptor blockers became established on the basis that reduction of aortic pressure and heart rate would help. Over-activity of proteinases, first suggested in 1980, has since been confirmed by evidence of over-expression of matrix metalloproteinases (MMP), notably MMP-2 and MMP-9. The search for MMP inhibitors led to the evaluation of doxycycline, and both animal studies and small trials, provided early evidence that this widely used antimicrobial agent was useful. Identification of the importance of TGF-β led to evaluation of angiotensin II type I receptor (AT1R) blockers with highly promising results. Combination prophylactic therapy would appear rational. Pharmacological and genomic research has provided good evidence that therapy with losartan and doxycycline would prevent the aortic complications of Marfan syndrome. If on-going well designed trials confirm their efficacy, the outlook for Marfan syndrome patients would be improved considerably. PMID:21276043

  20. The great imitator: IgG4 periaortitis masquerading as an acute aortic syndrome on computed tomographic angiography

    OpenAIRE

    Moore, Drew W.; Hansen, Neil J.; DiMaio, Dominick J.; Harrison, William L.

    2016-01-01

    We present the case of a 52-year-old woman who presented to the emergency department with chest and neck pain. Initial cervical spine magnetic resonance imaging shows an abnormal flow void in the left vertebral artery, which prompted a computed tomographic angiogram. This demonstrated a hyperdense thickened ascending aortic wall, which extended into the great vessel origins. Clinically and radiographically interpreted as an acute aortic syndrome and/or intramural hematoma, the patient underwe...

  1. Case report of a rarely seen long-segment middle aortic syndrome.

    Science.gov (United States)

    Yakut, Kahraman; Erdoğan, İlkay

    2017-03-01

    Middle aortic syndrome (MAS) follows a course with distal thoracic and abdominal aorta stenosis. It is a rare disease that is usually diagnosed after the first decade of life. Clinical reflection of MAS is often in the form of hypertension and claudication in the lower extremities. Its etiology is unclear, but is known to be associated with congenital or acquired diseases. This pathology, which is accompanied by malignant hypertension, often does not respond to medical treatment. In patients with MAS, surgical treatment is first line recommendation to prevent complications such as hypertension, heart failure, intracranial bleeding, or aortic rupture. In order to draw attention to this disease, presently described is case of high blood pressure detected during routine examination of a child who had no complaint, and discovery of long-segment stenosis in the abdominal aorta identified with echocardiography and conventional angiography.

  2. 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

  3. [Marfan syndrome: clinical and pathomorphological restructurings after surgical treatment of aortic aneurysm].

    Science.gov (United States)

    Zhuraev, R K; Zerbino, D D

    2014-01-01

    The authors examined the state of patients suffering from Marfan syndrome (MS) who endured operation for ascending aorta aneurysm with replacement of the ascending aorta and aortic valve (Bentall operation), studying alterations of the skeleton, face, heart and eyes, as well as pathomorphological restructurings in the aortic wall. The study was carried out 7.0 ± 4.2 years after the operation. We examined a total of 39 patients with MS - 27 (69.2%) men and 12 (30.8%) women aged from 22 to 70 years old (average age - 42.1 ± 13.4 years). All patients were operated on for dissecting aneurysm of the ascending aorta accompanied by a considerable degree of aortic valve insufficiency or aortic ostium stenosis. The mean diameter of the aorta at the level of the sinuses of Valsalva amounted to 7.0 ± 1.3 cm (minimal - 5.0 cm, maximal - 12.0 cm), the Z-score prior to operation was 12.7 ± 6.5. The time form making the diagnosis of MS to surgical intervention for aortic aneurysm amounted to 9.6 ± 5.9 years. The condition after operative treatment in all patients was satisfactory, with the haemodynamic indices stable: systolic AP - 133.5 ± 19.1 mm Hg, diastolic AP 85.1 ± 12.9 mm Hg, heart rate 74.8 ± 7.2 bpm. The average systemic score for the symptoms and tests of MS patients amounted to 8.2 ± 3.3 points. Pathohistological alterations of the aorta in patients with Marfan syndrome consisted in pronounced restructuring of the wall with deep irreversible alternative changes. The pathological process extended in the middle aortic layer all alone the length, but not only in the portions of rupture and dissection. The main pathomorphological signs in MS were as follows: focal accumulations of mucoid substances, dystrophic alterations of smooth-muscle cells, ribbon-like anuclear zones, formation of cystlike cavities, alterations of elastic fibres - fragmentation, hyperelastosis, multiplication, thinning and straightening, zones of elastolysis.

  4. AORTIC ANEURYSM: A RARE CAUSE OF ORTNER’S SYNDROME

    OpenAIRE

    Sukanta; Biswal Pradipta; Swetapadma; Jyoti; Manoranjan

    2014-01-01

    A 60 year old male patient presented with hoarseness of voice lasting for 2 months. First he was examined by the otorhinolaryngologist of our hospital, who noticed absence of movements of the left vocal cord. Chest X-ray showed a large opacity in the left upper zone and the patient was referred to the pulmonologist. His general physical examination was unremarkable not having any stigmata of Marfan's or Ehler Danlos syndrome. On chest auscultation, a systolic flow murmur was ...

  5. 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

  6. 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.

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

    Science.gov (United States)

    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.

  8. Endovascular Repair of Aortic Dissection in Marfan Syndrome: Current Status and Future Perspectives

    Directory of Open Access Journals (Sweden)

    Rosario Parisi

    2015-07-01

    Full Text Available Over the last decades, improvement of medical and surgical therapy has increased life expectancy in Marfan patients. Consequently, the number of such patients requiring secondary interventions on the descending thoracic aorta due to new or residual dissections, and distal aneurysm formation has substantially enlarged. Surgical and endovascular procedures represent two valuable options of treatment, both associated with advantages and drawbacks. The aim of the present manuscript was to review endovascular outcomes in Marfan syndrome and to assess the potential role of Thoracic Endovascular Aortic Repair (TEVAR in this subset of patients.

  9. Early Manifestation of Supravalvular Aortic and Pulmonary Artery Stenosis in a Patient with Williams Syndrome

    Directory of Open Access Journals (Sweden)

    Jong Uk Lee

    2016-04-01

    Full Text Available Williams syndrome (WS is a developmental disorder characterized by vascular abnormalities such as thickening of the vascular media layer in medium- and large-sized arteries. Supravalvular aortic stenosis (SVAS and peripheral pulmonary artery stenosis (PPAS are common vascular abnormalities in WS. The natural course of SVAS and PPAS is variable, and the timing of surgery or intervention is determined according to the progression of vascular stenosis. In our patient, SVAS and PPAS showed rapid concurrent progression within two weeks after birth. We report the early manifestation of SVAS and PPAS in the neonatal period and describe the surgical treatment for stenosis relief.

  10. Otofaciocervical syndrome and metachondromatosis in a girl: Presentation of a novel association and remarks on clinical variability of branchial-arch disorders.

    Science.gov (United States)

    Salinas-Torres, Victor M; Salinas-Torres, Rafael A

    2016-06-01

    Otofaciocervical syndrome (OFCS) is a rare disorder characterized by facial, ear, branchial, and musculoskeletal anomalies, along with hearing loss and mild intellectual disability. Clinically, its distinction from branchiootorenal syndrome can be difficult. To date, the coexistence of OFCS and metachondromatosis has not been reported. Here, we describe a sporadic patient with both OFCS and metachondromatosis. This novel association prompts us to do some remarks on the clinical variability of branchial-arch disorders; in fact, our observations are consistent with the highly variable expressivity of OFCS and illustrate the need of a more accurate characterization of these branchial-arch disorders. In the meantime, involvement of clavicles, scapulae and shoulders remains a distinctive feature of OFCS. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  11. Importance of absent ductus arteriosus in tetralogy of Fallot with absent pulmonary valve syndrome.

    Science.gov (United States)

    Qureshi, Muhammad Yasir; Burkhart, Harold M; Julsrud, Paul; Cetta, Frank

    2014-12-01

    Tetralogy of Fallot without pulmonary valve syndrome is almost always associated with an absent ductus arteriosus. Patients with right aortic arch and retroesophageal left subclavian artery have a vascular ring if the left ductus arteriosus or its remnant and the Kommerell diverticulum are present. We report the cases of 2 infants in whom the role of an absent ductus arteriosus or its remnant is noteworthy. Both patients had a combination of tetralogy of Fallot with absent pulmonary valve syndrome and right aortic arch with retroesophageal left subclavian artery without a vascular ring. The absence of the ductus arteriosus has a role in the pathogenesis of tetralogy of Fallot with absent pulmonary valve syndrome. The absence of a ductus arteriosus in the right aortic arch with retroesophageal left subclavian artery precludes a vascular ring.

  12. [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.

  13. Unlikely culprit: congenital middle aortic syndrome diagnosed in the sixth decade of life.

    Science.gov (United States)

    Ali, Muhammad Sajawal; Tchernodrinski, Stefan; Mohananey, Divyanshu; Ali, Ahya Sajawal

    2016-08-16

    A 58-year-old woman was admitted with acute heart failure. She had a long history of resistant hypertension, with an unremarkable work up for secondary causes in the past. Her brachial blood pressure was 210/70 mm Hg, with ankle blood pressure of 100/70 mm Hg. CT angiogram revealed marked narrowing of the descending thoracic aorta between the left subclavian artery and the diaphragm, consistent with middle aortic syndrome (MAS). She was initially managed with diuretics and antihypertensives. Subsequently thoracotomy revealed a severely hypoplastic segment of the descending aorta. The diseased segment was resected and aortic reconstruction performed. Histopathology showed fragmentation of the medial elastic fibres and fibrosis of the medial and intimal layers. These findings along with gross aortic hypoplasia and absence of features of Takayasu's arteritis, suggest that our patient had congenital MAS. The patient has done well since her surgery. We believe this is the first case of congenital MAS reported in the sixth decade of life. 2016 BMJ Publishing Group Ltd.

  14. 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.

  15. 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.

  16. [Maternal and fetal outcomes with aortic dissection in pregnant patients with Marfan syndrome].

    Science.gov (United States)

    Yang, Puyu; Zhang, Jun; Li, Yanna; Wang, Hui; Zheng, Jun

    2015-05-01

    To evaluate the clinical characteristics of aortic dissection in pregnant patients with Marfan syndrome and the maternal and fetal outcomes in cardiovascular surgery. Seven pregnant women with Marfan syndrome with aortic dissection were identified, who were treated in Beijing Anzhen Hospital Affiliated to Capital Medical University between January 2012 and September 2014. Patient charts were reviewed for cardiovascular surgery, occurrence of complications, clinical features and the maternal and fetal outcomes. (1) Among 7 patients, 4 cases were diagnosed as type A aortic dissection and 3 were cases diagnosed as type B aortic dissection. The diagnosis mainly depends on CT angiography. New York Heart Association (NYHA) classify into 5 of level II, 1 of level III, 1 of leveI IV. Except for 1 patient with cardiac tamponade lead to heart failure, the remaining 6 cases had no complications. (2) Three patients underwent heart surgery with cardiopulmonary bypass in second trimester and two patients underwent heart surgery in third trimester. Two patients terminated pregnancy before heart surgery (one of whom underwent artificial abortion, one of whom underwent cesarean section in second trimester). (3) The methods of cardiovascular surgeries were as follow: 3 of Bentall+Sun', 1 of Bentall+Sun'+ right coronary artery bypass grafting, 1 of Bentall, 1 of the whole chest aorta replacement surgery, and 1 of femoral artery catheter chest aorta with membrane mesh stent implantation. The diameter of aortic roots measured during operation were 5 cm in 2 cases, 7 cm in 2 cases and 10 cm in 2 cases respectively. Among the 7 cases, 3 were conducted cesarean sections during cardiovascular surgery, 1 was terminated pregnancy due to intrauterine fetal death after cardiovascular surgery, and 1 was conducted cesarean section due to severe early-onset preeclampsia at 30 weeks of pregnancy after cardiovascular surgery. (4) Among the 7 cases, 3 were conducted cesarean sections during

  17. 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.

  18. Fetal Aortic Valvuloplasty for Evolving Hypoplastic Left Heart Syndrome: Postnatal Outcomes of the First 100 Patients

    Science.gov (United States)

    Freud, Lindsay R.; McElhinney, Doff B.; Marshall, Audrey C.; Marx, Gerald R.; Friedman, Kevin G.; del Nido, Pedro J.; Emani, Sitaram M.; Lafranchi, Terra; Silva, Virginia; Wilkins-Haug, Louise E.; Benson, Carol B.; Lock, James E.; Tworetzky, Wayne

    2015-01-01

    Background Fetal aortic valvuloplasty (FAV) can be performed for severe mid-gestation aortic stenosis (AS) in an attempt to prevent progression to hypoplastic left heart syndrome (HLHS). A subset of patients has achieved a biventricular (BV) circulation after FAV. The postnatal outcomes and survival of the BV patients, compared to those managed as HLHS, have not been reported. Methods and Results We included 100 patients who underwent FAV for severe mid-gestation AS with evolving HLHS from March 2000 to January 2013. Patients were categorized based on postnatal management as BV or HLHS. Clinical records were reviewed. Eighty-eight fetuses were live-born, and 38 had a BV circulation (31 from birth, 7 converted after initial univentricular palliation). Left-sided structures, namely aortic and mitral valve sizes and LV volume, were significantly larger in the BV group at the time of birth (p-values <0.01). After a median follow-up of 5.4 years, freedom from cardiac death among all BV patients was 96±4% at 5 years and 84±12% at 10 years, which was better than HLHS patients (log-rank p=0.04). There was no cardiac mortality in patients with a BV circulation from birth. All but 1 of the BV patients required postnatal intervention; 42% underwent aortic and/or mitral valve replacement. On most recent echocardiogram, the median LV end-diastolic volume z-score was +1.7 (range: -1.3, +8.2), and 80% had normal ejection fraction. Conclusions Short- and intermediate-term survival among patients who underwent FAV and achieved a BV circulation postnatally is encouraging. However, morbidity still exists, and on-going assessment is warranted. PMID:25052401

  19. 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.

  20. 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)

  1. Phenotype in girls and women with Turner syndrome: Association between dysmorphic features, karyotype and cardio-aortic malformations.

    Science.gov (United States)

    Noordman, Iris; Duijnhouwer, Anthonie; Kapusta, Livia; Kempers, Marlies; Roeleveld, Nel; Schokking, Michiel; Smeets, Dominique; Freriks, Kim; Timmers, Henri; van Alfen-van der Velden, Janiëlle

    2018-06-01

    Turner syndrome (TS) is a genetic disorder characterized by the (partial) absence or a structural aberration of the second sex chromosome and is associated with a variety of phenotypes with specific physical features and cardio-aortic malformations. The objective of this study was to gain a better insight into the differences in dysmorphic features between girls and women with TS and to explore the association between these features, karyotype and cardio-aortic malformations. This prospective study investigated 14 dysmorphic features of TS girls and women using a checklist. Three major phenotypic patterns were recognized (severe phenotype, lymphatic phenotype and skeletal phenotype). Patient data including karyotype and cardio-aortic malformations (bicuspid aortic valve (BAV) and aortic coarctation (COA)) were collected. Associations between the prevalence of dysmorphic features, karyotype and cardio-aortic malformations were analysed using chi 2 -test and odds ratios. A total of 202 patients (84 girls and 118 women) were analysed prospectively. Differences in prevalence of dysmorphic features were found between girls and women. A strong association was found between monosomy 45,X and the phenotypic patterns. Furthermore, an association was found between COA and lymphatic phenotype, but no association was found between karyotype and cardio-aortic malformations. This study uncovered a difference in dysmorphic features between girls and women. Monosomy 45,X is associated with a more severe phenotype, lymphatic phenotype and skeletal phenotype. All patients with TS should be screened for cardio-aortic malformations, because in contrast to previous reports, karyotype and cardio-aortic malformations showed no significant association. Copyright © 2018 Elsevier Masson SAS. All rights reserved.

  2. Life-threatening subdural hematoma after aortic valve replacement in a patient with Heyde syndrome: a case report.

    Science.gov (United States)

    Uchida, Tetsuro; Hamasaki, Azumi; Ohba, Eiichi; Yamashita, Atsushi; Hayashi, Jun; Sadahiro, Mitsuaki

    2017-08-08

    Heyde syndrome is known as a triad of calcific aortic stenosis, anemia due to gastrointestinal bleeding from angiodysplasia, and acquired type 2A von Willebrand disease. This acquired hemorrhagic disorder is characterized by the loss of the large von Willebrand factor multimers due to the shear stress across the diseased aortic valve. The most frequently observed type of bleeding in these patients is mucosal or skin bleeding, such as epistaxis, followed by gastrointestinal bleeding. On the other hand, intracranial hemorrhage complicating Heyde syndrome is extremely rare. A 77-year-old woman presented to our hospital with severe aortic stenosis and severe anemia due to gastrointestinal bleeding and was diagnosed with Heyde syndrome. Although aortic valve replacement was performed without recurrent gastrointestinal bleeding, postoperative life-threatening acute subdural hematoma occurred with a marked midline shift. Despite prompt surgical evacuation of the hematoma, she did not recover consciousness and she died 1 month after the operation. Postoperative subdural hematoma is rare, but it should be kept in mind as a devastating hemorrhagic complication, especially in patients with Heyde syndrome.

  3. 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.

  4. [Case of neuroleptic malignant syndrome following open heart surgery for thoracic aortic aneurysm with parkinson's disease].

    Science.gov (United States)

    Shinoda, Maiko; Sakamoto, Mik; Shindo, Yuki; Ando, Yumi; Tateda, Takeshi

    2013-12-01

    An 80-year-old woman with Parkinson's disease was scheduled for open heart surgery to repair thoracic aortic aneurysm. Parkinson's symptoms were normally treated using oral levodopa (200 mg), selegiline-hydrochloride (5 mg), bromocriptine-mesilate (2 mg), and amantadine-hydrochloride (200 mg) daily. On the day before surgery, levodopa 50mg was infused intravenously. Another 25 mg of levodopa was infused immediately after surgery. Twenty hours later, the patient developed tremors, heyperventilation, but no obvious muscle rigidity. Two days after surgery, the patient exhibited high fever, hydropoiesis, elevated creatine kinase, and a rise in blood leukocytes. She was diagnosed with neuroleptic malignant syndrome. She was intubated, and received dantrolene sodium. Symptoms of neuroleptic malignant syndrome disappeared on the fourth postoperative day. The stress of open heart surgery, specifically extracorporeal circulation and concomitant dilution of levodopa, triggered neuroleptic malignant syndrome in this patient. Parkinson's patients require higher doses of levodopa prior to surgery to compensate and prevent neuroleptic malignant syndrome after surgery.

  5. Diagnostic Accuracy of the Aortic Dissection Detection Risk Score Plus D-Dimer for Acute Aortic Syndromes: The ADvISED Prospective Multicenter Study.

    Science.gov (United States)

    Nazerian, Peiman; Mueller, Christian; Soeiro, Alexandre de Matos; Leidel, Bernd A; Salvadeo, Sibilla Anna Teresa; Giachino, Francesca; Vanni, Simone; Grimm, Karin; Oliveira, Múcio Tavares; Pivetta, Emanuele; Lupia, Enrico; Grifoni, Stefano; Morello, Fulvio

    2018-01-16

    Acute aortic syndromes (AASs) are rare and severe cardiovascular emergencies with unspecific symptoms. For AASs, both misdiagnosis and overtesting are key concerns, and standardized diagnostic strategies may help physicians to balance these risks. D-dimer (DD) is highly sensitive for AAS but is inadequate as a stand-alone test. Integration of pretest probability assessment with DD testing is feasible, but the safety and efficiency of such a diagnostic strategy are currently unknown. In a multicenter prospective observational study involving 6 hospitals in 4 countries from 2014 to 2016, consecutive outpatients were eligible if they had ≥1 of the following: chest/abdominal/back pain, syncope, perfusion deficit, and if AAS was in the differential diagnosis. The tool for pretest probability assessment was the aortic dissection detection risk score (ADD-RS, 0-3) per current guidelines. DD was considered negative (DD-) if 1. Two hundred forty-one patients (13%) had AAS: 125 had type A aortic dissection, 53 had type B aortic dissection, 35 had intramural aortic hematoma, 18 had aortic rupture, and 10 had penetrating aortic ulcer. A positive DD test result had an overall sensitivity of 96.7% (95% confidence interval [CI], 93.6-98.6) and a specificity of 64% (95% CI, 61.6-66.4) for the diagnosis of AAS; 8 patients with AAS had DD-. In 294 patients with ADD-RS=0/DD-, 1 case of AAS was observed. This yielded a failure rate of 0.3% (95% CI, 0.1-1.9) and an efficiency of 15.9% (95% CI, 14.3-17.6) for the ADD-RS=0/DD- strategy. In 924 patients with ADD-RS ≤1/DD-, 3 cases of AAS were observed. This yielded a failure rate of 0.3% (95% CI, 0.1-1) and an efficiency of 49.9% (95% CI, 47.7-52.2) for the ADD-RS ≤1/DD- strategy. Integration of ADD-RS (either ADD-RS=0 or ADD-RS ≤1) with DD may be considered to standardize diagnostic rule out of AAS. URL: https://www.clinicaltrials.gov. Unique identifier: NCT02086136. © 2017 American Heart Association, Inc.

  6. 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.

  7. 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.

  8. 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.

  9. Ortner’s syndrome: a case report and literature review

    Directory of Open Access Journals (Sweden)

    Bruno Landim Dutra

    2015-08-01

    Full Text Available AbstractThe authors report the case of a 55-year-old female, hypertensive, smoker patient presenting with dysphonia, dysphagia and persistent dry cough. Laryngoscopy diagnosed left vocal cord paralysis. Computed tomography demonstrated saccular aneurysm of the inferior wall of the aortic arch, stretching the left recurrent laryngeal nerve, a finding compatible with Ortner’s syndrome.

  10. Clinical manifestation as acute coronary syndrome without electrocardiographically ischemia: a clue for aortic dissection

    Directory of Open Access Journals (Sweden)

    Hung Yi Chen

    2015-06-01

    Full Text Available Aortic dissection is a critical condition requiring immediate assessment and management. Clinical presentation is commonly associated with severe chest pain and high blood pressure. However, misdiagnosis is frequent because of various features. We presented a case of 51-year-old woman who complained of dyspnea for 3 d after she experienced back pain for one week. She was presented with severe respiration distress with impending respiration failure on arrival to our hospital. Her chest X-ray showed cardiomegaly with acute pulmonary edema. The laboratory data revealed elevated cardiac enzyme and electrocardiography demonstrated sinus tachycardia. She was hospitalized under the initial diagnosis of acute coronary syndrome. The patient remained hemodynamically stable, and experienced one episode of chest discomfort. After electrocardiography, she was found with bigeminy ventricular premature beats without ST-T change. Follow-up cardiac enzyme demonstrated progressive declined. Cardiac catheterization was performed on the third day of admission, and coronary angiography revealed large intimal flap on aortic root with bilateral coronary artery involvement. Surgical management was arranged after immediate chest computed tomography study.

  11. Beneficial Effects of High-Density Lipoproteins on Acquired von Willebrand Syndrome in Aortic Valve Stenosis.

    Science.gov (United States)

    Gebhard, C; Maafi, F; Stähli, B E; Bonnefoy, A; Gebhard, C E; Nachar, W; de Oliveira Moraes, A Benjamim; Mecteau, M; Mihalache-Avram, T; Lavoie, V; Kernaleguen, A E; Shi, Y; Busseuil, D; Chabot-Blanchet, M; Perrault, L P; Rhainds, D; Rhéaume, E; Tardif, J C

    2018-02-01

     Infusions of apolipoprotein A-I (apoA-I), the major protein component of high-density lipoproteins (HDL), result in aortic valve stenosis (AVS) regression in experimental models. Severe AVS can be complicated by acquired von Willebrand syndrome, a haemorrhagic disorder associated with loss of high-molecular-weight von Willebrand factor (vWF) multimers (HMWM), the latter being a consequence of increased shear stress and enhanced vWF-cleaving protease (ADAMTS-13) activity. Although antithrombotic actions of HDL have been described, its effects on ADAMTS-13 and vWF in AVS are unknown.  We assessed ADAMTS-13 activity in plasma derived from a rabbit model of AVS ( n  = 29) as well as in plasma collected from 64 patients with severe AVS (age 65.0 ± 10.4 years, 44 males) undergoing aortic valve replacement (AVR). In both human and rabbit AVS plasma, ADAMTS-13 activity was higher than that in controls ( p  AVS patients had less HMWM than controls (66.3 ± 27.2% vs. 97.2 ± 24.1%, p  AVS rabbits as compared with the placebo group (2.0 ± 0.5 RFU/sec vs. 3.8 ± 0.4 RFU/sec, p  AVS ( r  = -0.3, p  = 0.045).  Our data indicate that HDL levels are associated with reduced ADAMTS-13 activity and increased HMWM. HDL-based therapies may reduce the haematologic abnormalities of the acquired von Willebrand syndrome in AVS. Schattauer GmbH Stuttgart.

  12. [Anesthetic Management of a Patient Complicated with Marfan Syndrome and Suffering from Stanford Type A Aortic Dissection during Pregnancy].

    Science.gov (United States)

    Uozaki, Nako; Mizuno, Kaori; Shiraishi, Yoshito; Doi, Matsuyuki; Sato, Shigehito

    2015-04-01

    We report a case of a 36-year-old woman at 34 weeks of gestation complicated with Marfan syndrome who underwent Bentall type aortic replacement surgery due to Stanford type A aortic dissection after undergoing caesarean section. Since this patient exhibited severe hypotension before coming to the operating room, it was very difficult to determine whether the cardiac surgery or caesarean section should be performed first. In this case, the caesarean section was performed first, followed by Bentall's surgery. Although intra-aortic balloon pumping and percutaneous cardiopulmonary support were required after weaning from the cardiopulmonary bypass, she was discharged on post-operative day (POD) 40 and the baby was discharged on POD 60, without signs of cerebral palsy. Unfortunately, this patient died on POD 57, due to heart failure. We discuss how to determine the priority of surgeries for patients who require emergency surgery for cardiovascular disease during pregnancy.

  13. Evidence of aortic dissection and Marfan syndrome in a mummy from the Capuchin Catacombs of Palermo, Sicily.

    Science.gov (United States)

    Panzer, Stephanie; Thompson, Randall C; Hergan, Klaus; Zink, Albert R; Piombino-Mascali, Dario

    2018-06-08

    The authors report on the assessment of an anthropogenic mummy of a young man from the Capuchin Catacombs of Palermo, Sicily, tentatively dated from the mid- to late 19 th century AD. The mummy was investigated by full-body CT examination. CT images clearly showed aortic dissection classified as Stanford-A. Due to the relation of aortic dissection to inherited connective tissue diseases in young people, such as Marfan syndrome, conspicuous and pathological findings possibly indicating the presence of underlying Marfan syndrome were assessed. Several systemic features were scored that supported the presence of underlying Marfan syndrome in this mummy. These findings were: pectus carinatum and chest asymmetry, dural ectasia, protrusio acetabuli, dolichocephaly, down-slanting palpebral fissures, malar hypoplasia and (probable) reduced elbow extension. Aortic dissection, a cardinal feature of Marfan syndrome, turned out to be the diagnostic key for the paleoradiological diagnosis of this disease. The demonstrated CT findings contribute to the spectrum of cardiovascular diseases and inherited connective tissue disease in the fields of paleopathology and paleoradiology. Copyright © 2018 Elsevier Inc. All rights reserved.

  14. Endovascular treatment of type B dissection in patients with Marfan syndrome: mid-term outcomes and aortic remodeling.

    Science.gov (United States)

    Eid-Lidt, Guering; Gaspar, Jorge; Meléndez-Ramírez, Gabriela; Cervantes S, Jorge; González-Pacheco, Hector; Dámas de Los Santos, Félix; Meave-González, Aloha; Ramírez Marroquín, Samuel

    2013-12-01

    To evaluate the mid-term outcomes, and the aortic remodeling in Marfan syndrome (MFS) patients with type B dissection that were treated with endovascular repair. MFS is a relative contraindication to thoracic endovascular aortic repair (TEVAR). Mid-term aortic outcomes data in MFS after TEVAR are limited, and the occurrence of late events remains unclear. Of 89 patients that underwent TEVAR between September 2002 and February 2011, 10 patients with mid-term follow-up fulfilled the Ghent criteria for MFS and complicated type B dissection. High risk for open surgery was documented in 90%. The mean age was 35.1 ± 9.4 years and all patients presented with acute aortic syndrome complicating a chronic type B dissection (DeBakey type IIIb). Five patients underwent a Bentall surgical procedure previous to endovascular repair, and in four patients initial TEVAR was followed by surgery of the ascending aorta. Treatment was limited to endovascular repair in only one patient. In-hospital mortality was 10%. At a mean follow-up of 59.6 ± 38.9 months, the cumulated mortality was of 20% and late mortality 11.1%. The rate of secondary endoleak was 44.4%, and late reintervention of 33.3%. Survival freedom from cardiovascular death at 8 years was 80.0%, and positive remodeling was documented in 37.5% of patients. Our results suggest that TEVAR is feasible, safe, and associated with a high reintervention rate and reduced rate of positive aortic remodeling in patients with Marfan syndrome. Survival at 8 years was comparable to contemporary series of open repair. Copyright © 2013 Wiley Periodicals, Inc.

  15. Estratégia cirúrgica na transposição das grandes artérias associada à obstrução do arco aórtico Surgical strategy in transposition of the great arteries with aortic arch obstruction

    Directory of Open Access Journals (Sweden)

    Bayard Gontijo Filho

    2007-06-01

    Full Text Available OBJETIVO: Analisar nossa experiência no tratamento cirúrgico da transposição das grandes artérias (TGA associada à obstrução do arco aórtico. MÉTODO: Entre janeiro de 1998 e dezembro de 2005, realizamos 223 operações de Jatene para correção de TGA: 21 (9,4% pacientes apresentavam obstruções do arco aórtico. A anatomia do arco aórtico evidenciou: coarctação da aorta localizada (n=10; coarctação com hipoplasia tubular do arco aórtico (n=6; interrupção do arco aórtico (n=5. Comunicação interventricular (CIV: 19 pacientes (90,5%, sendo 11 do tipo Taussig-Bing. Desproporção importante entre aorta e artéria pulmonar e anomalias coronárias foram achados freqüentes. Houve 7 correções em dois estágios e 14 correções em um único estágio. A reconstrução do arco foi realizada por ressecção e anastomose término-terminal ampliada (13 ou por translocação da aorta ascendente (8. RESULTADO: Houve cinco (23,8% óbitos hospitalares; apenas um (11,1% nos últimos nove casos consecutivos. Reoperações no período hospitalar: revisão de hemostasia (5, CIV residual + coarctação não identificada (1, estenose residual de arco aórtico (1. Após a alta, houve dois óbitos e três pacientes foram submetidos a reintervenções para estenose da via de saída do ventrículo direito. CONCLUSÃO: O tratamento da transposição das grandes artérias associada à obstrução do arco aórtico apresenta alta complexidade e morbi-mortalidade. Empregamos as correções em um e em dois estágios, obtendo resultados comparáveis. Nossa preferência atual é pela correção precoce em um único estágio para todos os pacientes, independente de sua configuração anatômica.OBJECTIVE:To analyze our experience in the surgical correction of transposition of the great arteries associated with aortic arch obstruction. METHOD: From January 1998 to December 2005 we performed 223 arterial switch operations for transposition of the great

  16. 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.

  17. 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.

  18. 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.

  19. Ortner's syndrome: a case report and literature review

    Energy Technology Data Exchange (ETDEWEB)

    Dutra, Bruno Landim; Campos, Lenilton da Costa; Marques, Helder de Castro; Vilela, Vagner Moyses, E-mail: brunolandim@yahoo.com.br [Universidade Federal de Juiz de Fora (HU/UFJF), MG (Brazil). Hospital Universitario. Unidade de Radiologia e Diagnostico por Imagem; Carvalho, Rodolfo Elias Diniz da Silva [Centro de Diagnostico Medico (CRM), Sao Mateus, ES (Brazil); Duque, Andre Geraldo da Silva [Axial Medicina Diagnostica, Belo Horizonte, MG (Brazil)

    2015-07-15

    The authors report the case of a 55-year-old female, hypertensive, smoker patient presenting with dysphonia, dysphagia and persistent dry cough. Laryngoscopy diagnosed left vocal cord paralysis. Computed tomography demonstrated saccular aneurysm of the inferior wall of the aortic arch, stretching the left recurrent laryngeal nerve, a finding compatible with Ortner's syndrome. (author)

  20. Ortner's syndrome: a case report and literature review

    International Nuclear Information System (INIS)

    Dutra, Bruno Landim; Campos, Lenilton da Costa; Marques, Helder de Castro; Vilela, Vagner Moyses; Duque, Andre Geraldo da Silva

    2015-01-01

    The authors report the case of a 55-year-old female, hypertensive, smoker patient presenting with dysphonia, dysphagia and persistent dry cough. Laryngoscopy diagnosed left vocal cord paralysis. Computed tomography demonstrated saccular aneurysm of the inferior wall of the aortic arch, stretching the left recurrent laryngeal nerve, a finding compatible with Ortner's syndrome. (author)

  1. A comparison of Selective Aortic Arch Perfusion and Resuscitative Endovascular Balloon Occlusion of the Aorta for the management of hemorrhage-induced traumatic cardiac arrest: A translational model in large swine.

    Directory of Open Access Journals (Sweden)

    Ed B G Barnard

    2017-07-01

    Full Text Available Survival rates remain low after hemorrhage-induced traumatic cardiac arrest (TCA. Noncompressible torso hemorrhage (NCTH is a major cause of potentially survivable trauma death. Resuscitative Endovascular Balloon Occlusion of the Aorta (REBOA at the thoracic aorta (Zone 1 can limit subdiaphragmatic blood loss and allow for IV fluid resuscitation when intrinsic cardiac activity is still present. Selective Aortic Arch Perfusion (SAAP combines thoracic aortic balloon hemorrhage control with intra-aortic oxygenated perfusion to achieve return of spontaneous circulation (ROSC when cardiac arrest has occurred.Male Yorkshire Landrace cross swine (80.0 ± 6.0 kg underwent anesthesia, instrumentation for monitoring, and splenectomy. TCA was induced by laparoscopic liver lobe resection combined with arterial catheter blood withdrawal to achieve a sustained systolic blood pressure <10 mmHg, cardiac arrest. After 3 min of arrest, swine were allocated to one of three interventions: (1 REBOA plus 4 units of IV fresh whole blood (FWB, (2 SAAP with oxygenated lactated Ringer's (LR, 1,600 mL/2 min, or (3 SAAP with oxygenated FWB 1,600 mL/2 min. Primary endpoint was survival to the end of 60 min of resuscitation, a simulated prehospital phase. Thirty animals were allocated to 3 groups (10 per group-5 protocol exclusions resulted in a total of 35 animals being used. Baseline measurements and time to cardiac arrest were not different amongst groups. ROSC was achieved in 0/10 (0%, 95% CI 0.00-30.9 REBOA, 6/10 (60%, 95% CI 26.2-87.8 SAAP-LR and 10/10 (100%, 95% CI 69.2-100.0 SAAP-FWB animals, p < 0.001. Survival to end of simulated 60-minute prehospital resuscitation was 0/10 (0%, 95% CI 0.00-30.9 for REBOA, 1/10 (10%, 95% CI 0.25-44.5 for SAAP-LR and 9/10 (90%, 95% CI 55.5-99.7 for SAAP-FWB, p < 0.001. Total FWB infusion volume was similar for REBOA (2,452 ± 0 mL and SAAP-FWB (2,250 ± 594 mL. This study was undertaken in laboratory conditions, and as such may have

  2. Occlusive ascending aorta and arch atheroma treated with deep hypothermic circulatory arrest and thromboendarterectomy.

    LENUS (Irish Health Repository)

    O' Sullivan, Katie E

    2013-12-01

    We describe an uncommon presentation of severely advanced aortic atherosclerosis in a 48-year old man with a history of hypertension and heavy smoking. Initial presentation with upper limb ischaemia led to the diagnosis of an aortic arch atheroma occluding 90% of the aortic lumen, managed with deep hypothermic circulatory arrest and aortic thromboendarterectomy. To our knowledge, this is the first reported case of atherosclerotic plaque resulting in aortic occlusion and requiring emergent operative intervention.

  3. Forbidden word entropy of cerebral oximetric values predicts postoperative neurocognitive decline in patients undergoing aortic arch surgery under deep hypothermic circulatory arrest

    Directory of Open Access Journals (Sweden)

    Anna Dubovoy

    2017-01-01

    Full Text Available Purpose: Up to 53% of cardiac surgery patients experience postoperative neurocognitive decline. Cerebral oximetry is designed to detect changes in cerebral tissue saturation and therefore may be useful to predict which patients are at risk of developing neurocognitive decline. Methods: This is a retrospective analysis of a prospective study originally designed to determine if treatment of cerebral oximetry desaturation is associated with improvement in postoperative cognitive dysfunction in patients undergoing aortic reconstruction under deep hypothermic circulatory arrest. Cognitive function was measured, preoperatively and 3 months postoperatively, with 15 neuropsychologic tests administered by a psychologist; the individual test scores were summed and normalized. Bilateral cerebral oximetry data were stored and analyzed using measures of entropy. Cognitive decline was defined as any decrease in the summed normalized score from baseline to 3 months. Results: Seven of 17 (41% patients suffered cognitive decline. There was no association between baseline cerebral oximetry and postoperative cognitive dysfunction. Nor were changes in oximetry values associated with cognitive decline. However, cognitive decline was associated with loss of forbidden word entropy (FwEn (correlation: Rho ρ = 0.51, P = 0.037 for left cerebral oximetry FwEn and ρ = 0.54, P = 0.025 for right cerebral oximetry FwEn. Conclusion: Postoperative cognitive decline was associated with loss of complexity of the time series as shown by a decrease in FwEn from beginning to end of the case. This suggests that regulation of cerebral oximetry is different between those who do and those who do not develop cognitive decline.

  4. Surgical repair of supravalvular aortic stenosis in children with williams syndrome: a 30-year experience.

    Science.gov (United States)

    Fricke, Tyson A; d'Udekem, Yves; Brizard, Christian P; Wheaton, Gavin; Weintraub, Robert G; Konstantinov, Igor E

    2015-04-01

    Williams syndrome is an uncommon genetic disorder associated with supravalvular aortic stenosis (SVAS) in childhood. We reviewed outcomes of children with Williams syndrome who underwent repair of SVAS during a 30-year period at a single institution. Between 1982 and 2012, 28 patients with Williams syndrome were operated on for SVAS. Mean age at operation was 5.2 years (range, 3 months to 13 years), and mean weight at operation was 18.6 kg (range, 4.1 to 72.4 kg). Associated cardiac lesions in 11 patients (39.3%) were repaired at the time of the SVAS repair. The most common associated cardiac lesion was main pulmonary artery stenosis (8 of 28 [28%]). A 3-patch repair was performed in 10 patients, a Doty repair in 17, and a McGoon repair in 1 (3.6%). There were no early deaths. Follow-up was 96% complete (27 of 28). Overall mean follow-up was 11.2 years (range, 1 month to 27.3 years). Mean follow-up was 5 years (range, 1 month to 14.3 years) for the 3-patch repair patients and 14.7 years (range, 6 weeks to 27 years) for the Doty repair patients. Of the 17 Doty patients, there were 4 (24%) late deaths, occurring at 6 weeks, 3.5 years, 4 years, and 16 years after the initial operation. There were no late deaths in the 3-patch repair patients. Overall survival was 86% at 5, 10, and 15 years after repair. Survival was 82% at 5, 10 and 15 years for the Doty repair patients. Overall, 6 of 27 patients (22%) patients required late reoperation at a mean of 11.2 years (range, 3.6 to 23 years). No 3-patch repair patients required reoperation. Overall freedom from reoperation was 91% at 5 years and 73% at 10 and 15 years. Freedom from reoperation for the Doty repair patients was 93% at 5 years and 71% at 10 and 15 years. Surgical repair of SVAS in children Williams syndrome has excellent early results. However, significant late mortality and morbidity warrants close follow-up. Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights

  5. 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.

  6. Cardiovascular Structure and Function in Children With Middle Aortic Syndrome and Renal Artery Stenosis.

    Science.gov (United States)

    Rumman, Rawan K; Slorach, Cameron; Hui, Wei; Matsuda-Abedini, Mina; Langlois, Valerie; Radhakrishnan, Seetha; Lorenzo, Armando J; Amaral, Joao; Mertens, Luc; Parekh, Rulan S

    2017-12-01

    Middle aortic syndrome (MAS) is a narrowing of the abdominal aorta, often in conjunction with renal artery stenosis (RAS). Structure and function of the cardiovascular system are not well understood. In a prospective cross-sectional study, 35 children with MAS or RAS or both (MAS/RAS) were compared with 140 age-, sex-, and body surface area-matched healthy children. Vascular assessment included carotid intima-media thickness and carotid distensibility using B-mode ultrasound and central and peripheral pulse wave velocities using applanation tonometry. Left ventricular structure and function were assessed by 2-dimensional and speckle-tracking echocardiography. Children with MAS or RAS were 12.5±3.0 years old at enrollment, and 50% were men. Carotid intima-media thickness (0.54±0.10 versus 0.44±0.05 mm; P function (lower E/a ratio and lower e' velocities). Systolic parameters, including ejection fraction, global longitudinal and circumferential strain, were similar to controls. Our findings demonstrate that children with MAS or RAS have evidence of carotid and left ventricular remodeling, without peripheral arterial involvement, which suggests a localized disease process. Left ventricular systolic function is preserved; however, subtle changes in diastolic function are observed. Carotid vessel changes are consistent with a 5- to 10-year aging, which underscores the importance of blood pressure control. © 2017 American Heart Association, Inc.

  7. Digital subtraction angiography in patients with Marfan's syndrome

    International Nuclear Information System (INIS)

    Rauber, K.; Riemann, H.

    1987-01-01

    Marfan's syndrome is a rare inborn error of metabolism. Marfan patients are prone to aneurysms of the ascending aorta and run a high risk of rupture of the aortic arch. The diameter of the aneurysm is the most important predictor of the risk and therefore the leading point for surgical interventions. IV and IA-DSA according to our experiences are simple and effective methods in pre- and postoperative evaluation of patients with the syndrome. (orig.) [de

  8. Digital subtraction angiography in patients with Marfan's syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Rauber, K; Riemann, H

    1987-06-01

    Marfan's syndrome is a rare inborn error of metabolism. Marfan patients are prone to aneurysms of the ascending aorta and run a high risk of rupture of the aortic arch. The diameter of the aneurysm is the most important predictor of the risk and therefore the leading point for surgical interventions. IV and IA-DSA according to our experiences are simple and effective methods in pre- and postoperative evaluation of patients with the syndrome.

  9. Neurodevelopmental Outcome in Children after Fetal Cardiac Intervention for Aortic Stenosis with Evolving Hypoplastic Left Heart Syndrome.

    Science.gov (United States)

    Laraja, Kristin; Sadhwani, Anjali; Tworetzky, Wayne; Marshall, Audrey C; Gauvreau, Kimberlee; Freud, Lindsay; Hass, Cara; Dunbar-Masterson, Carolyn; Ware, Janice; Lafranchi, Terra; Wilkins-Haug, Louise; Newburger, Jane W

    2017-05-01

    To characterize neurodevelopmental outcomes after fetal aortic valvuloplasty for evolving hypoplastic left heart syndrome and determine the risk factors for adverse neurodevelopment. Questionnaires were mailed to families of children who underwent fetal aortic valvuloplasty from 2000 to 2012, and medical records were reviewed retrospectively. The primary outcome was the General Adaptive Composite score of the Adaptive Behavior Assessment System Questionnaire-Second Edition. Other questionnaires included the Behavior Assessment System for Children, Behavior Rating Inventory of Executive Function, Ages and Stages, and Pediatric Quality of Life Inventory. Among 69 eligible subjects, 52 (75%) completed questionnaires at median age of 5.5 (range 1.3-12) years; 30 (58%) had biventricular status circulation. The General Adaptive Composite mean score (92 ± 17) was lower than population norms (P neurodevelopmental questionnaires (Behavior Assessment System for Children, Behavior Rating Inventory of Executive Function, Ages and Stages, Pediatric Quality of Life Inventory), most subscale scores for patients with biventricular and single ventricular status were similar. Children who underwent fetal aortic valvuloplasty have neurodevelopmental delay, similar to patients with hypoplastic left heart syndrome without fetal intervention. Achievement of biventricular circulation was not associated with better outcomes. We infer that innate patient factors and morbidity during infancy have the greatest effect on neurodevelopmental outcomes. Copyright © 2017 Elsevier Inc. All rights reserved.

  10. Successful management of aortic thrombi resulting in spinal cord infarction in a patient with antiphospholipid antibody syndrome and acute cholecystitis

    Directory of Open Access Journals (Sweden)

    Izumi M

    2011-12-01

    Full Text Available Manabu Izumi, Shoko Teraoka, Keisuke Yamashita, Kenji Matsumoto, Tomohiro Muronoi, Yoshimitsu Izawa, Chikara Yonekawa, Masaki Ano, Masayuki SuzukawaDepartment of Emergency and Critical Care Medicine, Jichi Medical University, Tochigi, JapanAbstract: A 74-year-old man with coronary artery disease was suffering from acute nonobstructive cholecystitis and was admitted to a nearby hospital. Dual antiplatelet (aspirin and ticlopidine therapy was discontinued before preparation for surgical resection of the gall bladder. During his time in hospital he was aware of lumbar pain and weakness in both legs. He was transferred to our hospital for further evaluation and therapy. Diffuse intra-aortic thrombi were revealed by computed tomography with contrast media, and magnetic resonance imaging showed spinal cord infarction. However, computed tomography scans of the descending aorta obtained 4 months before admission exhibited no signs of atherosclerotic plaques or intra-aortic thrombi. Laboratory data suggest that antiphospholipid antibody syndrome might have caused these acute multiple intra-arterial thrombi. By restarting dual antiplatelet therapy and increasing the dose of heparin (from 10,000 IU/day to 15,000 IU/day we successfully managed the patient's clinical condition and symptoms. It is important to understand that stopping antiplatelet therapy may rapidly grow thrombi in patients with a hypercoagulative state.Keywords: intra-aortic thrombus, antiphospholipid antibody syndrome, spinal cord infarction

  11. Anesthetic Implications of Emergent Cesarean Section in a Parturient with Marfan Syndrome Complicated by Ascending Aortic Aneurysm and Heart Failure

    Directory of Open Access Journals (Sweden)

    Young Sung Kim

    2014-07-01

    Full Text Available Cardiovascular comorbidities to the Marfan syndrome may induce hemodynamic instability especially in the parturients during labor or delivery. For anesthesiologists, it is challenging to maintain hemodynamic stability during Cesarean section in those patients with Marfan syndrome. Remifentanil is an ultra-short-acting opioid with rapid onset and offset of action which provides cardiovascular stability during surgery. Together with remifentanil, the use of a laryngeal mask airway can reduce the risk of hypertensive response followed by tracheal intubation. We describe the successful administration of remifentanil and application of laryngeal mask airway for emergent Cesarean section performed under general anesthesia in a patient with Marfan syndrome complicated by ascending aortic aneurysm and heart failure. The use of remifentanil (loading dose of 1 μg/kg for 1 min, 2 min before induction; thereafter continuous infusion dose of 0.1 μg/kg/min was useful to maintain hemodynamic stability of the parturient throughout the surgery without neonatal respiratory depression. Keywords: Ascending Aortic Aneurysm; Cesarean section; Laryngeal mask airway; Marfan syndrome; Remifentanil

  12. Enhanced caspase activity contributes to aortic wall remodeling and early aneurysm development in a murine model of Marfan syndrome.

    Science.gov (United States)

    Emrich, Fabian C; Okamura, Homare; Dalal, Alex R; Penov, Kiril; Merk, Denis R; Raaz, Uwe; Hennigs, Jan K; Chin, Jocelyn T; Miller, Miquell O; Pedroza, Albert J; Craig, Juliana K; Koyano, Tiffany K; Blankenberg, Francis G; Connolly, Andrew J; Mohr, Friedrich W; Alvira, Cristina M; Rabinovitch, Marlene; Fischbein, Michael P

    2015-01-01

    Rupture and dissection of aortic root aneurysms remain the leading causes of death in patients with the Marfan syndrome, a hereditary connective tissue disorder that affects 1 in 5000 individuals worldwide. In the present study, we use a Marfan mouse model (Fbn1(C1039G/+)) to investigate the biological importance of apoptosis during aneurysm development in Marfan syndrome. Using in vivo single-photon emission computed tomographic-imaging and ex vivo autoradiography for Tc99m-annexin, we discovered increased apoptosis in the Fbn1(C1039G/+) ascending aorta during early aneurysm development peaking at 4 weeks. Immunofluorescence colocalization studies identified smooth muscle cells (SMCs) as the apoptotic cell population. As biological proof of concept that early aortic wall apoptosis plays a role in aneurysm development in Marfan syndrome, Fbn1(C1039G/+) mice were treated daily from 2 to 6 weeks with either (1) a pan-caspase inhibitor, Q-VD-OPh (20 mg/kg), or (2) vehicle control intraperitoneally. Q-VD-OPh treatment led to a significant reduction in aneurysm size and decreased extracellular matrix degradation in the aortic wall compared with control mice. In vitro studies using Fbn1(C1039G/+) ascending SMCs showed that apoptotic SMCs have increased elastolytic potential compared with viable cells, mostly because of caspase activity. Moreover, in vitro (1) cell membrane isolation, (2) immunofluorescence staining, and (3) scanning electron microscopy studies illustrate that caspases are expressed on the exterior cell surface of apoptotic SMCs. Caspase inhibition attenuates aneurysm development in an Fbn1(C1039G/+) Marfan mouse model. Mechanistically, during apoptosis, caspases are expressed on the cell surface of SMCs and likely contribute to elastin degradation and aneurysm development in Marfan syndrome. © 2014 American Heart Association, Inc.

  13. ArchE - An Architecture Design Assistant

    Science.gov (United States)

    2007-08-02

    Architecture Design Assistant Len Bass August 2, 2007 Report Documentation Page Form ApprovedOMB No. 0704-0188 Public reporting burden for the...ArchE - An Architecture Design Assistant 5a. CONTRACT NUMBER 5b. GRANT NUMBER 5c. PROGRAM ELEMENT NUMBER 6. AUTHOR(S) 5d. PROJECT NUMBER 5e. TASK...X, Module X 3 Author / Presenter, Date if Needed What is ArchE? ArchE is a software architecture design assistant, which: • Takes quality and

  14. Relationship of metabolic syndrome with incident aortic valve calcium and aortic valve calcium progression: the Multi-Ethnic Study of Atherosclerosis (MESA).

    Science.gov (United States)

    Katz, Ronit; Budoff, Matthew J; Takasu, Junichiro; Shavelle, David M; Bertoni, Alain; Blumenthal, Roger S; Ouyang, Pamela; Wong, Nathan D; O'Brien, Kevin D

    2009-04-01

    Metabolic syndrome (MetS) has been associated with increased prevalence of aortic valve calcium (AVC) and with increased progression of aortic stenosis. The purpose of this study was to determine whether MetS is associated with increased risks for the development of new ("incident") AVC or for progression of established AVC as assessed by CT. The relationships of MetS or its components as well as of diabetes to risks for incident AVC or AVC progression were studied among participants with CT scans performed at baseline and at either year 2 or year 3 examinations in the Multi-Ethnic Study of Atherosclerosis (MESA). Of 5,723 MESA participants meeting criteria for inclusion, 1,674 had MetS by Adult Treatment Panel III criteria, whereas 761 had diabetes. Among the 5,123 participants without baseline AVC, risks for incident AVC, adjusted for time between scans, age, sex, race/ethnicity, LDL cholesterol, lipid-lowering medications, and smoking, were increased significantly for MetS (odds ratio [OR] 1.67 [95% CI 1.21-2.31]) or diabetes (2.06 [1.39-3.06]). In addition, there was an increase in incident AVC risk with increasing number of MetS components. Similar results were found using the International Diabetes Federation MetS criteria. Among the 600 participants (10.5%) with baseline AVC, neither MetS nor diabetes was associated with AVC progression. In the MESA cohort, MetS was associated with a significant increase in incident ("new") AVC, raising the possibility that MetS may be a potential therapeutic target to prevent AVC development.

  15. Aortic valve-sparing operation after correction of heart displacement due to pectus excavatum using Nuss procedure in a Marfan syndrome patient.

    Science.gov (United States)

    Fukunaga, Naoto; Yuzaki, Mitsuru; Hamakawa, Hiroshi; Nasu, Michihiro; Takahashi, Yutaka; Okada, Yukikatsu

    2012-01-01

    Cardiovascular surgery in the setting of chest wall deformities is a clinical challenge. Pectus excavatum, for example, can cause heart displacement to the left thoracic cavity, following the poor operative field. This report highlights a case in which a successful aortic valve-sparing operation via conventional median sternotomy after correction of the heart displacement due to pectus excavatum using Nuss procedure in Marfan syndrome. This technique can be one surgical option in Marfan syndrome patients with pectus excavatum and thoracic aortic aneurysm under close follow up.

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

    Science.gov (United States)

    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.

  17. 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.

  18. Proteção cerebral no tratamento cirúrgico dos aneurismas do arco aórtico: estudo experimental em cães Cerebral protection to be used during aortic arch aneurysms resection

    Directory of Open Access Journals (Sweden)

    Henrique Murad

    1993-09-01

    Full Text Available Os autores realizaram estudo experimental comparativo entre dois métodos de proteção cerebral utilizados na abordagem cirúrgica dos aneurismas do arco aórtico, avaliando a sua eficácia. Os métodos comparados foram a hipotermia sistêmica profunda isolada (menor que 20ºC com pinçamento arterial braquiocefálico e a hipotermia sistêmica profunda associada à perfusão carotídea seletiva. Dois grupos de 15 cães cada foram submetidos, respectivamente, a hipotermia sistêmica profunda com pinçamento arterial braquiocefálico (GRUPO I e a hipotermia sistêmica profunda associada a perfusão seletiva da carótida direita (GRUPO II. Foram colhidas amostras seriadas de sangue para análise das alterações metabólicas de pH e PaCO2 que ocorreram no retorno venoso cerebral, aferidas na veia jugular interna, bem como as alterações histopatológicas encontradas com 45 min, 90 min e 135 min de cada procedimento. Os resultados demonstraram que, apesar de ambos os métodos de proteção cerebral serem eficazes por um período de 45 minutos, o método utilizado no GRUPO II mostrou ser superior em períodos de até 90 minutos. Em períodos de 135 minutos os métodos tiveram resultados semelhantes, não oferecendo proteção cerebral adequada.The authors proposition is to make an experimental study of two methods of cerebral protection to be used during aortic arch aneurysm resection. The methods to be evaluated were profound systemic hypothermia (under 20oC with great vessels occlusion and profound systemic hypothermia with selective right carotid artery perfusion. Two groups of 15 dogs each were submitted either to profound systemic hypothermia with great vessels occlusion (GROUP I or to profound systemic hypothermia with selective right carotid artery perfusion (GROUP II. Serial jugular vein samples for pH and PaC02 were analyzed to evaluate ischemic cerebral metabolic derangements. Hystopathological studies were also made at 45, 90 and 135

  19. Hypoplastic left heart syndrome

    Directory of Open Access Journals (Sweden)

    Thiagarajan Ravi

    2007-05-01

    Full Text Available Abstract Hypoplastic left heart syndrome(HLHS refers to the abnormal development of the left-sided cardiac structures, resulting in obstruction to blood flow from the left ventricular outflow tract. In addition, the syndrome includes underdevelopment of the left ventricle, aorta, and aortic arch, as well as mitral atresia or stenosis. HLHS has been reported to occur in approximately 0.016 to 0.036% of all live births. Newborn infants with the condition generally are born at full term and initially appear healthy. As the arterial duct closes, the systemic perfusion becomes decreased, resulting in hypoxemia, acidosis, and shock. Usually, no heart murmur, or a non-specific heart murmur, may be detected. The second heart sound is loud and single because of aortic atresia. Often the liver is enlarged secondary to congestive heart failure. The embryologic cause of the disease, as in the case of most congenital cardiac defects, is not fully known. The most useful diagnostic modality is the echocardiogram. The syndrome can be diagnosed by fetal echocardiography between 18 and 22 weeks of gestation. Differential diagnosis includes other left-sided obstructive lesions where the systemic circulation is dependent on ductal flow (critical aortic stenosis, coarctation of the aorta, interrupted aortic arch. Children with the syndrome require surgery as neonates, as they have duct-dependent systemic circulation. Currently, there are two major modalities, primary cardiac transplantation or a series of staged functionally univentricular palliations. The treatment chosen is dependent on the preference of the institution, its experience, and also preference. Although survival following initial surgical intervention has improved significantly over the last 20 years, significant mortality and morbidity are present for both surgical strategies. As a result pediatric cardiologists continue to be challenged by discussions with families regarding initial decision

  20. Compliance of the normal-sized aorta in adolescents with Marfan syndrome: comparison of MR measurements of aortic distensibility and pulse wave velocity

    International Nuclear Information System (INIS)

    Eichhorn, J.G.; Ruediger, H.J.; Gorenflo, M.; Khalil, M.; Ulmer, H.; Krissak, R.; Kauczor, H.U.; Ley, S.; Universitaetsklinik Heidelberg; Arnold, R.; Universitaetskinderklinik Freiburg; Boese, J.; Siemens AG, Medical Solutions, Forchheim; Krug, R.; Fink, C.

    2007-01-01

    Purpose: To compare the aortic compliance of the normal-sized aorta of adolescents with Marfan syndrome and healthy controls using MR measurements of the aortic distensibility and pulse wave velocity. Materials and Methods: Fourteen patients (median age: 15 [9-21] years) and 11 healthy subjects (23 [12-32] years) were examined at 1.5 T. The MR protocol included 2D steady-state free precession (SSFP)-CINE MRI of the aortic distensibility and PC-MRI of the pulse wave velocity. All measurements were positioned perpendicular to the descending aorta at the level of the diaphragm for assessing the changes in the aortic cross-sectional areas and additionally above and below this plane for assessing the pulse wave velocity. In addition contrast-enhanced 3D-MR angiography was performed in adolescents with Marfan syndrome to exclude morphologic changes and to prove normal-sized aorta. Results: Compared with control subjects, adolescents with Marfan syndrome had significantly decreased distensibility and significantly increased pulse wave velocity (χ 2 -test, p = 0.0002) using an age-related non-linear regression analysis. The related aortic compliance was significantly decreased (χ 2 -test, p = 0.0002). There was a good correlation between the two methods (r = 0.86). A low intraobserver variability was found for both methods (≤ 2 %). (orig.)

  1. Digital subtraction angiography in patients with Marfan's syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Rauber, K.; Riemann, H.

    1987-06-01

    Marfan's syndrome is a rare inborn error of metabolism. Marfan patients are prone to aneurysms of the ascending aorta and run a high risk of rupture of the aortic arch. The diameter of the aneurysm is the most important predictor of the risk and therefore the leading point for surgical interventions. IV and IA-DSA according to our experiences are simple and effective methods in pre- and postoperative evaluation of patients with the syndrome.

  2. 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

  3. 合并有主动脉弓及升主动脉倒撕裂的DeBakeyⅢ型主动脉夹层腔内介入治疗的时机和效果%Timing of TEVAR treatment in DeBakeyⅢtype aortic dissection associated with arch and ascending aorta anti-tearing

    Institute of Scientific and Technical Information of China (English)

    王志伟; 管生; 王稼祥; 李震

    2013-01-01

    Objective To explore the timing and the efficacy of TEVAR treatment in DeBakeyⅢtype aortic dissection associated with arch and ascending aorta anti-tearing. Methods Clinical data of 10 patients with DeBakeyⅢaortic dissection associated with arch and ascending aorta anti-tearing from January 2010 to January 2013 were retrospectively analyzed. All the patients had ifnally stepped stent therapy on the basis of conservative treatment, were erplored according to the follow up results. Results One case conducted TEVAR treatment three days after admission because of poor blood pressure control, but died of sudden pericardial tamponade postoperative two days. After 4 weeks’ conservative treatment all of 9 patients were performed TEVAR, the postoperative patients recovered well and were followed up for 6 to 30 months without adverse events. Conclusions False lumen thrombosis in ascending aorta and aortic arch are stable and partially absorbed after four weeks with conservative management in the patients who have DeBakeyⅢaortic dissection with arch and ascending aorta anti-tearing, and then the aortic arch can provide a relatively stable anchoring zone in the proximal end for TEVAR.%目的:探讨主动脉腔内修复术(TEVAR)治疗合并有主动脉弓和(或)升主动脉倒撕裂的DeBakeyⅢ型主动脉夹层的手术时机及疗效。方法对2010年1月至2013年1月郑州大学第一附属医院介入科收治的10例DeBakeyⅢ型主动脉夹层、同时合并有主动脉弓和(或)升主动脉逆向撕裂患者,在保守治疗的基础上行介入覆膜支架治疗,结合随访情况,探讨对该类患者行主动脉腔内修复术(TEVAR)的可行性、治疗时机及效果。结果1例患者血压控制差,入院后第3天行TEVAR治疗,术后第2天突发心包填塞而死亡;余9例患者在保守治疗4周,CT血管造影(CTA)显示逆向撕裂假腔内血栓稳定并部分吸收后,均安全实施了常规TEVAR,术

  4. 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.

  5. 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

  6. Variants of the left aortic arch branches

    African Journals Online (AJOL)

    . Type C (Courtesy of Rutherford. ... angiographic studies. The patients were selected randomly irrespective of age. Sixty cases were ... aorta, proximal to the origin of the right innominate artery. Forty millil- itres of contrast (Jopamiron 300 mg).

  7. 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.

  8. Beals Syndrome

    Science.gov (United States)

    ... the syndrome. How does Beals syndrome compare with Marfan syndrome? People with Beals syndrome have many of the ... bone) and aortic enlargement problems as people with Marfan syndrome, and treatments for these problems are the same. ...

  9. 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.

  10. Inactivation of Bmp4 from the Tbx1 Expression Domain Causes Abnormal Pharyngeal Arch Artery and Cardiac Outflow Tract Remodeling

    Science.gov (United States)

    Nie, Xuguang; Brown, Christopher B.; Wang, Qin; Jiao, Kai

    2011-01-01

    Maldevelopment of outflow tract and aortic arch arteries is among the most common forms of human congenital heart diseases. Both Bmp4 and Tbx1 are known to play critical roles during cardiovascular development. Expression of these two genes partially overlaps in pharyngeal arch areas in mouse embryos. In this study, we applied a conditional gene inactivation approach to test the hypothesis that Bmp4 expressed from the Tbx1 expression domain plays a critical role for normal development of outflow tract and pharyngeal arch arteries. We showed that inactivation of Bmp4 from Tbx1-expressing cells leads to the spectrum of deformities resembling the cardiovascular defects observed in human DiGeorge syndrome patients. Inactivation of Bmp4 from the Tbx1 expression domain did not cause patterning defects, but affected remodeling of outflow tract and pharyngeal arch arteries. Our further examination revealed that Bmp4 is required for normal recruitment/differentiation of smooth muscle cells surrounding the PAA4 and survival of outflow tract cushion mesenchymal cells. PMID:21123999

  11. MR imaging of the thoracic aorta in patients with Marfan syndrome

    International Nuclear Information System (INIS)

    Sommerhoff, B.A.; Sechtem, U.P.; Schiller, N.B.; Higgins, C.B.

    1986-01-01

    Gated MR imaging was used to evaluate the thoracic aorta in 11 with Marfan syndrome, eight patients with aneurysm of the ascending aorta, and 20 healthy subjects. The aortic diameter was measured on transverse and sagittal images at the levels of the sinuses of Valsalva, the caudal portion of the ascending aorta, the prearch region, the middle arch, and the descending aorta. The sinus of Valsalva-prearch region aortic diameter ratio in patients with Marfan syndrome was significantly greater than in the two other groups, indicating the characteristic shape of the Maranoid aorta. MR imaging allows definitive measurements' of aortic dimensions and is a valuable noninvasive method for monitoring the course of aortic enlargement

  12. Surgical treatment of ascending aortic complications in Marfan syndrome: early and long-term outcomes.

    Science.gov (United States)

    Favaloro, Roberto R; Casabé, J Horacio; Segura, Mónica; Abud, José; Casas, Jorge; López, Claudio; Dulbecco, Eduardo; Raffaelli, Héctor

    2008-08-01

    Findings in 54 patients (mean age 39 years, range 18-66 years, 25% female) were analyzed. Of these patients, 21 had dissection of the ascending aorta (15 acute and six chronic) and 33 had aneurysm of the ascending aorta. Surgery was classified as emergency surgery in three cases, as urgent in 15, and as scheduled surgery in 36. The Bentall-De Bono procedure was performed in 39 patients, aortic valve reimplantation was carried out in nine, Cabrol's operation was performed in three, and a homograft was used in three. The mean diameter of the ascending aorta was 66.6 mm. Overall, in-hospital mortality was 3.7% (33.3% for emergency surgery vs. 2.8% for scheduled surgery; P< .001). During the mean follow-up period of 4 years (range, 2 months-14 years), seven patients died, including four who died due to type-B aortic dissection. The actuarial survival rate at 2, 5 and 10 years was 94%, 83% and 75%, respectively, with 88%, 67% and 43% of patients, respectively, not requiring reoperation. Elective aortic root replacement was associated with a low risk and a good survival rate.

  13. 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.

  14. 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.

  15. 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.

  16. Coexistence of bilateral first and second branchial arch anomalies

    Science.gov (United States)

    Thakur, J S; Shekar, Vidya; Saluja, Manika; Mohindroo, N K

    2013-01-01

    Branchial arch anomalies are one of the most common congenital anomalies that are usually unilateral and bilateral presentation is rare. The simultaneous presence of bilateral second branchial arch anomalies along with bilateral first arch anomalies is extremely rare, with only three such cases reported in the literature. We present two non-syndromic cases of coexisting bilateral first and second arch anomalies. Developmental anomalies of the branchial apparatus account for 17% of all paediatric cervical masses and are the most common type of congenital cervical mass. They usually present in the paediatric age group. About 96–97% of these anomalies are unilateral. Bilateral presentation is seen in 2–3% having a strong familial association. Congenital syndromes also have been associated with first and second branchial arch anomalies. Thorough clinical examination and investigations should be done to rule out these syndromes. PMID:23580675

  17. Técnica de perfusión selectiva cerebral vía subclavia para la corrección de patologías del arco aórtico Selective cerebral perfusion technique by subclavian approach for correction of aortic arch pathology

    Directory of Open Access Journals (Sweden)

    Alejandro Escobar

    2007-08-01

    Full Text Available Objetivo: describir y evaluar la técnica de perfusión subclavia para protección cerebral selectiva con hipotermia moderada. Métodos: estudio descriptivo de 19 pacientes, a quienes se les practicó corrección de disección o aneurisma del arco aórtico mediante la utilización de esta técnica. Resultados: entre septiembre de 2002 y septiembre de 2005, se intervinieron 19 pacientes. El 68,4% eran hombres, con un promedio de edad de 54,05 ± 13,53 años. La disección de aorta tipo A correspondió al 57,8%; el 42,2% restante tenía aneurismas en alguna porción de la aorta con compromiso del cayado. El 73,7% tenía insuficiencia valvular aórtica. El 47,3% se encontró en clase funcional I, el 31,5% en clase II y el 21% en clase III. El 36,8% requirió revascularización coronaria. El tiempo promedio de perfusión cerebral selectiva fue de 28,95 ± 8,73 minutos; la perfusión sistémica fue de 163,31 ± 32,15 minutos, el pinzamiento aórtico fue de 135,36 ± 34,48 minutos y la temperatura promedio fue de 27º ± 0,94º centígrados. Hubo tres defunciones. No ocurrieron complicaciones neurológicas definitivas. Conclusión: esta técnica puede ser estandarizada para cirugías electivas o emergentes. Es una técnica simple, reproducible, que permite períodos de tiempo más prolongados para la reconstrucción del arco aórtico sin producir isquemia del tejido cerebral, tiempos de circulación extracorpórea más cortos, pocas complicaciones por sangrado, disminución del riesgo de embolización cerebral anterógrada y un excelente resultado neurológico final.Objective: describe and evaluate the subclavian perfusion technique for selective cerebral protection with moderate hypothermia. Methods: descriptive study of 19 patients to whom correction of the dissection or aneurysm of the aortic arch through the utilization of this technique was practiced. Results: between September 2002 and September 2005, 19 patients were operated. 68.4% were men

  18. Traumatic Aortic Injury

    Directory of Open Access Journals (Sweden)

    Brianna Miner

    2016-09-01

    Full Text Available History of present illness: A 48-year-old male with unknown past medical history presents as a trauma after being hit by a car traveling approximately 25 miles per hour. On initial presentation, the patient is confused, combative, and not answering questions appropriately. The patient is hypotensive with a blood pressure of 68/40 and a heart rate of 50 beats per minute, with oxygen saturation at 96% on room air. FAST scan is positive for fluid in Morrison’s pouch, splenorenal space, and pericardial space. Significant findings: The initial chest x-ray showed an abnormal superior mediastinal contour (blue line, suggestive of a possible aortic injury. The CT angiogram showed extensive circumferential irregularity and outpouching of the distal aortic arch (red arrows compatible with aortic transection. In addition, there was a circumferential intramural hematoma, which extended through the descending aorta to the proximal infrarenal abdominal aorta (green arrow. There was also an extensive surrounding mediastinal hematoma extending around the descending aorta and supraaortic branches (purple arrows. Discussion: Traumatic aortic injury is a life-threatening event. The incidence of blunt thoracic aortic injury is low, between 1 to 2 percent of those patients with blunt thoracic trauma.1 However, approximately 80% of patients with traumatic aortic injury die at the scene.2 Therefore it is imperative to diagnose traumatic aortic injury in a timely fashion. The diagnosis can be difficult due to the non-specific signs and symptoms and other distracting injuries. Clinical suspicion should be based on the mechanism of the injury and the hemodynamic status of the patient. In any patient with blunt or penetrating trauma to the chest that is hemodynamically unstable, traumatic aortic injury should be on the differential. Chest x-ray can be used as a screening tool. A normal chest x-ray has a negative predictive value of approximately 97%. CTA chest is the

  19. 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)

  20. Treatment of a Chronic Aneurysmal Aortic Dissection in a Patient with Marfan Syndrome Using a Staged Hybrid Procedure and a Fenestrated Endograft

    International Nuclear Information System (INIS)

    Walkden, R. Miles; Morgan, Rob A.; Loftus, Ian; Thompson, Matt

    2008-01-01

    Patients with aneurysmal dissections involving both the thoracic and the abdominal aorta are particularly challenging to treat with endovascular techniques because of the natural communications at the level of the visceral arteries. We present the case of a patient with Marfan syndrome with an aneurysmal aortic dissection involving the thoracic and abdominal aorta who was treated by a combination of endografts, surgical bypass, and a fenestrated tube graft.

  1. 12 min/week of high-intensity interval training reduces aortic reservoir pressure in individuals with metabolic syndrome: a randomized trial.

    Science.gov (United States)

    Ramos, Joyce S; Dalleck, Lance C; Ramos, Maximiano V; Borrani, Fabio; Roberts, Llion; Gomersall, Sjaan; Beetham, Kassia S; Dias, Katrin A; Keating, Shelley E; Fassett, Robert G; Sharman, James E; Coombes, Jeff S

    2016-10-01

    Decreased aortic reservoir function leads to a rise in aortic reservoir pressure that is an independent predictor of cardiovascular events. Although there is evidence that high-intensity interval training (HIIT) would be useful to improve aortic reservoir pressure, the optimal dose of high-intensity exercise to improve aortic reservoir function has yet to be investigated. Therefore, this study compared the effect of different volumes of HIIT and moderate-intensity continuous training (MICT) on aortic reservoir pressure in participants with the metabolic syndrome (MetS). Fifty individuals with MetS were randomized into one of the following 16-week training programs: MICT [n = 17, 30 min at 60-70% peak heart rate (HRpeak), five times/week]; 4 × 4-min high-intensity interval training (4HIIT) (n = 15, 4 × 4 min bouts at 85-95% HRpeak, interspersed with 3 min of active recovery at 50-70% HRpeak, three times/week); and 1 × 4-min high-intensity interval training (1HIIT) (n = 18, 1 × 4 min bout at 85-95% HRpeak, three times/week). Aortic reservoir pressure was calculated from radial applanation tonometry. Although not statistically significant, there was a trend for a small-to-medium group × time interaction effect on aortic reservoir pressure, indicating a positive adaptation following 1HIIT compared with 4HIIT and MICT [F (2,46) = 2.9, P = 0.07, η = 0.06]. This is supported by our within-group analysis wherein only 1HIIT significantly decreased aortic reservoir pressure from pre to postintervention (pre-post: 1HIIT 33 ± 16 to 31 ± 13, P = 0.03; MICT 29 ± 9-28 ± 8, P = 0.78; 4HIIT 28 ± 10-30 ± 9 mmHg, P = 0.10). Three sessions of 4 min of high-intensity exercise per week (12 min/week) was sufficient to improve aortic reservoir pressure, and thus may be a time-efficient exercise modality for reducing cardiovascular risk in individuals with MetS.

  2. [Surgical treatment of cardiovascular manifestations of Marfan syndrome].

    Science.gov (United States)

    Fukada, J; Morishita, K; Kawaharada, N; Yamada, A; Baba, T; Harada, N; Abe, T

    2002-07-01

    The present study determines the effect of surgical treatment of cardiovascular manifestations of Marfan syndrome in 72 patients by 114 operations, during 34-year period. This therapy resulted in aortic root repair, aortic arch replacement, or both in 78, mitral valve repair in 9, descending thoracic aortic replacement in 14, thoracoabdominal aortic replacement in 10, and abdominal aortic replacement in 6, including total aortic replacement in 4 and nearly total aortic replacement in 4 patients. Fusiform aneurysms were present in the the ascending aorta in 37, the aortic arch in 2, the thoracoabdominal aorta in 2, and the abdominal aorta in 6 patients. Aortic dissection occurred in 40 (55.6%), including type A aortic dissection in 29 patients. Aortic root repair included separate valve-graft in 8, Bentall composite valve-graft in 25, composite valve-graft with button technique in 26, composite valve-graft with interposition graft technique in 10, and valve sparing procedure in 5 patients. The overall early (30-day) mortality was 7.9%. The early survival was 75% in separate valve-graft procedure and 99.2% in composite valve-graft procedure. Late coronary dehiscence did not occur in the patients with Bentall technique in which the reattachments of coronary ostia were performed in 2 layers, but occurred in 50% of patients with the coronary anastomoses in 1 layer. Aortic valve regurgitation relapsed in 2 of the 5 patients with valve sparing procedure. Event free rate for the patients with composite valve-graft using button technique was 81.1% at 10 years. There were 14 late deaths; dissection or rupture of the residual aorta, composite graft endocarditis and cardiac failure were the principle causes of late deaths. In conclusion, Marfan patients with cardiovascular diseases can undergo surgical treatment with a low operative risk and low morbidity. Although late endocarditis remains a serious problem, we believe that Marfan syndrome is a contraindication for valve

  3. Comparative Study of Cerebral Protection during Surgery of Thoracic Aortic Aneurysm

    OpenAIRE

    Sueda, Taijiro; Nomimura, Takayuki; Kagawa, Tetsuya; Morita, Satoru; Hayashi, Saiho; Orihashi, Kazumasa; Shikata, Hiroo; Ryuu, Gou; Hamanaka, Yoshiharu; Matsuura, Yuichiro; Kawaue, Yasushi; Kanehiro, Keiichi; Ishihara, Hiroshi

    1992-01-01

    During the past 5 years, 30 cases of thoracic aortic aneurysm were treated. Selective cerebral perfusion (SCP) and retrograde cerebral perfusion (RCP) were conducted for cerebral protection during aortic cross clamping. SCP was carried out in 5 cases of dissecting aneurysm (all Stanford type A, including a case of AAE) and 3 cases of arch aneurysm. RCP was conducted in 5 cases of dissecting aneurysm (4 Stanford type A; 1 Stanford type B with retrograde dissection) and 2 cases of aortic arch a...

  4. Determination of entry site for acute type A aortic dissection by initial enhanced CT-scan

    International Nuclear Information System (INIS)

    Mase, Takenori; Narumiya, Chihiro; Aoyama, Takahiko; Nagata, Yoshihisa

    2002-01-01

    Acute type A aortic dissection presents a surgical emergency because conservative therapy is not effective in the majority of instances. Enhanced CT-scan of the chest is commonly available and is considered to be an optimal diagnostic method for this disease. The operative strategy is to resect the primary tear to close the entry site of the aortic dissection and replace it with a tubular Dacron graft. Therefore, the existence of the entry site is important in determining the operative procedure. Based on the numerical value of the enhanced CT-scan inspection, the present study seeks to preoperatively identify the location of the presumed entry site in aortic dissection. From May 1996 to June 1999, 21 consecutive patients (Marfan's syndrome excluded) with acute type A aortic dissection underwent surgical treatment. Nineteen patients were preoperatively examined by enhanced CT-scan: 11 men and 8 women, with a mean age of 61 years. CT-scan slices used for early diagnosis were of the ascending aorta, aortic arch, descending aorta, and thoracoabdominal aorta. The largest diameters of the whole and true lumen were measured from cross-sectional aortic images with a personal computer, and the areas of the whole and true lumen were obtained by the manual tracing method. The true ratio was calculated for the largest diameter and area of the whole lumen. The nineteen patients were divided into two groups according to the location of the entry site based on the operating views. Seven patients with the entry site in the ascending aorta were classified as group A, and twelve patients with the entry site further in the aortic arch and descending aorta were classified as group B. Comparisons were performed by non-parametric analysis. Moreover, a discriminant analysis was applied to evaluate the classification between the two groups. The ratio of the largest diameter of the true lumen in group A at the level of the ascending and descending aorta was significantly greater than that

  5. Determination of entry site for acute type A aortic dissection by initial enhanced CT-scan

    Energy Technology Data Exchange (ETDEWEB)

    Mase, Takenori; Narumiya, Chihiro; Aoyama, Takahiko; Nagata, Yoshihisa [Aichi Medical Univ., Nagakute (Japan). School of Medicine

    2002-01-01

    Acute type A aortic dissection presents a surgical emergency because conservative therapy is not effective in the majority of instances. Enhanced CT-scan of the chest is commonly available and is considered to be an optimal diagnostic method for this disease. The operative strategy is to resect the primary tear to close the entry site of the aortic dissection and replace it with a tubular Dacron graft. Therefore, the existence of the entry site is important in determining the operative procedure. Based on the numerical value of the enhanced CT-scan inspection, the present study seeks to preoperatively identify the location of the presumed entry site in aortic dissection. From May 1996 to June 1999, 21 consecutive patients (Marfan's syndrome excluded) with acute type A aortic dissection underwent surgical treatment. Nineteen patients were preoperatively examined by enhanced CT-scan: 11 men and 8 women, with a mean age of 61 years. CT-scan slices used for early diagnosis were of the ascending aorta, aortic arch, descending aorta, and thoracoabdominal aorta. The largest diameters of the whole and true lumen were measured from cross-sectional aortic images with a personal computer, and the areas of the whole and true lumen were obtained by the manual tracing method. The true ratio was calculated for the largest diameter and area of the whole lumen. The nineteen patients were divided into two groups according to the location of the entry site based on the operating views. Seven patients with the entry site in the ascending aorta were classified as group A, and twelve patients with the entry site further in the aortic arch and descending aorta were classified as group B. Comparisons were performed by non-parametric analysis. Moreover, a discriminant analysis was applied to evaluate the classification between the two groups. The ratio of the largest diameter of the true lumen in group A at the level of the ascending and descending aorta was significantly greater than

  6. 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.

  7. Marfan syndrome: pathologic features of aneurysm with dissection and aortic dissection

    Directory of Open Access Journals (Sweden)

    R. K. Zhuraev

    2012-08-01

    Full Text Available The article presents the pathologic changes in the aortic wall in patients with MFS (three men at the age of 25, 27, 35 years operated on the aneurysm with dissection of the ascending aorta. Morphological changes in the middle of the shell of the aorta in patients with MFS were characterized by severe restructuring with profound irreversible alterative changes in all of its components. The pathological process captured media of the aorta throughout its entire length, not just in the areas of rupture and separation. The main pathomorphological signs of MFS were the focal accumulation of mucoid substances, ribbon-like nuclear-free zones, degenerative changes in smooth muscle cells, the formation of cystic media degeneration cavities, changes in elastic fibers - fragmentation, hyperelastosis, multiplication, thinning and straightening, areas of significant elastolysis. Multiple CMD was detected in all patients with MFS, the changes revealed the same type, but at different stages of the process.

  8. Cable strengthened arches

    NARCIS (Netherlands)

    Kamerling, M.W.

    2013-01-01

    The structural efficiency of arches, subjected to several variable loads, can be increased by strengthening these arches with cables. For these structures it can be necessary, especially in case the permanent load is small, to post-tension the cables to avoid any compression acting on the cables. A

  9. Association of Aortic Calcification on Plain Chest Radiography with Obstructive Coronary Artery Disease

    Energy Technology Data Exchange (ETDEWEB)

    Kang, Yeong Han; Chang, Jeong Ho [Dept. of Diagnostic Radiology, Daegu Catholic University Hospital, Daegu (Korea, Republic of); Park, Jong Sam [Dept. of Radiologic Tecnology, Daegu Health College, Daegu (Korea, Republic of)

    2009-03-15

    This study was conducted to determine an association between aortic calcification viewed on plain chest radiography and obstructive coronary artery disease. Retrospective review of all chest radiography obtained from consecutive patients undergoing coronary angiography. Chest PA images were reviewed by technical radiologist and radiologist. Considering the presence of aortic arch calcification, images were compared with the results of coronary angiography. In addition, the size of aortic arch calcification were divided into two groups - the smaller and the larger than 10 mm. Among the total 846 patients, the number of the patients with obstructive coronary artery disease is total 417 (88.3%) in males and 312 (83.4%) in females. Considering the presence of aortic arch calcification, the positive predictive value of relation between aortic arch calcification and obstructive coronary artery disease was 91.4% and the relative risk of the group with aortic arch calcification to the opposite group was 1.10. According to the size of aortic arch calcification and obstructive coronary artery disease, the positive predictive value was 91.9% and the relative risk between two groups was 1.04. This study shows that aortic calcification was closely associated with obstructive coronary artery disease. If the aortic calcification is notified on plain chest radiography, we strongly recommend to consult with doctor.

  10. Can Brain Natriuretic Peptides and Osteoprotegerin Serve As Biochemical Markers for the Detection of Aortic Pathology in Children and Adolescents with Turner Syndrome?

    Directory of Open Access Journals (Sweden)

    Meenal Mavinkurve

    2017-07-01

    Full Text Available Turner syndrome (TS is a chromosomal disorder that affects 1:2,000 females. It results from either the complete or partial loss of the X chromosome as well as other aberrations. Clinical features of TS include short stature, delayed puberty, and congenital cardiac malformations. TS children also have an increased prevalence of cardiometabolic risk factors, which predisposes them to complications like coronary artery disease, cerebrovascular-related deaths, and aortic dissection. Early cardiac imaging, such as echocardiography and cardiac magnetic resonance imaging, are recommended to detect underlying aortic pathology. However, these modalities are limited by cost, accessibility, and are operator dependent. In view of these shortcomings, alternative methods, like vascular biomarkers, are currently being explored. There are only a few studies that have examined the relationship between B-type natriuretic peptide (BNP, N-terminal pro BNP (NT pro-BNP, and osteoprotegerin (OPG and aortic disease in TS, and thus the data are only in proof-of-concept stages. Further meticulous longitudinal studies are required before BNP, NT pro-BNP, and OPG are used as vascular biomarkers for the detection of aortic disease in childhood and adolescent TS.

  11. 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 ...

  12. Mid-aortic syndrome with renovascular hypertension and multisystem involvement in a girl with familiar neurofibromatosis von Recklinghausen type 1.

    Science.gov (United States)

    Petrak, Borivoj; Bendova, Sarka; Seeman, Tomas; Klein, Tibor; Lisy, Jiri; Zatrapa, Tomas; Marikova, Tana

    2007-12-01

    Neurofibromatosis von Recklinghausen type 1 (NF1) is an autosomal dominant neurocutaneous disorder affecting one in 3 000-4 000 individuals. Mid-aortic syndrome (MAS) is a rare condition characterized by segmental narrowing of abdominal aorta and stenosis of its major branches - mainly renal arteries, including manifestation of renovascular hypertension. MAS can be caused by different diseases, including NF1. A 9 years old girl with primary diagnosis of NF1 combined with renovascular hypertension due to MAS, suffered of bilateral optic and chiasm glioma, pubertas praecox, speech disorder, light mental retardation and scoliosis. We have found a mutation in exone 34 of the NF1 gene (17q11.2). Her father has been also diagnosed with NF1 and hypertension developed at early age. He has the same mutation in exone 34 of NF1 gene. The girl is currently treated with conservative antihypertensive medication with positive effect. Bilateral optic and chiasm glioma are asymptomatic at the time and they had been without progress over period of time. Any vascular surgery, neurosurgical and oncological therapy are not indicated at the present time. This article is a summary of clinical findings in patient with NF1 due to NF1 gene mutation in exone 34. It confirms the importance of complex multidisciplinar approach to examination and taking care of NF1 patients and their families.

  13. Ultrastructural pathology of aortic dissections in patients with Marfan syndrome: Comparison with dissections in patients without Marfan syndrome

    NARCIS (Netherlands)

    Dingemans, Koert P.; Teeling, Peter; van der Wal, Allard C.; Becker, Anton E.

    2006-01-01

    Despite the discovery in 1990 that mutations in the fibrillin-1 gene cause the Marfan syndrome, the pathogenesis of the life-threatening dissections associated with this disease is far from elucidated. Both the massive number of known fibrillin-1 mutations that result in a heterogeneous patient

  14. Neutron induced teratogenesis and spermatogenesis inhibitor fertilysin induced fetal bis-diamine syndrome in the rat. An animal model for DiGeorge and CATCH22 syndromes

    International Nuclear Information System (INIS)

    Shoji, Shuneki

    2003-01-01

    To develop preventive and regenerative medicine measures and to clarify the effect of neutron-irradiation and Fertilysin on vasculogenesis and teratogenesis, we decided to investigate the pathogenesis of these abnormalities in this study and compare them to abnormalities reported in humans. Pregnant rats were exposed to graded doses of 14.1 MeV neutron irradiation or Fertilysin on day 10 of gestation. The rats were sacrificed on day 18 of gestation, examined for lethality and surviving fetuses, and were microdissected for malformations. Our studies showed that neutron irradiation of rats commonly induced abnormalities whose types included eye, limb and tail defects, transposition of the great arteries, riding aorta, right aortic arch and aortic arch anomalies. These results suggest that maternal exposure to neutron-irradiation may have caused DNA damage and neural crest deficiency in offspring. These results are similar to those found in animal models with Retinoic acid syndrome and human fetuses with DiGeorge syndrome, a condition considered as a pharyngeal arch syndrome related to a cephalic neurocristopathy. In addition, multi-organ malformations associated with the highest incidences of abnormal vasculogenesis, cardiac outflow tracts and aortic arch anomalies such as right aortic arch and aberrant subclavian artery were found to be consistently produced following maternal exposure to Fertilysin on day 10 of gestation. Evidently the crucial scenario for administering Fertilysin to cause the cardiovascular defects of all surviving fetuses, in which over 80% of the fetuses were persistent truncus arteriosus (PTA) and the remainder was tetralogy of Fallot (TOF), is 200 mg for day 10 of gestation. This corresponds in humans to approximately day 21 after conception. A mechanism involving DNA damage, disruption of neural crest cells and growth and transcription factors, as well as growth failure of the branchial arches from apoptosis and neurocristopathy of the third

  15. Neutron induced teratogenesis and spermatogenesis inhibitor fertilysin induced fetal bis-diamine syndrome in the rat. An animal model for DiGeorge and CATCH22 syndromes

    Energy Technology Data Exchange (ETDEWEB)

    Shoji, Shuneki [Hiroshima Univ., Research Institute for Radiation Biology and Medicine, Hiroshima (Japan)

    2003-07-01

    To develop preventive and regenerative medicine measures and to clarify the effect of neutron-irradiation and Fertilysin on vasculogenesis and teratogenesis, we decided to investigate the pathogenesis of these abnormalities in this study and compare them to abnormalities reported in humans. Pregnant rats were exposed to graded doses of 14.1 MeV neutron irradiation or Fertilysin on day 10 of gestation. The rats were sacrificed on day 18 of gestation, examined for lethality and surviving fetuses, and were microdissected for malformations. Our studies showed that neutron irradiation of rats commonly induced abnormalities whose types included eye, limb and tail defects, transposition of the great arteries, riding aorta, right aortic arch and aortic arch anomalies. These results suggest that maternal exposure to neutron-irradiation may have caused DNA damage and neural crest deficiency in offspring. These results are similar to those found in animal models with Retinoic acid syndrome and human fetuses with DiGeorge syndrome, a condition considered as a pharyngeal arch syndrome related to a cephalic neurocristopathy. In addition, multi-organ malformations associated with the highest incidences of abnormal vasculogenesis, cardiac outflow tracts and aortic arch anomalies such as right aortic arch and aberrant subclavian artery were found to be consistently produced following maternal exposure to Fertilysin on day 10 of gestation. Evidently the crucial scenario for administering Fertilysin to cause the cardiovascular defects of all surviving fetuses, in which over 80% of the fetuses were persistent truncus arteriosus (PTA) and the remainder was tetralogy of Fallot (TOF), is 200 mg for day 10 of gestation. This corresponds in humans to approximately day 21 after conception. A mechanism involving DNA damage, disruption of neural crest cells and growth and transcription factors, as well as growth failure of the branchial arches from apoptosis and neurocristopathy of the third

  16. The syndromic child and anaesthesia

    African Journals Online (AJOL)

    fourth and sixth arches give rise to the larynx and trachea, and ... Keywords: anaesthesia, syndrome, atlantoaxial joint, branchial arches, Down's syndrome, 22q11 deletion .... which causes proximal weakness and a high risk of malignant.

  17. Plasma Lactate Dehydrogenase Levels Predict Mortality in Acute Aortic Syndromes: A Diagnostic Accuracy and Observational Outcome Study.

    Science.gov (United States)

    Morello, Fulvio; Ravetti, Anna; Nazerian, Peiman; Liedl, Giovanni; Veglio, Maria Grazia; Battista, Stefania; Vanni, Simone; Pivetta, Emanuele; Montrucchio, Giuseppe; Mengozzi, Giulio; Rinaldi, Mauro; Moiraghi, Corrado; Lupia, Enrico

    2016-02-01

    In acute aortic syndromes (AAS), organ malperfusion represents a key event impacting both on diagnosis and outcome. Increased levels of plasma lactate dehydrogenase (LDH), a biomarker of malperfusion, have been reported in AAS, but the performance of LDH for the diagnosis of AAS and the relation of LDH with outcome in AAS have not been evaluated so far.This was a bi-centric prospective diagnostic accuracy study and a cohort outcome study. From 2008 to 2014, patients from 2 Emergency Departments suspected of having AAS underwent LDH assay at presentation. A final diagnosis was obtained by aortic imaging. Patients diagnosed with AAS were followed-up for in-hospital mortality.One thousand five hundred seventy-eight consecutive patients were clinically eligible, and 999 patients were included in the study. The final diagnosis was AAS in 201 (20.1%) patients. Median LDH was 424 U/L (interquartile range [IQR] 367-557) in patients with AAS and 383 U/L (IQR 331-460) in patients with alternative diagnoses (P < 0.001). Using a cutoff of 450 U/L, the sensitivity of LDH for AAS was 44% (95% confidence interval [CI] 37-51) and the specificity was 73% (95% CI 69-76). Overall in-hospital mortality for AAS was 23.8%. Mortality was 32.6% in patients with LDH ≥ 450 U/L and 16.8% in patients with LDH < 450 U/L (P = 0.006). Following stratification according to LDH quartiles, in-hospital mortality was 12% in the first (lowest) quartile, 18.4% in the second quartile, 23.5% in the third quartile, and 38% in the fourth (highest) quartile (P = 0.01). LDH ≥ 450 U/L was further identified as an independent predictor of death in AAS both in univariate and in stepwise logistic regression analyses (odds ratio 2.28, 95% CI 1.11-4.66; P = 0.025), in addition to well-established risk markers such as advanced age and hypotension. Subgroup analysis showed excess mortality in association with LDH ≥ 450 U/L in elderly, hemodynamically stable and in nonsurgically

  18. Rarity of isolated pulmonary embolism and acute aortic syndrome occurring outside of the field of view of dedicated coronary CT angiography

    International Nuclear Information System (INIS)

    Lee, Hwa Yeon; Song, In Sup; Yoo, Seung Min; Rho, Ji Young; Moon, Jae Youn; Kim, In Jai; Lim, Sang Wook; Sung, Jung Hoon; Cha, Dong Hun; White, Charles S.

    2011-01-01

    Background Although triple rule-out CT angiography (TRO) to simultaneously evaluate acute coronary syndrome (ACS), pulmonary embolism (PE), and acute aortic syndrome (AAS) is increasingly used in many institutions, TRO is inevitably associated with increased radiation exposure due to extended z-axis coverage compared with dedicated coronary CT angiography (DCTA). Purpose To determine the frequency of exclusion of findings of AAS, PE, and significant incidental non-cardiac pathology that may be the cause of acute chest pain when using a restricted DCTA field of view (FOV). Material and Methods We retrospectively reviewed CT images and charts of 103 patients with acute PE and 50 patients with AAS. Either non-ECG gated dedicated pulmonary or aortic CT angiography was performed using 16- or 64-slice multidetector CT (MDCT). We analyzed the incidence of isolated PE, AAS, or significant non-cardiac pathology outside of DCTA FOV (i.e. from tracheal carina to the base of heart). Results There were two cases of isolated PE (2/103, 1.9%) excluded from the FOV of DCTA. One case of PE was isolated to the subsegmental pulmonary artery in the posterior segment of the right upper lobe. In the second case, pulmonary embolism in the left main pulmonary artery was located out of the FOV of DCTA because the left main pulmonary artery was retracted upwardly by fibrotic scar in the left upper lobe due to prior tuberculosis. There was no case of AAS and significant non-cardiac pathology excluded from the FOV of DCTA. AAS (n = 50) consisted of penetrating atherosclerotic ulcer (n = 7), intramural hematoma (n = 5) and aortic dissection (n = 38). Conclusion As isolated PE, AAS, and significant non-cardiac pathology outside of the DCTA FOV rarely occur, DCTA may replace TRO in the evaluation of patients with non-specific acute chest pain and a low pre-test probability of PE or aortic dissection

  19. Rarity of isolated pulmonary embolism and acute aortic syndrome occurring outside of the field of view of dedicated coronary CT angiography

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Hwa Yeon; Song, In Sup (Dept. of Diagnostic Radiology Chung-Ang Univ. College of Medicine, Seoul (Korea, Republic of)); Yoo, Seung Min; Rho, Ji Young (Dept. of Diagnostic Radiology CHA Medical Univ. Hospital, Bundang (Korea, Republic of)), email: smyoo68@hanmail.net; Moon, Jae Youn; Kim, In Jai; Lim, Sang Wook; Sung, Jung Hoon; Cha, Dong Hun (Dept. of Cardiology CHA Medical Univ. Hospital, Bundang (Korea, Republic of)); White, Charles S. (Dept. of Diagnostic Radiology Univ. of Maryland, Baltimore (United States))

    2011-05-15

    Background Although triple rule-out CT angiography (TRO) to simultaneously evaluate acute coronary syndrome (ACS), pulmonary embolism (PE), and acute aortic syndrome (AAS) is increasingly used in many institutions, TRO is inevitably associated with increased radiation exposure due to extended z-axis coverage compared with dedicated coronary CT angiography (DCTA). Purpose To determine the frequency of exclusion of findings of AAS, PE, and significant incidental non-cardiac pathology that may be the cause of acute chest pain when using a restricted DCTA field of view (FOV). Material and Methods We retrospectively reviewed CT images and charts of 103 patients with acute PE and 50 patients with AAS. Either non-ECG gated dedicated pulmonary or aortic CT angiography was performed using 16- or 64-slice multidetector CT (MDCT). We analyzed the incidence of isolated PE, AAS, or significant non-cardiac pathology outside of DCTA FOV (i.e. from tracheal carina to the base of heart). Results There were two cases of isolated PE (2/103, 1.9%) excluded from the FOV of DCTA. One case of PE was isolated to the subsegmental pulmonary artery in the posterior segment of the right upper lobe. In the second case, pulmonary embolism in the left main pulmonary artery was located out of the FOV of DCTA because the left main pulmonary artery was retracted upwardly by fibrotic scar in the left upper lobe due to prior tuberculosis. There was no case of AAS and significant non-cardiac pathology excluded from the FOV of DCTA. AAS (n = 50) consisted of penetrating atherosclerotic ulcer (n = 7), intramural hematoma (n = 5) and aortic dissection (n = 38). Conclusion As isolated PE, AAS, and significant non-cardiac pathology outside of the DCTA FOV rarely occur, DCTA may replace TRO in the evaluation of patients with non-specific acute chest pain and a low pre-test probability of PE or aortic dissection

  20. Coffin-Siris syndrome with the rarest constellation of congenital cardiac defects: A case report with review of literature

    OpenAIRE

    Nemani, Lalita; Barik, Ramachandra; Patnaik, Amar Narayana; Mishra, Ramesh C; Rao, Amaresh M; Kapur, Pragati

    2014-01-01

    We report a case of type-A Coffin-Siris syndrome (CSS) with a unique constellation of congenital heart defects. A 17-year-old Indian boy was referred to our hospital for central cyanosis with features of right heart failure. The cardiac abnormalities included biventricular outflow tract obstruction, small atrial septal defect (ASD), subaortic ventricular septal defect, drainage of left superior venacava to left atrial appendage, and aortic arch anomaly. Patient underwent successful right vent...

  1. Islamic Architecture and Arch

    Directory of Open Access Journals (Sweden)

    Mohammed Mahbubur Rahman

    2015-01-01

    Full Text Available The arch, an essential architectural element since the early civilizations, permitted the construction of lighter walls and vaults, often covering a large span. Visually it was an important decorative feature that was trans-mitted from architectural decoration to other forms of art worldwide. In early Islamic period, Muslims were receiving from many civilizations, which they improved and re-introduced to bring about the Renaissance. Arches appeared in Mesopotamia, Indus, Egyptian, Babylonian, Greek and Assyrian civilizations; but the Romans applied the technique to a wide range of structures. The Muslims mastered the use and design of the arch, employed for structural and functional purposes, progressively meeting decorative and symbolic pur-poses. Islamic architecture is characterized by arches employed in all types of buildings; most common uses being in arcades. This paper discusses the process of assimilation and charts how they contributed to other civilizations.

  2. Intraoral gothic arch tracing.

    Science.gov (United States)

    Rubel, Barry; Hill, Edward E

    2011-01-01

    In order to create optimum esthetics, function and phonetics in complete denture fabrication, it is necessary to record accurate maxillo-mandibular determinants of occlusion. This requires clinical skill to establish an accurate, verifiable and reproducible vertical dimension of occlusion (VDO) and centric relation (CR). Correct vertical relation depends upon a consideration of several factors, including muscle tone, inter-dental arch space and parallelism of the ridges. Any errors made while taking maxillo-mandibular jaw relation records will result in dentures that are uncomfortable and, possibly, unwearable. The application of a tracing mechanism such as the Gothic arch tracer (a central bearing device) is a demonstrable method of determining centric relation. Intraoral Gothic arch tracers provide the advantage of capturing VDO and CR in an easy-to-use technique for practitioners. Intraoral tracing (Gothic arch tracing) is a preferred method of obtaining consistent positions of the mandible in motion (retrusive, protrusive and lateral) at a comfortable VDO.

  3. 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.

  4. RARE BRANCHIAL ARCH ANOMALIES

    Directory of Open Access Journals (Sweden)

    Jayanta Kumar

    2016-03-01

    Full Text Available AIM Amongst the branchial arch anomalies third arch anomaly occurs rarely and more so the fourth arch anomalies. We present our experience with cases of rare branchial arch anomalies. PATIENTS AND METHODS From June 2006 to January 2016, cases having their external opening in the lower third of sternocleidomastoid muscle with the tract going through thyroid gland and directing to pyriform sinus (PFS or cysts with internal opening in the PFS were studied. RESULTS No fourth arch anomaly was encountered. One cyst with internal opening which later on formed a fistula, three fistulae from beginning and two sinuses were encountered. The main stay of diagnosis was the fistula in the PFS and the tract lying posterior to the internal carotid artery. Simple excision technique with a small incision around the external opening was done. There was no recurrence. CONCLUSION Third arch fistula is not very rare as it was thought. Internal fistula is found in most of the cases. Though radiological investigations are helpful, fistulae can be diagnosed clinically and during operation. Extensive operation of the neck, mediastinum and pharynx is not required.

  5. Stepwise Total Aortic Repairs With Fenestrated Endografts in a Patient With Loeys-Dietz Syndrome.

    Science.gov (United States)

    Hashizume, Kenichi; Shimizu, Hideyuki; Honda, Masanori; Inoue, Shinya; Takaki, Hidenobu; Hayashi, Kanako; Kaneyama, Hiroaki

    2017-07-01

    Loeys-Dietz syndrome (LDS) is a rare connective tissue disorder (CTD) caused by mutations in the gene encoding transforming growth factor-β receptors Ⅰ and Ⅱ. Patients with LDS manifest spontaneous aneurysms and dissections of the aorta and peripheral artery. We report a successful treatment with a hybrid endovascular repair for a rapidly expanding thoracoabdominal aneurysm in a 41-year-old woman affected by LDS. To overcome the difficulties of anatomical and surgical repair, we applied an original strategy using surgeon-modified fenestrated endografts. Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  6. Coffin-Siris syndrome with the rarest constellation of congenital cardiac defects: A case report with review of literature

    Directory of Open Access Journals (Sweden)

    Lalita Nemani

    2014-01-01

    Full Text Available We report a case of type-A Coffin-Siris syndrome (CSS with a unique constellation of congenital heart defects. A 17-year-old Indian boy was referred to our hospital for central cyanosis with features of right heart failure. The cardiac abnormalities included biventricular outflow tract obstruction, small atrial septal defect (ASD, subaortic ventricular septal defect, drainage of left superior venacava to left atrial appendage, and aortic arch anomaly. Patient underwent successful right ventricular infundibular resection, subaortic membrane resection, closure of atrial and ventricular septal defect, rerouting left superior vena cava to left pulmonary artery and aortic valve replacement.

  7. Coffin-Siris syndrome with the rarest constellation of congenital cardiac defects: A case report with review of literature.

    Science.gov (United States)

    Nemani, Lalita; Barik, Ramachandra; Patnaik, Amar Narayana; Mishra, Ramesh C; Rao, Amaresh M; Kapur, Pragati

    2014-09-01

    We report a case of type-A Coffin-Siris syndrome (CSS) with a unique constellation of congenital heart defects. A 17-year-old Indian boy was referred to our hospital for central cyanosis with features of right heart failure. The cardiac abnormalities included biventricular outflow tract obstruction, small atrial septal defect (ASD), subaortic ventricular septal defect, drainage of left superior venacava to left atrial appendage, and aortic arch anomaly. Patient underwent successful right ventricular infundibular resection, subaortic membrane resection, closure of atrial and ventricular septal defect, rerouting left superior vena cava to left pulmonary artery and aortic valve replacement.

  8. 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.

  9. An alternative surgical approach for the combined treatment of pectus excavatum and acute aortic dissection type-A in Marfan syndrome.

    Science.gov (United States)

    Schwill, Simon; Kallenbach, Klaus; Beller, Carsten J; Karck, Matthias

    2011-04-01

    Acute aortic dissection type-A (AADA) is a life-threatening condition especially in patients with Marfan syndrome (MFS) simultaneously suffering from severe pectus excavatum (PE). We report on emergency surgery for combined treatment of PE and AADA in a patient with MFS using an alternative approach. It leads to excellent exposure of the dislocated heart and great vessels enabling Bentall procedure followed by funnel chest repair with modified technique of Adkins and Blades. We achieved favorable functional and cosmetic results. Therefore, we conclude the surgical approach presented is feasible for standard treatment of AADA and consecutive repair of PE.

  10. Repair of left coronary artery aneurysm, recurrent ascending aortic aneurysm, and mitral valve prolapse 19 years after Bentall's procedure in a patient with Marfan syndrome.

    Science.gov (United States)

    Badmanaban, Balaji; Mallon, Peter; Campbell, Norman; Sarsam, Mazin A I

    2004-01-01

    A 45-year-old female with Marfan syndrome had a Bentall's procedure performed 19 years ago. She presented with a 4-year history of gradually worsening dyspnea and decreasing exercise tolerance. Investigations revealed severe mitral valve prolapse, a left main stem coronary artery (LMSCA) aneurysm, and a recurrent aneurysm of the ascending aorta. The mitral valve was replaced and the aortic aneurysmal sac and the LMSCA aneurysm were then repaired by a modified Bentall procedure. The patient made an uneventful recovery and was discharged home.

  11. 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.

  12. 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

  13. Glossary to ARCH (GARCH)

    DEFF Research Database (Denmark)

    Bollerslev, Tim

    The literature on modeling and forecasting time-varying volatility is ripe with acronyms and abbreviations used to describe the many different parametric models that have been put forth since the original linear ARCH model introduced in the seminal Nobel Prize winning paper by Engle (1982......).  The present paper provides an easy-to-use encyclopedic reference guide to this long list of ARCH acronyms.  In addition to the acronyms associated with specific parametric models, I have also included descriptions of various abbreviations associated with more general statistical procedures and ideas...

  14. Clarifying the anatomy of the fifth arch artery

    Directory of Open Access Journals (Sweden)

    Saurabh Kumar Gupta

    2016-01-01

    Full Text Available The artery allegedly forming in the fifth pharyngeal arch has increasingly been implicated as responsible for various vascular malformations in patients with congenitally malformed hearts. Observations from studies on developing embryos, however, have failed to provide support to substantiate several of these inferences such that the very existence of the fifth arch artery remains debatable. To the best of our knowledge, in only a solitary human embryo has a vascular channel been found that truly resembled the artery of the fifth arch. Despite the meager evidence to support its existence, the fifth arch artery has been invoked to explain the morphogenesis of double-barreled aorta, some unusual forms of aortopulmonary communications, and abnormalities of the brachiocephalic arteries. In most of these instances, the interpretations have proved fallible when examined in the light of existing knowledge of cardiac development. In our opinion, there are more plausible alternative explanations for the majority of these descriptions. Double-barreled aorta is more likely to result from retention of the recently identified dorsal collateral channels while abnormalities of brachiocephalic arteries are better explained on the basis of extensive remodeling of aortic arches during fetal development. Some examples of aortopulmonary communications, nonetheless, may well represent persistence of the developing artery of the fifth pharyngeal arch. We here present one such case - a patient with tetralogy of Fallot and pulmonary atresia, in whom the fifth arch artery provided a necessary communication between the ascending aorta and the pulmonary arteries. In this light, we discuss the features we consider to be essential before attaching the tag of "fifth arch artery" to a candidate vascular channel.

  15. Exact monitoring of aortic diameters in Marfan patients without gadolinium contrast: intraindividual comparison of 2D SSFP imaging with 3D CE-MRA and echocardiography

    International Nuclear Information System (INIS)

    Veldhoen, Simon; Behzadi, Cyrus; Derlin, Thorsten; Henes, Frank Oliver; Adam, Gerhard; Bannas, Peter; Rybczinsky, Meike; Kodolitsch, Yskert von; Sheikhzadeh, Sara; Bley, Thorsten Alexander

    2015-01-01

    To assess whether ECG-gated non-contrast 2D steady-state free precession (SSFP) imaging allows for exact monitoring of aortic diameters in Marfan syndrome (MFS) patients using non-ECG-gated contrast-enhanced 3D magnetic resonance angiography (CE-MRA) and echocardiography for intraindividual comparison. Non-ECG-gated CE-MRA and ECG-gated non-contrast SSFP at 1.5 T were prospectively performed in 50 patients. Two readers measured aortic diameters on para-sagittal images identically aligned with the aortic arch at the sinuses of Valsalva, sinotubular junction, ascending/descending aorta and aortic arch. Image quality was assessed on a three-point scale. Aortic root diameters acquired by echocardiography were used as reference. Intra- and interobserver variances were smaller for SSFP at the sinuses of Valsalva (p = 0.002; p = 0.002) and sinotubular junction (p = 0.014; p = 0.043). Image quality was better in SSFP than in CE-MRA at the sinuses of Valsalva (p < 0.0001), sinotubular junction (p < 0.0001) and ascending aorta (p = 0.02). CE-MRA yielded higher diameters than SSFP at the sinuses of Valsalva (mean bias, 2.5 mm; p < 0.0001), and comparison with echocardiography confirmed a higher bias for CE-MRA (7.2 ± 3.4 mm vs. SSFP, 4.7 ± 2.6 mm). ECG-gated non-contrast 2D SSFP imaging provides superior image quality with higher validity compared to non-ECG-gated contrast-enhanced 3D imaging. Since CE-MRA requires contrast agents with potential adverse effects, non-contrast SSFP imaging is an appropriate alternative for exact and riskless aortic monitoring of MFS patients. (orig.)

  16. Exact monitoring of aortic diameters in Marfan patients without gadolinium contrast: intraindividual comparison of 2D SSFP imaging with 3D CE-MRA and echocardiography

    Energy Technology Data Exchange (ETDEWEB)

    Veldhoen, Simon [University Medical Center Wuerzburg, Department of Diagnostic and Interventional Radiology, Bavaria (Germany); University Medical Center Hamburg-Eppendorf, Department of Diagnostic and Interventional Radiology, Hamburg (Germany); Behzadi, Cyrus; Derlin, Thorsten; Henes, Frank Oliver; Adam, Gerhard; Bannas, Peter [University Medical Center Hamburg-Eppendorf, Department of Diagnostic and Interventional Radiology, Hamburg (Germany); Rybczinsky, Meike; Kodolitsch, Yskert von; Sheikhzadeh, Sara [University Medical Center Hamburg-Eppendorf, Department of General and Interventional Cardiology, Hamburg (Germany); Bley, Thorsten Alexander [University Medical Center Wuerzburg, Department of Diagnostic and Interventional Radiology, Bavaria (Germany)

    2014-10-15

    To assess whether ECG-gated non-contrast 2D steady-state free precession (SSFP) imaging allows for exact monitoring of aortic diameters in Marfan syndrome (MFS) patients using non-ECG-gated contrast-enhanced 3D magnetic resonance angiography (CE-MRA) and echocardiography for intraindividual comparison. Non-ECG-gated CE-MRA and ECG-gated non-contrast SSFP at 1.5 T were prospectively performed in 50 patients. Two readers measured aortic diameters on para-sagittal images identically aligned with the aortic arch at the sinuses of Valsalva, sinotubular junction, ascending/descending aorta and aortic arch. Image quality was assessed on a three-point scale. Aortic root diameters acquired by echocardiography were used as reference. Intra- and interobserver variances were smaller for SSFP at the sinuses of Valsalva (p = 0.002; p = 0.002) and sinotubular junction (p = 0.014; p = 0.043). Image quality was better in SSFP than in CE-MRA at the sinuses of Valsalva (p < 0.0001), sinotubular junction (p < 0.0001) and ascending aorta (p = 0.02). CE-MRA yielded higher diameters than SSFP at the sinuses of Valsalva (mean bias, 2.5 mm; p < 0.0001), and comparison with echocardiography confirmed a higher bias for CE-MRA (7.2 ± 3.4 mm vs. SSFP, 4.7 ± 2.6 mm). ECG-gated non-contrast 2D SSFP imaging provides superior image quality with higher validity compared to non-ECG-gated contrast-enhanced 3D imaging. Since CE-MRA requires contrast agents with potential adverse effects, non-contrast SSFP imaging is an appropriate alternative for exact and riskless aortic monitoring of MFS patients. (orig.)

  17. How to Perfuse: Concepts of Cerebral Protection during Arch Replacement

    Directory of Open Access Journals (Sweden)

    Andreas Habertheuer

    2015-01-01

    Full Text Available Arch surgery remains undoubtedly among the most technically and strategically challenging endeavors in cardiovascular surgery. Surgical interventions of thoracic aneurysms involving the aortic arch require complete circulatory arrest in deep hypothermia (DHCA or elaborate cerebral perfusion strategies with varying degrees of hypothermia to achieve satisfactory protection of the brain from ischemic insults, that is, unilateral/bilateral antegrade cerebral perfusion (ACP and retrograde cerebral perfusion (RCP. Despite sophisticated and increasingly individualized surgical approaches for complex aortic pathologies, there remains a lack of consensus regarding the optimal method of cerebral protection and circulatory management during the time of arch exclusion. Many recent studies argue in favor of ACP with various degrees of hypothermic arrest during arch reconstruction and its advantages have been widely demonstrated. In fact ACP with more moderate degrees of hypothermia represents a paradigm shift in the cardiac surgery community and is widely adopted as an emergent strategy; however, many centers continue to report good results using other perfusion strategies. Amidst this important discussion we review currently available surgical strategies of cerebral protection management and compare the results of recent European multicenter and single-center data.

  18. How to Perfuse: Concepts of Cerebral Protection during Arch Replacement

    Science.gov (United States)

    Habertheuer, Andreas; Wiedemann, Dominik; Kocher, Alfred; Laufer, Guenther; Vallabhajosyula, Prashanth

    2015-01-01

    Arch surgery remains undoubtedly among the most technically and strategically challenging endeavors in cardiovascular surgery. Surgical interventions of thoracic aneurysms involving the aortic arch require complete circulatory arrest in deep hypothermia (DHCA) or elaborate cerebral perfusion strategies with varying degrees of hypothermia to achieve satisfactory protection of the brain from ischemic insults, that is, unilateral/bilateral antegrade cerebral perfusion (ACP) and retrograde cerebral perfusion (RCP). Despite sophisticated and increasingly individualized surgical approaches for complex aortic pathologies, there remains a lack of consensus regarding the optimal method of cerebral protection and circulatory management during the time of arch exclusion. Many recent studies argue in favor of ACP with various degrees of hypothermic arrest during arch reconstruction and its advantages have been widely demonstrated. In fact ACP with more moderate degrees of hypothermia represents a paradigm shift in the cardiac surgery community and is widely adopted as an emergent strategy; however, many centers continue to report good results using other perfusion strategies. Amidst this important discussion we review currently available surgical strategies of cerebral protection management and compare the results of recent European multicenter and single-center data. PMID:26713319

  19. Substituição do arco aórtico sem parada circulatória total: técnicas, táticas e resultados Substitution of the aortic arch without total circulatory arrest: techniques, tactics and results

    Directory of Open Access Journals (Sweden)

    Fernando Antônio Roquette REIS FILHO

    2001-09-01

    Full Text Available INTRODUÇÃO: A despeito dos avanços da cirurgia cardiovascular, das técnicas de circulação extracorpórea e dos métodos de proteção cerebral, a mortalidade nas operações de substituição ou reparo do arco aórtico permanece elevada. As alterações decorrentes da hipotermia profunda e as lesões neurológicas ainda são a maior causa de morbi-mortalidade. OBJETIVO: Demonstrar um conjunto de técnicas e táticas cirúrgicas que permite realizar a substituição do arco aórtico sem a necessidade de hipotermia e parada circulatória total e apresentar os resultados alcançados em um grupo de 10 pacientes. CASUÍSTICA E MÉTODOS: Dez pacientes do sexo masculino, com idade média de 48,7 anos, foram submetidos a substituição do arco aórtico, utilizando-se hipotermia moderada, canulação arterial em artéria subclávia direita e femoral esquerda, perfusão cerebral seletiva pela artéria subclávia e confecção da anastomose dos vasos arco no primeiro tempo. RESULTADOS: O tempo médio de perfusão cerebral seletiva foi de 14,1 minutos, de isquemia miocárdica 39,6 minutos e de circulação extracorpórea 98,9 minutos. A temperatura esofágica média foi de 26,6º C (24º C a 30º C. O tempo médio de internação foi de 18,4 dias (8 a 40. Não ocorreram óbitos imediatos ou tardios. Dois pacientes apresentaram confusão mental temporária e um apresentou hemiparesia que foi revertida. CONCLUSÕES: A combinação de técnicas e táticas empregadas permitiu uma efetiva correção das lesões em um campo cirúrgico amplo e seco. Ofereceu uma excelente proteção cerebral sem a necessidade de hipotermia profunda o que diminuiu substancialmente as complicações pós-operatórias.INTRODUCTION: Despite the development in cardiovascular techniques such as in cardiopulmonary bypass and cerebral protection the mortality in the correlations of the diseases of the aortic arch remains high. Deep hypothermic circulatory arrest and cardiopulmonary

  20. Imaging of acute aortic diseases; L'imagerie de la pathologie aortique aigue

    Energy Technology Data Exchange (ETDEWEB)

    Semlali, S.; Ennafae, I.; Mahi, M.; Benaissa, L.; Hanine, A.; Akjouj, S.; Jidal, M.; Chaouir, S. [Service d' imagerie medicale, hopital militaire Mohamed V, CHU, Rabat (Morocco)

    2010-09-15

    We report a review of computed tomography (CT) and magnetic resonance (MR) imaging findings in acute aortic syndrome. Contrast-enhanced multidetector CT is a highly accurate imaging method for determining the cause of acute aortic syndrome. (authors)

  1. Partial subclavian steal syndrome in a congenitally anomalous subclavian artery

    International Nuclear Information System (INIS)

    Krnic, A.; Sucic, Z.; Vucic, N.; Krolo, I.

    2006-01-01

    Background. A subclavian steal syndrome results from the abnormal flow of blood due to the occlusion in the subclavian artery proximal to the origin of the vertebral artery. A case of a male patient with a partial subclavian steal syndrome is presented. Case report. The syndrome was caused by a stenotic lesion of an aberrant right subclavian artery (the so called lusorian artery). The partial subclavian steal was recognized using the duplex ultrasound which showed the to and fro pattern in the right vertebral artery. Angiography of the aortic arch revealed the arterial anomaly. In our case, duplex ultrasound was a crucial method in diagnosing the partial subclavian steal syndrome. However, in order to show the arterial anomaly, the final evaluation had to be performed using arteriography. Conclusions. The early recognized partial subclavian steal syndrome provides good understanding of patient's symptoms, successful follow up, and a variety of treatment options. (author)

  2. Electrothermally Tunable Arch Resonator

    KAUST Repository

    Hajjaj, Amal Z.

    2017-03-18

    This paper demonstrates experimentally, theoretically, and numerically a wide-range tunability of electrothermally actuated microelectromechanical arch beams. The beams are made of silicon and are intentionally fabricated with some curvature as in-plane shallow arches. An electrothermal voltage is applied between the anchors of the beam generating a current that controls the axial stress caused by thermal expansion. When the electrothermal voltage increases, the compressive stress increases inside the arch beam. This leads to an increase in its curvature, thereby increasing its resonance frequencies. We show here that the first resonance frequency can increase monotonically up to twice its initial value. We show also that after some electrothermal voltage load, the third resonance frequency starts to become more sensitive to the axial thermal stress, while the first resonance frequency becomes less sensitive. These results can be used as guidelines to utilize arches as wide-range tunable resonators. Analytical results based on the nonlinear Euler Bernoulli beam theory are generated and compared with the experimental data and the results of a multi-physics finite-element model. A good agreement is found among all the results. [2016-0291

  3. [Loeys-Dietz syndrome (TGFβR2 mutation) in a 4-year-old child with thoracic aortic aneurysm].

    Science.gov (United States)

    De Potter, M-J; Edouard, T; Amadieu, R; Plaisancié, J; Julia, S; Hadeed, K; Hascoët, S; Acar, P; Dulac, Y

    2016-05-01

    Loeys-Dietz syndrome is a rare form of connective tissue disorder, whose clinical features can resemble those of Marfan syndrome, but with a more unpolished appearance. Recently brought out, this pathology remains little known; however, its consequences may be dramatic. We report on the case of a 4-year-old girl followed for a congenital hip dislocation, in which a systematic exam found increased cutaneous elasticity and a bifid uvula, suggesting a connective tissue disorder. Symptoms were unpolished, as the child's height was normal, without any positive cardiac, rheumatological, or ophthalmological family history. Cardiovascular tests found a thoracic aortic aneurysm at the Valsalva sinus (26mm, Z-score=+4.24). A genetic investigation found a TGFβR2 gene mutation, leading to the diagnosis of Loeys-Dietz syndrome type 2. Skeletal damage associated with bifid uvula and/or hypertelorism and an aneurysm of the ascending aorta should guide the genetic investigation to the search for TGF-β vasculopathy such as Loeys-Dietz syndrome. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

  4. Value and limitations of chimney grafts to treat arch lesions.

    Science.gov (United States)

    Mangialardi, N; Ronchey, S; Malaj, A; Fazzini, S; Alberti, V; Ardita, V; Orrico, M; Lachat, M

    2015-08-01

    The endovascular debranching with chimney stents provides a minimally invasive alternative to open surgery with readily available devices and has extended the option of endoluminal therapy into the realm of the aortic arch. But a critical observation at the use of this technique at the aortic arch is important and necessary because of the lack of long-term results and long term patency of the stents. Our study aims to review the results of chimney grafts to treat arch lesions. A systematic health database search was performed in December 2014 according to the Prisma Guidelines. Papers were sought through a meticulous search of the MEDLINE database (National Library of Medicine, Bethesda, MA) using the Pubmed search engine. Twenty-two articles were eligible for detailed analysis and data extraction. A total of 182 patients underwent chimney techniques during TEVAR (Thoracic Endovascular Aneurysm Repair). A total of 217 chimney grafts were implanted: 36 to the IA, 1 to the RCCA, 91 to the LCCA and 89 to the LSA. The type of stent-graft used for TEVAR was described in 132 patients. The type and name of chimney graft was described in 126 patients. In 53 patients information was limited to the type. Primary technical success, defined as a complete chimney procedure was achieved in 171 patients (98%). In 8 patients it was not clearly reported. The overall stroke rate was 5.3%. The overall endoleak rate, in those papers were it was clearly reported, was 18.4% (31 patients); 23(13,6%) patients developed a type IA endoleak, 1 patient (0.6%) developed type IB endoleak and 7 patients (4.1%) developed a type II endoleak The total endovascular aortic arch debranching technique represent a good option to treat high-risk patients, because it dramatically reduces the aggressiveness of the procedure in the arch. Many concerns are still present, mainly related to durability and material interaction during time. Long-term follow-up is exceptionally important in light of the

  5. Outcomes of single-stage total arch replacement via clamshell incision

    Directory of Open Access Journals (Sweden)

    Ishizaka Toru

    2011-09-01

    Full Text Available Abstract Background Treatment of complex aortic pathologies involving the transverse arch with extensive involvement of the descending aorta remains a surgical challenge. Since clamshell incision provides superior exposure of the entire thoracic aorta, we evaluated the use of this technique for single-stage total arch replacement by arch vessel reconstruction. Methods The arch-first technique combined with clamshell incision was used in 38 cases of aneurysm and aortic disease in 2008 and 2009. Extensive total arch replacement was used with clamshell incision for reconstruction of arch vessels under deep hypothermic circulatory arrest. Results Overall 30-day mortality was 13%. The mean operating time was approximately 8 hours. Deep hypothermia resulted in mean CPB time exceeding 4.5 hours and mean duration of circulatory arrest was 25 minutes. The overall postoperative temporary and permanent neurologic dysfunction rates were 3% and 3% for elective and 3% and 0% for emergency surgery, respectively. All patients except the five who died in hospital were discharged without nursing care after an average post-operative hospital stay of 35 days. Conclusions The arch-first technique, combined with clamshell incision, provides expeditious replacement of the thoracic aorta with an acceptable duration of hypothermic circulatory arrest and minimizes the risk of retrograde atheroembolism by using antegrade perfusion.

  6. Recurrent acute pulmonary oedema after aortic and mitral valve surgery due to trachea malacia and obstructive sleep apnoea syndrome

    NARCIS (Netherlands)

    Sankatsing, S. U. C.; Hanselaar, W. E. J. J.; van Steenwijk, R. P.; Van der Sloot, J. A. P.; Broekhuis, E.; Kok, W. E. M.

    2008-01-01

    In this report we describe a patient with recurrent episodes of acute pulmonary oedema after aortic and mitral valve surgery. The first episode of pulmonary oedema was caused by mitral valve dysfunction. The second episode of pulmonary oedema was not clearly associated with a mitral valve problem,

  7. 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

  8. Chest pain in patients with arterial hypertension, angiographically normal coronary arteries and stiff aorta: the aortic pain syndrome.

    Science.gov (United States)

    Stakos, Dimitrios A; Tziakas, Dimitrios N; Chalikias, George; Mitrousi, Konstantina; Tsigalou, Christina; Boudoulas, Harisios

    2013-01-01

    Arterial hypertension is often associated with a stiff aorta as a result of collagen accumulation in the aortic wall and may produce chest pain. In the present study, possible interrelationships between aortic function, collagen turnover and exercise-induced chest pain in patients with arterial hypertension and angiographically normal coronary arteries were investigated. Ninety-seven patients with arterial hypertension, angiographically normal coronary arteries and no evidence of myocardial ischemia on nuclear cardiac imaging during exercise test were studied. Of these, 43 developed chest pain during exercise (chest pain group) while 54 did not (no chest pain group). Carotid femoral pulse-wave velocity (PWVc-f) was used to assess the elastic properties of the aorta. Amino-terminal pro-peptides of pro-collagen type I, (PINP, reflecting collagen synthesis), serum telopeptides of collagen type I (CITP, reflecting collagen degradation), pro-metalloproteinase 1 (ProMMP-1), and tissue inhibitor of metalloproteinase 1 (TIMP-1, related to collagen turnover) were measured in plasma by immunoassay. The chest pain group had higher PWVc-f, higher and /CITP ratio, and lower proMMP-1/ TIMP-1 ratio compared to the no chest pain group. PWVc-f (t=2.53, p=0.02) and PINP (t=2.42, p=0.02) were independently associated with the presence of chest pain in multiple regression analysis. Patients with arterial hypertension, exercise-induced chest pain and angiographically normal coronary arteries, without evidence of exercise-induced myocardial ischemia, had a stiffer aorta compared to those without chest pain. Alterations in collagen type I turnover that favor collagen accumulation in the aortic wall may contribute to aortic stiffening and chest pain in these patients.

  9. Cardiovascular operations for Loeys-Dietz syndrome: Intermediate-term results.

    Science.gov (United States)

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

    2017-02-01

    Early experience with Loeys-Dietz syndrome (LDS) suggested an aggressive aortopathy with high risk of aneurysm dissection and rupture at young ages and at smaller aortic diameters than in other connective tissue disorders. We reviewed our experience with LDS to re-examine our indications and outcomes of surgical management. We reviewed all patients with a diagnosis of LDS who underwent cardiovascular surgery at our institution. The primary endpoint was mortality, and secondary endpoints included postoperative complications and need for reintervention. Seventy-nine operated patients with LDS were identified. Mean age at first operation was 25 years, 39 (49%) were female, and 38 (48%) were children (age <18 years). Six (8%) patients presented with acute dissection. Five (6%) patients had a bicuspid aortic valve, and all presented with an ascending aortic aneurysm with a mean root diameter of 3.5cm. Twenty (25%) patients had a previous sternotomy. Sixty-five (82%) patients underwent aortic root replacement, of whom 52 underwent a valve-sparing operation and 4 had concomitant arch replacement. Mean aortic root diameter in this group was 4.2 cm. Nine (11%) patients underwent aortic arch replacement, 2 (3%) had isolated ascending aorta replacement, and 3 (4%) underwent open thoracoabdominal repair. There were 2 (3%) operative and 8 late deaths. Nineteen patients underwent subsequent operations for late aneurysm and/or dissection. Mean follow-up was 6 years (range 0-24 years). Kaplan-Meier survival was 88% at 10 years. Growing experience with LDS has confirmed early impressions of its aggressive nature and proclivity toward aortic catastrophe. Surgical outcomes are favorable, but reintervention rates are high. Meticulous follow-up with cardiovascular surveillance imaging remain important for management, particularly as clinical LDS subtypes are characterized and more tailored treatment is developed. Copyright © 2016 The American Association for Thoracic Surgery

  10. 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.

  11. 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.

  12. Aortic reconstruction with bovine pericardial grafts

    Directory of Open Access Journals (Sweden)

    Silveira Lindemberg Mota

    2003-01-01

    Full Text Available INTRODUCTION: Glutaraldehyde-treated crimped bovine pericardial grafts are currently used in aortic graft surgery. These conduits have become good options for these operations, available in different sizes and shapes and at a low cost. OBJECTIVE:To evaluate the results obtained with bovine pericardial grafts for aortic reconstruction, specially concerning late complications. METHOD: Between January 1995 and January 2002, 57 patients underwent different types of aortic reconstruction operations using bovine pericardial grafts. A total of 29 (50.8% were operated on an urgent basis (mostly acute Stanford A dissection and 28 electively. Thoracotomy was performed in three patients for descending aortic replacement (two patients and aortoplasty with a patch in one. All remaining 54 underwent sternotomy, cardiopulmonary bypass and aortic resection. Deep hypothermia and total circulatory arrest was used in acute dissections and arch operations. RESULTS: Hospital mortality was 17.5%. Follow-up was 24.09 months (18.5 to 29.8 months confidence interval and complication-free actuarial survival curve was 92.3% (standard deviation ± 10.6. Two patients lately developed thoracoabdominal aneurysms following previous DeBakey II dissection and one died from endocarditis. One "patch" aortoplasty patient developed local descending aortic pseudoaneurysm 42 months after surgery. All other patients are asymptomatic and currently clinically evaluated with echocardiography and CT scans, showing no complications. CONCLUSION: Use of bovine pericardial grafts in aortic reconstruction surgery is adequate and safe, with few complications related to the conduits.

  13. Diagnosis of aortic interruption by CT angiography

    International Nuclear Information System (INIS)

    Shirani, Shapour; Soleymanzadeh, Maryam

    2013-01-01

    Interrupted aortic arch (IAA) is a rare congenital malformation of the aortic arch, which might be accompanied with other coexisting cardiovascular anomalies. Many cases with IAA are diagnosed at their neonatal and newborn period but in rare cases the diagnosis is not established until adulthood. The patients may have no clinical symptoms but the signs of heart failure will gradually appear and may cause death. The development of imaging methods such as computed tomography (CT) and magnetic resonance (MR) imaging has dramatically changed the diagnostics. Here we report a 20-year-old young man with IAA associated with sinus venosus atrial septal defect (SVD) and partial anomalous pulmonary venous connection (PAPVC) referred to our hospital

  14. Morphological risk factors of stroke during thoracic endovascular aortic repair.

    Science.gov (United States)

    Kotelis, Drosos; Bischoff, Moritz S; Jobst, Bertram; von Tengg-Kobligk, Hendrik; Hinz, Ulf; Geisbüsch, Philipp; Böckler, Dittmar

    2012-12-01

    This study aims to identify independent factors correlating to an increased risk of perioperative stroke during thoracic endovascular aortic repair (TEVAR). A prospective maintained TEVAR database, medical records, and imaging studies of 300 patients (205 men; median age of all, 66 years, range 21-89), who underwent TEVAR between March 1997 and February 2011, were reviewed. Preoperative CT data sets were reviewed by two experienced radiologists with focus on the atheroma burden in the aortic arch (grade I, normal, to grade V, ulcerated or pedunculated atheroma). Aortic arch geometry (arch types I-III) was documented. Further parameters included in the univariate analysis were age, gender, urgency of repair, duration of procedure, adenosine-induced cardiac arrest or rapid pacing, proximal landing zone, left subclavian artery (LSA) coverage, and number of stent grafts. Multivariate logistic regression analysis was performed to assess the independent correlations of potential risk factors. Atherosclerotic aneurysm was the most common pathology (44%). One hundred and fifty-four of our patients (51%) were treated under urgent or emergent conditions. Seventeen percent of all patients had significant arch atheroma (grade IV or V), and 43% had a steep type III aortic arch. The perioperative stroke was 4% (12 patients; median age, 73 years, range 31-78). Two strokes were lethal (0.7%). All strokes were classified as embolic based on imaging characteristics. In eight patients, strokes were located in the left cerebral hemisphere (seven of them in the anterior and one in the posterior circulation). Four stroke patients (one in the left posterior circulation) underwent LSA coverage without revascularization. Three stroke patients had severe arch atheroma grade V. Five patients suffering stroke were recognized to have a type III aortic arch. Strokes were equally distributed between zones 0-2 vs. 3-4 (n = 6 each, 5 vs. 3.3%). The highest incidence was found in zone 1 (11

  15. Compliance of the normal-sized aorta in adolescents with Marfan syndrome: comparison of MR measurements of aortic distensibility and pulse wave velocity; Compliance der morphologisch unauffaelligen Aorta bei Jugendlichen mit Marfan Syndrom: Vergleich von MR-Messungen der aortalen Dehnbarkeit und der Pulswellengeschwindigkeit

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    Eichhorn, J.G.; Ruediger, H.J.; Gorenflo, M.; Khalil, M.; Ulmer, H. [Universitaetskinderklinik Heidelberg (Germany). Kardiologie; Krissak, R.; Kauczor, H.U. [Deutsches Krebsforschungszentrum (DKFZ), Heidelberg (Germany). Radiologie; Ley, S. [Deutsches Krebsforschungszentrum (DKFZ), Heidelberg (Germany). Radiologie; Universitaetsklinik Heidelberg (Germany). Paediatrische Kardiologie; Arnold, R. [Universitaetskinderklinik Heidelberg (Germany). Kardiologie; Universitaetskinderklinik Freiburg (Germany). Paediatrische Kardiologie; Boese, J. [Deutsches Krebsforschungszentrum (DKFZ), Heidelberg (Germany). Medizinische Physik in der Radiologie; Siemens AG, Medical Solutions, Forchheim (Germany). Angiography, Fluoroscopic and Radiographic Systems; Krug, R. [Deutsches Krebsforschungszentrum (DKFZ), Heidelberg (Germany). Medizinische Physik in der Radiologie; Fink, C. [Medizinische Fakultaet Mannheim der Univ. Heidelberg, Mannheim (Germany). Inst. fuer Klinische Radiologie

    2007-08-15

    Purpose: To compare the aortic compliance of the normal-sized aorta of adolescents with Marfan syndrome and healthy controls using MR measurements of the aortic distensibility and pulse wave velocity. Materials and Methods: Fourteen patients (median age: 15 [9-21] years) and 11 healthy subjects (23 [12-32] years) were examined at 1.5 T. The MR protocol included 2D steady-state free precession (SSFP)-CINE MRI of the aortic distensibility and PC-MRI of the pulse wave velocity. All measurements were positioned perpendicular to the descending aorta at the level of the diaphragm for assessing the changes in the aortic cross-sectional areas and additionally above and below this plane for assessing the pulse wave velocity. In addition contrast-enhanced 3D-MR angiography was performed in adolescents with Marfan syndrome to exclude morphologic changes and to prove normal-sized aorta. Results: Compared with control subjects, adolescents with Marfan syndrome had significantly decreased distensibility and significantly increased pulse wave velocity ({chi}{sup 2}-test, p = 0.0002) using an age-related non-linear regression analysis. The related aortic compliance was significantly decreased ({chi}{sup 2}-test, p = 0.0002). There was a good correlation between the two methods (r = 0.86). A low intraobserver variability was found for both methods ({<=} 2 %). (orig.)

  16. Aortic stenosis

    Science.gov (United States)

    ... Images Aortic stenosis Heart valves References Carabello BA. Valvular heart disease. In: Goldman L, Schafer AI, eds. Goldman-Cecil ... ACC guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/ ...

  17. Early and late results of graft replacement for dissecting aneurysm of thoracoabdominal aorta in patients with Marfan syndrome.

    Science.gov (United States)

    Omura, Atsushi; Tanaka, Akiko; Miyahara, Shunsuke; Sakamoto, Toshihito; Nomura, Yoshikatsu; Inoue, Takeshi; Oka, Takanori; Minami, Hitoshi; Okada, Kenji; Okita, Yutaka

    2012-09-01

    When treating dissecting aneurysm of the thoracoabdominal aorta surgically in patients with Marfan syndrome, we have usually performed graft replacement- including the entire thoracoabdominal aorta and reconstruction of all visceral branches, even if dilatation is mild in some segments-to avoid further aortic operations in the follow-up period. From October 1999 through July 2011, 20 consecutive patients with Marfan syndrome underwent repair of dissecting aneurysm of the thoracoabdominal aorta (median age, 45 years; range, 19-65 years). All patients underwent surgical intervention with cerebrospinal fluid (CSF) drainage and distal aortic and selective organ perfusion. Deep hypothermia was used in 13 patients for spinal cord protection. No in-hospital mortality was observed. One patient had temporary spinal cord ischemia but was fully recovered by discharge. Other complications included exploration for bleeding (n=1), prolonged ventilation (n=1), and graft infection (n=1). At a mean follow-up of 54 months (range, 9-129 months), 1 patient had died of interstitial pneumonia at 38 months postoperatively. Survival at 8% years was 91.2±9.0%. Two patients required additional aortic procedures (total arch replacement and aortic valve-sparing surgery). Actuarial rate of freedom from aortic operations at 8 years was 83.9%±10.5%, but no patient needed required repeated thoracotomy for an aortic procedure. Neither false nor patch aneurysms were observed using computed tomography (CT) during follow-up surveillance. Graft replacement for dissecting aneurysm of the thoracoabdominal aorta in Marfan syndrome offers good early and long-term results. We believe total aortic replacement including the entire thoracoabdominal aorta and reconstruction of all visceral arteries should be recommended for selected patients with Marfan syndrome. Copyright © 2012 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  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. 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

  20. Proteção cerebral no tratamento cirúrgico dos aneurismas do arco aórtico: estudo experimental em cães Cerebral protection during surgical approach to the aortic arch aneurysms resection: experimental study in dogs

    Directory of Open Access Journals (Sweden)

    Gaudêncio Lopes Espinosa

    1994-12-01

    Full Text Available É realizado estudo experimental comparativo entre dois métodos de proteção cerebral na abordagem cirúrgica dos aneurismas do arco aórtico, avaliando a sua eficácia: hipotermia sistêmica profunda isolada (menor que 20ºC com pinçamento arterial braquiocefálico e hipotermia sistêmica profunda associada à perfusão carotídea seletiva. Dois grupos de 15 cães cada foram submetidos, respectivamente, à hipotermia sistêmica profunda com pinçamento arterial braquiocefálico (Grupo I e à hipotermia sistêmica profunda associada à perfusão seletiva da carótida direita (Grupo II. Foram analisadas amostras seriadas das alterações metabólicas de pH e PaCO2 que ocorreram no retorno venoso cerebral aferidas na veia julgular interna, bem como as alterações histopatológicas encontradas com 45 min., 90 min. e 135 min. de cada procedimento. Os resultados demonstram que, apesar de ambos os métodos de proteção cerebral serem eficazes por um período de 45 minutos, o método utilizado no Grupo II mostrou ser superior em períodos de até 90 minutos de isquemia cerebral. Em períodos de 135 minutos os métodos tiveram resultados semelhantes, não oferecendo proteção cerebral adequada.The authors proposition is to make an experimental study of two methods of cerebral protection to be used during aortic arch aneurysm resection. The methods to be evaluated were profound systemic hypothermia (under 20ºC whith great vessels occlusion and profound systemic hypothermia with selective right carotid artery perfusion. Two groups of 15 dogs each were submitted either to profound systemic hypothermia with great vessels occlusion (Group I, or to profound systemic hypothermia with selective right carotid artery perfusion (Group II. Serial jugular vein samples for pH and PaCO2 were analyzed to evaluate ischemic cerebral metabolic derangements. Hystopathological studies were also performed 45, 90 and 135 minutes, through animal sacrifice in each

  1. Dimorphic effects of transforming growth factor-β signaling during aortic aneurysm progression in mice suggest a combinatorial therapy for Marfan syndrome.

    Science.gov (United States)

    Cook, Jason R; Clayton, Nicholas P; Carta, Luca; Galatioto, Josephine; Chiu, Emily; Smaldone, Silvia; Nelson, Carol A; Cheng, Seng H; Wentworth, Bruce M; Ramirez, Francesco

    2015-04-01

    Studies of mice with mild Marfan syndrome (MFS) have correlated the development of thoracic aortic aneurysm (TAA) with improper stimulation of noncanonical (Erk-mediated) TGFβ signaling by the angiotensin type I receptor (AT1r). This correlation was largely based on comparable TAA modifications by either systemic TGFβ neutralization or AT1r antagonism. However, subsequent investigations have called into question some key aspects of this mechanism of arterial disease in MFS. To resolve these controversial points, here we made a head-to-head comparison of the therapeutic benefits of TGFβ neutralization and AT1r antagonism in mice with progressively severe MFS (Fbn1(mgR/mgR) mice). Aneurysm growth, media degeneration, aortic levels of phosphorylated Erk and Smad proteins and the average survival of Fbn1(mgR/mgR) mice were compared after a ≈3-month-long treatment with placebo and either the AT1r antagonist losartan or the TGFβ-neutralizing antibody 1D11. In contrast to the beneficial effect of losartan, TGFβ neutralization either exacerbated or mitigated TAA formation depending on whether treatment was initiated before (postnatal day 16; P16) or after (P45) aneurysm formation, respectively. Biochemical evidence-related aneurysm growth with Erk-mediated AT1r signaling, and medial degeneration with TGFβ hyperactivity that was in part AT1r dependent. Importantly, P16-initiated treatment with losartan combined with P45-initiated administration of 1D11 prevented death of Fbn1(mgR/mgR) mice from ruptured TAA. By demonstrating that promiscuous AT1r and TGFβ drive partially overlapping processes of arterial disease in MFS mice, our study argues for a therapeutic strategy against TAA that targets both signaling pathways although sparing the early protective role of TGFβ. © 2015 American Heart Association, Inc.

  2. White blood cell and platelet count as adjuncts to standard clinical evaluation for risk assessment in patients at low probability of acute aortic syndrome.

    Science.gov (United States)

    Morello, Fulvio; Cavalot, Giulia; Giachino, Francesca; Tizzani, Maria; Nazerian, Peiman; Carbone, Federica; Pivetta, Emanuele; Mengozzi, Giulio; Moiraghi, Corrado; Lupia, Enrico

    2017-08-01

    Pre-test probability assessment is key in the approach to suspected acute aortic syndromes (AASs). However, most patients with AAS-compatible symptoms are classified at low probability, warranting further evaluation for decision on aortic imaging. White blood cell count, platelet count and fibrinogen explore pathophysiological pathways mobilized in AASs and are routinely assayed in the workup of AASs. However, the diagnostic performance of these variables for AASs, alone and as a bundle, is unknown. We tested the hypothesis that white blood cell count, platelet count and/or fibrinogen at presentation may be applied as additional tools to standard clinical evaluation for pre-test risk assessment in patients at low probability of AAS. This was a retrospective observational study conducted on consecutive patients managed in our Emergency Department from 2009 to 2014 for suspected AAS. White blood cell count, platelet count and fibrinogen were assayed during evaluation in the Emergency Department. The final diagnosis was obtained by computed tomography angiography. The pre-test probability of AAS was defined according to guidelines. Of 1210 patients with suspected AAS, 1006 (83.1%) were classified at low probability, and 271 (22.4%) were diagnosed with AAS. Within patients at low probability, presence of at least one alteration among white blood cell count >9*10 3 /µl, platelet count probability, white blood cell count >9*10 3 /µl and platelet count probability, the estimated risk of AAS based on the number of alterations amongst white blood cell count >9*10 3 /µl and platelet count probability to fine-tune risk assessment of AAS.

  3. Endovascular Treatment of Late Thoracic Aortic Aneurysms after Surgical Repair of Congenital Aortic Coarctation in Childhood

    Science.gov (United States)

    Juszkat, Robert; Perek, Bartlomiej; Zabicki, Bartosz; Trojnarska, Olga; Jemielity, Marek; Staniszewski, Ryszard; Smoczyk, Wiesław; Pukacki, Fryderyk

    2013-01-01

    Background In some patients, local surgery-related complications are diagnosed many years after surgery for aortic coarctation. The purposes of this study were: (1) to systematically evaluate asymptomatic adults after Dacron patch repair in childhood, (2) to estimate the formation rate of secondary thoracic aortic aneurysms (TAAs) and (3) to assess outcomes after intravascular treatment for TAAs. Methods This study involved 37 asymptomatic patients (26 female and 11 male) who underwent surgical repair of aortic coarctation in the childhood. After they had reached adolescence, patients with secondary TAAs were referred to endovascular repair. Results Follow-up studies revealed TAA in seven cases (19%) (including six with the gothic type of the aortic arch) and mild recoarctation in other six (16%). Six of the TAA patients were treated with stentgrafts, but one refused to undergo an endovascular procedure. In three cases, stengrafts covered the left subclavian artery (LSA), in another the graft was implanted distally to the LSA. In two individuals, elective hybrid procedures were performed with surgical bypass to the supraaortic arteries followed by stengraft implantation. All subjects survived the secondary procedures. One patient developed type Ia endoleak after stentgraft implantation that was eventually treated with a debranching procedure. Conclusions The long-term course of clinically asymptomatic patients after coarctation patch repair is not uncommonly complicated by formation of TAAs (particularly in individuals with the gothic pattern of the aortic arch) that can be treated effectively with stentgrafts. However, in some patients hybrid procedures may be necessary. PMID:24386233

  4. Unusual rapid evolution of type B aortic dissection in a marfan patient following heart transplantation: successful endovascular treatment.

    Science.gov (United States)

    Botta, L; Russo, V; Grigioni, F; Arpesella, G; Rocchi, G; Di Bartolomeo, R; Fattori, R

    2006-10-01

    A patient with Marfan syndrome with previous Bentall operation for mitral and tricuspid valve repair, required orthotopic cardiac transplantation for end stage cardiomyopathy. Postoperatively he suff