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Sample records for thoracic aortic syndromes

  1. 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

  2. [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.

  3. 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.

  4. 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

  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. Thoracic aortic aneurysm

    Science.gov (United States)

    ... age Connective tissue disorders such as Marfan or Ehlers-Danlos syndrome Inflammation of the aorta Injury from falls or ... of connective tissue disorders (such as Marfan or Ehlers-Danlos syndrome) Chest or back discomfort Prevention To prevent atherosclerosis: ...

  7. Thoracic aortic aneurysms and dissections: endovascular treatment.

    Science.gov (United States)

    Baril, Donald T; Cho, Jae S; Chaer, Rabih A; Makaroun, Michel S

    2010-01-01

    The treatment of thoracic aortic disease has changed radically with the advances made in endovascular therapy since the concept of thoracic endovascular aortic repair was first described 15 years ago. Currently, there is a diverse array of endografts that are commercially available to treat the thoracic aorta. Multiple studies, including industry-sponsored and single-institution reports, have demonstrated excellent outcomes of thoracic endovascular aortic repair for the treatment of thoracic aortic aneurysms, with less reported perioperative morbidity and mortality in comparison with conventional open repair. Additionally, similar outcomes have been demonstrated for the treatment of type B dissections. However, the technology remains relatively novel, and larger studies with longer term outcomes are necessary to more fully evaluate the role of endovascular therapy for the treatment of thoracic aortic disease. This review examines the currently available thoracic endografts, preoperative planning for thoracic endovascular aortic repair, and outcomes of thoracic endovascular aortic repair for the treatment of both thoracic aortic aneurysms and type B aortic dissections. Mt Sinai J Med 77:256-269, 2010. (c) 2010 Mount Sinai School of Medicine.

  8. A History of Thoracic Aortic Surgery.

    Science.gov (United States)

    McFadden, Paul Michael; Wiggins, Luke M; Boys, Joshua A

    2017-08-01

    Ancient historical texts describe the presence of aortic pathology conditions, although the surgical treatment of thoracic aortic disease remained insurmountable until the 19th century. Surgical treatment of thoracic aortic disease then progressed along with advances in surgical technique, conduit production, cardiopulmonary bypass, and endovascular technology. Despite radical advances in aortic surgery, principles established by surgical pioneers of the 19th century hold firm to this day. Copyright © 2017 Elsevier Inc. All rights reserved.

  9. 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

  10. Imaging of thoracic aortic dissection

    International Nuclear Information System (INIS)

    Vu, F.H.; Young, N.; Soo, Y.S.

    1994-01-01

    Acute thoracic aortic dissection has a high mortality rate if untreated, so the diagnosis must be rapidly made. Multiple imaging techniques are often used. This retrospective study from 1988 to 1993 assesses the usefulness in diagnosis of chest X-rays, computed tomography (CT) scanning, aortography, magnetic resonance imaging (MRI), trans-thoracic (TTE) and trans-oesophageal (TOE) echocardiography. Forty-two patients with a final clinical diagnosis of dissection were studied. The diagnosis was confirmed in 16 (13 at surgery and three at autopsy). Three died with dissection given as the only cause of death. Chest X-ray abnormalities were seen in all 19 patients with surgery or death from dissection, with a widened mediastinum and/or dilated aorta being present in 17. In the group of 16 patients with surgery or autopsy proof, CT scans found dissections in 9 out of 12 patients studied and correctly classified the type in only five. Aortography was preformed in five, with accurate depiction of dissection and type in all. TTE found dissections in three of eight patients imaged by this method. MRI and TOE were preformed each on two patients, with accurate depiction of dissection and type in each. Because of the relatively low sensitivity of CT scanning in defining aortic dissections Westmead Hospital is currently assessing the use of TOE as the prime imaging modality prior to surgical intervention. 17 refs., 4 tabs., 4 figs

  11. Myocardial ischemia due to compression of an unruptured thoracic aortic aneurysm in a patient with Marfan syndrome.

    Science.gov (United States)

    Minami, Hiroya; Asada, Tatsuro; Gan, Kunio; Abe, Koichiro; Izumi, Satoshi

    2007-06-01

    We report a 33-year-old woman who had a 60-mm thoracic aneurysm of the ascending aorta with Marfan syndrome and effort angina due to compression of the right coronary artery (RCA) by the aneurysm. Surgery was performed using the Bentall procedure and a coronary artery bypass graft to the RCA. Postoperatively, coronary angiography showed that the coronary flow of the RCA was restored by removing the aneurysmal compression. The patient was discharged without angina on postoperative day 21.

  12. [Thoracic aortic dissection revealed by systemic cholesterol embolism].

    Science.gov (United States)

    Braem, L; Paule, P; Héno, P; Morand, J J; Mafart, B; La Folie, T; Varlet, P; Mioulet, D; Fourcade, L

    2006-10-01

    Systemic cholesterol embolism is a rare complication of atherosclerosis, and has various presentations. Arterial catheterisms are a common cause. However, the association with an aortic dissection has been exceptionally reported. We report the observation of a 70 year-old man, with coronary artery disease, hypertension, diabetes and dyslipidemia. Six months before hospitalization, a coronary angioplasty was performed due to recurrent angina. The association of purpuric lesions on the feet, with acute renal failure confirmed cholesterol embolism syndrome. Transoesophageal echocardiography showed a dissection of the descending thoracic aorta associated with complex atheroma. The evolution was marked by the pulpar necrosis of a toe and by a worsening of the renal failure, requiring definitive hemodialysis. Further echographic control highlighted the rupture of the intimal veil of the dissection. Cholesterol embolism syndrome may reveal an aortic dissection in patients without thoracic symptoms. In such cases, transoesophageal echocardiography is a useful and non-invasive examination.

  13. 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.

  14. [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.

  15. Wall stress on ascending thoracic aortic aneurysms with bicuspid compared with tricuspid aortic valve.

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    Xuan, Yue; Wang, Zhongjie; Liu, Raymond; Haraldsson, Henrik; Hope, Michael D; Saloner, David A; Guccione, Julius M; Ge, Liang; Tseng, Elaine

    2018-03-08

    Guidelines for repair of bicuspid aortic valve-associated ascending thoracic aortic aneurysms have been changing, most recently to the same criteria as tricuspid aortic valve-ascending thoracic aortic aneurysms. Rupture/dissection occurs when wall stress exceeds wall strength. Recent studies suggest similar strength of bicuspid aortic valve versus tricuspid aortic valve-ascending thoracic aortic aneurysms; thus, comparative wall stress may better predict dissection in bicuspid aortic valve versus tricuspid aortic valve-ascending thoracic aortic aneurysms. Our aim was to determine whether bicuspid aortic valve-ascending thoracic aortic aneurysms had higher wall stresses than their tricuspid aortic valve counterparts. Patients with bicuspid aortic valve- and tricuspid aortic valve-ascending thoracic aortic aneurysms (bicuspid aortic valve = 17, tricuspid aortic valve = 19) greater than 4.5 cm underwent electrocardiogram-gated computed tomography angiography. Patient-specific 3-dimensional geometry was reconstructed and loaded to systemic pressure after accounting for prestress geometry. Finite element analyses were performed using the LS-DYNA solver (LSTC Inc, Livermore, Calif) with user-defined fiber-embedded material model to determine ascending thoracic aortic aneurysm wall stress. Bicuspid aortic valve-ascending thoracic aortic aneurysms 99th-percentile longitudinal stresses were 280 kPa versus 242 kPa (P = .028) for tricuspid aortic valve-ascending thoracic aortic aneurysms in systole. These stresses did not correlate to diameter for bicuspid aortic valve-ascending thoracic aortic aneurysms (r = -0.004) but had better correlation to tricuspid aortic valve-ascending thoracic aortic aneurysms diameter (r = 0.677). Longitudinal stresses on sinotubular junction were significantly higher in bicuspid aortic valve-ascending thoracic aortic aneurysms than in tricuspid aortic valve-ascending thoracic aortic aneurysms (405 vs 329 kPa, P = .023). Bicuspid

  16. 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.

  17. Endovascular repair of traumatic thoracic aortic tears.

    Science.gov (United States)

    Mansour, M Ashraf; Kirk, Jeffrey S; Cuff, Robert F; Banegas, Shonda L; Ambrosi, Gavin M; Liao, Timothy H; Chambers, Christopher M; Wong, Peter Y; Heiser, John C

    2012-03-01

    Patients with thoracic aorta injuries (TAI) present a unique challenge. The purpose of this study was to review the outcomes of thoracic endovascular aortic repair (TEVAR) in patients with TAI. A retrospective chart review of all patients admitted for TEVAR for trauma was performed. In a 5-year period, 19 patients (6 women and 13 men; average age, 42 y) were admitted to our trauma center with TAI. Mechanism of injury was a motor vehicle crash in 12 patients, motorcycle crash in 2 patients, automobile-pedestrian accident in 2 patients, 1 fall, 1 crush injury, and 1 stab wound to the back. A thoracic endograft was used in 6 patients and proximal aortic cuffs were used in 13 patients (68%). One patient (5%) died. There were no strokes, myocardial infarctions, paraplegia, or renal failure. TEVAR for TAI appears to be a safe option for patients with multiple injuries. TEVAR in young patients is still controversial because long-term endograft behavior is unknown. Copyright © 2012 Elsevier Inc. All rights reserved.

  18. Aortic dose constraints when reirradiating thoracic tumors

    International Nuclear Information System (INIS)

    Evans, Jaden D.; Gomez, Daniel R.; Amini, Arya; Rebueno, Neal; Allen, Pamela K.; Martel, Mary K.; Rineer, Justin M.; Ang, Kie Kian; McAvoy, Sarah; Cox, James D.; Komaki, Ritsuko; Welsh, James W.

    2013-01-01

    Background and purpose: Improved radiation delivery and planning has allowed, in some instances, for the retreatment of thoracic tumors. We investigated the dose limits of the aorta wherein grade 5 aortic toxicity was observed after reirradiation of lung tumors. Material and methods: In a retrospective analysis, 35 patients were identified, between 1993 and 2008, who received two rounds of external beam irradiation that included the aorta in the radiation fields of both the initial and retreatment plans. We determined the maximum cumulative dose to 1 cm 3 of the aorta (the composite dose) for each patient, normalized these doses to 1.8 Gy/fraction, and corrected them for long-term tissue recovery between treatments (NID R ). Results: The median time interval between treatments was 30 months (range, 1–185 months). The median follow-up of patients alive at analysis was 42 months (range, 14–70 months). Two of the 35 patients (6%) were identified as having grade 5 aortic toxicities. There was a 25% rate of grade 5 aortic toxicity for patients receiving composite doses ⩾120.0 Gy (vs. 0% for patients receiving R ⩾90.0 Gy) to 1 cm 3 of the aorta

  19. Thoracic Endovascular Aortic Repair (TEVAR) in Proximal (Type A) Aortic Dissection: Ready for a Broader Application?

    OpenAIRE

    Nienaber, Christoph A.; Sakalihasan, Natzi; Clough, Rachel E.; Aboukoura, Mohamed; Mancuso, Enrico; Yeh, James S.M.; Defraigne, Jean-Olivier; Cheshire, Nick; Rosendahl, Ulrich Peter; Quarto, Cesare; Pepper, John

    2016-01-01

    ObjectiveThoracic endovascular aortic repair (TEVAR) has demonstrated encouraging results and is gaining increasing acceptance as a treatment option for aortic aneurysms and dissections. Yet, its role in managing proximal aortic pathologies is unknown - this is important because in proximal (Stanford type A) aortic dissections, 10-30% are not accepted for surgery, and 30-50% are technically amenable for TEVAR. We describe our case series of type A aortic dissections treated using TEVAR.Method...

  20. Clinical Studies in Risk Stratification & Therapy of Thoracic Aortic Disease

    NARCIS (Netherlands)

    Kamman, AV

    2017-01-01

    For this thesis we aimed to summarize outcomes and optimal treatment modality for thoracic aortic disease, discuss new imaging techniques and improve the use of current imaging modalities. Furthermore, we aimed to improve risk stratification for uncomplicated type B aortic dissection (TBAD) and

  1. [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.

  2. 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)

  3. Predictive factors for cerebrovascular accidents after thoracic endovascular aortic repair.

    Science.gov (United States)

    Mariscalco, Giovanni; Piffaretti, Gabriele; Tozzi, Matteo; Bacuzzi, Alessandro; Carrafiello, Giampaolo; Sala, Andrea; Castelli, Patrizio

    2009-12-01

    Cerebrovascular accidents are devastating and worrisome complications after thoracic endovascular aortic repair. The aim of this study was to determine cerebrovascular accident predictors after thoracic endovascular aortic repair. Between January 2001 and June 2008, 76 patients treated with thoracic endovascular aortic repair were prospectively enrolled. The study cohort included 61 men; mean age was 65.4 +/- 16.8 years. All patients underwent a specific neurologic assessment on an hourly basis postoperatively to detect neurologic deficits. Cerebrovascular accidents were diagnosed on the basis of physical examination, tomography scan or magnetic resonance imaging, or autopsy. Cerebrovascular accidents occurred in 8 (10.5%) patients, including 4 transient ischemic attack and 4 major strokes. Four cases were observed within the first 24-hours. Multivariable analysis revealed that anatomic incompleteness of the Willis circle (odds ratio [OR] 17.19, 95% confidence interval [CI] 2.10 to 140.66), as well as the presence of coronary artery disease (OR 6.86, 95 CI% 1.18 to 40.05), were independently associated with postoperative cerebrovascular accident development. Overall hospital mortality was 9.2%, with no significant difference for patients hit by cerebrovascular accidents (25.0% vs 7.3%, p = 0.102). Preexisting coronary artery disease, reflecting a severe diseased aorta and anomalies of Willis circle are independent cerebrovascular accident predictors after thoracic endovascular aortic repair procedures. A careful evaluation of the arch vessels and cerebral vascularization should be mandatory for patients suitable for thoracic endovascular aortic repair.

  4. 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.

  5. Association Between Thoracic Aortic Disease and Inguinal Hernia

    OpenAIRE

    Olsson, Christian; Eriksson, Per; Franco?Cereceda, Anders

    2014-01-01

    Background The study hypothesis was that thoracic aortic disease (TAD) is associated with a higher?than?expected prevalence of inguinal hernia. Such an association has been reported for abdominal aortic aneurysm (AAA) and hernia. Unlike AAA, TAD is not necessarily detectable with clinical examination or ultrasound, and there are no population?based screening programs for TAD. Therefore, conditions associated with TAD, such as inguinal hernia, are of particular clinical relevance. Methods and ...

  6. 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.

  7. Thoracic outlet syndrome: Case report

    International Nuclear Information System (INIS)

    Marquez, Juan Camilo; Acosta, Mauricio Fernando; Uribe Jorge Ricardo

    2009-01-01

    We report a case of vascular thoracic outlet syndrome in a young man, diagnosed with upper limb arteriography, leading to repeated arterio-arterial emboli originating from a post-stenotic subclavian artery aneurysm. It is of our interest due to its low incidence and the small number of cases reported that have been diagnosed by arteriography. The thoracic outlet is the path through which vascular and neural structures goes from the neck to the axilla, and it has three anatomical strictures, that when pronounced, can compress the brachial plexus or subclavian vessels, leading to different symptoms and signs.

  8. 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.

  9. Aneurysm growth after late conversion of thoracic endovascular aortic repair

    Directory of Open Access Journals (Sweden)

    Hirofumi Kasahara

    2015-01-01

    Full Text Available A 69-year-old man underwent thoracic endovascular aortic repair of a descending aortic aneurysm. Three years later, he developed impending rupture due to aneurysmal expansion that included the proximal landing zone. Urgent open surgery was performed via lateral thoracotomy, and a Dacron graft was sewn to the previous stent graft distally with Teflon felt reinforcement. Postoperatively, four sequential computed tomography scans demonstrated that the aneurysm was additionally increasing in size probably due to continuous hematoma production, suggesting a possibility of endoleaks. This case demonstrates the importance of careful radiologic surveillance after endovascular repair, and also after partial open conversion.

  10. Re-interventions on the thoracic and thoracoabdominal aorta in patients with Marfan syndrome

    OpenAIRE

    Schoenhoff, Florian S.; Carrel, Thierry P.

    2017-01-01

    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...

  11. 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.

  12. 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.

  13. Massive aggrecan and versican accumulation in thoracic aortic aneurysm and dissection.

    Science.gov (United States)

    Cikach, Frank S; Koch, Christopher D; Mead, Timothy J; Galatioto, Josephine; Willard, Belinda B; Emerton, Kelly B; Eagleton, Matthew J; Blackstone, Eugene H; Ramirez, Francesco; Roselli, Eric E; Apte, Suneel S

    2018-03-08

    Proteoglycan accumulation is a hallmark of medial degeneration in thoracic aortic aneurysm and dissection (TAAD). Here, we defined the aortic proteoglycanome using mass spectrometry, and based on the findings, investigated the large aggregating proteoglycans aggrecan and versican in human ascending TAAD and a mouse model of severe Marfan syndrome. The aortic proteoglycanome comprises 20 proteoglycans including aggrecan and versican. Antibodies against these proteoglycans intensely stained medial degeneration lesions in TAAD, contrasting with modest intralamellar staining in controls. Aggrecan, but not versican, was increased in longitudinal analysis of Fbn1mgR/mgR aortas. TAAD and Fbn1mgR/mgR aortas had increased aggrecan and versican mRNAs, and reduced expression of a key proteoglycanase gene, ADAMTS5, was seen in TAAD. Fbn1mgR/mgR mice with ascending aortic dissection and/or rupture had dramatically increased aggrecan staining compared with mice without these complications. Thus, aggrecan and versican accumulation in ascending TAAD occurs via increased synthesis and/or reduced proteolytic turnover, and correlates with aortic dissection/rupture in Fbn1mgR/mgR mice. Tissue swelling imposed by aggrecan and versican is proposed to be profoundly deleterious to aortic wall mechanics and smooth muscle cell homeostasis, predisposing to type-A dissections. These proteoglycans provide potential biomarkers for refined risk stratification and timing of elective aortic aneurysm repair.

  14. Application of thoracic endovascular aortic repair (TEVAR) in treating dwarfism with Stanford B aortic dissection

    Science.gov (United States)

    Qiu, Jian; Cai, Wenwu; Shu, Chang; Li, Ming; Xiong, Qinggen; Li, Quanming; Li, Xin

    2018-01-01

    Abstract Rationale: To apply thoracic endovascular aortic repair (TEVAR) to treat dwarfism complicated with Stanford B aortic dissection. Patient concerns: In this report, we presented a 63-year-old male patient of dwarfism complicated with Stanford B aortic dissection successfully treated with TEVAR. Diagnoses: He was diagnosed with dwarfism complicated with Stanford B aortic dissection. Interventions: After conservative treatment, the male patient underwent TEVAR at 1 week after hospitalization. After operation, he presented with numbness and weakness of his bilateral lower extremities, and these symptoms were significantly mitigated after effective treatment. At 1- and 3-week after TEVAR, the aorta status was maintained stable and restored. Outcomes: The patient obtained favorable clinical prognosis and was smoothly discharged. During subsequent follow-up, he remained physically stable. Lessons: TEVAR is probably an option for treating dwarfism complicated with Stanford B aortic dissection, which remains to be validated by subsequent studies with larger sample size. PMID:29703033

  15. 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

  16. Endovascular stent-graft management of thoracic aortic diseases

    International Nuclear Information System (INIS)

    Dake, Michael D.

    2001-01-01

    The traditional standard therapy for descending thoracic aortic aneurysm (TAA) is open operative repair with graft replacement of the diseased aortic segment. Despite important advances in surgical techniques, anesthetic management, and post-operative care over the last 30 years, the mortality and morbidity of surgery remains considerable, especially in patients at high risk for thoracotomy because of coexisting severe cardiopulmonary abnormalities or other medical diseases. The advent of endovascular stent-graft technology provides an alternative to open surgery for selected patients with TAA. The initial experience suggests that stent-graft therapy potentially may reduce the operative risk, hospital stay and procedural expenses of TAA repair. These potential benefits are especially attractive for patients at high risk for open TAA repair. Current results of endovascular TAA therapy document operative mortalities of between 0 and 4%, aneurysm thrombosis in 90 and 100% of cases, and paraplegia as a complication in 0 and 1.6% of patients. The early success of stent-graft repair of TAA has fostered the application of these devices for the management of a wide variety of thoracic aortic pathologies, including acute and chronic dissection, intramural hematoma, penetrating ulcer, traumatic injuries, and other diseases. The results of prospective controlled trials that compare the outcomes of stent-graft therapy with those of surgical treatment in patients with specific types of aortic disease are anxiously awaited before recommendations regarding the general use of these new devices can be made with confidence

  17. Factors Affecting Optimal Aortic Remodeling After Thoracic Endovascular Aortic Repair of Type B (IIIb) Aortic Dissection

    International Nuclear Information System (INIS)

    Chen, I-Ming; Chen, Po-Lin; Huang, Chun-Yang; Weng, Shih-Hsien; Chen, Wei-Yuan; Shih, Chun-Che

    2017-01-01

    PurposeThe purpose of this study was to determine factors associated with entire aortic remodeling after thoracic endovascular aortic repair (TEVAR) in patients with type B dissection.Materials and MethodsThe patients with type B (IIIb) dissections who underwent TEVAR from 2006 to 2013 with minimum of 2 years of follow-up computed tomography data were retrospectively reviewed. Based on the status of false lumen remodeling of entire aorta, patients were divided into three groups: complete regression, total thrombosis, and inadequate regression with patent abdominal false lumen.ResultsA total of 90 patients (72 males, 18 females; mean age 56.6 ± 16.4 years) were included and divided into the complete regression (n = 22), total thrombosis (n = 18), and inadequate regression (n = 50) groups. Multivariate logistic regression analysis indicated that dissection extension to iliac arteries, increased preoperative number of dissection tear over abdominal aorta, and decreased preoperative abdominal aorta bifurcation true lumen ratio, as compared between the inadequate and complete regression groups, were associated with a persistent false lumen (odds ratio = 33.33, 2.304, and 0.021; all, p ≤ 0.012). Comparison of 6, 12, and 24 months postoperative data revealed no significant differences at any level, suggesting that the true lumen area ratio might not change after 6 months postoperatively.ConclusionsIncreased preoperative numbers of dissection tear around the abdominal visceral branches, dissection extension to the iliac arteries, and decreased preoperative true lumen area ratio of abdominal aorta are predictive of entire aortic remodeling after TEVAR in patients with type B dissection.Level of EvidenceIII.

  18. Factors Affecting Optimal Aortic Remodeling After Thoracic Endovascular Aortic Repair of Type B (IIIb) Aortic Dissection

    Energy Technology Data Exchange (ETDEWEB)

    Chen, I-Ming [National Yang Ming University, Institute of Clinical Medicine, School of Medicine (China); Chen, Po-Lin; Huang, Chun-Yang [National Yang Ming University, Department of Medicine, School of Medicine (China); Weng, Shih-Hsien; Chen, Wei-Yuan; Shih, Chun-Che, E-mail: ccshih@vghtpe.gov.tw [National Yang Ming University, Institute of Clinical Medicine, School of Medicine (China)

    2017-05-15

    PurposeThe purpose of this study was to determine factors associated with entire aortic remodeling after thoracic endovascular aortic repair (TEVAR) in patients with type B dissection.Materials and MethodsThe patients with type B (IIIb) dissections who underwent TEVAR from 2006 to 2013 with minimum of 2 years of follow-up computed tomography data were retrospectively reviewed. Based on the status of false lumen remodeling of entire aorta, patients were divided into three groups: complete regression, total thrombosis, and inadequate regression with patent abdominal false lumen.ResultsA total of 90 patients (72 males, 18 females; mean age 56.6 ± 16.4 years) were included and divided into the complete regression (n = 22), total thrombosis (n = 18), and inadequate regression (n = 50) groups. Multivariate logistic regression analysis indicated that dissection extension to iliac arteries, increased preoperative number of dissection tear over abdominal aorta, and decreased preoperative abdominal aorta bifurcation true lumen ratio, as compared between the inadequate and complete regression groups, were associated with a persistent false lumen (odds ratio = 33.33, 2.304, and 0.021; all, p ≤ 0.012). Comparison of 6, 12, and 24 months postoperative data revealed no significant differences at any level, suggesting that the true lumen area ratio might not change after 6 months postoperatively.ConclusionsIncreased preoperative numbers of dissection tear around the abdominal visceral branches, dissection extension to the iliac arteries, and decreased preoperative true lumen area ratio of abdominal aorta are predictive of entire aortic remodeling after TEVAR in patients with type B dissection.Level of EvidenceIII.

  19. Extra-Thoracic Supra-aortic Bypass Surgery Is Safe in Thoracic Endovascular Aortic Repair and Arterial Occlusive Disease Treatment.

    Science.gov (United States)

    Gombert, Alexander; van Issum, Lea; Barbati, Mohammad E; Grommes, Jochen; Keszei, Andras; Kotelis, Drosos; Jalaie, Houman; Greiner, Andreas; Jacobs, Michael J; Kalder, Johannes

    2018-04-20

    The safety and feasibility of supra-aortic debranching as part of endovascular aortic surgery or as a treatment option for arterial occlusive disease (AOD) remains controversial. The aim of this study was to assess the clinical outcome of this surgery. This single centre, retrospective study included 107 patients (mean age 69.2 years, 38.4% women) who underwent supra-aortic bypass surgery (carotid-subclavian bypass, carotid-carotid bypass, and carotid-carotid-subclavian bypass) because of thoracic or thoraco-abdominal endovascular aortic repair (57%; 61/107) or as AOD treatment (42.9%; 46/107) between January 2006 and January 2015. Mortality, morbidity with a focus on neurological complications, and patency rate were assessed. Twenty-six of 107 (14.2%) of the debranching patients were treated under emergency conditions because of acute type B dissection or symptomatic aneurysm. Follow up, conducted by imaging interpretation and telephone interviews, continued till March 2017 (mean 42.1, 0-125, months). The in hospital mortality rate was 10.2% (11/107), all of these cases from the debranching group and related to emergency procedures (p supra-aortic bypass surgery involves low complication rates and high mid-term bypass patency rates. It is a safe and feasible treatment option in the form of debranching in combination with endovascular aortic aneurysm repair and in AOD. Copyright © 2018 European Society for Vascular Surgery. Published by Elsevier B.V. All rights reserved.

  20. Neurological Complications Following Endoluminal Repair of Thoracic Aortic Disease

    International Nuclear Information System (INIS)

    Morales, J. P.; Taylor, P. R.; Bell, R. E.; Chan, Y. C.; Sabharwal, T.; Carrell, T. W. G.; Reidy, J. F.

    2007-01-01

    Open surgery for thoracic aortic disease is associated with significant morbidity and the reported rates for paraplegia and stroke are 3%-19% and 6%-11%, respectively. Spinal cord ischemia and stroke have also been reported following endoluminal repair. This study reviews the incidence of paraplegia and stroke in a series of 186 patients treated with thoracic stent grafts. From July 1997 to September 2006, 186 patients (125 men) underwent endoluminal repair of thoracic aortic pathology. Mean age was 71 years (range, 17-90 years). One hundred twenty-eight patients were treated electively and 58 patients had urgent procedures. Anesthesia was epidural in 131, general in 50, and local in 5 patients. Seven patients developed paraplegia (3.8%; two urgent and five elective). All occurred in-hospital apart from one associated with severe hypotension after a myocardial infarction at 3 weeks. Four of these recovered with cerebrospinal fluid (CSF) drainage. One patient with paraplegia died and two had permanent neurological deficit. The rate of permanent paraplegia and death was 1.6%. There were seven strokes (3.8%; four urgent and three elective). Three patients made a complete recovery, one had permanent expressive dysphasia, and three died. The rate of permanent stroke and death was 2.1%. Endoluminal treatment of thoracic aortic disease is an attractive alternative to open surgery; however, there is still a risk of paraplegia and stroke. Permanent neurological deficits and death occurred in 3.7% of the patients in this series. We conclude that prompt recognition of paraplegia and immediate insertion of a CSF drain can be an effective way of recovering spinal cord function and improving the prognosis

  1. 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:

  2. Endograft Collapse After Endovascular Treatment for Thoracic Aortic Disease

    International Nuclear Information System (INIS)

    Bandorski, Dirk; Brueck, Martin; Guenther, Hans-Ulrich; Manke, Christoph

    2010-01-01

    Endovascular treatment is an established therapy for thoracic aortic disease. Collapse of the endograft is a potentially fatal complication. We reviewed 16 patients with a thoracic endograft between 2001 and 2006. Medical records of the treated patients were studied. Data collected include age, gender, diagnosis, indication for endoluminal treatment, type of endograft, and time of follow up. All patients (n = 16; mean age, 61 years; range, 21-82 years) underwent computed tomography (CT) for location of the lesion and planning of the intervention. Time of follow-up with CT scan ranged from 1 to 61 months. Indications for endovascular treatment were degenerative aneurysm (n = 7; 44%), aortic dissection (n = 2; 12%), perforated aortic ulcer (n = 4; 25%), and traumatic aortic injury (n = 3; 19%). Three patients suffered from a collapse of the endograft (one patient distal, two patients proximal) between 3 and 8 days after endovascular treatment. These patients were younger (mean age, 37 ± 25 years vs. 67 ± 16 years; P 0.05]; distal, 45 ± 23.5% vs. 38 ± 21.7% [P > 0.05]). Proximal collapse was corrected by placing a bare stent. In conclusion, risk factors for stent-graft collapse are a small lumen of the aorta and a small radius of the aortic arch curvature (young patients), as well as oversizing, which is an important risk factor and is described for different types of endografts and protheses (Gore TAG and Cook Zenith). Dilatation of the collapsed stent-graft is not sufficient. Following therapy implantation of a second stent or surgery is necessary in patients with a proximal endograft collapse. Distal endograft collapse can possibly be treated conservatively under close follow-up.

  3. Increased interleukin-11 levels in thoracic aorta and plasma from patients with acute thoracic aortic dissection.

    Science.gov (United States)

    Xu, Yao; Ye, Jing; Wang, Menglong; Wang, Yuan; Ji, Qingwei; Huang, Ying; Zeng, Tao; Wang, Zhen; Ye, Di; Jiang, Huimin; Liu, Jianfang; Lin, Yingzhong; Wan, Jun

    2018-06-01

    Interleukin (IL) 11 is closely related to tumor and hematological system diseases. Recent studies have demonstrated that IL-11 also participates in cardiovascular diseases, including ischemia-reperfusion mediated heart injury and acute myocardial infarction. This study aimed to investigate whether IL-11 is involved in acute thoracic aortic dissection (TAD). Aortic tissue samples from normal donors and acute TAD patients were collected, and the expression of IL-11 in all aortic tissue was analyzed. In addition, blood samples from patients with chest pain were collected and divided into a non-AD (NAD) group and a TAD group according to the results of computed tomography angiography of the thoracic aorta. The plasma IL-11, IL-17 and interferon (IFN) γ in all blood samples were measured. Compared with aortic tissue of normal controls, IL-11 was significantly increased in aortic tissue of acute TAD patients, especially in the torn section. The IL-11 was derived from aorta macrophages in TAD. In addition, the plasma IL-11, IL-17 and IFN-γ were significantly higher in acute TAD patients than in NAD patients, and the correlation analysis showed that IL-11 levels were positively correlated with levels of IFN-γ, IL-17, glucose, systolic blood pressure, diastolic blood pressure, white blood cells, C-reactive proteins and D-dimers. Binary logistic regression analyses showed that elevated IL11 in patients who may have diagnostic value of TAD, but less that D-dimer. IL-11 was increased in thoracic aorta and plasma of TAD patients and may be a promising biomarker for diagnosis in patients with TAD. Copyright © 2018. Published by Elsevier B.V.

  4. RELEVANCIA DEL DIAGNÓSTICO DIFERENCIAL ENTRE EL SÍNDROME AÓRTICO AGUDO Y EL SÍNDROME CORONARIO AGUDO EN PACIENTES CON DOLOR TORÁCICO Y CRISIS HIPERTENSIVA: REVISIÓN A PROPÓSITO DE 2 CASOS / Relevance of the differential diagnosis between acute aortic syndrome and acute coronary syndrome in patients with thoracic pain and hypertensive crisis: review on 2 case reports

    Directory of Open Access Journals (Sweden)

    Borja Simó Sánchez

    2011-11-01

    Full Text Available Acute aortic syndrome is a pathological process with low incidence compared with acute coronary syndrome, although with a worse prognosis in the short term, which is why its early diagnosis and urgent treatment are essential to the favorable evolution of thepatient. Electrocardiographic changes suggestive of myocardial ischemia, with acute evolution, are rare in acute aortic syndrome. Even in the presence of a suggestive thoracic pain and high levels of blood pressure, an adequate differential diagnosis is of utmost importance, since the administration of anticoagulation and antiplatelet therapy in high doses can have a dramatic impact on patient outcome. Two cases are presented in which electrocardiographic changes determined the action to take in patients with acute aortic syndrome.

  5. Thoracic aortic coarctation: MR evaluation and follow-up

    International Nuclear Information System (INIS)

    Papavero, R.; Kastler, B.; Clair, C.; Livolsi, A.; Papavero, R.; Site, O.; Kastler, B.; Clair, C.; Litzler, J.F.; Delabrousse, E.; Scheneider, P.; Bernard, Y.

    2001-01-01

    Purpose: Report our experience in the evaluation and follow-up of thoracic aortic coarctation with MRI and describe its role to estimate trans-stenotic flow. Material and methods: 43 MR examinations were performed in 30 patients (age range 15 days to 73 years) referred to our institution in the last 7 years. Results: MRI visualized the ascending, horizontal and descending portions of the aorta and the supra-aortic vessels in 42/43 patients. MRI clearly identified preoperatively an aortic coarctation in 11/12 cases confirmed by surgery. Postoperatively MRI depicted 4 restenosis and one aneurysm. In 5 patients MRI demonstrated pseudo-coarctation. A significant correlation was established between the maximal trans-stenotic pressure gradient when measured by Doppler US or angiography and the size of the signal void measured on cine-MR images (r=0.72; p<0.01). Conclusion: MRI is a reliable non invasive investigation method for the diagnosis and semi-quantitative evaluation of aortic coarctation particularly when colour Doppler US is not satisfactory. (authors)

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

    Science.gov (United States)

    Backer, Julie De; Braverman, Alan C

    2018-05-01

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

  7. Overexpression of interleukin-1β and interferon-γ in type I thoracic aortic dissections and ascending thoracic aortic aneurysms: possible correlation with matrix metalloproteinase-9 expression and apoptosis of aortic media cells.

    Science.gov (United States)

    Zhang, Lei; Liao, Ming-fang; Tian, Lei; Zou, Si-li; Lu, Qing-sheng; Bao, Jun-min; Pei, Yi-fei; Jing, Zai-ping

    2011-07-01

    To examine the expression of interleukin-1β and interferon-γ and their possible roles in aortic dissections and aneurysms. Aortic specimens were obtained from patients with type I thoracic aortic dissection, ascending thoracic aortic aneurysms, and control organ donors. The expression of interleukin-1β, interferon-γ, matrix metalloproteinase-9, and signal transduction factors phospho-p38 and phosphorylated c-jun N-terminal kinase (phospho-JNK) were detected by real time reverse transcription-polymerase chain reaction (real time RT-PCR), Western blot, and immunohistochemistry, respectively. Terminal deoxynucleotidyl transferase dUTP nick-end labeling (TUNEL) staining was performed to detect apoptosis of media cells. The correlation of these factors and apoptosis was also studied. Apoptosis in the media of thoracic aortic dissection and in ascending thoracic aortic aneurysms was dramatically higher than in the control group. The expression of interleukin-1β gradually increased from the control group, thoracic aortic dissection to ascending thoracic aortic aneurysms (p matrix metalloproteinase-9 was significantly increased in the media of thoracic aortic dissection and ascending thoracic aortic aneurysms compared with the control group (p correlations between interleukin-1β versus matrix metalloproteinase-9, interleukin-1β versus phospho-p38 in thoracic aortic dissection (p matrix metalloproteinase-9, interferon-γ versus phospho-JNK, interferon-γ versus apoptosis, and interleukin-1β versus apoptosis in ascending thoracic aortic aneurysms (p = 0.02, 0.02, p matrix metalloproteinase-9 and the apoptosis of media cells in humans. Copyright © 2010 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

  8. Thoracic aortic aneurysm in a child due to cystic medial necrosis

    International Nuclear Information System (INIS)

    Kuribayashi, Sachio; Watabe, Tsuneya; Ohtaki, Makoto; Matsuyama, Shoya; Ogawa, Junichi

    1983-01-01

    The valuable role of computed tomography (CT) was stressed in the diagnosis of thoracic aortic aneurysm in an asymptomatic 12-year-old child. She initially presented mediastinal mass on plain chest film. A saccular thoracic aortic aneurysm was highly suspected from the CT findings, and it was confirmed on angiography. Pathological examination of the aneurysmal wall revealed cystic medial necrosis. (author)

  9. Retrograde ascending aortic dissection during or after thoracic aortic stent graft placement: insight from the European registry on endovascular aortic repair complications

    DEFF Research Database (Denmark)

    Eggebrecht, Holger; Thompson, Matt; Rousseau, Hervé

    2009-01-01

    BACKGROUND: Single-center reports have identified retrograde ascending aortic dissection (rAAD) as a potentially lethal complication of thoracic endovascular aortic repair (TEVAR). METHODS AND RESULTS: Between 1995 and 2008, 28 centers participating in the European Registry on Endovascular Aortic...

  10. Diagnostic and therapy of acute thoracic aortic diseases; Diagnostik und Therapie akuter Erkrankungen der thorakalen Aorta

    Energy Technology Data Exchange (ETDEWEB)

    Schotten, Sebastian; Pitton, Michael B. [Universitaetsmedizin Mainz Univ. (Germany). Klinik und Poliklinik fuer Diagnostische und Interventionelle Radiologie

    2017-09-15

    Acute diseases of the thoracic aorta represent a relatively rare but life threatening spectrum of pathologies. The non-traumatic diseases are usually summarized by the term ''acute aortic syndrome''. A timely diagnosis and initiation of therapy are cornerstones for the patient outcome. CT has become the standard imaging procedure due do its widespread availability and excellent sensitivity. Furthermore, CT is able to discriminate the variants of acute aortic diseases and to detect the wide spectrum of complications. The volumetric CT dataset is also the basis for planning of interventional procedures. Open surgical repair still represents the standard of care for acute pathologies of the ascending aorta while endovascular therapy, due to minimally invasive character and good outcome, has replaced open surgery in most cases of complicated lesions of the descending aorta.

  11. MRI of thoracic outlet syndrome in children

    Energy Technology Data Exchange (ETDEWEB)

    Chavhan, Govind B.; Batmanabane, Vaishnavi [The Hospital for Sick Children and University of Toronto, Department of Diagnostic Imaging, Toronto, ON (Canada); Muthusami, Prakash [The Hospital for Sick Children and University of Toronto, Department of Diagnostic Imaging, Toronto, ON (Canada); The Hospital for Sick Children, Division of Image Guided Therapy, Department of Diagnostic Imaging, Toronto, ON (Canada); Towbin, Alexander J. [Cincinnati Children' s Hospital Medical Center, Department of Radiology and Medical Imaging, Cincinnati, OH (United States); Borschel, Gregory H. [The Hospital for Sick Children and University of Toronto, Division of Plastic Surgery, Department of Pediatric Surgery, Toronto, ON (Canada)

    2017-09-15

    Thoracic outlet syndrome is caused by compression of the neurovascular bundle as it passes from the upper thorax to the axilla. The neurovascular bundle can be compressed by bony structures such as the first rib, cervical ribs or bone tubercles, or from soft-tissue abnormalities like a fibrous band, muscle hypertrophy or space-occupying lesion. Thoracic outlet syndrome commonly affects young adults but can be seen in the pediatric age group, especially in older children. Diagnosis is based on a holistic approach encompassing clinical features, physical examination findings including those triggered by various maneuvers, electromyography, nerve conduction studies and imaging. Imaging is performed to confirm the diagnosis, exclude mimics and classify thoracic outlet syndrome into neurogenic, arterial, venous or mixed causes. MRI and MR angiography are useful in this process. A complete MRI examination for suspected thoracic outlet syndrome should include the assessment of anatomy and any abnormalities using routine sequences, vessel assessment with the arms in adduction by MR angiography and assessment of dynamic compression of vessels with abduction of the arms. The purpose of this paper is to describe the anatomy of the thoracic outlet, causes of thoracic outlet syndrome, the MR imaging techniques used in its diagnosis and the principles of image interpretation. (orig.)

  12. MRI of thoracic outlet syndrome in children

    International Nuclear Information System (INIS)

    Chavhan, Govind B.; Batmanabane, Vaishnavi; Muthusami, Prakash; Towbin, Alexander J.; Borschel, Gregory H.

    2017-01-01

    Thoracic outlet syndrome is caused by compression of the neurovascular bundle as it passes from the upper thorax to the axilla. The neurovascular bundle can be compressed by bony structures such as the first rib, cervical ribs or bone tubercles, or from soft-tissue abnormalities like a fibrous band, muscle hypertrophy or space-occupying lesion. Thoracic outlet syndrome commonly affects young adults but can be seen in the pediatric age group, especially in older children. Diagnosis is based on a holistic approach encompassing clinical features, physical examination findings including those triggered by various maneuvers, electromyography, nerve conduction studies and imaging. Imaging is performed to confirm the diagnosis, exclude mimics and classify thoracic outlet syndrome into neurogenic, arterial, venous or mixed causes. MRI and MR angiography are useful in this process. A complete MRI examination for suspected thoracic outlet syndrome should include the assessment of anatomy and any abnormalities using routine sequences, vessel assessment with the arms in adduction by MR angiography and assessment of dynamic compression of vessels with abduction of the arms. The purpose of this paper is to describe the anatomy of the thoracic outlet, causes of thoracic outlet syndrome, the MR imaging techniques used in its diagnosis and the principles of image interpretation. (orig.)

  13. Intensive Care Management of Thoracic Aortic Surgical Patients, Including Thoracic and Infradiaphragmatic Endovascular Repair (EVAR/TEVAR).

    Science.gov (United States)

    Cole, Sheela Pai

    2015-12-01

    The patient with thoracic aortic disease can present for open or endovascular repair. Thoracic endovascular aortic repair (TEVAR) has emerged as a minimally invasive option for a multitude of aortic pathology, including dissections, aneurysms, traumatic injuries, and ulcers. Postoperative management of these patients depends on the extent of procedure, whether it was open or endovascular, and, finally, on the preoperative comorbidities present. While procedural success has catapulted TEVAR to popularity, midterm results have been mixed. Additionally, periprocedural complications such as paraplegia and renal failure remain a significant morbidity in these patients. © The Author(s) 2015.

  14. Inflammatory Cell Infiltrates in Acute and Chronic Thoracic Aortic Dissection.

    Science.gov (United States)

    Wu, Darrell; Choi, Justin C; Sameri, Aryan; Minard, Charles G; Coselli, Joseph S; Shen, Ying H; LeMaire, Scott A

    2013-12-01

    Thoracic aortic dissection (TAD) is a highly lethal cardiovascular disease. Injury to the intima and media allows pulsatile blood to enter the media, leading to dissection formation. Inflammatory cells then infiltrate the site of aortic injury to clear dead cells and damaged tissue. This excessive inflammation may play a role in aneurysm formation after dissection. Using immunohistochemistry, we compared aortic tissues from patients with acute TAD (n = 11), patients with chronic TAD (n = 35), and donor controls (n = 20) for the presence of CD68+ macrophages, neutrophils, mast cells, and CD3+ T lymphocytes. Tissue samples from patients with acute or chronic TAD generally had significantly more inflammatory cells in both the medial and adventitial layers than did the control samples. In tissues from patients with acute TAD, the adventitia had more of the inflammatory cells studied than did the media. The pattern of increase in inflammatory cells was similar in chronic and acute TAD tissues, except for macrophages, which were seen more frequently in the adventitial layer of acute TAD tissue than in the adventitia of chronic TAD tissue. The inflammatory cell content of both acute and chronic TAD tissue was significantly different from that of control tissue. However, the inflammatory cell profile of aneurysmal chronic TAD was similar to that of acute TAD. This may reflect a sustained injury response that contributes to medial degeneration and aneurysm formation.

  15. Thoracic aortic aneurysm in a buck associated with caseous lymphadenitis

    Directory of Open Access Journals (Sweden)

    R.R. Pinheiro

    2013-06-01

    Full Text Available This paper reports the clinical, bacteriological and pathological findings of a thoracic aortic aneurysm in a four-year-old Anglo-Nubian goat buck, related to a framework of visceral caseous lymphadenitis. General clinical examination showed heart rate of 75 beats per minute, respiratory rate of 20 movements per minute and ruminal movements of four movements per minute. Superficial lymph nodes were normal upon palpation. Rectal temperature was slightly high (40.5°C. Blood test showed an intense leukocytosis (54,000/µL, characterized by strong neutrophil shift to the left. At necropsy, a large blood clot was detected in the thoracic cavity. The thickening of the myocardium and dilatation of the aorta in the thoracic portion, presenting a saculiform format was also observed. A large number of abscesses were disseminated in the media and intima layers of aorta. The aorta lumen obstruction by arterial plaques consisting of inflammatory infiltrate, predominantly neutrophilic was also detected. Abscesses were found spread in turbinate, rumen, reticulum, kidneys, liver, spleen, testicles and aorta wall. The microbiological exam of exudate confirmed Corynebacterium pseudotuberculosis as the causal agent.

  16. 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

  17. Long-term results after proximal thoracic aortic redo surgery.

    Directory of Open Access Journals (Sweden)

    Martin Czerny

    Full Text Available OBJECTIVE: To evaluate early and mid-term results in patients undergoing proximal thoracic aortic redo surgery. METHODS: We analyzed 60 patients (median age 60 years, median logistic EuroSCORE 40 who underwent proximal thoracic aortic redo surgery between January 2005 and April 2012. Outcome and risk factors were analyzed. RESULTS: In hospital mortality was 13%, perioperative neurologic injury was 7%. Fifty percent of patients underwent redo surgery in an urgent or emergency setting. In 65%, partial or total arch replacement with or without conventional or frozen elephant trunk extension was performed. The preoperative logistic EuroSCORE I confirmed to be a reliable predictor of adverse outcome- (ROC 0.786, 95%CI 0.64-0.93 as did the new EuroSCORE II model: ROC 0.882 95%CI 0.78-0.98. Extensive individual logistic EuroSCORE I levels more than 67 showed an OR of 7.01, 95%CI 1.43-34.27. A EuroSCORE II larger than 28 showed an OR of 4.44 (95%CI 1.4-14.06. Multivariate logistic regression analysis identified a critical preoperative state (OR 7.96, 95%CI 1.51-38.79 but not advanced age (OR 2.46, 95%CI 0.48-12.66 as the strongest independent predictor of in-hospital mortality. Median follow-up was 23 months (1-52 months. One year and five year actuarial survival rates were 83% and 69% respectively. Freedom from reoperation during follow-up was 100%. CONCLUSIONS: Despite a substantial early attrition rate in patients presenting with a critical preoperative state, proximal thoracic aortic redo surgery provides excellent early and mid-term results. Higher EuroSCORE I and II levels and a critical preoperative state but not advanced age are independent predictors of in-hospital mortality. As a consequence, age alone should no longer be regarded as a contraindication for surgical treatment in this particular group of patients.

  18. 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

  19. Relation of thoracic aortic and aortic valve calcium to coronary artery calcium and risk assessment.

    Science.gov (United States)

    Wong, Nathan D; Sciammarella, Maria; Arad, Yadon; Miranda-Peats, Romalisa; Polk, Donna; Hachamovich, Rory; Friedman, John; Hayes, Sean; Daniell, Anthony; Berman, Daniel S

    2003-10-15

    Aortic calcium, aortic valve calcium (AVC), and coronary artery calcium (CAC) have been associated with cardiovascular event risk. We examined the prevalence of thoracic aortic calcium (TAC) and AVC in relation to the presence and extent of CAC, cardiovascular risk factors, and estimated risk of coronary heart disease (CHD). In 2,740 persons without known CHD aged 20 to 79 years, CAC was assessed by electron beam- or multidetector-computed tomography. We determined the prevalence of TAC and AVC in relation to CAC, CHD risk factors, and predicted 10-year risk of CHD. A close correspondence of TAC and AVC was observed with CAC. TAC and AVC increased with age; by the eighth decade of life, the prevalence of TAC was similar to that of CAC (>80%), and 36% of men and 24% of women had AVC. Age, male gender, and low-density lipoprotein cholesterol were directly related to the likelihood of CAC, TAC, and AVC; higher diastolic blood pressure and cigarette smoking additionally predicted CAC. Body mass index and higher systolic and lower diastolic blood pressures were also related to TAC, and higher body mass index and lower diastolic blood pressure were related to AVC. Calculated risk of CHD increased with the presence of AVC and TAC across levels of CAC. TAC and AVC provided incremental value over CAC in association with the 10-year calculated risk of CHD. If longitudinal studies show an incremental value of aortic and aortic valve calcium over that of CAC for prediction of cardiovascular events, future guidelines for risk assessment incorporating CAC assessment may additionally incorporate the measurement of aortic and/or aortic valve calcium.

  20. Bio-chemo-mechanics of thoracic aortic aneurysms.

    Science.gov (United States)

    Wagenseil, Jessica E

    2018-03-01

    Most thoracic aortic aneurysms (TAAs) occur in the ascending aorta. This review focuses on the unique bio-chemo-mechanical environment that makes the ascending aorta susceptible to TAA. The environment includes solid mechanics, fluid mechanics, cell phenotype, and extracellular matrix composition. Advances in solid mechanics include quantification of biaxial deformation and complex failure behavior of the TAA wall. Advances in fluid mechanics include imaging and modeling of hemodynamics that may lead to TAA formation. For cell phenotype, studies demonstrate changes in cell contractility that may serve to sense mechanical changes and transduce chemical signals. Studies on matrix defects highlight the multi-factorial nature of the disease. We conclude that future work should integrate the effects of bio-chemo-mechanical factors for improved TAA treatment.

  1. Thoracic aortic aneurysm: A rare cause of elevated hemidiaphragm

    Directory of Open Access Journals (Sweden)

    Md Arshad Ejazi

    2016-01-01

    Full Text Available Phrenic nerve palsy causing hemidiaphragm paralysis is a very uncommon feature of thoracic aortic aneurysm. In one case, a 30 year male complained of chronic dull aching chest pain, and hoarseness of voice; posteroanterior view chest radiograph revealed large spherical radiopacity on the left upper lung zone with smooth lobulated margin with elevated left hemidiaphragm. On Colour Doppler sonography, lesion was anechoic on gray scale sonography but on Doppler analysis revealed intense internal vascularity within it with characteristic "Ying Yang" sign. The finding favor the vascular origin of the lesion and a diagnosis of an arterial aneurysm was made Contrast-enhanced computed tomography (CT of the thorax revealed a large well defined spherical lesion of 8 × 10 cm size with smooth well defined margin arising from the aortic arch and attenuation of impending rupture or dissection were lesion on immediate post contrast and delayed scan was similar to that of aorta. Left hemidiaphragm elevation was explained by the gross mass effect of the aneurysm causing right phrenic nerve palsy.

  2. Association between thoracic aortic disease and inguinal hernia.

    Science.gov (United States)

    Olsson, Christian; Eriksson, Per; Franco-Cereceda, Anders

    2014-08-21

    The study hypothesis was that thoracic aortic disease (TAD) is associated with a higher-than-expected prevalence of inguinal hernia. Such an association has been reported for abdominal aortic aneurysm (AAA) and hernia. Unlike AAA, TAD is not necessarily detectable with clinical examination or ultrasound, and there are no population-based screening programs for TAD. Therefore, conditions associated with TAD, such as inguinal hernia, are of particular clinical relevance. The prevalence of inguinal hernia in subjects with TAD was determined from nation-wide register data and compared to a non-TAD group (patients with isolated aortic stenosis). Groups were balanced using propensity score matching. Multivariable statistical analysis (logistic regression) was performed to identify variables independently associated with hernia. Hernia prevalence was 110 of 750 (15%) in subjects with TAD versus 29 of 301 (9.6%) in non-TAD, P=0.03. This statistically significant difference remained after propensity score matching: 21 of 159 (13%) in TAD versus 14 of 159 (8.9%) in non-TAD, PTAD, OR 1.8 (1.1 to 2.8), P=0.015. The prevalence of inguinal hernia (15%) in TAD is higher than expected in a general population and higher in TAD, compared to non-TAD. TAD is independently associated with hernia in multivariable analysis. Presence or history of hernia may be of importance in detecting TAD, and the association warrants further study. © 2014 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley Blackwell.

  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. [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

  5. Blunt Cardiac Injury in Trauma Patients with Thoracic Aortic Injury

    Directory of Open Access Journals (Sweden)

    Rathachai Kaewlai

    2011-01-01

    Full Text Available Trauma patients with thoracic aortic injury (TAI suffer blunt cardiac injury (BCI at variable frequencies. This investigation aimed to determine the frequency of BCI in trauma patients with TAI and compare with those without TAI. All trauma patients with TAI who had admission electrocardiography (ECG and serum creatine kinase-MB (CK-MB from January 1999 to May 2009 were included as a study group at a level I trauma center. BCI was diagnosed if there was a positive ECG with either an elevated CK-MB or abnormal echocardiography. There were 26 patients (19 men, mean age 45.1 years, mean ISS 34.4 in the study group; 20 had evidence of BCI. Of 52 patients in the control group (38 men, mean age 46.9 years, mean ISS 38.7, eighteen had evidence of BCI. There was a significantly higher rate of BCI in trauma patients with TAI versus those without TAI (77% versus 35%, P<0.001.

  6. Endovascular treatment of thoracic aortic pseudoaneurysm due to brucellosis: a rare case report.

    Science.gov (United States)

    Wang, Shuai; Wang, Qi; Liu, Han; Sun, Siqiao; Sun, Xiwei; Zhang, Yang; Wang, Zhongying; Cheng, Zhihua

    2017-06-02

    Arterial damage is a known complication of brucellosis, but the occurrence of a thoracic aortic pseudoaneurysm secondary to brucellosis has not been previously reported. A 65-year-old Chinese man presented with a pseudoaneurysm in the descending segment of the thoracic aorta that caused symptoms of chest pain and intermittent fever. He was diagnosed with a thoracic aortic pseudoaneurysm secondary to brucellosis based on a positive brucella serology test (standard-tube agglutination test) and imaging examination (computed tomography angiography). Anti-brucellosis treatment and covered stent graft implantation were attempted to eliminate the brucellosis and pseudoaneurysm, respectively, and were ultimately successful, with no symptoms after 6 months of follow-up. Endovascular repair may be effective and safe for treating a thoracic aortic pseudoaneurysm resulting from brucellosis.

  7. 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

  8. A Rare Entity: Traumatic Thoracic Aortic Injury in a Patient with Aberrant Right Subclavian Artery.

    Science.gov (United States)

    Patel, Hiten Mohanbhai; Banerjee, Shubhabrata; Bulsara, Shahzad; Sahu, Tapish; Sheorain, Virender K; Grover, Tarun; Parakh, Rajiv

    2017-05-01

    Aberrant right subclavian artery is an uncommon entity incidence ranging from 0.5 to 2.5%. Management of thoracic aortic injury in the presence of such anomalies can be a challenge. We present here a case of traumatic aortic injury, which was incidentally found to have an asymptomatic aberrant right subclavian artery. The patient was managed by an endovascular repair of thoracic aortic injury with an endograft and a right carotid to subclavian artery bypass as a hybrid procedure. A 40-year male patient was brought to the emergency in shock with an alleged history of road traffic accident an hour back. After initial resuscitation as per advance trauma life support protocol, imaging revealed thoracic aortic injury with aberrant right subclavian artery with multiple rib and bilateral humerus fracture. After primary stabilization of arm fractures, the patient was shifted to a hybrid operation room. As the aortic injury was within 10 mm of the origin of both subclavian arteries, it was decided to cover the origin of both subclavian arteries and land the endograft distal to the left carotid artery origin. Since there was a right dominant vertebral artery on imaging, right carotid to right subclavian artery bypass was done with expanded polytetrafluoroethylene graft to prevent posterior circulatory stroke along with thoracic endovascular aortic repair to seal the thoracic aortic injury. After endovascular repair of thoracic aortic injury, left subclavian artery perfusion was maintained through left vertebral artery; and hence, revascularization of left subclavian artery was deferred. After management of all fractures, the patient was discharged 3 weeks after the date of admission without any complications. At 6 months follow-up, patient was stable and images showed patent bypass graft and sealed aortic injury. In a trauma setting with multiple injuries, hybrid procedure with a thoracic endograft is associated with low mortality and morbidity; hence, it is the treatment

  9. Thoracic endometriosis syndrome: CT and MRI features

    International Nuclear Information System (INIS)

    Rousset, P.; Rousset-Jablonski, C.; Alifano, M.; Mansuet-Lupo, A.; Buy, J.-N.; Revel, M.-P.

    2014-01-01

    Thoracic endometriosis is considered to be rare, but is the most frequent form of extra-abdominopelvic endometriosis. Thoracic endometriosis syndrome affects women of reproductive age. Diagnosis is mainly based on clinical findings, which can include catamenial pneumothorax and haemothorax, non-catamenial endometriosis-related pneumothorax, catamenial haemoptysis, lung nodules, and isolated catamenial chest pain. Symptoms are typically cyclical and recurrent, with a right-sided predominance. Computed tomography (CT) is the first-line imaging method, but is poorly specific; therefore, its main role is to rule out other pulmonary diseases. However, in women with a typical clinical history, some key CT findings may help to confirm this often under-diagnosed syndrome. MRI can also assist with the diagnosis, by showing signal changes typical of haemorrhage within diaphragmatic or pleural lesions

  10. Application of thoracic endovascular aortic repair (TEVAR) in treating dwarfism with Stanford B aortic dissection: A case report.

    Science.gov (United States)

    Qiu, Jian; Cai, Wenwu; Shu, Chang; Li, Ming; Xiong, Qinggen; Li, Quanming; Li, Xin

    2018-04-01

    To apply thoracic endovascular aortic repair (TEVAR) to treat dwarfism complicated with Stanford B aortic dissection. In this report, we presented a 63-year-old male patient of dwarfism complicated with Stanford B aortic dissection successfully treated with TEVAR. He was diagnosed with dwarfism complicated with Stanford B aortic dissection. After conservative treatment, the male patient underwent TEVAR at 1 week after hospitalization. After operation, he presented with numbness and weakness of his bilateral lower extremities, and these symptoms were significantly mitigated after effective treatment. At 1- and 3-week after TEVAR, the aorta status was maintained stable and restored. The patient obtained favorable clinical prognosis and was smoothly discharged. During subsequent follow-up, he remained physically stable. TEVAR is probably an option for treating dwarfism complicated with Stanford B aortic dissection, which remains to be validated by subsequent studies with larger sample size.

  11. JAG Tearing Technique with Radiofrequency Guide Wire for Aortic Fenestration in Thoracic Endovascular Aneurysm Repair

    International Nuclear Information System (INIS)

    Ricci, Carmelo; Ceccherini, Claudio; Leonini, Sara; Cini, Marco; Vigni, Francesco; Neri, Eugenio; Tucci, Enrico; Benvenuti, Antonio; Tommasino, Giulio; Sassi, Carlo

    2012-01-01

    An innovative approach, the JAG tearing technique, was performed during thoracic endovascular aneurysm repair in a patient with previous surgical replacement of the ascending aorta with a residual uncomplicated type B aortic dissection who developed an aneurysm of the descending thoracic aorta with its lumen divided in two parts by an intimal flap. The proximal landing zone was suitable to place a thoracic stent graft. The distal landing zone was created by cutting the intimal flap in the distal third of the descending thoracic aorta with a radiofrequency guide wire and intravascular ultrasound catheter.

  12. Thoracic manifestations of ovarian hyperstimulation syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Levin, M.F.; Hutton, L.C.; Kaplan, B.R. [University of Western Ontario, London, ON (Canada)

    1995-02-01

    In order to determine the thoracic manifestations of severe ovarian hyperstimulation syndrome, the medical records and available images of 771 patients who had received gonadotropins to induce superovulation, were reviewed. In 22 patients (3%) severe hyperstimulation syndrome was diagnosed clinically and confirmed with ultrasonography (US). Pleural effusion occurred in five of these (23%), one of whom required thoracentesis. Atelectasis and internal jugular vein thrombosis developed in one patient, and ventilation-perfusion mismatch occurred in another. The study concluded that respiratory distress in patients with ovarian hyperstimulation syndrome was most likely due to lung restriction. Pulmonary manifestations formed an important part of this syndrome, and radiologic input were considered necessary for assessment, monitoring and management. 10 refs., 2 figs., 1 tab.

  13. 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...

  14. Endovascular stent graft treatment of acute thoracic aortic transections due to blunt force trauma.

    LENUS (Irish Health Repository)

    Bjurlin, Marc A

    2012-02-01

    Endovascular stent graft treatment of acute thoracic aortic transections is an encouraging minimally invasive alternative to open surgical repair. Between 2006 and 2008, 16 patients with acute thoracic aortic transections underwent evaluation at our institution. Seven patients who were treated with an endovascular stent graft were reviewed. The mean Glasgow Coma Score was 13.0, probability of survival was .89, and median injury severity score was 32. The mean number of intensive care unit days was 7.7, mean number of ventilator support days was 5.4, and hospital length of stay was 10 days. Mean blood loss was 285 mL, and operative time was 143 minutes. Overall mortality was 14%. Procedure complications were a bleeding arteriotomy site and an endoleak. Endovascular treatment of traumatic thoracic aortic transections appears to demonstrate superior results with respect to mortality, blood loss, operative time, paraplegia, and procedure-related complications when compared with open surgical repair literature.

  15. 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

  16. Potential Long-Term Complications of Endovascular Stent Grafting for Blunt Thoracic Aortic Injury

    Directory of Open Access Journals (Sweden)

    Larry E. Miller

    2012-01-01

    Full Text Available Blunt thoracic aortic injury (BTAI is a rare, but lethal, consequence of rapid deceleration events. Most victims of BTAI die at the scene of the accident. Of those who arrive to the hospital alive, expedient aortic intervention significantly improves survival. Thoracic endovascular aortic repair (TEVAR has been accepted as the standard of care for BTAI at many centers, primarily due to the convincing evidence of lower mortality and morbidity in comparison to open surgery. However, less attention has been given to potential long-term complications of TEVAR for BTAI. This paper focuses on these complications, which include progressive aortic expansion with aging, inadequate stent graft characteristics, device durability concerns, long-term radiation exposure concerns from follow-up computed tomography scans, and the potential for (Victims of Modern Imaging Technology VOMIT.

  17. 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...

  18. "Elephant trunk" and endovascular stentgrafting : a hybrid approach to the treatment of extensive thoracic aortic aneurysm

    OpenAIRE

    Holubec, Tomás; Raupach, Jan; Dominik, Jan; Vojácek, Jan

    2013-01-01

    A hybrid approach to elephant trunk technique for treatment of thoracic aortic aneurysms combines a conventional surgical and endovascular therapy. Compared to surgery alone, there is a presumption that mortality and morbidity is reduced. We present a case report of a 42-year-old man with a giant aneurysm of the entire thoracic aorta, significant aortic and tricuspid regurgitation and ventricular septum defect. The patient underwent multiple consecutive operations and interventions having, am...

  19. 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.)

  20. 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.)

  1. 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.

  2. Operativ behandling af thoracic outlet syndrome

    DEFF Research Database (Denmark)

    Birkeland, Peter; Stiasny, Jerzy

    2012-01-01

    of the brachial plexus. At surgery, we found and severed a fibrous band that compressed the inferior trunk. Postoperatively, the pain subsided and fine hand movements improved. One patient had no cervical rib, however, in the two other cases we found rudimentary cervical ribs. Magnetic resonance imaging......We present three cases with longstanding true neurogenic thoracic outlet syndrome. All patients had aching pain in the shoulder, arm and ulnar border of the hand. On examination, we found atrophy of the hand muscles. Electromyography revealed signs of compromised function of the inferior trunk...

  3. Epidemiology and pathogenesis of thoracic outlet syndrome

    Directory of Open Access Journals (Sweden)

    Wojcik Gustaw

    2015-03-01

    Full Text Available The superior thoracic aperture is a place particularly vulnerable to the occurrence of tissue conflict and the development of a number of neurovascular changes carrying a risk of upper limb dysfunction. The triggering factor in this case is the pressure on the nerve vascular elements brought about by too large muscles of the chest and neck, clavicle fracture and dislocation of the upper ribs, anomalies in the form of ribs, in the neck, or by apex of the lung tumors. Each anatomical anomaly may be a cause of a number of lesions and lead to the development of the disease. Due to the nature of the oppressed structures, there are two basic groups: neurogenic and vascular. The most common variant giving clinical symptoms is neurogenic thoracic outlet syndrome. In this, the compression ratio, the brachial plexus, and for this reason, the vascular surface of the upper limb dysfunction is often overlooked. However, the vascular variant, and especially arterial sub-variant, is very dangerous because it can give complications even in the form of aneurysms, and even upper limb ischemia. The aim of the study is to present the most common changes in the thoracic outlet causing functional disorders of the upper limb.

  4. 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

  5. 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...

  6. Thoracic Aorta 3D Hemodynamics in Pediatric and Young Adult Patients With Bicuspid Aortic Valve

    NARCIS (Netherlands)

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

    2015-01-01

    Background: To evaluate the 3D hemodynamics in the thoracic aorta of pediatric and young adult bicuspid aortic valve (BAV) patients. Methods: 4D flow MRI was performed in 30 pediatric and young adult BAV patients (age: 13.9 +/- 4.4 (range: [3.4, 20.7]) years old, M:F = 17:13) as part of this

  7. 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

  8. Outcomes of endovascular management of acute thoracic aortic emergencies in an academic level 1 trauma center.

    Science.gov (United States)

    Echeverria, Angela B; Branco, Bernardino C; Goshima, Kay R; Hughes, John D; Mills, Joseph L

    2014-12-01

    Thoracic aortic emergencies account for 10% of thoracic-related admissions in the United States and remain associated with high morbidity and mortality rates. Open repair has declined owing to the emergence of thoracic endovascular aortic repair (TEVAR), but data on emergency TEVAR use for acute aortic pathology remain limited. We therefore reviewed our experience. We retrospectively evaluated emergency descending thoracic aortic endovascular interventions performed at a single academic level 1 trauma center between January 2005 and August 2013 including all cases of traumatic aortic injury, ruptured descending thoracic aneurysm, penetrating atherosclerotic ulcer, aortoenteric fistula, and acute complicated type B dissection. Demographics, clinical data, and outcomes were extracted. Stepwise logistic regression was used to identify independent risk factors for death. During the study period, 51 patients underwent TEVAR; 22 cases (43.1%) were performed emergently (11 patients [50.0%] traumatic aortic injury; 4 [18.2%] ruptured descending thoracic aneurysm; 4 [18.2%] complicated type B dissection; 2 [9.1%] penetrating aortic ulcer; and 1 [4.5%] aortoenteric fistula). Overall, 72.7% (n = 16) were male with a mean age of 54.8 ± 15.9 years. Nineteen patients (86.4%) required only a single TEVAR procedure, whereas 2 (9.1%) required additional endovascular therapy, and 1 (4.5%) open thoracotomy. Four traumatic aortic injury patients required exploratory laparotomy for concomitant intra-abdominal injuries. During a mean hospital length of stay of 18.9 days (range, 1 to 76 days), 3 patients (13.6%) developed major complications. In-hospital mortality was 27.2%, consisting of 6 deaths from traumatic brain injury (1); exsanguination in the operating room before repair could be achieved (2); bowel ischemia (1) and multisystem organ failure (1); and family withdrawal of care (1). A stepwise logistic regression model identified 24-hour packed red blood cell requirements ≥4

  9. Cardiovascular Disease in Ageing: An Overview on Thoracic Aortic Aneurysm as an Emerging Inflammatory Disease

    Directory of Open Access Journals (Sweden)

    Calogera Pisano

    2017-01-01

    Full Text Available Medial degeneration associated with thoracic aortic aneurysm and acute aortic dissection was originally described by Erdheim as a noninflammatory lesion related to the loss of smooth muscle cells and elastic fibre fragmentation in the media. Recent evidences propose the strong role of a chronic immune/inflammatory process in aneurysm evocation and progression. The coexistence of inflammatory cells with markers of apoptotic vascular cell death in the media of ascending aorta with aneurysms and type A dissections raises the possibility that activated T cells and macrophages may contribute to the elimination of smooth muscle cells and degradation of the matrix. On the other hand, several inflammatory pathways (including TGF-β, TLR-4 interferon-γ, chemokines, and interferon-γ seem to be involved in the medial degeneration related to aged and dilated aorta. This is an overview on thoracic aortic aneurysm as an emerging inflammatory disease.

  10. Preoperative Evaluation and Endovascular Procedure of Intraoperative Aneurysm Rupture During Thoracic Endovascular Aortic Repair

    Energy Technology Data Exchange (ETDEWEB)

    Zha, Bin-Shan, E-mail: binszha2013@163.com; Zhu, Hua-Gang, E-mail: huagzhu@yeah.net; Ye, Yu-Sheng, E-mail: yeyusheng@aliyun.com; Li, Yong-Sheng, E-mail: 872868848@qq.com; Zhang, Zhi-Gong, E-mail: zzgedward@sina.com; Xie, Wen-Tao, E-mail: 345344347@qq.com [The First Affiliated Hospital of Anhui Medical University, Department of Vascular Surgery (China)

    2017-03-15

    Thoracic aortic aneurysms are now routinely repaired with endovascular repair if anatomically feasible because of advantages in safety and recovery. However, intraoperative aneurysm rupture is a severe complication which may have an adverse effect on the outcome of treatment. Comprehensive preoperative assessment and considerate treatment are keys to success of endovascular aneurysm repair, especially during unexpected circumstances. Few cases have reported on intraoperative aortic rupture, which were successfully managed by endovascular treatment. Here, we present a rare case of an intraoperative aneurysm rupture during endovascular repair of thoracic aortic aneurysm with narrow neck and angulated aorta arch (coarctation-associated aneurysm), which was successfully treated using double access route approach and iliac limbs of infrarenal devices.Level of EvidenceLevel 5.

  11. 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.

  12. 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.

  13. Intentional left subclavian artery coverage during thoracic endovascular aortic repair for traumatic aortic injury.

    Science.gov (United States)

    McBride, Cameron L; Dubose, Joseph J; Miller, Charles C; Perlick, Alexa P; Charlton-Ouw, Kristofer M; Estrera, Anthony L; Safi, Hazim J; Azizzadeh, Ali

    2015-01-01

    Thoracic endovascular aortic repair (TEVAR) is widely used for treatment of traumatic aortic injury (TAI). Stent graft coverage of the left subclavian artery (LSA) may be required in up to 40% of patients. We evaluated the long-term effects of intentional LSA coverage (LSAC) on symptoms and return to normal activity in TAI patients compared with a similarly treated group whose LSA was uncovered (LSAU). Patients were identified from a prospective institutional trauma registry between September 2005 and July 2012. TAI was confirmed using computed tomography angiography. The electronic medical records, angiograms, and computed tomography angiograms were reviewed in a retrospective fashion. In-person or telephone interviews were conducted using the SF-12v2 (Quality Metrics, Lincoln, RI) to assess quality of life. An additional questionnaire was used to assess specific LSA symptoms and the ability to return to normal activities. Data were analyzed by Spearman rank correlation and multiple linear and logistic regression analysis with appropriate transformations using SAS software (SAS Institute, Cary, NC). During the study period, 82 patients (57 men; mean age 40.5 ± 20 years, mean Injury Severity Score, 34 ± 10.0) underwent TEVAR for treatment of TAI. Among them, LSAC was used in 32 (39.5%) and LSAU in 50. A group of the LSAU patients (n = 22) served as matched controls in the analysis. We found no statistically significant difference in SF-12v2 physical health scores (ρ = -0.08; P = .62) between LSAC and LSAU patients. LSAC patients had slightly better mental health scores (ρ = 0.62; P = .037) than LSAU patients. LSAC patients did not have an increased likelihood of experiencing pain (ρ = -0.0056; P = .97), numbness (ρ = -0.12; P = .45), paresthesia (ρ = -0.11; P = .48), fatigue (ρ = -0.066; P = .69), or cramping (ρ = -0.12; P = .45). We found no difference between groups in the ability to return to activities. The mean follow-up time was 3.35 years. Six LSAC

  14. Society of Thoracic Surgeons Risk Score predicts hospital charges and resource use after aortic valve replacement.

    Science.gov (United States)

    Arnaoutakis, George J; George, Timothy J; Alejo, Diane E; Merlo, Christian A; Baumgartner, William A; Cameron, Duke E; Shah, Ashish S

    2011-09-01

    The impact of Society of Thoracic Surgeons predicted mortality risk score on resource use has not been previously studied. We hypothesize that increasing Society of Thoracic Surgeons risk scores in patients undergoing aortic valve replacement are associated with greater hospital charges. Clinical and financial data for patients undergoing aortic valve replacement at The Johns Hopkins Hospital over a 10-year period (January 2000 to December 2009) were reviewed. The current Society of Thoracic Surgeons formula (v2.61) for in-hospital mortality was used for all patients. After stratification into risk quartiles, index admission hospital charges were compared across risk strata with rank-sum and Kruskal-Wallis tests. Linear regression and Spearman's coefficient assessed correlation and goodness of fit. Multivariable analysis assessed relative contributions of individual variables on overall charges. A total of 553 patients underwent aortic valve replacement during the study period. Average predicted mortality was 2.9% (±3.4) and actual mortality was 3.4% for aortic valve replacement. Median charges were greater in the upper quartile of patients undergoing aortic valve replacement (quartiles 1-3, $39,949 [interquartile range, 32,708-51,323] vs quartile 4, $62,301 [interquartile range, 45,952-97,103], P < .01]. On univariate linear regression, there was a positive correlation between Society of Thoracic Surgeons risk score and log-transformed charges (coefficient, 0.06; 95% confidence interval, 0.05-0.07; P < .01). Spearman's correlation R-value was 0.51. This positive correlation persisted in risk-adjusted multivariable linear regression. Each 1% increase in Society of Thoracic Surgeons risk score was associated with an added $3000 in hospital charges. This is the first study to show that increasing Society of Thoracic Surgeons risk score predicts greater charges after aortic valve replacement. As competing therapies, such as percutaneous valve replacement, emerge to

  15. 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.

  16. 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.

  17. Spinal Ischemia in Thoracic Aortic Procedures: Impact of Radiculomedullary Artery Distribution.

    Science.gov (United States)

    Kari, Fabian A; Wittmann, Karin; Krause, Sonja; Saravi, Babak; Puttfarcken, Luisa; Förster, Katharina; Rylski, Bartosz; Maier, Sven; Göbel, Ulrich; Siepe, Matthias; Czerny, Martin; Beyersdorf, Friedhelm

    2017-12-01

    The aim of this study was to assess the influence of thoracic anterior radiculomedullary artery (tARMA) distribution on spinal cord perfusion in a thoracic aortic surgical model. Twenty-six pigs (34 ± 3 kg; study group, n = 20; sham group, n = 6) underwent ligation of the left subclavian artery and thoracic segmental arteries. End points were spinal cord perfusion pressure (SCPP), regional spinal cord blood flow (SCBF), and neurologic outcome with an observation time of 3 hours. tARMA distribution patterns tested for an effect on end points included (1) maximum distance between any 2 tARMAs within the treated aortic segment (0 or 1 segment = small-distance group; >1 segment = large-distance group) and (2) distance between the end of the treated aortic segment and the first distal tARMA (at the level of the distal simulated stent-graft end = group 0; gap of 1 or more segments = group ≥1). The number of tARMA ranged from 3 to 13 (mean, 8). In the large-distance group, SCBF dropped from 0.48 ± 0.16 mL/g/min to 0.3 ± 0.08 mL/g/min (p distribution patterns of tARMAs correlate with the degree of SCBF drop and insufficient reactive parenchymal hyperemia in aortic procedures. Individual ARMA distribution patterns along the treated aortic segment could help us predict the individual risk of spinal ischemia. Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  18. 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

  19. Endovascular treatment of type II endoleak following thoracic endovascular aortic repair for thoracic aortic aneurysm: Case report of squeeze technique to reach the aneurysmal sac

    Energy Technology Data Exchange (ETDEWEB)

    Kang, Hyun Jung; Kim, Chang Won; Lee, Tae Hong; Song, Seung Hwan; Lee, Chung Won; Chung, Sung Woon [Pusan National University Hospital, School of Medicine, Pusan National University, Busan (Korea, Republic of)

    2014-12-15

    Type II endoleaks are common after thoracic endovascular aortic repair (TEVAR). Various strategies are introduced to manage type II endoleaks, such as the use of coils, plugs, or liquid embolic agents (histoacryl, thrombin, onyx, etc.) through a transarterial approach or a direct puncture of the aneurysmal sac. We herein report a case of a type II endoleak caused by reverse blood flow through intercostal artery after TEVAR which was successfully treated with n-butyl cyanoacrylate (histoacryl)-lipiodol mixture by a squeeze technique to reach the aneurismal sac using a microcatheter.

  20. Quantification of progression and regression of descending thoracic aortic wall thickness by enhanced computed tomography

    International Nuclear Information System (INIS)

    Yokoyama, Kenichi; Takasu, Junichiro; Yamamoto, Rie; Taguchi, Rie; Itani, Yasutaka; Ito, Yuichi; Watanabe, Shigeru; Masuda, Yoshiaki

    2001-01-01

    The purpose of this study is to verify the usefulness of the quantification of aortic wall involvement by enhanced computed tomography (CT). One-hundred thirteen Japanese patients underwent two enhanced CT of the descending thoracic aorta at intervals. We sliced the descending thoracic aorta continuously from the level of the tracheal bifurcation with 1 cm intervals, and we defined aortic wall volume (AWV) (cm 3 ) as the sum of a 7-slice area of aortic wall involving calcification. The average of AWV increased from 7.95±2.92 cm 3 to 8.70±2.98 cm 3 . The developmental rate of AWV (ΔAWV) was 0.270±0.281 cm 3 /year. ΔAWV did not have a significant correlation with any risk factor at the baseline. ΔAWV had significant correlation with total cholesterol, (LDL-C) low-density lipoprotein cholesterol and LDL-C/(HDL-C) high-density lipoprotein cholesterol ratio at the follow-up, and by multivariate analysis with only the LDL-C/HDL-C ratio. ΔAWV was not correlated with the intake status of hypoglycemic, antihypertensive or lipid-lowering drugs. The cut-off level of total cholesterol with the most significant odds ratio for progression of aortic wall was 190 mg/dl, and that of LDL-C was 130 mg/dl. This method proved to be useful for the non-invasive assessment of aortic wall thickness. (author)

  1. CT appearance of complications related to thoracic endovascular aortic repair (TEVAR): a pictorial essay

    International Nuclear Information System (INIS)

    Pua, U.; Tay, K.H.; Tan, B.S.; Htoo, M.M.; Sebastian, M.; Sin, K.; Chua, Y.L.

    2009-01-01

    Thoracic endovascular aortic repair (TEVAR) is a recognized treatment for various diseases involving the thoracic aorta. Patients treated with TEVAR require lifelong surveillance for potential complications, with CT being highly utilized in most centres. Endoleak is the most common complication and can be detected using CT. However, other complications such as stent strut perforations and end organ ischemia can also be detected on CT. The purpose of this pictorial essay is to illustrate the CT appearance of post-TEVAR complications encountered in our institution and to highlight their significance. (orig.)

  2. CT appearance of complications related to thoracic endovascular aortic repair (TEVAR): a pictorial essay

    Energy Technology Data Exchange (ETDEWEB)

    Pua, U. [Singapore General Hospital, Department of Diagnostic Radiology, Singapore (Singapore); Tan Tock Seng Hospital, Department of Diagnostic Radiology, Singapore (Singapore); Tay, K.H.; Tan, B.S.; Htoo, M.M. [Singapore General Hospital, Department of Diagnostic Radiology, Singapore (Singapore); Sebastian, M. [Singapore General Hospital, Department of General Surgery, Singapore (Singapore); Sin, K.; Chua, Y.L. [National Heart Centre, Department of Cardiothoracic and Vascular Surgery, Singapore (Singapore)

    2009-05-15

    Thoracic endovascular aortic repair (TEVAR) is a recognized treatment for various diseases involving the thoracic aorta. Patients treated with TEVAR require lifelong surveillance for potential complications, with CT being highly utilized in most centres. Endoleak is the most common complication and can be detected using CT. However, other complications such as stent strut perforations and end organ ischemia can also be detected on CT. The purpose of this pictorial essay is to illustrate the CT appearance of post-TEVAR complications encountered in our institution and to highlight their significance. (orig.)

  3. [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.

  4. 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

  5. Increased levels of interleukin-22 in thoracic aorta and plasma from patients with acute thoracic aortic dissection.

    Science.gov (United States)

    Ye, Jing; Wang, Menglong; Jiang, Huimin; Ji, Qingwei; Huang, Ying; Liu, Jianfang; Zeng, Tao; Xu, Yao; Wang, Zhen; Lin, Yingzhong; Wan, Jun

    2017-11-03

    Interleukin (IL)-22 plays important roles in the development of arterial disease, including atherosclerosis and hypertension. However, the relationship between IL-22 and acute thoracic aortic dissection (TAD) remains unknown. Blood samples were collected from patients with chest pain who underwent computed tomography angiography of the thoracic aorta but had no known preoperative diagnosis of coronary artery disease, peripheral artery disease, arthritis, and/or membranous nephropathy. Patients were divided into non-AD (NAD) and TAD groups, and the plasma concentrations of IL-22, IL-6 and tumor necrosis factor (TNF)-α were measured. In addition, aortic tissue samples from acute TAD patients and normal donors were collected, and the expression levels of IL-22 and IL-22 receptor 1 (IL-22R1) were measured. IL-22, IL-6 and TNF-α levels were significantly higher in acute TAD patients than in NAD patients (IL-22, NAD group: 27.0 (19.1, 38.6) pg/ml vs. TAD group: 32.9 (20.6, 58.3) pg/ml, pTAD (OR 1.169, 95% CI 1.069 to 1.277; p=0.001). In addition, compared with aortic tissue of normal controls, TAD aortas showed increased expression of IL-22 and IL-22R1, especially in the torn section (IL-22, non-torn section: 2.8±0.5/HPF vs. torn section 2.8±0.5/HPF, pTAD. Copyright © 2017 Elsevier B.V. All rights reserved.

  6. 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.

  7. Impact of Discordant Views in the Management of Descending Thoracic Aortic Aneurysm.

    Science.gov (United States)

    Chiu, Peter; Sailer, Anna-Margaretha; Baiocchi, Michael; Goldstone, Andrew B; Schaffer, Justin M; Trojan, Jeff; Fleischmann, Dominik; Mitchell, R Scott; Miller, D Craig; Dake, Michael D; Woo, Y Joseph; Lee, Jason T; Fischbein, Michael P

    2017-01-01

    Thoracic endovascular aortic repair has a lower perceived risk than open surgical repair and has become an increasingly popular alternative. Whether general consensus exists regarding candidacy for either operation among open and endovascular specialists is unknown. A retrospective review of isolated descending thoracic aortic aneurysm at our institution between January 2005 and October 2015 was performed, excluding trauma and dissection. Two cardiac surgeons, 2 cardiovascular surgeons, 1 vascular surgeon, and 1 interventional radiologist gave their preference for open vs endovascular repair. Interobserver agreement was assessed with the kappa coefficient. k-means clustering agnostically grouped various patterns of agreement. The mean rating was predicted using least absolute shrinkage and selection operator regression. Negative binomial regression predicted the discrepancy between our panel of raters and the historical operation. Generalized estimating equation modeling was then used to evaluate the association between the extent of discrepancy and the adverse perioperative outcome. There were 77 patients with preoperative imaging studies. Pairwise interobserver agreement was only fair (median weighted kappa 0.270 [interquartile range 0.211-0.404]). Increasing age and proximal neck length predicted an increasing preference for thoracic endovascular aortic repair in our panel; larger proximal neck diameter predicted a general preference for open surgical repair. Increasing proximal neck diameter predicted a larger discrepancy between our panel and the historical operation. Greater discrepancy was associated with adverse outcome. Substantial disagreement existed among our panel, and an exploratory analysis of the effect of increasing discrepancy demonstrated an association with adverse perioperative outcome. An investigation of the effect of a thoracic aortic team with open and endovascular specialists is warranted. Copyright © 2017 Elsevier Inc. All rights

  8. Thoracic aortic stent-graft placement combined with left subclavian artery 'chimney operation': therapeutic analysis of 15 cases with insufficient proximal anchor area

    International Nuclear Information System (INIS)

    Liu Jiayi; Huang Lianjun; Fan Zhanming; Zhang Zhaoqi

    2012-01-01

    Objective: To discuss the strategies for the management of insufficient proximal anchoring area during the performance of transluminal stent-graft placement (TSGP), and to evaluate the feasibility of intentional coverage of the left subclavian artery (LSA) together with left subclavian artery stent-graft placement by using 'chimney operation' technique. Methods: A total of 15 patients with thoracic aortic diseases complicated by insufficient proximal anchoring area, who were encountered in authors' hospital during the period from Dec. 2009 to April 2011, were enrolled in this study. The clinical data were retrospectively analyzed. The thoracic aortic diseases included aortic dissection (n=6), aortic pseudoaneurysm (n=1), aortic aneurysm (n=4) and penetrating ulcer (n=4). Of the 15 patients, the distance between the lesion and LSA anchoring site 15 mm in 2. TSGP was carried out. The ostium of LSA was intentionally and completely covered by thoracic aortic stent-graft and left subclavian artery stent-graft placement was subsequently performed. The patients were kept under observation for symptoms of cerebral and upper limb ischemia. The postoperative complications such as endoleak and the patency of LSA were assessed with angiography. Results: Thoracic aortic stent-graft placement was successfully carried out in all 15 patients. In addition, one 'chimney' stent was properly implanted in LSA in each patient. After the procedure, no complications of nervous system or severe ischemia of upper extremity occurred. Follow-up examinations performed between 5 days to 3 months after the treatment revealed that the aortic stent-graft remained in stable condition and no type Ⅰ endoleak occurred, meanwhile the blood flow in 'chimney' stent was unobstructed. Conclusion: Intentional LSA coverage with 'chimney operation' can expand the applicability of TSGP with high tolerability. It is especially useful for patients with left vertebral artery blood supply dominance or with

  9. 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.

  10. Genetic variants in FBN-1 and risk for thoracic aortic aneurysm and dissection.

    Science.gov (United States)

    Iakoubova, Olga A; Tong, Carmen H; Rowland, Charles M; Luke, May M; Garcia, Veronica E; Catanese, Joseph J; Moomiaie, Remo M; Sotonyi, Peter; Ascady, Gyorgy; Nikas, Demitrios; Dedelias, Panagiotis; Tranquilli, Maryann; Elefteriades, John A

    2014-01-01

    A recent genome wide association study (GWAS) by LeMaire et al. found that two single nucleotide polymorphisms (SNPs), rs2118181 and rs10519177 in the FBN-1 gene (encoding Fibrillin-1), were associated with thoracic aortic dissection (TAD), non-dissecting thoracic aortic aneurysm (TAA), and thoracic aortic aneurysm or dissection (TAAD); the largest effect was observed for the association of rs2118181 with TAD. We investigated whether rs2118181 and rs10519177 were associated with TAD, TAA, and TAAD in the Yale study. The genotypes of rs2118181 and rs10519177 were determined for participants in the Yale study: 637 TAAD cases (140 TAD, 497 TAA) and 275 controls from the United States, Hungary, and Greece. The association of the genotypes with TAD, TAA and TAAD were assessed using logistic regression models adjusted for sex, age, study center and hypertension. In the Yale study, rs2118181 was associated with TAD: compared with non-carriers, carriers of the risk allele had an unadjusted odds ratio for TAD of 1.80 (95% CI 1.15-2.80) and they had odds ratio for TAD of 1.87 (95% CI 1.09-3.20) after adjusting for sex, age, study center and hypertension. We did not find significant differences in aortic size, a potential confounder for TAD, between rs2118181 risk variant carriers and non-carriers: mean aortic size was 5.56 (95% CI: 5.37-5.73) for risk variant carriers (CC+CT) and was 5.48 (95% CI: 5.36-5.61) for noncarriers (TT) (p = 0.56). rs2118181 was not associated with TAA or TAAD. rs10519177 was not associated with TAD, TAA, or TAAD in the Yale study. Thus, the Yale study provided further support for the association of the FBN-1 rs2118181SNP with TAD.

  11. 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.

  12. The Benefits of Internal Thoracic Artery Catheterization in Patients With Chronic Abdominal Aortic Occlusion

    International Nuclear Information System (INIS)

    Ilic, Nikola; Davidovic, Lazar; Koncar, Igor; Dragas, Marko; Markovic, Miroslav; Colic, Momcilo; Cinara, Ilijas

    2011-01-01

    Occlusion of the abdominal aorta may be caused by an embolic lesion, but more commonly by thrombotic disease at the aortoiliac area, progressing retrograde. However, the visualization of the distal run-off via internal thoracic-epigastric inferior artery collateral channel may be a very important diagnostic tool, especially in countries with poor technical equipment. This study was designed to show the benefit of the selective internal thoracic angiography in cases with complete aortic occlusion. We present 30 patients with chronic aortic abdominal occlusion who were submitted to the transaxillary aortography and selective ITA angiography with purpose of distal run off evaluation. Angiographic evaluation was performed by two independent radiologists according to previously defined classification. Good angiographic score via internal thoracic angiography by first observer was achieved in 19 (63.3%) patients and in 18 (60%) by a second observer. Transaxillary aortography showed inferior results: good angiographic score by the first observer in six (20%) patients and by the second observer in three (3%) patients. Low extremity run-off is better visualized during internal thoracic angiography than during transaxillary aortography.

  13. 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,

  14. “ELEPHANT TRUNK” AND ENDOVASCULAR STENTGRAFTING – A HYBRID APPROACH TO THE TREATMENT OF EXTENSIVE THORACIC AORTIC ANEURYSM

    Directory of Open Access Journals (Sweden)

    Tomáš Holubec

    2013-01-01

    Full Text Available A hybrid approach to elephant trunk technique for treatment of thoracic aortic aneurysms combines a conventional surgical and endovascular therapy. Compared to surgery alone, there is a presumption that mortality and morbidity is reduced. We present a case report of a 42-year-old man with a giant aneurysm of the entire thoracic aorta, significant aortic and tricuspid regurgitation and ventricular septum defect. The patient underwent multiple consecutive operations and interventions having, among others, finally replaced the entire thoracic aorta with the use of the hybrid elephant trunk technique.

  15. 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.

  16. Endovascular stenting of a chronic ruptured type B thoracic aortic dissection, a second chance: a case report.

    Science.gov (United States)

    Arshad, Ali; Khan, Sumaira L; Whitaker, Simon C; Macsweeney, Shane T

    2008-02-07

    We aim to highlight the need for awareness of late complications of endovascular thoracic aortic stenting and the need for close follow-up of patients treated by this method. We report the first case in the English literature of an endovascular repair of a previously stented, ruptured chronic Stanford type B thoracic aortic dissection re-presenting with a type III endoleak of the original repair. Endovascular thoracic stenting is now a widely accepted technique for the treatment of thoracic aortic dissection and its complications. Long term follow up is necessary to ensure that late complications are identified and treated appropriately. In this case of type III endoleak, although technically challenging, endovascular repair was feasible and effective.

  17. MRI findings in thoracic outlet syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Aralasmak, Ayse; Sharifov, Rasul; Kilicarslan, Rukiye; Alkan, Alpay [Bezmialem Vakif University, Department of Radiology, Fatih/Istanbul (Turkey); Cevikol, Can; Karaali, Kamil; Senol, Utku [Akdeniz University, Department of Radiology, Antalya (Turkey)

    2012-11-15

    We discuss MRI findings in patients with thoracic outlet syndrome (TOS). A total of 100 neurovascular bundles were evaluated in the interscalene triangle (IS), costoclavicular (CC), and retropectoralis minor (RPM) spaces. To exclude neurogenic abnormality, MRIs of the cervical spine and brachial plexus (BPL) were obtained in neutral. To exclude compression on neurovascular bundles, sagittal T1W images were obtained vertical to the longitudinal axis of BPL from spinal cord to the medial part of the humerus, in abduction and neutral. To exclude vascular TOS, MR angiography (MRA) and venography (MRV) of the subclavian artery (SA) and vein (SV) in abduction were obtained. If there is compression on the vessels, MRA and MRV of the subclavian vessels were repeated in neutral. Seventy-one neurovascular bundles were found to be abnormal: 16 arterial-venous-neurogenic, 20 neurogenic, 1 arterial, 15 venous, 8 arterial-venous, 3 arterial-neurogenic, and 8 venous-neurogenic TOS. Overall, neurogenic TOS was noted in 69%, venous TOS in 66%, and arterial TOS in 39%. The neurovascular bundle was most commonly compressed in the CC, mostly secondary to position, and very rarely compressed in the RPM. The cause of TOS was congenital bone variations in 36%, congenital fibromuscular anomalies in 11%, and position in 53%. In 5%, there was unilateral brachial plexitis in addition to compression of the neurovascular bundle. Severe cervical spondylosis was noted in 14%, contributing to TOS symptoms. For evaluation of patients with TOS, visualization of the brachial plexus and cervical spine and dynamic evaluation of neurovascular bundles in the cervicothoracobrachial region are mandatory. (orig.)

  18. Differences in the Thoracic Aorta by Region and Sex in a Murine Model of Marfan Syndrome

    Directory of Open Access Journals (Sweden)

    Francesc Jiménez-Altayó

    2017-11-01

    Full Text Available Marfan syndrome (MFS is a hereditary disorder of the connective tissue that causes life-threatening aortic aneurysm, which initiates at the aortic root and can progress into the ascending portion. However, analysis of ascending aorta reactivity in animal models of MFS has remained elusive. Epidemiologic evidence suggests that although MFS is equally prevalent in men and women, men are at a higher risk of aortic complications than non-pregnant women. Nevertheless, there is no experimental evidence to support this hypothesis. The aim of this study was to explore whether there are regional and sex differences in the thoracic aorta function of mice heterozygous for the fibrillin 1 (Fbn1 allele encoding a missense mutation (Fbn1C1039G/+, the most common class of mutation in MFS. Ascending and descending thoracic aorta reactivity was evaluated by wire myography. Ascending aorta mRNA and protein levels, and elastic fiber integrity were assessed by qRT-PCR, Western blotting, and Verhoeff-Van Gieson histological staining, respectively. MFS differently altered reactivity in the ascending and descending thoracic aorta by either increasing or decreasing phenylephrine contractions, respectively. When mice were separated by sex, contractions to phenylephrine increased progressively from 3 to 6 months of age in MFS ascending aortas of males, whereas contractions in females were unchanged. Endothelium-dependent relaxation was unaltered in the MFS ascending aorta of either sex; an effect related to augmented endothelium-dependent hyperpolarization-type dilations. In MFS males, the non-selective cyclooxygenase (COX inhibitor indomethacin prevented the MFS-induced enhancement of phenylephrine contractions linked to increased COX-2 expression. In MFS mice of both sexes, the non-selective nitric oxide synthase inhibitor L-NAME revealed negative feedback of nitric oxide on phenylephrine contractions, which was associated with upregulation of eNOS in females. Finally

  19. Differences in the Thoracic Aorta by Region and Sex in a Murine Model of Marfan Syndrome

    Science.gov (United States)

    Jiménez-Altayó, Francesc; Siegert, Anna-Maria; Bonorino, Fabio; Meirelles, Thayna; Barberà, Laura; Dantas, Ana P.; Vila, Elisabet; Egea, Gustavo

    2017-01-01

    Marfan syndrome (MFS) is a hereditary disorder of the connective tissue that causes life-threatening aortic aneurysm, which initiates at the aortic root and can progress into the ascending portion. However, analysis of ascending aorta reactivity in animal models of MFS has remained elusive. Epidemiologic evidence suggests that although MFS is equally prevalent in men and women, men are at a higher risk of aortic complications than non-pregnant women. Nevertheless, there is no experimental evidence to support this hypothesis. The aim of this study was to explore whether there are regional and sex differences in the thoracic aorta function of mice heterozygous for the fibrillin 1 (Fbn1) allele encoding a missense mutation (Fbn1C1039G/+), the most common class of mutation in MFS. Ascending and descending thoracic aorta reactivity was evaluated by wire myography. Ascending aorta mRNA and protein levels, and elastic fiber integrity were assessed by qRT-PCR, Western blotting, and Verhoeff-Van Gieson histological staining, respectively. MFS differently altered reactivity in the ascending and descending thoracic aorta by either increasing or decreasing phenylephrine contractions, respectively. When mice were separated by sex, contractions to phenylephrine increased progressively from 3 to 6 months of age in MFS ascending aortas of males, whereas contractions in females were unchanged. Endothelium-dependent relaxation was unaltered in the MFS ascending aorta of either sex; an effect related to augmented endothelium-dependent hyperpolarization-type dilations. In MFS males, the non-selective cyclooxygenase (COX) inhibitor indomethacin prevented the MFS-induced enhancement of phenylephrine contractions linked to increased COX-2 expression. In MFS mice of both sexes, the non-selective nitric oxide synthase inhibitor L-NAME revealed negative feedback of nitric oxide on phenylephrine contractions, which was associated with upregulation of eNOS in females. Finally, MFS ascending

  20. [Change of paradigms in the surgical treatment of complex thoracic aortic disease].

    Science.gov (United States)

    Herold, Ulf; Tsagakis, Konstantinos; Kamler, Markus; Massoudy, Parwis; Assenmacher, Eva; Eggebrecht, Holger; Buck, Thomas; Jakob, Heinz

    2006-08-01

    One of the main issues in complex thoracic aortic disease, requiring the replacement of the ascending aorta, the entire aortic arch and the descending aorta, is the vast amount of surgery necessary to cure the patient. Though one-stage repair is feasible by a clamshell thoracotomy, the associated surgical trauma and perioperative morbidity limit this approach to younger patients only. Classic surgical repair consist of a two-stage strategy, whereby, in the first step, the ascending aorta and the aortic arch are replaced via a midline sternotomy. In the second step, via a lateral thoracotomy, the descending aorta is replaced. The two stages may sum up to a mortality of 20%; furthermore, the waiting period between the stages is associated with a mortality rate of 10% of its own. Additionally, the two-stage strategy has an inherent limitation, due to the comorbidity and advanced age of the majority of patients. Therefore, the second stage cannot be offered to up to 30% of patients. New developments and improvements in aortic surgery were introduced to overcome these shortcomings and to simplify the surgical repair. The "elephant trunk" principle, introduced by Borst et al. in 1983, was an important step to facilitate surgical repair, but still required the second step. With the introduction of endovascular repair of thoracic aortic disease with stent grafts implanted retrograde via the femoral artery, new therapeutic concepts emerged. In the late 1990s, two Japanese groups reported first trials to stabilize the free-floating "elephant trunk" prosthesis by implantation of nitinol stent grafts into the vascular graft. The applied devices were purely custom-made and nonstandardized. The availability of industrially made and CE-marked stent-graft devices raised the possibility to apply them in open aortic arch surgery. The experience with stent-graft devices implanted antegrade into the descending aorta (Medtronic Talent) was reported first by the Essen and the Vienna

  1. Multidisciplinary Treatment Approach for Prosthetic Vascular Graft Infection in the Thoracic Aortic Area

    Science.gov (United States)

    Watanabe, Yoshinori

    2015-01-01

    Prosthetic vascular graft infection in the thoracic aortic area is a rare but serious complication. Adequate management of the complication is essential to increase the chance of success of open surgery. While surgical site infection is suggested as the root cause of the complication, it is also related to decreased host tolerance, especially as found in elderly patients. The handling of prosthetic vascular graft infection has been widely discussed to date. This paper mainly provides a summary of literature reports published within the past 5 years to discuss issues related to multidisciplinary treatment approaches, including surgical site infection, timing of onset, diagnostic methods, causative pathogens, auxiliary diagnostic methods, antibiotic treatment, anti-infective structures of vascular prostheses, surgical treatment, treatment strategy against infectious aortic aneurysms, future surgical treatment, postoperative systemic therapy, and antimicrobial stewardship. A thorough understanding of these issues will enable us to prevent prosthetic vascular graft infection in the thoracic aortic area as far as possible. In the event of its occurrence, the early introduction of appropriate treatment is expected to cure the disease without worsening of the underlying pathological condition. PMID:26356686

  2. Aortic rupture complicating a fracture of an ankylosed thoracic spine. A case report.

    Science.gov (United States)

    Savolaine, E R; Ebraheim, N A; Stitgen, S; Jackson, W T

    1991-11-01

    A 34-year-old man was injured in a motorcycle accident and suffered both aortic rupture and thoracic spinal fracture, complicated by an underlying undetected ankylosing spondylitis. The latter disease can affect the integrity of vascular and spinal structure. Aortography is recommended as a high priority for the patient in an unstable cardiovascular condition requiring a definitive diagnosis. Aortic rupture and thoracic spine fracture may occur from high energy deceleration trauma. Motor vehicle passenger and pedestrian injuries are most commonly involved, although airline accidents and high falls also generate some cases. Mediastinal widening, displacement of esophagus and trachea, apical dissection of blood, and, especially, paravertebral pleural space widening are common to both injuries. Whereas most mediastinal hematomas are nonaortic in origin, a combined injury must be considered because clinical features may also overlap. These include hypotension (hypovolemic or spinal shock), paraplegia, and severe back pain. In light of the high mortality and time constraints associated with aortic rupture, immediate diagnostic resolution is necessary for appropriate management and priority of investigation.

  3. 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 ...

  4. Impact of Surgical Stroke on the Early and Late Outcomes After Thoracic Aortic Operations.

    Science.gov (United States)

    Okada, Noritaka; Oshima, Hideki; Narita, Yuji; Abe, Tomonobu; Araki, Yoshimori; Mutsuga, Masato; Fujimoto, Kazuro L; Tokuda, Yoshiyuki; Usui, Akihiko

    2015-06-01

    Thoracic aortic operations still remain associated with substantial risks of death and neurologic injury. This study investigated the impact of surgical stroke on the early and late outcomes, focusing on the physical status and quality of life (QOL). From 1986 to 2008, 500 patients (aged 63 ± 13 years) underwent open thoracic aortic repair for root and ascending (31%), arch (39%), extended arch (10%), and descending and thoracoabdominal (19%) aneurysms. Brain protection consisted of retrograde cerebral perfusion (52%), antegrade cerebral perfusion (29%), and simple deep hypothermic circulatory arrest (19%). Surgical stroke was defined as a neurologic deficit persisting more than 72 hours after the operation. QOL was assessed with the Short-Form 36 Health Survey Questionnaire 5.9 ± 4.2 years after the operation. Stroke occurred in 10.3% of patients. Hospital mortality was 21% in the stroke group and 2.7% in the nonstroke group (p mental component was similar in both groups (14% vs 14%). Surgical stroke is associated with high hospital mortality and PNDs that decrease late survival and the physical component score of the QOL survey. Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  5. 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.

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

    Science.gov (United States)

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

    2008-08-01

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

  7. 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.

  8. 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.)

  9. 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.)

  10. Prevention of paraplegia after endovascular exclusion for stanford B thoracic aortic dissection aneurism

    International Nuclear Information System (INIS)

    Feng Rui; Jing Zaiping; Bao Junmin; Zhao Zhiqing; Zhao Jun; Feng Xiang; Lu Qingsheng; Huang Cheng

    2003-01-01

    Objective: To assess the prophylactic measures of paraplegia and paralysis after endovascular graft exclusion (EVE) for Stanford B thoracic aortic dissections (TAD). Methods: The records of 116 consecutive patients undergoing endovascular TAD repair from 1998 to 2001 were retrospectively reviewed. Steroids were administrated postoperatively in high risk patients likely to be candidates for paraplegia or paralysis. Results: No paraplegia or paralysis occurred postoperatively in all cases, including the patient undergone selective spinal artery angiography (SSAA). Conclusions: Transluminal repair can avoid spinal cord ischemia due to aortic cross-clamping, there is still a risk of spinal cord injury caused by occlusion of intercostal arteries under the cover of endograft. A combination of the prophylactic measures, including SSAA and steroids, have been able to reduce the risk of paraplegia and paralysis. A graft-stent of appropriate length is the key point of this procedure

  11. Relation of murine thoracic aortic structural and cellular changes with aging to passive and active mechanical properties.

    Science.gov (United States)

    Wheeler, Jason B; Mukherjee, Rupak; Stroud, Robert E; Jones, Jeffrey A; Ikonomidis, John S

    2015-02-25

    Maintenance of the structure and mechanical properties of the thoracic aorta contributes to aortic function and is dependent on the composition of the extracellular matrix and the cellular content within the aortic wall. Age-related alterations in the aorta include changes in cellular content and composition of the extracellular matrix; however, the precise roles of these age-related changes in altering aortic mechanical function are not well understood. Thoracic aortic rings from the descending segment were harvested from C57BL/6 mice aged 6 and 21 months. Thoracic aortic diameter and wall thickness were higher in the old mice. Cellular density was reduced in the medial layer of aortas from the old mice; concomitantly, collagen content was higher in old mice, but elastin content was similar between young and old mice. Stress relaxation, an index of compliance, was reduced in aortas from old mice and correlated with collagen fraction. Contractility of the aortic rings following potassium stimulation was reduced in old versus young mice. Furthermore, collagen gel contraction by aortic smooth muscle cells was reduced with age. These results demonstrate that numerous age-related structural changes occurred in the thoracic aorta and were related to alterations in mechanical properties. Aortic contractility decreased with age, likely because of a reduction in medial cell number in addition to a smooth muscle contractile deficit. Together, these unique findings provide evidence that the age-related changes in structure and mechanical function coalesce to provide an aortic substrate that may be predisposed to aortopathies. © 2015 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley Blackwell.

  12. 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.

  13. Aortopulmonary Fistula Presenting without an Endoleak after Thoracic Endovascular Aortic Repair

    Directory of Open Access Journals (Sweden)

    Giacomo Sica

    2017-08-01

    Full Text Available Herein, we report the case of a 60-year-old man, a smoker with a history of arterial hypertension and diabetes mellitus. After computed tomography (CT for an episode of hemoptysis, the patient underwent elective thoracic endovascular aortic repair (TEVAR because of a degenerative aneurysm of the descending thoracic aorta. The area of perianeurysmal pulmonary atelectasis reported on the CT scan was not considered. Three months later, he developed an aortopulmonary fistula without endoleaks. Although TEVAR is a relatively safe procedure, no detail should be overlooked in the preoperative evaluation in order to avoid life-threatening complications. Further, the effectiveness and modality of prolonged antibiotic prophylaxis and/or preoperative respiratory physiotherapy should be assessed in such cases.

  14. Relation between thoracic aortic inflammation and features of plaque vulnerability in the coronary tree in patients with non-ST-segment elevation acute coronary syndrome undergoing percutaneous coronary intervention. An FDG-positron emission tomography and optical coherence tomography study

    Energy Technology Data Exchange (ETDEWEB)

    Taglieri, Nevio; Ghetti, Gabriele; Saia, Francesco; Bacchi Reggiani, Maria Letizia; Rapezzi, Claudio [Alma Mater Studiorum Universita di Bologna, Istituto di Cardiologia, Dipartimento di Medicina Specialistica, Diagnostica e Sperimentale, Bologna (Italy); Nanni, Cristina; Bonfiglioli, Rachele; Lima, Giacomo Maria; Fanti, Stefano [Alma Mater Studiorum Universita di Bologna, Istituto di Medicina Nucleare, Dipartimento di Medicina Specialistica, Diagnostica e Sperimentale, Bologna (Italy); Marco, Valeria [CLI Foundation, Rome (Italy); Prati, Francesco [CLI Foundation, Rome (Italy); Ettore Sansavini Health Science Foundation, GVM Care and Research, Cotignola (Italy)

    2017-10-15

    To evaluate the relationship between aortic inflammation as assessed by {sup 18}F-fluorodeoxyglucose-positron emission tomography ({sup 18}F-FDG-PET) and features of plaque vulnerability as assessed by frequency domain-optical coherence tomography (FD-OCT). We enrolled 30 consecutive non-ST-segment elevation acute coronary syndrome patients undergoing percutaneous coronary intervention. All patients underwent three-vessel OCT before intervention and {sup 18}F-FDG-PET before discharge. Univariable and C-reactive protein (CRP)-adjusted linear regression analyses were performed between features of vulnerability [namely:lipid-rich plaques with and without macrophages and thin cap fibroatheromas (TCFA)] and {sup 18}F-FDG uptake in both ascending (AA) and descending aorta (DA) [measured either as averaged mean and maximum target-to-blood ratio (TBR) or as active slices (TBR{sub max} ≥ 1.6)]. Mean age was 62 years, and 26 patients were male. On univariable linear regression analysis TBR{sub mean} and TBR{sub max} in DA was associated with the number of lipid-rich plaques (β = 4.22; 95%CI 0.05-8.39; p = 0.047 and β = 3.72; 95%CI 1.14-6.30; p = 0.006, respectively). TBR{sub max} in DA was also associated with the number of lipid-rich plaques containing macrophages (β = 2.40; 95%CI 0.07-4.72; p = 0.044). A significant CRP adjusted linear association between the TBR{sub max} in DA and the number of lipid-rich plaques was observed (CRP-adjusted β = 3.58; 95%CI -0.91-6.25; p = 0.01). TBR{sub max} in DA showed a trend towards significant CRP-adjusted association with number of lipid-rich plaques with macrophages (CRP-adjusted β = 2.30; 95%CI -0.11-4.71; p = 0.06). We also observed a CRP-adjusted (β = 2.34; 95%CI 0.22-4.47; p = 0.031) linear association between the number of active slices in DA and the number of lipid-rich plaques. No relation was found between FDG uptake in the aorta and the number of TCFAs. In patients with first NSTEACS{sup ,} {sup 18}F-FDG uptake in

  15. When and how should we manage thoracic aortic injuries in the modern era?

    Science.gov (United States)

    Bottet, Benjamin; Bouchard, François; Peillon, Christophe; Baste, Jean-Marc

    2016-12-01

    A best evidence topic in cardiovascular surgery was written according to a structured protocol. The question addressed was what are the optimum treatment modality and timing of intervention for blunt thoracic aortic injury (BTAI) in the modern era? Of the 697 papers found using the reported search, 14 (5 meta-analyses, 2 prospective and 7 retrospective studies) represented the best evidence to answer the clinical question. The author, journal, country, date of publication, patient group studied, study type, relevant outcomes, results and weakness of these papers are tabulated. All five meta-analyses reported a reduction in mortality with thoracic endovascular aortic repair (TEVAR) compared with open repair (OR), but only four found the same benefit on paraplegia rate. Similarly, the two prospective and four retrospective studies showed significantly lower mortality with TEVAR than with OR. Only one study (a meta-analysis) reported a significantly lower stroke rate with TEVAR than with OR, whereas the 13 others reported a similar or even higher stroke rate. Other complication rates were identical. Four studies demonstrated that non-operative management (NOM) as a treatment option for BTAI was associated with increased mortality, even if it has declined in recent years. One study emphasized that some cases with minimal aortic injuries (Grade I and II on CT scan) could benefit from NOM. Regarding the timing of repair, only three studies analysed outcomes of delayed repair and reported significantly lower mortality than for early repair. We conclude that with lower mortality and similar overall complications including paraplegia but higher stroke rate, TEVAR is the most suitable treatment for BTAI in the modern era, where expertise exists, especially for cases of multiple associated injuries and in the older age group. Delayed aortic repair can be proposed based on CT scan analysis, but emergent repair should still be advocated for imminent free aortic rupture. NOM

  16. The Role at Rehabilitation in Treatment of Thoracic Outlet Syndrome

    Directory of Open Access Journals (Sweden)

    Mohammad Ali Hosseinian

    2003-01-01

    Full Text Available Objective: Thoracic outlet syndrome is a complex disorder caused by neurovascular irritation in the region of the thoracic outlet. The syndrome have been said to be mainly due to anomalous structures in the thoracic outlet, treatment for thoracic outlet syndrome varies among different institutions, and there has not been any standard program. In general conservative and surgical treatment can be do if necessary. Materials & Methods: The rehabilitation program consists of exercise and physiotherapy and brace designed to hold the posture in which thoracic outlet is enlarged. Exercise program was designed simple enough to be performed in the daily living or during work after minimal training and isometric exercises of Serratus anterior, Levator Scapulae and Erector Spinae muscles to be performed in one posture: flexion and elevation of scapular girdle and correction position of upper-thoracic spine. During 7 years, 131 cases of (T.O.S. were evaluated that 26 cases (20% have operated and 84 cases (64% have treated with conservative treatment and 21 cases (16% have been candidate for surgery but they didn't accepted. Results: All of the cases have treated with conservative treatment for four months. 84 cases responded well and no further treatment was needed. 47 cases were not satisfied with. The outcome of their treatment, that 26 cases have operated and 21 cases have not accepted the operation and continued the conservative treatment, they have had pain and slightly disability. 23 cases of operated group responded well and they have resumed to work, one case has had neuropraxia for about one year. Conclusion: Most cases of thoracic outlet syndrome (T.O.S. can be treated conservatively. Surgically treatment is indicated only in cases severe enough to make them disable to work. It is better all the patients undergo conservative treatment for at least four months then will decided for surgical treatment.

  17. One-stage sequential bilateral thoracic expansion for asphyxiating thoracic dystrophy (Jeune syndrome).

    Science.gov (United States)

    Muthialu, Nagarajan; Mussa, Shafi; Owens, Catherine M; Bulstrode, Neil; Elliott, Martin J

    2014-10-01

    Jeune syndrome (asphyxiating thoracic dystrophy) is a rare disorder characterized by skeletal dysplasia, reduced diameter of the thoracic cage and extrathoracic organ involvement. Fatal, early respiratory insufficiency may occur. Two-stage lateral thoracic expansion has been reported, addressing each side sequentially over 3-12 months. While staged repair theoretically provides less invasive surgery in a small child with respiratory distress, we utilized a single stage, bilateral procedure aiming to rapidly maximize lung development. Combined bilateral surgery also offered the chance of rapid recovery, and reduced hospital stay. We present our early experience of this modification of existing surgical treatment for an extremely rare condition, thought to be generally fatal in early childhood. Nine children (6 males, 3 females; median age 30 months [3.5-75]) underwent thoracic expansion for Jeune syndrome in our centre. All patients required preoperative respiratory support (5 with tracheostomy, 8 requiring positive pressure ventilation regularly within each day/night cycle). Two children underwent sequential unilateral (2-month interval between stages) and 7 children bilateral thoracic expansion by means of staggered osteotomies of third to eighth ribs and plate fixation of fourth to fifth rib and sixth to seventh rib, leaving the remaining ribs floating. There was no operative mortality. There were 2 deaths within 3 months of surgery, due to pulmonary hypertension (1 following two-stage and 1 following single-stage thoracic expansion). At the median follow-up of 11 months (1-15), 3 children have been discharged home from their referring unit and 2 have significantly reduced respiratory support. One child remains on non-invasive ventilation and another is still ventilated with a high oxygen requirement. Jeune syndrome is a difficult condition to manage, but bilateral thoracic expansion offers an effective reduction in ventilator requirements in these children

  18. 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.

  19. 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.

  20. 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...

  1. Glutathione system participation in thoracic aneurysms from patients with Marfan syndrome.

    Science.gov (United States)

    Zúñiga-Muñoz, Alejandra María; Pérez-Torres, Israel; Guarner-Lans, Verónica; Núñez-Garrido, Elías; Velázquez Espejel, Rodrigo; Huesca-Gómez, Claudia; Gamboa-Ávila, Ricardo; Soto, María Elena

    2017-05-01

    Aortic dilatation in Marfan syndrome (MFS) is progressive. It is associated with oxidative stress and endothelial dysfunction that contribute to the early acute dissection of the vessel and can result in rupture of the aorta and sudden death. We evaluated the participation of the glutathione (GSH) system, which could be involved in the mechanisms that promote the formation and progression of the aortic aneurysms in MFS patients. Aortic aneurysm tissue was obtained during chest surgery from eight control subjects and 14 MFS patients. Spectrophotometrical determination of activity of glutathione peroxidase (GPx), glutathione-S-transferase (GST), glutathione reductase (GR), lipid peroxidation (LPO) index, carbonylation, total antioxidant capacity (TAC), and concentration of reduced and oxidized glutathione (GSH and GSSG respectively), was performed in the homogenate from aortic aneurysm tissue. LPO index, carbonylation, TGF-β1, and GR activity were increased in MFS patients (p < 0.04), while TAC, GSH/GSSG ratio, GPx, and GST activity were significantly decreased (p < 0.04). The depletion of GSH, in spite of the elevated activity of GR, not only diminished the activity of GSH-depend GST and GPx, but increased LPO, carbonylation and decreased TAC. These changes could promote the structural and functional alterations in the thoracic aorta of MFS patients.

  2. Risk factors and early outcomes of acute renal injury after thoracic aortic endograft repair for type B aortic dissection

    Directory of Open Access Journals (Sweden)

    Luo S

    2017-08-01

    Full Text Available Songyuan Luo,* Huanyu Ding,* Jianfang Luo, Wei Li, Bing Ning, Yuan Liu, Wenhui Huang, Ling Xue, Ruixin Fan, Jiyan Chen Cardiology Department, Guangdong Cardiovascular Institute, Guangdong Provincial Key Laboratory of Coronary Heart Disease Prevention, Guangdong General Hospital, Guangdong Academy of Medical Sciences, Guangzhou, Guangdong, China *These authors contributed equally to this work Background: Thoracic endovascular aortic repair (TEVAR has become an emerging treatment modality for acute type B aortic dissection (TBAD patients in recent years. The risk factors and impacts of acute kidney injury (AKI after percutaneous TEVAR, however, have not been widely established.Methods: We retrospectively studied the clinical records of 305 consecutive patients who admitted to our institution and had TEVAR for TBAD between December 2009 and June 2013. The patients were routinely monitored for their renal functions preoperatively until 7 days after TEVAR. The Kidney Disease Improving Global Guidelines (KDIGO criteria were used for AKI.Results: Of the total 305 consecutive patients, 84 (27.5% developed AKI after TEVAR, comprising 66 (21.6% patients in KDIGO stage 1, 6 (2.0% patients in stage 2 and 12 (3.9% patients in stage 3. From the logistic regression analysis, systolic blood pressure (SBP on admission >140 mmHg (odds ratio [OR], 2.288; 95% CI, 1.319–3.969 and supra-aortic branches graft bypass hybrid surgery (OR, 3.228; 95% CI, 1.526–6.831 were independent risk factors for AKI after TEVAR. Local anesthesia tended to be a protective factor (OR, 0.563; 95% CI, 0.316–1.001. The preoperative renal function, angiotensin-converting enzyme inhibitor/angiotensin receptor blocker or statin administration, volume of contrast agent, range of TBAD and false lumen involving renal artery were not associated with post-operation AKI. The in-hospital mortality and major adverse events were markedly increased with the occurrence of AKI (7.1% vs 0.9%, P=0

  3. Zone zero thoracic endovascular aortic repair: A proposed modification to the classification of landing zones.

    Science.gov (United States)

    Roselli, Eric E; Idrees, Jay J; Johnston, Douglas R; Eagleton, Matthew J; Desai, Milind Y; Svensson, Lars G

    2018-04-01

    Endovascular stent-grafting provides an alternative treatment option for high-risk patients with ascending aortic disease. The feasibility of this approach has been demonstrated before. We assess the updated experience with ascending thoracic endovascular aortic repair and propose a modification of the landing zone classification based on the outcomes. From 2006 to 2016, 39 patients deemed very high risk for open replacement underwent endovascular repair of ascending aorta for acute type A dissection (12, 31%), intramural hematoma (2, 5%), pseudoaneurysm (22, 56%), and chronic dissection suture line entry tear (3, 8%). Ascending thoracic endovascular aortic repair was performed in 36 patients. In 3 patients with pseudoaneurysm, occluder devices were used. Computed tomography imaging analysis was performed, and the extent of aortic pathology was designated by segmental proximity to the left ventricle. Segmental anatomy of the proximal aorta was designed as zone 0A from the annulus to the distal margin of highest coronary, 0B extends from above the coronary to the distal margin of right pulmonary artery, and 0C extends from the right pulmonary artery border to the innominate artery. Multivariable time to event Cox regression analysis was performed to predict mortality, and long-term survival was estimated using the Kaplan-Meier method. Operative mortality was 13%; all 5 deaths occurred after emergency ascending thoracic endovascular aortic repair for type A dissection. Other complications included stroke in 4 patients (10%), myocardial infarction in 2 patients (5%), tracheostomy in 2 patients (5%), and dialysis in 2 patients (5%). In patients with acute type A dissection, the ascending pathology extended into zone 0A in 10 (71%) and 0B in 4 (29%). Among those with pseudoaneurysm, the location of the defect was in 0B in 11 (50%), 0C in 10 (45%), and 0A in 1. Among the patients with chronic dissection, the defect was located in 0C in all 3 (100%). After multivariable

  4. Thoracic endometriosis syndrome: Current concept in ...

    African Journals Online (AJOL)

    Background: Thoracic endometriosis is a rare pathology. The diagnosis is often delayed or missed, however recently, there has been significant advances in the knowledge of this condition and hence, an improvement in the diagnosis and treatment. Objective: To review the current concepts in the pathophysiology and ...

  5. 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

  6. 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.

  7. 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

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

    Science.gov (United States)

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

    2017-06-09

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

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

    Science.gov (United States)

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

    2015-10-01

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

  10. Improvement of thoracic aortic vasoreactivity by continuous and intermittent exercise in high-fat diet-induced obese rats.

    Science.gov (United States)

    Liu, Hongpeng; Yang, Zhen; Hu, Jian; Luo, Yan; Zhu, Lingqin; Yang, Huifang; Li, Guanghua

    2015-07-01

    The aim of the present study was to explore the effects of continuous and intermittent exercise on the thoracic aortic vasoreactivity and free radical metabolism in rats fed with a high-fat diet (HD). Sprague-Dawley (SD) rats were randomly divided into four groups (n=8, each group): Conventional diet (CD), HD, HD with continuous exercise (HCE) and HD with intermittent exercise (HIE). HCE rats swam once/day for 90 min; HIE rats performed swimming exercises 3 times/day, 30 min each time with an interval of 4 h. In these two groups, the exercise was conducted 5 days a week for 8 weeks. Rats in the CD and HD groups were fed without swimming training. At the end of the exercise, all the rats were sacrificed and the blood, thoracic aorta and myocardium were collected immediately. The thoracic aortic vasoreactivity, the plasma total cholesterol (TC), triglyceride (TG), high-density lipoprotein (HDL), low-density lipoprotein (LDL), superoxide dismutase (SOD), malondialdehyde (MDA) and vascular endothelial nitric oxide synthase (eNOS) gene expression were measured. Compared to the control group, in the HD group the enhanced contractile response of the thoracic aortic rings to noradrenaline (NA) was observed (Pimprove the activity of the thoracic aorta in obese rats, which may be associated with enhanced antioxidant enzyme activity and reduced free radical generating. Additionally, intermittent exercise is better than the continuous exercise in improving the thoracic aorta vasoreactivity.

  11. 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.

  12. Hybrid repair of a very late, post-aortic coarctation surgery thoracic aneurysm: a case report

    Directory of Open Access Journals (Sweden)

    Tilea Ioan

    2012-08-01

    Full Text Available Abstract Introduction Local aneurysms after surgical repair of coarctation of the aorta occur mainly in patients surgically treated by Dacron patch plasty during adulthood. The management of these patients is always problematic, with frequent complications and increased mortality rates. Percutaneous stent-graft implantation avoids the need for surgical reintervention. Case presentation We report a case involving the hybrid treatment by stent-graft implantation and transposition of the left subclavian artery to the left common carotid artery of an aneurysmal dilatation of the thoracic aorta that occurred in a 64-year-old Caucasian man, operated on almost 40 years earlier with a Dacron patch plasty for aortic coarctation. Our patient presented to our facility for evaluation with back pain and shortness of breath after minimal physical effort. A physical examination revealed stony dullness to percussion of the left posterior thorax, with no other abnormalities. The results of chest radiography, followed by contrast-enhanced computed tomography and aortography, led to a diagnosis of giant aortic thoracic aneurysm. Successful treatment of the aneurysm was achieved by percutaneous stent-graft implantation combined with transposition of the left subclavian artery to the left common carotid artery. His post-procedural recovery was uneventful. Three months after the procedure, computed tomography showed complete thrombosis of the excluded aneurysm, without any clinical signs of left lower limb ischemia or new onset neurological abnormalities. Conclusions Our patient’s case illustrates the clinical outcomes of surgical interventions for aortic coarctation. However, the very late appearance of a local aneurysm is rather unusual. Management of such cases is always difficult. The decision-making should be multidisciplinary. A hybrid approach was considered the best solution for our patient.

  13. [Midterm results of thoracic aortic dissection endovascular repair in conjunctions with the location of Adamkiewicz artery].

    Science.gov (United States)

    Jia, Ying-bin; Li, Jian; Su, Yong-hui; Ma, Jie-fei; Guan, Xiao-dong; Zhang, Bai-meng

    2012-10-23

    To evaluate the effects of using longer xenografts in conjunctions with the location of Adamkiewicz artery (AKA) on midterm outcomes of endovascular treatment for thoracic aortic dissection. From March 2005 to September 2011, 217 patients with type B dissection were recruited. There were 143 males and 74 females with a mean age of 65 ± 11 years. Among them, 43 patients were from Fifth Affiliated Hospital of Sun Yat-Sen University while another 174 patients from Affiliated Zhongshan Hospital of Fudan University. They were divided into 2 groups according to whether AKA was identified or not pre-operatively. Endovascular repairs were performed for all patients. Distal landing levels of xenografts were recorded. The thrombosis of false lumen and the complications of spinal cord injury and endoleak were analyzed. AKA was detected in 121 (55.8%) patients (group A) but not in 96 (44.2%) patients (group B). According to the levels of AKA, the patients of group A obtained the stabilization of affected thoracic aorta over a longer distance. And the ratio of patients with distal landing levels at T8-T10 was significantly higher than in group B (59.5% vs 12.5%, χ² = 49.85, P < 0.01). Also, during the follow-up period of 7.3 months, the ratio of patients with total thrombosis of false lumen in group A was significantly higher than that in group B (32.1% vs 19.1%, χ² = 4.34, P < 0.05). During the endovascular repair of thoracic aortic dissection, selecting a longer device may provide a better structural stability of affected aorta and promote false lumen thrombosis.

  14. The utility of chest X-ray as a screening tool for blunt thoracic aortic injury.

    Science.gov (United States)

    Gutierrez, Adam; Inaba, Kenji; Siboni, Stefano; Effron, Zachary; Haltmeier, Tobias; Jaffray, Paul; Reddy, Sravanthi; Lofthus, Alexander; Benjamin, Elizabeth; Dubose, Joseph; Demetriades, Demetrios

    2016-01-01

    The early and accurate identification of patients with blunt thoracic aortic injury (BTAI) remains a challenge. Traditionally, a portable AP chest X-ray (CXR) is utilized as the initial screening modality for BTAI, however, there is controversy surrounding its sensitivity. The purpose of this study was to assess the sensitivity of CXR as a screening modality for BTAI. After IRB approval, all adult (≥18 yo) blunt trauma patients admitted to LAC+USC (01/2011-12/2013) who underwent CXR and chest CT were retrospectively reviewed. Final radiology attending CXR readings were reviewed for mediastinal abnormalities (widened mediastinum, mediastinal to chest width ratio greater than 0.25, irregular aortic arch, blurred aortic contour, opacification of the aortopulmonary window, and apical pleural haematoma) suggestive of aortic injury. Chest CT final attending radiologist readings were utilized as the gold standard for diagnosis of BTAI. The primary outcome analyzed was CXR sensitivity. A total of 3728 patients were included in the study. The majority of patients were male (72.6%); mean age was 43 (SD 20). Median ISS was 9 (IQR 4-17) and median GCS was 15 (IQR 14-15). The most common mechanism of injury was MVC (48.0%), followed by fall (20.6%), and AVP (16.9%). The total number of CXRs demonstrating a mediastinal abnormality was 200 (5.4%). Widened mediastinum was present on 191 (5.1%) of CXRs, blurred aortic contour on 10 (0.3%), and irregular aortic arch on 4 (0.1%). An acute aortic injury confirmed by chest CT was present in 17 (0.5%) patients. Only 7 of these with CT-confirmed BTAI had a mediastinal abnormality identified on CXR, for a sensitivity of 41% (95% CI: 19-67%). The results from this study suggest that CXR alone is not a reliable screening modality for BTAI. A combination of screening CXR and careful consideration of other factors, such as mechanism of injury, will be required to effectively discriminate between those who should and should not undergo chest

  15. Abnormal secretion or extracellular matrix incorporation of fibrillin by dermal fibroblasts from patients with thoracic aortic aneurysms

    Energy Technology Data Exchange (ETDEWEB)

    Milewicz, D.; Cao, S.; Cosselli, J. [Univ. of Texas Medical School, Houston, TX (United States)

    1994-09-01

    Abnormal synthesis, secretion, and extracellular matrix incorporation of fibrillin is observed in the majority of fibroblast cell strains obtained from individuals with the Marfan syndrome (>85%). These fibrillin protein abnormalities are due to mutations in the FBN1 gene. We have screened fibroblast cell strains from patients with thoracic aortic aneurysms (TAA) without skeletal or ocular features of the Marfan syndrome for defects in fibrillin synthesis or processing. Dermal fibroblasts obtained from biopsies were pulse labeled with [{sup 35}S]cysteine for 30 minutes and then chased for 0, 4, and 20 hours. The media, cell lysate and extracellular matrix were harvested separately, then analyzed by SDS-PAGE. We selected fibroblasts from 17 TAA patients to study based on the development of a TAA at a young age or a family history of TAAs. Cells from 3 patients synthesized and secreted fibrillin normally, but did not incorporate the fibrillin in the extracellular matrix. None of the cell strains were found to have diminished synthesis of fibrillin when compared with control cells. We were unable to detect abnormalities in the synthesis, secretion, or matrix incorporation of fibrillin by cells from 9 of the 17 patients. These results indicate that fibrillin protein defects are found in a significant number of patients with TAAs who are young or have a family history of TAAs. Analysis of the FBN1 gene for mutations in these patients with fibrillin protein defects will determine if the observed protein abnormalities are the result of FBN1 gene mutations.

  16. Descending aortic injury by a thoracic pedicle screw during posterior reconstructive surgery: a case report.

    Science.gov (United States)

    Watanabe, Kei; Yamazaki, Akiyoshi; Hirano, Toru; Izumi, Tomohiro; Sano, Atsuki; Morita, Osamu; Kikuchi, Ren; Ito, Takui

    2010-09-15

    Case report. To describe an iatrogenic aortic injury by pedicle screw instrumentation during posterior reconstructive surgery of spinal deformity. Iatrogenic major vascular injuries during anterior instrumentation procedures have been reported by several authors, but there have been few reports regarding iatrogenic major vascular injuries during posterior instrumentation procedures. A 57-year-old woman with thoracolumbar kyphosis due to osteoporotic T12 vertebral fracture underwent posterior correction and fusion (T10-L2), using segmental pedicle screw construct concomitant with T12 pedicle subtraction osteotomy. Postoperative routine plain radiographs and computed tomography myelography demonstrated a misplaced left T10 pedicle screw, which was in contact with the posteromedial aspect of the thoracic aorta, and suspected penetration of the aortic wall. The patient underwent removal of the pedicle screw, and repair of the penetrated aortic wall through a simultaneous anterior-posterior approach. The patient tolerated the procedure well without neurologic sequelae, and was discharged several days after removal of a left tube thoracostomy. Plain radiographs demonstrated solid fusion at the osteotomy site and no loosening of hardware. Preoperative neurologic symptoms improved completely at 18-months follow-up. Use of pedicle screw instrumentation has the potential to cause major vascular injury during posterior spinal surgery, and measures to prevent this complication must be taken. Timely diagnosis and treatment are essential to prevent both early and delayed complications and death.

  17. Assessment of thoracic aortic elasticity: a preliminary study using electrocardiographically gated dual-source CT

    International Nuclear Information System (INIS)

    Li, Ning; Guo, Lijun; Sun, Haitao; Gao, Fei; Liu, Cheng; Beck, Thomas; Chen, Jiuhong; Biermann, Christina

    2011-01-01

    To gain a new insight into the elastic properties of the thoracic aorta in patients without aortic diseases using electrocardiographically (ECG)-gated dual-source (DS) CT. 56 subjects with no cardiovascular disease, selected from 2,700 people undergoing ECG-gated DSCT examination, were divided into three groups according to their age. CT data were reconstructed in 5% step throughout the RR interval. Diameter and area were measured at the curve of the ascending aorta (AA) and at the same level of the descending aorta (DA). The pulsation and elasticity of the aorta were evaluated. Aortic diameter changes were noted throughout the cardiac cycle. The maximum average diameter was seen at an RR interval of 24.02 ± 4.99% for the AA and 25.63 ± 4.77% for the DA. The minimum was at 93.5 ± 4.04% for the AA and 96.6 ± 4.58% for the DA. There was an age-dependent decrease in elasticity, while different correlation coefficients were found between various age groups and different elastic parameters. The properties of aortic pulsation and wall elasticity could be well shown by ECG-gated DSCT. The new findings regarding segment difference and age relevance were significant and should be taken into account in clinical trials and treatments for the elasticity related cardiovascular diseases. (orig.)

  18. Role of contrast-enhanced helical CT in the evaluation of acute thoracic aortic injuries after blunt chest trauma

    International Nuclear Information System (INIS)

    Scaglione, M.; Pinto, A.; Pinto, F.; Romano, L.; Ragozzino, A.; Grassi, R.

    2001-01-01

    The purpose of this retrospective study was to determine the value of contrast-enhanced helical CT for detecting and managing acute thoracic aortic injury (ATAI). Between June 1995 and February 2000, 1419 consecutive chest CT examinations were performed in the setting of major blunt trauma. The following CT findings were considered indicative of ATAI: intimal flap; pseudoaneurysm; contour irregularity; lumen abnormality; and extravasation of contrast material. On the basis of these direct findings no further diagnostic investigations were performed. Isolated mediastinal hematoma on CT scans was considered an indirect sign of ATAI: In these cases, thoracic aortography was performed even if CT indicated normal aorta. Seventy-seven patients had abnormal CT scans: Among the 23 patients with direct CT signs, acute thoracic aortic injuries was confirmed at thoracotomy in 21. Two false-positive cases were observed. The 54 remaining patients had isolated mediastinal hematoma without aortic injuries at CT and corresponding negative angiograms. The 1342 patients with negative CT scans were included in the 8-month follow-up program and did not show any adverse sequela based on clinical and radiographic criteria. Contrast-enhanced helical CT has a critical role in the exclusion of thoracic aortic injuries in patient with major blunt chest trauma and prevents unnecessary thoracic aortography. Direct CT signs of ATAI do not require further diagnostic investigations to confirm the diagnosis: Isolated aortic bands or contour vessel abnormalities should be first considered as possible artifacts or related to non-traumatic etiologies especially when mediastinal hematoma is absent. In cases of isolated mediastinal hematoma other possible sources of bleeding should be considered before directing patients to thoracic aortography. (orig.)

  19. [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.

  20. 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.

  1. Evaluation of chemokine receptors (CCRs expression on peripheral blood T-lymphocyte subsets in patients with thoracic aortic aneurysm

    Directory of Open Access Journals (Sweden)

    Kaushal Kishore Tiwari

    2016-03-01

    Full Text Available Background & Objectives: Mortality and morbidity from the complication of aortic aneurysm remain very high. Aortic size index, which classify thoracic aortic aneurysm patients in three risk groups for aortic rupture prediction. Recent data support that aortic wall remodeling is a dynamic process with active involvement of the chronic inflammation and immunological system. Aim of our study is to evaluate expression level of chemokine receptors known to be involved in the T-cells migration and to correlate them with aortic size index. Materials & Methods: Total 20 patients undergoing surgery for ascending aortic aneurysm and/or aortic valve surgery were enrolled. Aortic size index was calculated. Preoperatively blood samples collected. By flowcytometry and dual parameter dot plot technology percentage of positivity of CCR5 on these T-cell subsets were quantified. Results: Mean age of the patients was 67±5.93 years. Majority of patients had hypertension. Mean ascending aortic diameter was 42.1±8.14 mm. Mean Aortic size Index was 22.21±3.38 mm/m2. A statistical significance has observed between aortic size index and the expression of CCR5 on total CD4 positive T-cells (p-0.0949, and between aortic size index and CCR5 expression on the total CD3 positive T-cells (p-0.0293. Significant correlation observed between ASI and CCR5 expression on the CD8+/CD3+ T-cell subset (p-0.0183. Similarly, strong positive relationship between ASI and the expression of CCR5 on the cytotoxic CD28-/CD4+ T-cell subset (p-0.0055. Activated state of cytotoxic CD28-/CD4+ cell also correlated with aortic size index (p-0.0668.Conclusion: We conclude that T-cell mediated cytotoxic mechanism driven by CCR5 play an important role in the pathophysiology of the thoracic aortic aneurysm.JCMS Nepal. 2016;12(1:23-27.

  2. 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.

  3. Innovative postmarket device evaluation using a quality registry to monitor thoracic endovascular aortic repair in the treatment of aortic dissection.

    Science.gov (United States)

    Beck, Adam W; Lombardi, Joseph V; Abel, Dorothy B; Morales, J Pablo; Marinac-Dabic, Danica; Wang, Grace; Azizzadeh, Ali; Kern, John; Fillinger, Mark; White, Rodney; Cronenwett, Jack L; Cambria, Richard P

    2017-05-01

    United States Food and Drug Administration (FDA)-mandated postapproval studies have long been a mainstay of the continued evaluation of high-risk medical devices after initial marketing approval; however, these studies often present challenges related to patient/physician recruitment and retention. Retrospective single-center studies also do not fully represent the spectrum of real-world performance nor are they likely to have a sufficiently large enough sample size to detect important signals. In recent years, The FDA Center for Devices and Radiological Health has been promoting the development and use of patient registries to advance infrastructure and methodologies for medical device investigation. The FDA 2012 document, "Strengthening the National System for Medical Device Post-market Surveillance," highlighted registries as a core foundational infrastructure when linked to other complementary data sources, including embedded unique device identification. The Vascular Quality Initiative (VQI) thoracic endovascular aortic repair for type B aortic dissection project is an innovative method of using quality improvement registries to meet the needs of device evaluation after market approval. Here we report the organization and background of this project and highlight the innovation facilitated by collaboration of physicians, the FDA, and device manufacturers. This effort used an existing national network of VQI participants to capture patients undergoing thoracic endovascular aortic repair for acute type B aortic dissection within a registry that aligns with standard practice and existing quality efforts. The VQI captures detailed patient, device, and procedural data for consecutive eligible cases under the auspices of a Patient Safety Organization (PSO). Patients were divided into a 5-year follow-up group (200 acute; 200 chronic dissections) and a 1-year follow-up group (100 acute; 100 chronic). The 5-year cohort required additional imaging details, and the 1-year

  4. 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.

  5. 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.

  6. Use of Suture-Mediated Closure Device in Percutaneous Direct Carotid Puncture During Chimney-Thoracic Endovascular Aortic Repair

    International Nuclear Information System (INIS)

    Chan, Gabriel; Quek, Lawrence Hwee Han; Tan, Glenn Leong Wei; Pua, Uei

    2016-01-01

    BackgroundInsertion of a carotid chimney graft during thoracic endovascular aortic repair (Ch-TEVAR) is a recognized technique to extend the proximal landing zone into the aortic arch in the treatment of thoracic aortic disease. Conventional technique requires surgical exposure of the carotid artery for insertion of the carotid chimney graft.MethodologyWe describe our experience in the use of a suture-mediated closure device in percutaneous Ch-TEVAR in four patients.ResultsSuccessful hemostasis was achieved in all four patients. No complications related to the carotid puncture were recorded.ConclusionWe conclude that using suture-mediated closure device for carotid closure appears feasible and deserves further studies as a potential alternative to conventional surgical approach.

  7. Use of Suture-Mediated Closure Device in Percutaneous Direct Carotid Puncture During Chimney-Thoracic Endovascular Aortic Repair

    Energy Technology Data Exchange (ETDEWEB)

    Chan, Gabriel, E-mail: dr.changabriel@gmail.com; Quek, Lawrence Hwee Han, E-mail: lawrence-quek@ttsh.com.sg [Tan Tock Seng Hospital, Department of Diagnostic Radiology (Singapore); Tan, Glenn Leong Wei, E-mail: glenn-tan@ttsh.com.sg [Tan Tock Seng Hospital, Department of General Surgery (Singapore); Pua, Uei, E-mail: druei@yahoo.com [Tan Tock Seng Hospital, Department of Diagnostic Radiology (Singapore)

    2016-07-15

    BackgroundInsertion of a carotid chimney graft during thoracic endovascular aortic repair (Ch-TEVAR) is a recognized technique to extend the proximal landing zone into the aortic arch in the treatment of thoracic aortic disease. Conventional technique requires surgical exposure of the carotid artery for insertion of the carotid chimney graft.MethodologyWe describe our experience in the use of a suture-mediated closure device in percutaneous Ch-TEVAR in four patients.ResultsSuccessful hemostasis was achieved in all four patients. No complications related to the carotid puncture were recorded.ConclusionWe conclude that using suture-mediated closure device for carotid closure appears feasible and deserves further studies as a potential alternative to conventional surgical approach.

  8. The implantation of separating aortic stent-graft into the canine thoracic aorta: an experimental study

    International Nuclear Information System (INIS)

    Xia Jinguo; Shi Haibin; Yang Zhengqiang; Li Chao; Liu Sheng; Zu Qingquan; Li Linsun

    2011-01-01

    Objective: to assess the technical feasibility of implanting separating stent-graft into the canine thoracic aorta and to study its biocompatibility. Methods: Twelve adult dogs were randomly and equally divided into three groups. The right femoral artery was cut open, through which the separating stent-graft was inserted and deployed in the canine thoracic aorta, with the proximal end of the graft being quite close to the origin of the left subclavian artery. the technical feasibility of the deployment process was assessed. Angiography was performed at 4, 8 and 12 weeks after stent-graft placement to evaluate the position and patency of the stent-graft. Then the dogs were sacrificed and the specimens were collected for pathologic study. Both gross and microscopic examinations were made to evaluate the fixation of the stent-graft with the vessel wall, the endothelialization of stent-graft surface and the pathologic changes of the vascular wall. Results: A total of ten separating stent-grafts were successfully deployed in the canine thoracic aorta, no migration or deformation occurred. One dog died of massive bleeding due to the rupture of the right femoral artery which occurred when the delivery system containing the inner bare stent was inserted through the right femoral artery. Death occurred in another dog as a result of the ascending aorta rupture caused by the migration of outer-layer stent-graft. Angiography was conducted at 4, 8 and 12 weeks after stent-graft placement. No migration, deformation, rupture or stenosis of the implanted stent-grafts were observed. The formation of intima on the inner surface of the bare stent appeared at 4 weeks, which became more and more obvious with the time passing, and at 12 weeks complete endothelialization of stent-graft surface was observed. Conclusion: Technically, it is feasible to deploy the separating aortic stent-graft into the canine thoracic aorta. Moreover, the separating aortic stent-graft carries excellent

  9. 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

  10. Endovascular graft exclusion in treating thoracic aortic dissection: a report of 25 cases

    International Nuclear Information System (INIS)

    Chen Wei; Yang Jianyong; Zhuang Wenquan; Guo Wenbo; Li Heping; Zhong Lizhen; Huang Qiuping

    2003-01-01

    Objective: To evaluate the usefulness and efficacy of endovascular graft exclusion (EVGE) in treating thoracic aortic dissection (TAD). Methods: Twenty-five cases of TAD, including 24 cases of Standford B and 1 case of A, were treated by EVGE. The clinical outcome and morphological changes of the lesions were analyzed during a 2-20 months' follow-up. Results: Procedures were technically successful in all 25 cases, while a total of 28 stent-grafts were deployed (3 cases with 2 stent-grafts in each). Complete disappearance of the false lumen or remarkable decrease of the endoleak was noted on the angiograms after stent placement. No severe procedure-related complication was observed, and thrombosis of the false lumen was noted during the follow-up. Conclusion: EVGE is effective and reliable in treating TAD, especially for patients with sub-acute or chronic courses

  11. Management of aorto-esophageal fistula secondary after thoracic endovascular aortic repair: a review of literature.

    Science.gov (United States)

    Uno, Kaname; Koike, Tomoyuki; Takahashi, Seiichi; Komazawa, Daisuke; Shimosegawa, Tooru

    2017-10-01

    Aorto-esophageal fistula (AEF) is a rare and lethal entity, and the difficulty of making diagnosis of AEF is well-known. As promising results in the short-term effectiveness of thoracic endovascular aortic repair (TEVAR) promote its usage, the occurrence of AEF after TEVAR (post-TEVAR AEF) increases as one of the major complications. Therefore, we provide a review concerning the management strategy of post-TEVAR AEF. Although its representative symptom was reported as the triad of mid-thoracic pain and sentinel hematemesis followed by massive hematemesis, the symptom-free interval between sentinel hemorrhage and massive exsanguination is unpredictable. However, the physiological condition represents a surgical contraindication. Accordingly, early diagnosis is important, but either CT or esophago-gastro-duodenoscopy rarely depicts a typical image. The formation of post-TEVAR AEF might be associated with the infection of micro-organisms, which is uncontrollable with anti-biotic administration. The current first-line strategy is combination therapy as follows, (1) to control bleeding by TEVAR in the urgent phase, and (2) radical debridement and aortic/esophageal re-construction in the semi-urgent phase. In view of the high mortality and morbidity rate, it is proposed that the choice in treatment strategies might be affected by patient`s condition, size of the wall defects and the etiology of AEF. Practically, we should keep in mind the importance of making an early diagnosis and, once a suspicious symptom has occurred in a patient with a history of TEVAR, the existence of post-TEVAR AEF should be suspected. A prospective registry together with more developed technologies will be needed to establish a future strategy.

  12. Celiac Trunk Embolization, as a Means of Elongating Short Distal Descending Thoracic Aortic Aneurysm Necks, Prior to Endovascular Aortic Repair

    International Nuclear Information System (INIS)

    Belenky, Alexander; Haddad, Menashe; Idov, Igor; Knizhnik, Michael; Litvin, Sergey; Bachar, Gil N.; Atar, Eli

    2009-01-01

    The purpose of this study was to report our experience in elongating short distal necks of descending thoracic aortic aneurysms (DTAAs) by coil embolization of the celiac trunk prior to endovascular aneurysm repair (EVAR). During 6 years seven patients (five men and two women; mean age, 74) who had DTAAs with short distal necks unsuitable for conventional EVAR, and well patent superior and inferior mesenteric arteries based on CT, were treated in one session with EVAR after the celiac trunk was coil embolized to elongate the neck. All patients were followed by CT every 3 months in the first year and every 6 months thereafter. Technical success was achieved in all patients, and no early or late ischemic complications were noted. No procedural complications occurred and good aneurysm sealing was obtained in all patients. Three endoleaks were identified after 3 months (one patient) and 6 months (two patients); all were treated successfully with insertion of an additional stent-graft. In patients with DTAAs who are candidates for EVAR but have short aneurysm distal necks, celiac trunk embolization-only if the superior and inferior mesenteric arteries are patent-is a good and safe way to elongate the neck and enable EVAR.

  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. 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 ...

  16. Importance of designated thoracic trauma surgeons in the management of traumatic aortic transection.

    Science.gov (United States)

    Albrink, M H; Rodriguez, E; England, G J; McKeown, P P; Hurst, J M; Rosemurgy, A S

    1994-04-01

    The medical literature is replete with reports on traumatic aortic transection. These reports have delineated many factors regarding the morbidity and high mortality of this ominous injury. Most reports are reviews of the collective experience of a single institution over a period of years. It is likely that many authors writing on the subject of traumatic aortic transection have no experience with operative repair of the lesion. There has been debate about the various techniques of primary repair versus graft insertion, as well as the question of whether cardiopulmonary bypass is superior to the "clamp and sew" methods. No studies have directly examined the skills of individual surgeons with respect to outcome. We present the results of a study from a university-affiliated level I trauma center in which the outcomes from various groups of surgeons were compared over a 5-year period. The information in this study strongly suggests that designated thoracic trauma surgeons who are promptly available and have dedicated interests in trauma patients achieve better results.

  17. Endovascular Treatment of Descending Thoracic Aortic Aneurysms with the EndoFit Stent-Graft

    International Nuclear Information System (INIS)

    Saratzis, N.; Saratzis, Athanasios; Melas, N.; Ginis, G.; Lioupis, A.; Lykopoulos, D.; Lazaridis, J.; Kiskinis, Dimitrios

    2007-01-01

    Objective. To evaluate the mid-term feasibility, efficacy, and durability of descending thoracic aortic aneurysm (DTAA) exclusion using the EndoFit device (LeMaitre Vascular). Methods. Twenty-three (23) men (mean age 66 years) with a DTAA were admitted to our department for endovascular repair (21 were ASA III+ and 2 refused open repair) from January 2003 to July 2005. Results. Complete aneurysm exclusion was feasible in all subjects (100% technical success). The median follow-up was 18 months (range 8-40 months). A single stent-graft was used in 6 cases. The deployment of a second stent-graft was required in the remaining 17 patients. All endografts were attached proximally, beyond the left subclavian artery, leaving the aortic arch branches intact. No procedure-related deaths have occurred. A distal type I endoleak was detected in 2 cases on the 1 month follow-up CT scan, and was repaired with reintervention and deployment of an extension graft. A nonfatal acute myocardial infarction occurred in 1 patient in the sixth postoperative month. Graft migration, graft infection, paraplegia, cerebral or distal embolization, renal impairment or any other major complications were not observed. Conclusion. The treatment of DTAAs using the EndoFit stent-graft is technically feasible. Mid-term results in this series are promising

  18. LncRNA Expression Profile of Human Thoracic Aortic Dissection by High-Throughput Sequencing.

    Science.gov (United States)

    Sun, Jie; Chen, Guojun; Jing, Yuanwen; He, Xiang; Dong, Jianting; Zheng, Junmeng; Zou, Meisheng; Li, Hairui; Wang, Shifei; Sun, Yili; Liao, Wangjun; Liao, Yulin; Feng, Li; Bin, Jianping

    2018-01-01

    In this study, the long non-coding RNA (lncRNA) expression profile in human thoracic aortic dissection (TAD), a highly lethal cardiovascular disease, was investigated. Human TAD (n=3) and normal aortic tissues (NA) (n=3) were examined by high-throughput sequencing. Bioinformatics analyses were performed to predict the roles of aberrantly expressed lncRNAs. Quantitative real-time polymerase chain reaction (qRT-PCR) was applied to validate the results. A total of 269 lncRNAs (159 up-regulated and 110 down-regulated) and 2, 255 mRNAs (1 294 up-regulated and 961 down-regulated) were aberrantly expressed in human TAD (fold-change> 1.5, PTAD than in NA. The predicted binding motifs of three up-regulated lncRNAs (ENSG00000248508, ENSG00000226530, and EG00000259719) were correlated with up-regulated RUNX1 (R=0.982, PTAD. These findings suggest that lncRNAs are novel potential therapeutic targets for human TAD. © 2018 The Author(s). Published by S. Karger AG, Basel.

  19. 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.

  20. Thoracic aortic dissection and rupture in conotruncal cardiac defects: A population-based study.

    Science.gov (United States)

    Frischhertz, Benjamin P; Shamszad, Pirouz; Pedroza, Claudia; Milewicz, Dianna M; Morris, Shaine A

    2015-04-01

    Although the risk of thoracic aortic dissection and rupture (TAD) is well-known in bicuspid aortic valve (BAV), the risk of TAD in other congenital heart diseases (CHD), particularly conotruncal lesions like tetralogy of Fallot (TOF), truncus arteriosus, D-transposition of the great arteries (D-TGA), and double outlet right ventricle is currently unknown. The primary purpose of this study was to describe TAD in conotruncal CHD, and the secondary purpose was to explore whether an association exists between TAD and conotruncal CHD. Using the Texas Inpatient Public Use Data File, an administrative database of all Texas hospitalizations, including >37.9 million hospitalizations from January 1999 through June 2012, 12,016 cases of TAD and 214 cases of TAD in CHD were identified. The most common lesions were BAV (42%), atrial septal defect (21%), aortic coarctation (7%), ventricular septal defect (6%), and patent ductus arteriosus (4%). Three patients with TOF, 2 with D-TGA, and 1 with truncus arteriosus were admitted with TAD. An exploratory case-control study in patients older than 1 year using multilevel logistic regression models to evaluate the association between CHD and TAD that controlled for known TAD risk factors demonstrated a significant association between TAD and BAV (OR 10, 95% CI 8.2-13) but not coarctation of the aorta or any conotruncal lesion. TAD in conotruncal CHD is exquisitely rare. In our hospitalized population, there was no increased occurrence of TAD in conotruncal CHD above what would be expected in the rest of the hospitalized population. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

  1. Aortic valve-sparing in 37 patients with Marfan syndrome: midterm results with David operation.

    Science.gov (United States)

    Forteza, Alberto; De Diego, Javier; Centeno, Jorge; López, Maria Jesus; Pérez, Enrique; Martín, Carlos; Sánchez, Violeta; Rufilanchas, Juan J; Cortina, Jose

    2010-01-01

    We reviewed our experience with aortic valve-sparing operations in Marfan syndrome during last 5 years. Between March 2004 and June 2009, 94 patients with aortic root aneurysms underwent valve-sparing operations. Of these, 37 (68% male) were diagnosed with Marfan syndrome, according to the Ghent diagnostic criteria. Mean age was 30 +/- 10 years (range, 11 to 59 years). Moderate/severe aortic regurgitation was present in 13%, and the mean diameter of the Valsalva sinuses was 50 +/- 4 mm (range, 42 to 62 mm). The David V modification was performed in the last 28 patients. Additional procedures were mitral valve repair in 6, tricuspid valve repair in 3, closure of septal atrial defect in 2, and closure of a patent foramen ovale in 13. Mean follow-up was 27 +/- 16 months (range, 1 to 61 months). There were no in-hospital deaths and no major adverse outcomes. One patient required implantation of a mechanical prosthesis during the same procedure because of moderate aortic regurgitation. One late death occurred. No patients required reoperation. In the last follow-up, 23 patients did not have aortic regurgitation, 12 had grade I, and 1 had grade II. No thromboembolic complications have been documented, and 97% of the patients are free from anticoagulation. Short-term and midterm results with the reimplantation technique for aortic root aneurysms in Marfan patients are excellent. If long-term results are similar, this technique could be the treatment of choice for these patients. 2010 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  2. 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.

  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. Dynamic autophagic activity affected the development of thoracic aortic dissection by regulating functional properties of smooth muscle cells

    International Nuclear Information System (INIS)

    Wang, Yang; Zhao, Zhi-Min; Zhang, Guan-Xin; Yang, Fan; Yan, Yan; Liu, Su-Xuan; Li, Song-Hua; Wang, Guo-Kun; Xu, Zhi-Yun

    2016-01-01

    The aortic medial degeneration is the key histopathologic feature of Thoracic aortic dissection (TAD). The aim of this study was to identify the change of autophagic activity in the aortic wall during TAD development, and to explore the roles of autophagy on regulating functional properties of smooth muscle cells (SMCs). Firstly, compared with control group (n = 11), the increased expression of autophagic markers Beclin1 and LC3 was detected in the aortic wall from TAD group (n = 23) by immunochemistry and western blot. We found that more autophagic vacuoles were present in the aortic wall of TAD patients using Transmission electron microscopy. Next, autophagic activity was examined in AD mice model established by β-aminopropionitrile fumarate (BAPN) and angiotensin II. Immunochemistry proved that autophagic activity was dynamically changed during AD development. Beclin1 and LC3 were detected up-regulated in the aortic wall in the second week after BAPN feeding, earlier than the fragmentation or loss of elastic fibers. When AD occurred in the 4th week, the expression of Beclin1 and LC3 began to decrease, but still higher than the control. Furthermore, autophagy was found to inhibit starvation-induced apoptosis of SMCs. Meanwhile, blockage of autophagy could suppress PDGF-induced phenotypic switch of SMCs. Taken together, autophagic activity was dynamically changed in the aortic wall during TAD development. The abnormal autophagy could regulate the functional properties of aortic SMCs, which might be the potential pathogenesis of TAD. - Highlights: • Autophagy is up-regulated in aorta wall from thoracic aorta dissection (TAD) patient. • Autophagic activity is dynamically changed during TAD development. • Dynamically change of autophagy is associated with pathological process of TAD. • Autophagy participate in the development of TAD by regulating function of SMCs.

  5. Intraoperative use of low-dose recombinant activated factor VII during thoracic aortic operations.

    Science.gov (United States)

    Andersen, Nicholas D; Bhattacharya, Syamal D; Williams, Judson B; Fosbol, Emil L; Lockhart, Evelyn L; Patel, Mayur B; Gaca, Jeffrey G; Welsby, Ian J; Hughes, G Chad

    2012-06-01

    Numerous studies have supported the effectiveness of recombinant activated factor VII (rFVIIa) for the control of bleeding after cardiac procedures; however safety concerns persist. Here we report the novel use of intraoperative low-dose rFVIIa in thoracic aortic operations, a strategy intended to improve safety by minimizing rFVIIa exposure. Between July 2005 and December 2010, 425 consecutive patients at a single referral center underwent thoracic aortic operations with cardiopulmonary bypass (CPB); 77 of these patients received intraoperative low-dose rFVIIa (≤60 μg/kg) for severe coagulopathy after CPB. Propensity matching produced a cohort of 88 patients (44 received intraoperative low-dose rFVIIa and 44 controls) for comparison. Matched patients receiving intraoperative low-dose rFVIIa got an initial median dose of 32 μg/kg (interquartile range [IQR], 16-43 μg/kg) rFVIIa given 51 minutes (42-67 minutes) after separation from CPB. Patients receiving intraoperative low-dose rFVIIa demonstrated improved postoperative coagulation measurements (partial thromboplastin time 28.6 versus 31.5 seconds; p=0.05; international normalized ratio, 0.8 versus 1.2; pproduct transfusions (2.5 versus 5.0 units; p=0.05) compared with control patients. No patient receiving intraoperative low-dose rFVIIa required postoperative rFVIIa administration or reexploration for bleeding. Rates of stroke, thromboembolism, myocardial infarction, and other adverse events were equivalent between groups. Intraoperative low-dose rFVIIa led to improved postoperative hemostasis with no apparent increase in adverse events. Intraoperative rFVIIa administration in appropriately selected patients may correct coagulopathy early in the course of refractory blood loss and lead to improved safety through the use of smaller rFVIIa doses. Appropriately powered randomized studies are necessary to confirm the safety and efficacy of this approach. Copyright © 2012 The Society of Thoracic Surgeons

  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. 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.

  8. Familial Thoracic Aortic Aneurysm with Dissection Presenting as Flash Pulmonary Edema in a 26-Year-Old Man

    Directory of Open Access Journals (Sweden)

    Sabry Omar

    2014-01-01

    Full Text Available We are reporting a case of familial thoracic aortic aneurysm and dissection in a 26-year-old man with no significant past medical history and a family history of dissecting aortic aneurysm in his mother at the age of 40. The patient presented with cough, shortness of breath, and chest pain. Chest X-ray showed bilateral pulmonary infiltrates. CT scan of the chest showed a dissection of the ascending aorta. The patient underwent aortic dissection repair and three months later he returned to our hospital with new complaints of back pain. CT angiography showed a new aortic dissection extending from the left carotid artery through the bifurcation and into the iliac arteries. The patient underwent replacement of the aortic root, ascending aorta, total aortic arch, and aortic valve. The patient recovered well postoperatively. Genetic studies of the patient and his children revealed no mutations in ACTA2, TGFBR1, TGFBR2, TGFB2, MYH11, MYLK, SMAD3, or FBN1. This case report focuses on a patient with familial TAAD and discusses the associated genetic loci and available screening methods. It is important to recognize potential cases of familial TAAD and understand the available screening methods since early diagnosis allows appropriate management of risk factors and treatment when necessary.

  9. Application of rapid artificial cardiac pacing in thoracic endovascular aortic repair in aged patients

    Directory of Open Access Journals (Sweden)

    Chen J

    2013-12-01

    Full Text Available Jun Chen,1,* Wenhui Huang,2,* Songyuan Luo,2,* Dahao Yang,1 Zhengrong Xu,1 Jianfang Luo21Department of Angiocardiopathy, Affiliated Baoan Hospital of Southern Medical University, Shenzhen City, People's Republic of China; 2Department of Cardiology, Guangdong Cardiovascular Institute, Guangdong General Hospital, Guangdong Academy of Medical Sciences, Guangzhou, People's Republic of China*These authors contributed equally to this workObjective: To compare the safety, efficacy, and impact on stent graft positioning between rapid artificial cardiac pacing (RACP, induced hypotension and sodium nitroprusside (SNP induced hypotension during thoracic endovascular aortic repair (TEVAR for Stanford B aortic dissection.Methods: One hundred and sixty-eight patients, who were diagnosed with Stanford B aortic dissection and who underwent selective TEVAR in Guangdong General Hospital and the People's Hospital of Baoan District, Shenzhen, People's Republic of China, were enrolled in this study. Patients were randomly divided into a RACP group (n=77 and a SNP group (n=91. During localization and deployment of the stent graft, hypotension was induced by RACP or intravenous SNP, according to randomization. Hemodynamics, landing precision (deviation from planned placement site, duration of procedure, renal function, neurocognitive function, and incidence of endoleaks and paraplegia/hemiplegia were compared. Except for methods of inducing hypotension, TEVAR was performed according to the same protocol in each group.Results: RACP was successfully performed in all patients assigned to the RACP group. Compared with the SNP group, blood pressure was significantly lower (43±5 versus 81±6 mmHg, P=0.003 and the restoration time of blood pressure and the operation duration were significantly shorter (7±2 versus 451±87 seconds, P<0.001; 87±15 versus 106±18 minutes, P<0.001, respectively in the RACP group. Stent graft localization/deployment was more precise in the RACP

  10. Delayed endovascular aortic repair is associated with reduced in-hospital mortality in patients with blunt thoracic aortic injury.

    Science.gov (United States)

    Marcaccio, Christina L; Dumas, Ryan P; Huang, Yanlan; Yang, Wei; Wang, Grace J; Holena, Daniel N

    2018-02-13

    The traditional approach to stable blunt thoracic aortic injury (BTAI) endorsed by the Society for Vascular Surgery is early (<24 hours) thoracic endovascular aortic repair (TEVAR). Recently, some studies have shown improved mortality in stable BTAI patients repaired in a delayed manner (≥24 hours). However, the indications for use of delayed TEVAR for BTAI are not well characterized, and its overall impact on the patient's survival remains poorly understood. We sought to determine whether delayed TEVAR is associated with a decrease in mortality compared with early TEVAR in this population. We conducted a retrospective cohort study of adult patients with BTAI (International Classification of Diseases, Ninth Revision diagnosis code 901.0) who underwent TEVAR (International Classification of Diseases, Ninth Revision procedure code 39.73) from 2009 to 2013 using the National Sample Program data set. Missing physiologic data were imputed using chained multiple imputation. Patients were parsed into groups based on the timing of TEVAR (early, <24 hours, vs delayed, ≥24 hours). The χ 2 , Mann-Whitney, and Fisher exact tests were used to compare baseline characteristics and outcomes of interest between groups. Multivariable logistic regression for mortality was performed that included all variables significant at P ≤ .2 in univariate analyses. A total of 2045 adult patients with BTAI were identified, of whom 534 (26%) underwent TEVAR. Patients with missing data on TEVAR timing were excluded (n = 27), leaving a total of 507 patients for analysis (75% male; 69% white; median age, 40 years [interquartile range, 27-56 years]; median Injury Severity Score [ISS], 34 [interquartile range, 26-41]). Of these, 378 patients underwent early TEVAR and 129 underwent delayed TEVAR. The two groups were similar with regard to age, sex, race, ISS, and presenting physiology. Mortality was 11.9% in the early TEVAR group vs 5.4% in the delayed group, with the early group

  11. 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

  12. [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.

  13. 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

  14. Supraclavicular scalenectomy for thoracic outlet syndrome--functional outcomes assessed using the DASH scoring system.

    LENUS (Irish Health Repository)

    Glynn, Ronan W

    2012-02-01

    To evaluate supraclavicular scalenectomy ± cervical rib excision for thoracic outlet syndrome (TOS), employing Disability of Arm, Shoulder, and Hand (DASH) scoring for functional assessment post-decompression.

  15. Problems and prospects of managing thoracic aortic aneurysm at the Nnamdi Azikiwe University Teaching Hospital, Nnewi, Nigeria.

    Science.gov (United States)

    Chianakwana, Gabriel U; Odike, Maxy A C; Nwofor, Alexander M E

    2004-03-01

    The objective of this report is to highlight the problems encountered in managing thoracic aortic aneurysm in a third-generation teaching hospital serving a purely rural community in the heartland of Anambra State, in the southeastern part of Nigeria. This report also proffers solutions aimed at assisting in providing better care for patients afflicted with this condition. From time to time, patients present with vascular diseases, including aneurysm, but the condition is only occasionally suspected and sparingly investigated. This is a report of two cases within the setting of the Nnamdi Azikiwe University Teaching Hospital (NAUTH), Nnewi Nigeria. After the death of these two patients, one of whom was a member of the staff of the hospital, post mortem examinations revealed that they had died of ruptured thoracic aortic aneurysm. Their medical records were retrieved from the Medical Records Department and reviewed with the aim of analyzing their clinical features and management in the light of the unexpected post-mortem examination results. The survey of the patient records revealed that the diagnosis of thoracic aortic aneurysm was not suspected in either patient even though both had symptoms pointing to this condition. The staff member was a 55-year old man and the other patient was a 31-year old woman in her 30th week of pregnancy. We conclude by drawing the attention of medical practitioners in our community to the fact that thoracic aortic aneurysms are probably more common than we thought. Only a high index of suspicion will lead to clinical diagnosis and treatment.

  16. Computational predictions of damage propagation preceding dissection of ascending thoracic aortic aneurysms.

    Science.gov (United States)

    Mousavi, S Jamaleddin; Farzaneh, Solmaz; Avril, Stéphane

    2018-04-01

    Dissections of ascending thoracic aortic aneurysms (ATAAs) cause significant morbidity and mortality worldwide. They occur when a tear in the intima-media of the aorta permits the penetration of the blood and the subsequent delamination and separation of the wall in 2 layers, forming a false channel. To predict computationally the risk of tear formation, stress analyses should be performed layer-specifically and they should consider internal or residual stresses that exist in the tissue. In the present paper, we propose a novel layer-specific damage model based on the constrained mixture theory, which intrinsically takes into account these internal stresses and can predict appropriately the tear formation. The model is implemented in finite-element commercial software Abaqus coupled with user material subroutine. Its capability is tested by applying it to the simulation of different exemplary situations, going from in vitro bulge inflation experiments on aortic samples to in vivo overpressurizing of patient-specific ATAAs. The simulations reveal that damage correctly starts from the intimal layer (luminal side) and propagates across the media as a tear but never hits the adventitia. This scenario is typically the first stage of development of an acute dissection, which is predicted for pressures of about 2.5 times the diastolic pressure by the model after calibrating the parameters against experimental data performed on collected ATAA samples. Further validations on a larger cohort of patients should hopefully confirm the potential of the model in predicting patient-specific damage evolution and possible risk of dissection during aneurysm growth for clinical applications. Copyright © 2017 John Wiley & Sons, Ltd.

  17. Time-resolved three-dimensional magnetic resonance velocity mapping of chronic thoracic aortic dissection. A preliminary investigation

    International Nuclear Information System (INIS)

    Amano, Yasuo; Sekine, Tetsuro; Tanaka, Keiji; Takagi, Ryo; Kumita, Shinichiro; Suzuki, Yuriko

    2011-01-01

    The blood flow patterns of chronic thoracic aortic dissection are complicated, and their clinical significance remains unknown. We evaluated the technical and clinical potentials of time-resolved 3-dimensional (3D) magnetic resonance (MR) velocity mapping for assessing these patterns. We used data collected from time-resolved 3D phase-contrast MR imaging of 16 patients with chronic thoracic aortic dissection to generate time-resolved 3D MR velocity mapping that included 3D streamline and path line. We investigated blood flow patterns of this disease in the mapping and compared them with the morphological changes of the patent false lumen. Time-resolved 3D MR velocity mapping visualized rapid flow at the entry and in the true lumen immediately distal to the entry. We observed slower helical or laminar flow in the patent false lumen. In patients with disease progression, slower helical flow following rapid entry jet collided with the outer wall of the false lumen and was also observed in a growing ulcer-like projection. We showed the potential of time-resolved 3D MR velocity mapping for visualizing pathologic flow patterns related to chronic thoracic aortic dissection. (author)

  18. Heartbeat-related displacement of the thoracic aorta in patients with chronic aortic dissection type B: Quantification by dynamic CTA

    Energy Technology Data Exchange (ETDEWEB)

    Weber, Tim F. [University of Heidelberg, Department of Diagnostic and Interventional Radiology, Im Neuenheimer Feld 110, 69120 Heidelberg (Germany)], E-mail: tim.weber@med.uni-heidelberg.de; Ganten, Maria-Katharina [German Cancer Research Center, Department of Radiology, Im Neuenheimer Feld 280, 69120 Heidelberg (Germany)], E-mail: m.ganten@dkfz.de; Boeckler, Dittmar [University of Heidelberg, Department of Vascular and Endovascular Surgery, Im Neuenheimer Feld 110, 69120 Heidelberg (Germany)], E-mail: dittmar.boeckler@med.uni-heidelberg.de; Geisbuesch, Philipp [University of Heidelberg, Department of Vascular and Endovascular Surgery, Im Neuenheimer Feld 110, 69120 Heidelberg (Germany)], E-mail: philipp.geisbuesch@med.uni-heidelberg.de; Kauczor, Hans-Ulrich [University of Heidelberg, Department of Diagnostic and Interventional Radiology, Im Neuenheimer Feld 110, 69120 Heidelberg (Germany)], E-mail: hu.kauczor@med.uni-heidelberg.de; Tengg-Kobligk, Hendrik von [German Cancer Research Center, Department of Radiology, Im Neuenheimer Feld 280, 69120 Heidelberg (Germany)], E-mail: h.vontengg@dkfz.de

    2009-12-15

    Purpose: The purpose of this study was to characterize the heartbeat-related displacement of the thoracic aorta in patients with chronic aortic dissection type B (CADB). Materials and methods: Electrocardiogram-gated computed tomography angiography was performed during inspiratory breath-hold in 11 patients with CADB: Collimation 16 mm x 1 mm, pitch 0.2, slice thickness 1 mm, reconstruction increment 0.8 mm. Multiplanar reformations were taken for 20 equidistant time instances through both ascending (AAo) and descending aorta (true lumen, DAoT; false lumen, DAoF) and the vertex of the aortic arch (VA). In-plane vessel displacement was determined by region of interest analysis. Results: Mean displacement was 5.2 {+-} 1.7 mm (AAo), 1.6 {+-} 1.0 mm (VA), 0.9 {+-} 0.4 mm (DAoT), and 1.1 {+-} 0.4 mm (DAoF). This indicated a significant reduction of displacement from AAo to VA and DAoT (p < 0.05). The direction of displacement was anterior for AAo and cranial for VA. Conclusion: In CADB, the thoracic aorta undergoes a heartbeat-related displacement that exhibits an unbalanced distribution of magnitude and direction along the thoracic vessel course. Since consecutive traction forces on the aortic wall have to be assumed, these observations may have implications on pathogenesis of and treatment strategies for CADB.

  19. The effects of aprotinin on blood product transfusion associated with thoracic aortic surgery requiring deep hypothermic circulatory arrest.

    LENUS (Irish Health Repository)

    Seigne, P W

    2012-02-03

    OBJECTIVE: To compare the effects of aprotinin on blood product use and postoperative complications in patients undergoing thoracic aortic surgery requiring deep hypothermic circulatory arrest. DESIGN: A retrospective study. SETTING: A university hospital. PARTICIPANTS: Nineteen patients who underwent elective or urgent thoracic aortic surgery. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The total number of units of packed red blood cells, fresh frozen plasma, and platelets was significantly less in the group that received aprotinin (p = 0.01, 0.04, and 0.01). The intraoperative transfusion of packed red blood cells and platelets, collection and retransfusion of cell saver, and postoperative transfusion of fresh frozen plasma were also significantly less in the aprotinin group (p = 0.01, 0.02, 0.01, and 0.05). No patient in either group sustained renal dysfunction or a myocardial infarction. Two patients who had not received aprotinin suffered from chronic postoperative seizures, and one patient who had received aprotinin sustained a perioperative stroke. CONCLUSIONS: Low-dose aprotinin administration significantly decreases blood product transfusion requirements in the setting of thoracic aortic surgery requiring deep hypothermic circulatory arrest, and it does not appear to be associated with renal or myocardial dysfunction.

  20. Structure of the Elastin-Contractile Units in the Thoracic Aorta and How Genes That Cause Thoracic Aortic Aneurysms and Dissections Disrupt This Structure.

    Science.gov (United States)

    Karimi, Ashkan; Milewicz, Dianna M

    2016-01-01

    The medial layer of the aorta confers elasticity and strength to the aortic wall and is composed of alternating layers of smooth muscle cells (SMCs) and elastic fibres. The SMC elastin-contractile unit is a structural unit that links the elastin fibres to the SMCs and is characterized by the following: (1) layers of elastin fibres that are surrounded by microfibrils; (2) microfibrils that bind to the integrin receptors in focal adhesions on the cell surface of the SMCs; and (3) SMC contractile filaments that are linked to the focal adhesions on the inner side of the membrane. The genes that are altered to cause thoracic aortic aneurysms and aortic dissections encode proteins involved in the structure or function of the SMC elastin-contractile unit. Included in this gene list are the genes encoding protein that are structural components of elastin fibres and microfibrils, FBN1, MFAP5, ELN, and FBLN4. Also included are genes that encode structural proteins in the SMC contractile unit, including ACTA2, which encodes SMC-specific α-actin and MYH11, which encodes SMC-specific myosin heavy chain, along with MYLK and PRKG1, which encode kinases that control SMC contraction. Finally, mutations in the gene encoding the protein linking integrin receptors to the contractile filaments, FLNA, also predispose to thoracic aortic disease. Thus, these data suggest that functional SMC elastin-contractile units are important for maintaining the structural integrity of the aorta. Copyright © 2016 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.

  1. Image quality and diagnostic accuracy of unenhanced SSFP MR angiography compared with conventional contrast-enhanced MR angiography for the assessment of thoracic aortic diseases

    Energy Technology Data Exchange (ETDEWEB)

    Krishnam, Mayil S. [University of California, Cardiovascular and Thoracic Imaging, UCI Medical Center, Irvine, CA (United States); Tomasian, Anderanik; Malik, Sachin; Ruehm, Stefan G. [University of California at Los Angeles, Department of Radiological Sciences, Ronald Reagan Medical Center, Los Angeles, CA (United States); Desphande, Vibhas; Laub, Gerhard [Siemens Medical Solutions, Los Angeles, CA (United States)

    2010-06-15

    The purpose of this study was to determine the image quality and diagnostic accuracy of three-dimensional (3D) unenhanced steady state free precession (SSFP) magnetic resonance angiography (MRA) for the evaluation of thoracic aortic diseases. Fifty consecutive patients with known or suspected thoracic aortic disease underwent free-breathing ECG-gated unenhanced SSFP MRA with non-selective radiofrequency excitation and contrast-enhanced (CE) MRA of the thorax at 1.5 T. Two readers independently evaluated the two datasets for image quality in the aortic root, ascending aorta, aortic arch, descending aorta, and origins of supra-aortic arteries, and for abnormal findings. Signal-to-noise ratio (SNR) and contrast-to-noise ratio (CNR) were determined for both datasets. Sensitivity, specificity, and diagnostic accuracy of unenhanced SSFP MRA for the diagnosis of aortic abnormalities were determined. Abnormal aortic findings, including aneurysm (n = 47), coarctation (n = 14), dissection (n = 12), aortic graft (n = 6), intramural hematoma (n = 11), mural thrombus in the aortic arch (n = 1), and penetrating aortic ulcer (n = 9), were confidently detected on both datasets. Sensitivity, specificity, and diagnostic accuracy of SSFP MRA for the detection of aortic disease were 100% with CE-MRA serving as a reference standard. Image quality of the aortic root was significantly higher on SSFP MRA (P < 0.001) with no significant difference for other aortic segments (P > 0.05). SNR and CNR values were higher for all segments on SSFP MRA (P < 0.01). Our results suggest that free-breathing navigator-gated 3D SSFP MRA with non-selective radiofrequency excitation is a promising technique that provides high image quality and diagnostic accuracy for the assessment of thoracic aortic disease without the need for intravenous contrast material. (orig.)

  2. 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.

  3. Volumetric analysis demonstrates that true and false lumen remodeling persists for 12 months after thoracic endovascular aortic repair

    Directory of Open Access Journals (Sweden)

    Ga-Young Suh, PhD

    2016-09-01

    Full Text Available A 62-year-old man underwent an elephant trunk procedure followed by thoracic endovascular aortic repair (TEVAR. Computed tomography angiography-based models were built to quantify volume of the whole aorta and true and false lumens preoperatively, before TEVAR, after TEVAR, and at follow-up at 3, 6, and 12 months. With TEVAR, descending aortic true lumen volume increased by 54%, then increased additionally by 60% during 12 months. The descending aortic false lumen volume regressed continuously for 12 months following TEVAR, with the most rapid rate from 6 to 12 months at 16 cm3/month. TEVAR immediately increased true lumen volume and continued to remodel the true and false lumens throughout the following 12 months.

  4. Fate of patients with spinal cord ischemia complicating thoracic endovascular aortic repair.

    Science.gov (United States)

    DeSart, Kenneth; Scali, Salvatore T; Feezor, Robert J; Hong, Michael; Hess, Philip J; Beaver, Thomas M; Huber, Thomas S; Beck, Adam W

    2013-09-01

    Spinal cord ischemia (SCI) is a potentially devastating complication of thoracic endovascular aortic repair (TEVAR) that can result in varying degrees of short-term and permanent disability. This study was undertaken to describe the clinical outcomes, long-term functional impact, and influence on survival of SCI after TEVAR. A retrospective review of all TEVAR patients at the University of Florida from 2000 to 2011 was performed to identify individuals experiencing SCI, defined by any new lower extremity neurologic deficit not attributable to another cause. SCI was dichotomized into immediate or delayed onset, with immediate onset defined as SCI noted upon awakening from anesthesia, and delayed characterized as a period of normal function, followed by development of neurologic injury. Ambulatory status was determined using database query, record review, and phone interviews with patients and/or family. Mortality was estimated using life-table analysis. A total of 607 TEVARs were performed for various indications, with 57 patients (9.4%) noted to have postoperative SCI (4.3% permanent). SCI patients were more likely to be older (63.9 ± 15.6 vs 70.5 ± 11.2 years; P = .002) and have a number of comorbidities, including chronic obstructive pulmonary disease, hypertension, dyslipidemia, and cerebrovascular disease (P impact on postdischarge FI or long-term mortality. Copyright © 2013 Society for Vascular Surgery. Published by Mosby, Inc. All rights reserved.

  5. Infective endocarditis and thoracic aortic disease: A review on forgotten psychological aspects.

    Science.gov (United States)

    Suárez Bagnasco, Mariana; Núñez-Gil, Iván J

    2017-07-26

    To summarize the current evidence on psychological issues in thoracic aortic disease (TAD) and infective endocarditis (IE) setting. We performed a narrative review about psychological issues in adults with IE and TAD. Through the electronic databases, PubMed and PsycINFO, we searched full manuscripts in English and published until September 1, 2014. We found sixteen studies exploring psychological issues in patients with IE (six studies) and in TAD (ten papers). Psychological issues assessed were quality of life, depression, anxiety and posttraumatic stress disorder. Quality of life was explored in IE (four papers) and in TAD (eight papers). Depression and anxiety were analyzed in TAD only (five papers). Post-traumatic stress disorder was assessed in IE (one study). Quality of life was found impaired in three of four studies about IE and in three of eight studies about TAD. Posttraumatic stress disorder was present in 11% and was associated with lower levels of quality of life in IE patients. In TAD patients, anxiety and depression levels after different invasive interventions did not differ. Sixteen studies report about psychological issues in IE and TAD. Most of them explore quality of life and to a less extent anxiety and depression.

  6. Thoracic Stent Graft Implantation for Aortic Coarctation with Patent Ductus Arteriosus via Retroperitoneal Iliac Approach in the Presence of Small Sized Femoral Artery

    Directory of Open Access Journals (Sweden)

    Ozge Korkmaz

    2016-01-01

    Full Text Available Endovascular stent graft implantation is a favorable method for complex aortic coarctation accompanied by patent ductus arteriosus. Herein, an 18-year-old woman with complex aortic coarctation and patent ductus arteriosus was successfully treated by endovascular thoracic stent graft via retroperitoneal approach. The reason for retroperitoneal iliac approach was small sized common femoral arteries which were not suitable for stent graft passage. This case is the first aortic coarctation plus patent ductus arteriosus case described in the literature which is treated by endovascular thoracic stent graft via retroperitoneal approach.

  7. Percutaneous implantation of thoracic and abdominal aortic prostheses in patients at high surgical risk

    Directory of Open Access Journals (Sweden)

    Juan C Ortiz

    2013-02-01

    intraluminal stent implantation is an alternative. Objective: to analyze the impact of percutaneous implantation of aortic stents in high-risk surgical patients with a minimum of one y ear follow-up. Method: Descriptive study conducted from December 2005 to March 2010 which included 125 patients with thoracic or abdominal aortic aneurysm, meeting surgical criteria by its diameter and that were rejected from surgery due to their high risk. The outcomes were intraoperative death from any cause and aneurysm-related at one, six and twelve months. Complications were defined as vascular occurred during the first thirty days. Results: Abdominal aneurysm was more frequent (70.4%. The overall mortality at 25.7 months follow-up was 14.8%. Of this percentage, 5.2% died from causes related to the aneurysm. One patient died during surgery. 4.3% were reoperated for leaks. There was higher aneurysm-related mortality in the thoracic (14.7 vs. 1.2% p = 0.003 and a trend in those of larger diameter (6.9 vs. 5.7 cm p = 0.210. There was no association between mortality and diabetes mellitus, smoking, heart disease, hypertension or dyslipidemia. Conclusions: aneurysm-related mortality in patients undergoing aortic stent graft is low. Mortality was associated with thoracic aneurysm and to its greater diameter. Complications did not imply an increase in mortality. In conclusion, in patients with aortic aneurysm and high surgical risk rejected for open surgery, percutaneous approach is a safe and effective treatment in a medium-term follow-up.

  8. 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.

  9. Current Evidence and Insights about Genetics in Thoracic Aorta Disease

    Science.gov (United States)

    Muneretto, Claudio

    2013-01-01

    Thoracic aortic aneurysms have been historically considered to be caused by etiologic factors similar to those implied in abdominal aortic aneurysms. However, during the past decade, there has been increasing evidence that almost 20% of thoracic aortic aneurysms may be associated with a genetic disease, often within a syndromic or familial disorder. Moreover, the presence of congenital anomalies, such as bicuspid aortic valve, may have a unique common genetic underlying cause. Finally, also sporadic forms have been found to be potentially associated with genetic disorders, as highlighted by the analysis of rare variants and expression of specific microRNAs. We therefore sought to perform a comprehensive review of the role of genetic causes in the development of thoracic aortic aneurysms, by analyzing in detail the current evidence of genetic alterations in syndromes such as Marfan, Loeys-Dietz, and Ehler-Danlos, familial or sporadic forms, or forms associated with bicuspid aortic valve. PMID:24453931

  10. 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.

  11. Image quality and diagnostic accuracy of unenhanced SSFP MR angiography compared with conventional contrast-enhanced MR angiography for the assessment of thoracic aortic diseases

    International Nuclear Information System (INIS)

    Krishnam, Mayil S.; Tomasian, Anderanik; Malik, Sachin; Ruehm, Stefan G.; Desphande, Vibhas; Laub, Gerhard

    2010-01-01

    The purpose of this study was to determine the image quality and diagnostic accuracy of three-dimensional (3D) unenhanced steady state free precession (SSFP) magnetic resonance angiography (MRA) for the evaluation of thoracic aortic diseases. Fifty consecutive patients with known or suspected thoracic aortic disease underwent free-breathing ECG-gated unenhanced SSFP MRA with non-selective radiofrequency excitation and contrast-enhanced (CE) MRA of the thorax at 1.5 T. Two readers independently evaluated the two datasets for image quality in the aortic root, ascending aorta, aortic arch, descending aorta, and origins of supra-aortic arteries, and for abnormal findings. Signal-to-noise ratio (SNR) and contrast-to-noise ratio (CNR) were determined for both datasets. Sensitivity, specificity, and diagnostic accuracy of unenhanced SSFP MRA for the diagnosis of aortic abnormalities were determined. Abnormal aortic findings, including aneurysm (n = 47), coarctation (n = 14), dissection (n = 12), aortic graft (n = 6), intramural hematoma (n = 11), mural thrombus in the aortic arch (n = 1), and penetrating aortic ulcer (n = 9), were confidently detected on both datasets. Sensitivity, specificity, and diagnostic accuracy of SSFP MRA for the detection of aortic disease were 100% with CE-MRA serving as a reference standard. Image quality of the aortic root was significantly higher on SSFP MRA (P 0.05). SNR and CNR values were higher for all segments on SSFP MRA (P < 0.01). Our results suggest that free-breathing navigator-gated 3D SSFP MRA with non-selective radiofrequency excitation is a promising technique that provides high image quality and diagnostic accuracy for the assessment of thoracic aortic disease without the need for intravenous contrast material. (orig.)

  12. 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.

  13. Multidetector-row computed tomography of thoracic aortic anomalies in dogs and cats: Patent ductus arteriosus and vascular rings

    Directory of Open Access Journals (Sweden)

    Nolte Ingo

    2011-09-01

    Full Text Available Abstract Background Diagnosis of extracardiac intrathoracic vascular anomalies is of clinical importance, but remains challenging. Traditional imaging modalities, such as radiography, echocardiography, and angiography, are inherently limited by the difficulties of a 2-dimensional approach to a 3-dimensional object. We postulated that accurate characterization of malformations of the aorta would benefit from 3-dimensional assessment. Therefore, multidetector-row computed tomography (MDCT was chosen as a 3-dimensional, new, and noninvasive imaging technique. The purpose of this study was to evaluate patients with 2 common diseases of the intrathoracic aorta, either patent ductus arteriosus or vascular ring anomaly, by contrast-enhanced 64-row computed tomography. Results Electrocardiography (ECG-gated and thoracic nongated MDCT images were reviewed in identified cases of either a patent ductus arteriosus or vascular ring anomaly. Ductal size and morphology were determined in 6 dogs that underwent ECG-gated MDCT. Vascular ring anomalies were characterized in 7 dogs and 3 cats by ECG-gated MDCT or by a nongated thoracic standard protocol. Cardiac ECG-gated MDCT clearly displayed the morphology, length, and caliber of the patent ductus arteriosus in 6 affected dogs. Persistent right aortic arch was identified in 10 animals, 8 of which showed a coexisting aberrant left subclavian artery. A mild dilation of the proximal portion of the aberrant subclavian artery near its origin of the aorta was present in 4 dogs, and a diverticulum analogous to the human Kommerell's diverticulum was present in 2 cats. Conclusions Contrast-enhanced MDCT imaging of thoracic anomalies gives valuable information about the exact aortic arch configuration. Furthermore, MDCT was able to characterize the vascular branching patterns in dogs and cats with a persistent right aortic arch and the morphology and size of the patent ductus arteriosus in affected dogs. This additional

  14. 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 ...

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2017-04-15

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

  16. 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 ...

  17. 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.

  18. 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.

  19. The repair of a type Ia endoleak following thoracic endovascular aortic repair using a stented elephant trunk procedure.

    Science.gov (United States)

    Qi, Rui-Dong; Zhu, Jun-Ming; Liu, Yong-Min; Chen, Lei; Li, Cheng-Nan; Xing, Xiao-Yan; Sun, Li-Zhong

    2018-04-01

    Type Ia endoleaks are not uncommon complications that occur after thoracic endovascular aortic repair (TEVAR). Because aortic arch vessels prevent extension of the landing zone, it is very difficult to manipulate a type Ia endoleak using an extension cuff or stent-graft, especially when the aortic arch is involved. Here, we retrospectively review our experience of surgical treatment of type Ia endoleak after TEVAR using a stented elephant trunk procedure. From July 2010 to August 2016, we treated 17 patients diagnosed with a type Ia endoleak following TEVAR using stented elephant trunk procedure. The mean age of our patients was 52 ± 8 years. The mean interval between TEVAR and the open surgical repair was 38 ± 43 months. All cases of type Ia endoleak (100%) were repaired successfully. There were no in-hospital deaths. One case required reintubation and continuous renal replacement therapy due to renal failure; this patient recovered smoothly before discharge. One other patient suffered a stroke and renal failure and did not fully recover following discharge, or follow-up. During follow-up, there were 3 deaths. Acceptable results were obtained using a stented elephant trunk procedure in patients with a type Ia endoleak after TEVAR. This technique allowed us to repair the proximal aortic arch lesions, surgically correct the type Ia endoleak, and promote false lumen thrombosis in the distal aorta. Implantation of a stented elephant trunk, with or without a concomitant aortic arch procedure, is an alternative approach for this type of lesion. Copyright © 2017 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.

  20. 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 suffered type-B aortic dissection, despite normal aortic diameters. Following sudden increase of aortic diameters, two years later, he underwent successful stent graft implantation. In patients with Marfan syndrome, post transplantation morbidity is high, with a 40% incidence of thoracic aortic dissection. This case highlights the potential of endovascular approach for treating post-transplantation aortic dissection.

  1. 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.

  2. Wall Shear Stress Estimation of Thoracic Aortic Aneurysm Using Computational Fluid Dynamics

    Directory of Open Access Journals (Sweden)

    J. Febina

    2018-01-01

    Full Text Available An attempt has been made to evaluate the effects of wall shear stress (WSS on thoracic aortic aneurysm (TAA using Computational Fluid Dynamics (CFD. Aneurysm is an excessive localized swelling of the arterial wall due to many physiological factors and it may rupture causing shock or sudden death. The existing imaging modalities such as MRI and CT assist in the visualization of anomalies in internal organs. However, the expected dynamic behaviour of arterial bulge under stressed condition can only be effectively evaluated through mathematical modelling. In this work, a 3D aneurysm model is reconstructed from the CT scan slices and eventually the model is imported to Star CCM+ (Siemens, USA for intensive CFD analysis. The domain is discretized using polyhedral mesh with prism layers to capture the weakening boundary more accurately. When there is flow reversal in TAA as seen in the velocity vector plot, there is a chance of cell damage causing clots. This is because of the shear created in the system due to the flow pattern. It is observed from the proposed mathematical modelling that the deteriorating WSS is an indicator for possible rupture and its value oscillates over a cardiac cycle as well as over different stress conditions. In this model, the vortex formation pattern and flow reversals are also captured. The non-Newtonian model, including a pulsatile flow instead of a steady average flow, does not overpredict the WSS (15.29 Pa compared to 16 Pa for the Newtonian model. Although in a cycle the flow behaviour is laminar-turbulent-laminar (LTL, utilizing the non-Newtonian model along with LTL model also overpredicted the WSS with a value of 20.1 Pa. The numerical study presented here provides good insight of TAA using a systematic approach to numerical modelling and analysis.

  3. Contemporary economic and clinical evaluations of endovascular repair for intact descending thoracic aortic aneurysms.

    Science.gov (United States)

    Silingardi, Roberto; Gennai, Stefano; Coppi, Giovanni; Chester, Johanna; Marcheselli, Luigi; Brunetti, Massimo

    2017-12-01

    The aim of this study was to assess clinical and contemporary costs associated with elective endovascular repair of intact descending thoracic aortic aneurysms (DTAA) into the mid-term follow-up. A retrospective review of a prospectively maintained clinical database including 29 consecutive patients from July 2005 to December 2009 treated with elective endovascular repair (TEVAR) or TEVAR and surgical infrarenal repair (hybrid) of intact DTAA was performed. Mean age was 74.5 years old (±7.1). Primary clinical endpoints include mortality and major morbidity. Additionally a comprehensive economic appraisal of individual in-hospital and follow-up costs was executed. Economic endpoints include in-hospital and follow-up costs and patient discharge status. Elective endovascular and open repairs' clinical and economical outcomes in contemporary literature were assessed for comparison according to PRISMA standards. Immediate mortality was 6.9% (1/24 TEVAR and 1/5 hybrid). Three respiratory complications were recorded (11%; 2 TEVAR, 1 hybrid). Renal and cardiac complication rates were 7.4% (1 TEVAR, 1 hybrid) and 3.7% (1 TEVAR) respectively. Routine discharge home was achieved for 85% of patients (95.7% TEVAR, 25% hybrid). Three endoleaks were treated throughout the follow-up (2 TEVAR, 1 hybrid; mean 30.4 mo, ±19.9) rendering an 11% (3/27) reintervention rate. Average immediate cost was €21,976.87 for elective endovascular repair and €33,783.21 for elective endovascular hybrid repair. Additional reintervention and routine follow-up costs augmented immediate costs by 12.4%. This study supports satisfying immediate clinical outcomes for TEVAR and TEVAR+surgical infrarenal procedures. Although limited by a small population size and difficulties in economic comparisons, this study presents the real world social and economic cost scenario for both elective TEVAR and TEVAR hybrid treatment of DTAA of both the in-hospital and at mid term follow-up periods.

  4. Expression of platelet-derived growth factor B is upregulated in patients with thoracic aortic dissection.

    Science.gov (United States)

    Meng, Weixin; Liu, Shangdian; Li, Dandan; Liu, Zonghong; Yang, Hui; Sun, Bo; Liu, Hongyu

    2018-04-20

    Thoracic aortic dissection (TAD) is a serious condition requiring urgent treatment to avoid catastrophic consequences. The inflammatory response is involved in the occurrence and development of TAD, possibly potentiated by platelet-derived growth factors (PDGFs). This study aimed to determine whether expression of PDGF-B (a subunit of PDGF-BB) was increased in TAD patients and to explore the factors responsible for its upregulation and subsequent effects on TAD. Full-thickness ascending aorta wall specimens from TAD patients (n = 15) and control patients (n = 10) were examined for expression of PDGF-B and its receptor (PDGFRB) and in terms of morphology, inflammation, and fibrosis. Blood samples from TAD and control patients were collected to detect plasma levels of PDGF-BB and soluble elastins. Expression levels of PDGF-B, PDGFRB, and collagen I were significantly enhanced in ascending aorta wall specimens from TAD patients compared with controls. Furthermore, soluble elastic fragments and PDGF-BB were significantly increased in plasma from TAD patients compared with controls, and numerous irregular elastic fibers and macrophages were seen in the ascending aorta wall in TAD patients. An increase in elastic fragments in the aorta wall might be responsible for inducing the activation and migration of macrophages to injured sites, leading to elevated expression of PDGF-B, which in turn induces deposition of collagen, disrupts extracellular matrix homeostasis, and increases the stiffness of the aorta wall, resulting in compromised aorta compliance. Copyright © 2018 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.

  5. Physiologic Tolerance of Descending Thoracic Aortic Balloon Occlusion in a Swine Model of Hemorrhagic Shock

    Science.gov (United States)

    2013-01-10

    Endovascular proximal control of ruptured abdominal aortic aneurysms: the internal aortic clamp. J Cardiovasc Surg (Torino) 2009;50(3):381. 14. Arthurs Z...Starnes B, See C, Andersen C. Clamp Before You Cut: Proximal Control of Ruptured Abdominal Aortic Aneurysms Using Endovascular Balloon Occlusion

  6. 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.

  7. Three-dimensional thoracic aorta principal strain analysis from routine ECG-gated computerized tomography: feasibility in patients undergoing transcatheter aortic valve replacement.

    Science.gov (United States)

    Satriano, Alessandro; Guenther, Zachary; White, James A; Merchant, Naeem; Di Martino, Elena S; Al-Qoofi, Faisal; Lydell, Carmen P; Fine, Nowell M

    2018-05-02

    Functional impairment of the aorta is a recognized complication of aortic and aortic valve disease. Aortic strain measurement provides effective quantification of mechanical aortic function, and 3-dimenional (3D) approaches may be desirable for serial evaluation. Computerized tomographic angiography (CTA) is routinely performed for various clinical indications, and offers the unique potential to study 3D aortic deformation. We sought to investigate the feasibility of performing 3D aortic strain analysis in a candidate population of patients undergoing transcatheter aortic valve replacement (TAVR). Twenty-one patients with severe aortic valve stenosis (AS) referred for TAVR underwent ECG-gated CTA and echocardiography. CTA images were analyzed using a 3D feature-tracking based technique to construct a dynamic aortic mesh model to perform peak principal strain amplitude (PPSA) analysis. Segmental strain values were correlated against clinical, hemodynamic and echocardiographic variables. Reproducibility analysis was performed. The mean patient age was 81±6 years. Mean left ventricular ejection fraction was 52±14%, aortic valve area (AVA) 0.6±0.3 cm 2 and mean AS pressure gradient (MG) 44±11 mmHg. CTA-based 3D PPSA analysis was feasible in all subjects. Mean PPSA values for the global thoracic aorta, ascending aorta, aortic arch and descending aorta segments were 6.5±3.0, 10.2±6.0, 6.1±2.9 and 3.3±1.7%, respectively. 3D PSSA values demonstrated significantly more impairment with measures of worsening AS severity, including AVA and MG for the global thoracic aorta and ascending segment (panalysis is clinically feasible from routine ECG-gated CTA. Appropriate reductions in PSSA were identified with increasing AS hemodynamic severity. Expanded study of 3D aortic PSSA for patients with various forms of aortic disease is warranted.

  8. Detection of thoracic aortic prosthetic graft infection with 18F-fluorodeoxyglucose positron emission tomography/computed tomography.

    Science.gov (United States)

    Tokuda, Yoshiyuki; Oshima, Hideki; Araki, Yoshimori; Narita, Yuji; Mutsuga, Masato; Kato, Katsuhiko; Usui, Akihiko

    2013-06-01

    To investigate the diagnostic value of (18)F-fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT) in detecting thoracic aortic prosthetic graft infection. Nine patients with clinically suspected thoracic aortic graft infection underwent FDG-PET/CT scanning. In these patients, the diagnoses could not be confirmed using conventional modalities. The patients' clinical courses were retrospectively reviewed. On the basis of surgical, microbiological and clinical follow-up findings, the aortic grafts were considered infected in 4 patients and not infected in 5. All 4 patients with graft infection (root: 2 cases, arch: 1 case and descending: 1 case) eventually underwent in situ re-replacement. Two of the 4 patients also had abdominal grafts; however, only the thoracic grafts were replaced because uptake was low around the abdominal grafts. The maximal standardized uptake value (SUVmax) in the perigraft area was higher in the infected group than in the non-infected group (11.4 ± 4.5 vs 6.9 ± 6.4), although the difference was not statistically significant. According to the receiver operating characteristic analysis, SUVmax >8 appeared to be the cut-off value in distinguishing the two groups (sensitivity: 1.0 and specificity: 0.8). FDG-PET/CT is useful for confirming the presence of graft infection by detecting high uptake around grafts and excluding other causes of inflammation. An SUVmax value greater than 8 around a graft suggests the presence of graft infection. In addition, FDG-PET/CT can be used to clarify the precise extent of infection. This is especially useful if multiple separated prosthetic grafts have been implanted.

  9. 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.

  10. 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.

  11. 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.

  12. 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

  13. 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.

  14. 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

  15. Aortic valve sparing root surgery for Marfan syndrome.

    Science.gov (United States)

    Matalanis, George; Perera, Nisal K

    2017-11-01

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

  16. Society of Thoracic Surgeons Risk Score Predicts Hospital Charges and Resource Utilization After Aortic Valve Replacement

    Science.gov (United States)

    Arnaoutakis, George J.; George, Timothy J.; Alejo, Diane E.; Merlo, Christian A.; Baumgartner, William A.; Cameron, Duke E.; Shah, Ashish S.

    2011-01-01

    Context The impact of Society of Thoracic Surgeons (STS) predicted mortality risk score on resource utilization after aortic valve replacement (AVR) has not been previously studied. Objective We hypothesize that increasing STS risk scores in patients having AVR are associated with greater hospital charges. Design, Setting, and Patients Clinical and financial data for patients undergoing AVR at a tertiary care, university hospital over a ten-year period (1/2000–12/2009) were retrospectively reviewed. The current STS formula (v2.61) for in-hospital mortality was used for all patients. After stratification into risk quartiles (Q), index admission hospital charges were compared across risk strata with Rank-Sum tests. Linear regression and Spearman’s coefficient assessed correlation and goodness of fit. Multivariable analysis assessed relative contributions of individual variables on overall charges. Main Outcome Measures Inflation-adjusted index hospitalization total charges Results 553 patients had AVR during the study period. Average predicted mortality was 2.9% (±3.4) and actual mortality was 3.4% for AVR. Median charges were greater in the upper Q of AVR patients [Q1–3,$39,949 (IQR32,708–51,323) vs Q4,$62,301 (IQR45,952–97,103), p=<0.01]. On univariate linear regression, there was a positive correlation between STS risk score and log-transformed charges (coefficient: 0.06, 95%CI 0.05–0.07, p<0.01). Spearman’s correlation R-value was 0.51. This positive correlation persisted in risk-adjusted multivariable linear regression. Each 1% increase in STS risk score was associated with an added $3,000 in hospital charges. Conclusions This study showed increasing STS risk score predicts greater charges after AVR. As competing therapies such as percutaneous valve replacement emerge to treat high risk patients, these results serve as a benchmark to compare resource utilization. PMID:21497834

  17. 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.

  18. 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.

  19. Thoracic aortic injury in motor vehicle crashes: the effect of impact direction, side of body struck, and seat belt use.

    Science.gov (United States)

    Fitzharris, Michael; Franklyn, Melanie; Frampton, Richard; Yang, King; Morris, Andrew; Fildes, Brian

    2004-09-01

    Using in-depth, real-world motor vehicle crash data from the United States and the United Kingdom, we aimed to assess the incidence and risk factors associated with thoracic aorta injuries. De-identified National Automotive Sampling System Crashworthiness Data System (U.S.) and Co-operative Crash Injury Study (U.K.) data formed the basis of this retrospective analysis. Logistic regression was used to assess the level of risk of thoracic aorta injury associated with impact direction, seat belt use and, given the asymmetry of the thoracic cavity, whether being struck toward the left side of the body was associated with increased risk in side-impact crashes. A total of 13,436 U.S. and 3,756 U.K. drivers and front seat passengers were analyzed. The incidence of thoracic aorta injury in the U.S. and U.K. samples was 1.5% (n = 197) and 1.9% (n = 70), respectively. The risk was higher for occupants seated on the side closest to the impact than for occupants involved in frontal impact crashes. This was the case irrespective of whether the force was applied toward the left (belted: relative risk [RR], 4.6; 95% confidence interval [CI], 2.9-7.1; p direction. Thoracic aorta injuries were found to be associated with high impact severity, and being struck by a sports utility vehicle relative to a passenger vehicle (RR, 1.7; 95% CI, 1.2-2.3; p = 0.001). Aortic injuries have been conventionally associated with frontal impacts. However, emergency clinicians should be aware that occupants of side-impact crashes are at greater risk, particularly if the occupant was unbelted and involved in a crash of high impact severity.

  20. The management of thoracic inlet syndrome associated with Hurler's syndrome: a novel surgical technique.

    LENUS (Irish Health Repository)

    Ahsan, Rauf M

    2012-02-01

    A 21-year-old male developed significant swelling of his tongue after a respiratory arrest. The patient had a history of Hurler\\'s syndrome. Magnetic resonance imaging (MRI) angiogram delineated that the swelling was due to compression of his internal jugular veins at the level of the first rib, resulting in thoracic inlet obstruction. The standard surgical treatment of thoracic inlet obstruction was not suitable in this patient\\'s case due to his short thick neck and his characteristic Hurler\\'s syndrome body habitus. Therefore, a novel surgical strategy was used to decompress his head and neck vessels. The manubrium was widened using an iliac crest bone graft, stabilised using internal fixation plates and reconstructed with a pectoral muscle flap.

  1. 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.

  2. Commentary on “Inhibition of interleukin-1beta decreases aneurysm formation and progression in a novel model of thoracic aortic aneurysms”

    Directory of Open Access Journals (Sweden)

    Yonghua Bi

    2015-09-01

    Full Text Available Aortic aneurysm is a silent but life-threatening disease, whose pathogenesis remains poorly understood. Aneurysm models have been induced in small animals to study its pathogenesis, Johnston WF et al. successfully induced a novel model of thoracic aortic aneurysms (TAA by periadventitial application of elastase in mice. We comment on this model according to our experiment. We hypothesize that endogenous MMPs, especially MMP2, play a vital role in complex repair process of aneurysmal wall, which should be a key target in the investigation and treatment of aortic aneurysms.

  3. Lethal Progressive Thoracic Insufficiency in a Neonate Due to Jarcho Levin Syndrome

    Science.gov (United States)

    Bhutia, Euden; Maria, Arti; Verma, Arushi; Sethi, Sidharth Kumar

    2014-01-01

    A rare case of Jarcho Levin syndrome (JLS) presenting as a lethal progressive respiratory insufficiency in early neonatal period is reported. The neonate had classical features of this syndrome including vertebral segmentation defects, typical costo-vertebral fusion defects and scoliosis resulting in small thoracic volume and limited chest expansion; all consistent with a clinical diagnosis of JLS with thoracic insufficiency. In addition, our case had a rare association of dextrocardia and acyanotic congenital heart disease. PMID:24741543

  4. Thoracic and abdominal aortic diameters in a general population: MRI-based reference values and association with age and cardiovascular risk factors

    Energy Technology Data Exchange (ETDEWEB)

    Mensel, Birger; Hesselbarth, Lydia; Wenzel, Michael; Kuehn, Jens-Peter; Hegenscheid, Katrin [University Medicine Greifswald, Institute of Diagnostic Radiology and Neuroradiology, Greifswald (Germany); Doerr, Marcus [University Medicine Greifswald, Department of Internal Medicine, Greifswald (Germany); DZHK (German Center for Cardiovascular Research), partner site Greifswald, Greifswald (Germany); Voelzke, Henry [University Medicine Greifswald, Institute for Community Medicine, Greifswald (Germany); DZHK (German Center for Cardiovascular Research), partner site Greifswald, Greifswald (Germany); Lieb, Wolfgang [Christian Albrechts University, Institute of Epidemiology, Kiel (Germany); Lorbeer, Roberto [Ludwig-Maximilians-University Hospital, Institute of Clinical Radiology, Munich (Germany)

    2016-04-15

    To generate reference values for thoracic and abdominal aortic diameters determined by magnetic resonance imaging (MRI) and analyse their association with cardiovascular risk factors in the general population. Data from participants (n = 1759) of the Study of Health in Pomerania were used for analysis in this study. MRI measurement of thoracic and abdominal aortic diameters was performed. Parameters for calculation of reference values according to age and sex analysis were provided. Multivariable linear regression models were used for determination of aortic diameter-related risk factors, including smoking, blood pressure (BP), high-density lipoprotein cholesterol (HDL-C). For the ascending aorta (β = -0.049, p < 0.001), the aortic arch (β = -0.061, p < 0.001) and the subphrenic aorta (β = -0.018, p = 0.004), the body surface area (BSA)-adjusted diameters were lower in men. Multivariable-adjusted models revealed significant increases in BSA-adjusted diameters with age for all six aortic segments (p < 0.001). Consistent results for all segments were observed for the positive associations of diastolic BP (β = 0.001; 0.004) and HDL (β = 0.035; 0.087) with BSA-adjusted aortic diameters and for an inverse association of systolic BP (β = -0.001). Some BSA-adjusted median aortic diameters are smaller in men than in women. All diameters increase with age, diastolic blood pressure and HDL-C and decrease as systolic BP increases. (orig.)

  5. 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.

  6. 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.

  7. 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.

  8. 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.)

  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. 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....

  11. 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...

  12. 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.

  13. Mean blood velocities and flow impedance in the fetal descending thoracic aortic and common carotid artery in normal pregnancy.

    Science.gov (United States)

    Bilardo, C M; Campbell, S; Nicolaides, K H

    1988-12-01

    A linear array pulsed Doppler duplex scanner was used to establish reference ranges for mean blood velocities and flow impedance (Pulsatility Index = PI) in the descending thoracic aorta and in the common carotid artery from 70 fetuses in normal pregnancies at 17-42 weeks' gestation. The aortic velocity increased with gestation up to 32 weeks, then remained constant until term, when it decreased. In contrast, the velocity in the common carotid artery increased throughout pregnancy. The PI in the aorta remained constant throughout pregnancy, while in the common carotid artery it fell steeply after 32 weeks. These results suggest that with advancing gestation there is a redistribution of the fetal circulation with decreased impedance to flow to the fetal brain, presumably to compensate for the progressive decrease in fetal blood PO2.

  14. 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.

  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. Interleukin-6 downregulated vascular smooth muscle cell contractile proteins via ATG4B-mediated autophagy in thoracic aortic dissection.

    Science.gov (United States)

    An, Zhao; Qiao, Fan; Lu, Qijue; Ma, Ye; Liu, Yang; Lu, Fanglin; Xu, Zhiyun

    2017-12-01

    Interleukin-6 (IL-6) overexpression played an important role in the pathogenesis of thoracic aortic dissection (TAD). Our previous study found enhanced autophagy accompanying with contractile proteins α smooth muscle actin (α-SMA) and smooth muscle 22α (SM22α) degradation in TAD aortic vascular smooth muscle cells (VSMCs). Autophagy is an important way for intracellular proteins degradation, while IL-6 has been found as a contributing factor of autophagy in some cancers. These indicated IL-6 might contribute to the occurrence of TAD by promoting autophagy-induced contractile proteins degradation, which has not been investigated. The aim of the present study is to verify this hypothesis and investigate the mechanism of it. We collected 10 TAD and 10 control aortic specimens from patients underwent TAD surgical repair and coronary artery bypass grafting, respectively. Quantitative real-time polymerase chain reaction was used to detect mRNA expression. Protein expression level was assessed by enzyme-linked immunosorbent assay, western blot, and immunohistochemistry. Microtubule-associated protein 1 light chain 3 beta overexpression adenovirus with green and red fluorescent protein tags and transmission electron microscopy were used to detect autophagy level in VSMCs. 3-Methyladenine (3-MA) and chloroquine were used to block autophagy in human VSMCs. Experiment results showed that the expression of IL-6 was significantly increased accompanying with up-regulated autophagy in TAD aortic wall compared with controls. In vitro results showed that IL-6 stimulation decreased the expression of VSMCs contractile proteins α-SMA and SM22α accompanying with up-regulated autophagy. Blocking autophagy with 3-MA or chloroquine inhibited IL-6 induced α-SMA and SM22α degradation. Further investigation showed that autophagy-related 4B cysteine peptidase (ATG4B) was significantly overexpressed in TAD aortic wall and played important role in IL-6 induced autophagy up

  18. Ortner's Syndrome: Secondary Laryngeal Paralysis Caused by a Great Thoracic Aorta Aneurysm

    Directory of Open Access Journals (Sweden)

    Zangirolami, Ana Claudia Alves

    2015-01-01

    Full Text Available Introduction Recurrent laryngeal nerve injury caused by cardiovascular disease is a rare condition, and often it is the only prominent sign of an imminent break of an aortic artery aneurysm. Objective To report left laryngeal paralysis caused by a great aortic arch aneurysm and to highlight the importance of an otorhinolaryngologic evaluation along with a thoracic radiologic study. Resumed Report A 42-year-old man complained of thickness of his voice and dysphagia for 3 months, but no thoracic pain or other relevant complaints. Video laryngoscopy revealed immobility of his left vocal fold in the paramedian position. Imaging was obtained for investigation, including magnetic resonance imaging of his thorax, which showed a fusiform aneurysm in the aortic arch, leading to recurrent compression of the left laryngeal nerve. The patient was successfully treated with endovascular repair of the aneurysm. At 2-month follow-up, there was still no recovery of the laryngeal mobility. Conclusion An aortic artery aneurysm can suddenly break, requiring emergency heart surgery, and the results can be fatal in many cases. We suggest routine exam of the vocal folds in all patients with a heart condition, and we review the literature and suggest the use of imaging to reduce the number of emergency procedures.

  19. Ortner's Syndrome: Secondary Laryngeal Paralysis Caused by a Great Thoracic Aorta Aneurysm.

    Science.gov (United States)

    Zangirolami, Ana Claudia Alves; Oliveira, Frederico Vieira de; Tepedino, Miguel Soares

    2015-04-01

    Introduction Recurrent laryngeal nerve injury caused by cardiovascular disease is a rare condition, and often it is the only prominent sign of an imminent break of an aortic artery aneurysm. Objective To report left laryngeal paralysis caused by a great aortic arch aneurysm and to highlight the importance of an otorhinolaryngologic evaluation along with a thoracic radiologic study. Resumed Report A 42-year-old man complained of thickness of his voice and dysphagia for 3 months, but no thoracic pain or other relevant complaints. Video laryngoscopy revealed immobility of his left vocal fold in the paramedian position. Imaging was obtained for investigation, including magnetic resonance imaging of his thorax, which showed a fusiform aneurysm in the aortic arch, leading to recurrent compression of the left laryngeal nerve. The patient was successfully treated with endovascular repair of the aneurysm. At 2-month follow-up, there was still no recovery of the laryngeal mobility. Conclusion An aortic artery aneurysm can suddenly break, requiring emergency heart surgery, and the results can be fatal in many cases. We suggest routine exam of the vocal folds in all patients with a heart condition, and we review the literature and suggest the use of imaging to reduce the number of emergency procedures.

  20. Preoperative evaluation of the artery of adamkiewicz by MR angiography and CT angiography in patients with a thoracic aortic aneurysm

    International Nuclear Information System (INIS)

    Niinuma, Hiroyuki; Ohira, Atsushi; Makita, Shinji; Moriai, Yoshiteru; Hiramori, Katsuhiko; Yoshioka, Kunihiro; Nakajima, Takayuki; Kawazoe, Kohei

    2002-01-01

    Paraplegia is known as an extremely serious and important complication of surgical repair in patients with a thoraco-abdominal aortic aneurysm. It is important to evaluate the artery of Adamkiewicz (AdA) before surgical repair to prevent paraplegia. But the AdA is difficult to visualize by the invasive and hazardous, conventional selective angiography. The aim of this study was to visualize AdA by MR angiography (MRA) and CT angiography (CTA). Twenty-one consecutive patients with a thoracic aortic aneurysm underwent both gadolinium-enhanced, three-dimensional MRA and CTA using multislice helical CT. The AdA was successfully visualized in 15 of the 21 patients (71.4%) by MRA, and in 17 of those 21 patients (80.9%) by CTA. Its continuity was depicted in 12 of 15 patients (80%) by MRA, and in 9 of 17 patients (47%) by CTA. AdA was visualized at 85.7% by MRA or CTA, respectively. This study shows that CTA is a much more sensitive method to detect AdA than MRA. On the other hand, MRA is better to evaluate the continuity of AdA from the descending aorta to the anterior spinal artery, than CTA. Therefore, MRA and CTA are both useful for a preoperative evaluation of AdA and its detailed vascular anatomy from the aorta to the anterior spinal artery. (author)

  1. 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

  2. Radiofrequency thermocoagulation of the thoracic splanchnic nerve in functional abdominal pain syndrome -A case report-

    OpenAIRE

    Choi, Ji-Won; Joo, Eun-Young; Lee, Sang-Hyun; Lee, Chul-Joong; Kim, Tae-Hyeong; Sim, Woo-Seok

    2011-01-01

    The thoracic splanchnic nerve block has been used in managing abdominal pain, especially for pains arising from abdominal cancers. A 27-year-old male patient who had a constant abdominal pain was referred to our clinic for pain management but had no organic disease. The numeric rating scale (NRS) for pain scored 7/10. We applied a diagnostic thoracic splanchnic nerve block under the diagnosis of functional abdominal pain syndrome. Since the block reduced the pain, we applied a radiofrequency ...

  3. Intravascular ultrasound is a critical tool for accurate endograft sizing in the management of blunt thoracic aortic injury.

    Science.gov (United States)

    Wallace, Gabriel A; Starnes, Benjamin W; Hatsukami, Thomas S; Sobel, Michael; Singh, Niten; Tran, Nam T

    2015-03-01

    Accurate measurement of true aortic luminal diameter (ALD) is critical for endograft sizing in endovascular treatment of blunt thoracic aortic injury (BTAI), but ALD is dynamic and changes with respect to patients' hemodynamic status. This study aimed to characterize how ALD at the time of diagnosis of BTAI compares with ALD at the time of endovascular repair and later at follow-up. This is an Institutional Review Board-approved, single-institution retrospective analysis of prospectively obtained data. Patients were included who presented between July 2007 and December 2012 with computed tomography angiography (CTA)-diagnosed BTAI; who underwent thoracic endovascular aortic repair (TEVAR); and who underwent preoperative CTA, intraoperative intravascular ultrasound (IVUS), and postimplantation CTA. Comparison measurements of the ALD were made among CTA and IVUS images at the level of the left subclavian artery (LSCA) and between initial CTA and postimplantation CTA at 10, 15, and 20 cm distal to the LSCA. Theoretical endograft sizes were determined and compared for each ALD at the LSCA. Twenty-two patients were included in the analysis. Mean age was 38 ± 14 years (range, 17-61 years), with 82% men and mean Injury Severity Score of 43 ± 11 (range, 24-66). Mean time from emergency department admission to initial CTA was -1.2 ± 5 hours (range, -13 to 11.5 hours; negative time implies imaging at an outside facility before admission). Mean time from initial CTA to IVUS was 1.2 ± 1.4 days (range, 2.5 hours-5.7 days) and from IVUS to postimplantation CTA 33 ± 45 days (range, 17 hours-169 days). Overall, ALD measured by IVUS was significantly larger than that by initial CTA (Δ2.5 ± 3.1 mm; P < .05). ALD was also larger at 10, 15, and 20 cm distal to the LSCA in comparing the postimplantation CTA with the initial CTA (Δ2.4, 2.0, and 2.0 mm, respectively; all P < .05). More than half the devices would be sized differently with ALD measured by IVUS at the time of TEVAR

  4. Type 1a endoleak following Zone 1 and Zone 2 thoracic endovascular aortic repair: effect of bird-beak configuration.

    Science.gov (United States)

    Kudo, Tomoaki; Kuratani, Toru; Shimamura, Kazuo; Sakamoto, Tomohiko; Kin, Keiwa; Masada, Kenta; Shijo, Takayuki; Torikai, Kei; Maeda, Koichi; Sawa, Yoshiki

    2017-10-01

    Type 1a endoleak is one of the most severe complications after thoracic endovascular aortic repair (TEVAR), because it carries the risk of aortic rupture. The association between bird-beak configuration and Type 1a endoleak remains unclear. The purpose of this study was to analyse the predictors of Type 1a endoleak following Zone 1 and Zone 2 TEVAR, with a particular focus on the effect of bird-beak configuration. From April 2008 to July 2015, 105 patients (mean age 68.6 years) who underwent Zone 1 and 2 landing TEVAR were enrolled, with a mean follow-up period of 4.3 years. The patients were categorized into 2 groups, according to the presence (Group B, n = 32) or the absence (Group N, n = 73) of bird-beak configuration on the first postoperative multidetector computed tomography. The Kaplan-Meier event-free rate curve showed that Type 1a endoleak and bird-beak progression occurred less frequently in Group N than in Group B. Five-year freedom from Type 1a endoleak rates were 79.7% and 100% for Groups B and N, respectively (P = 0.007). Multivariable logistic regression analysis showed that dissecting aortic aneurysm (odds ratio 3.72, 95% confidence interval 1.30-11.0; P = 0.014) and shorter radius of inner curvature (odds ratio 1.09, 95% confidence interval 0.85-0.99; P = 0.025) were significant risk factors for bird-beak configuration. Multivariable Cox proportional hazard regression showed that Z-type stent graft (hazard ratio 2.69, 95% confidence interval 1.11-6.51; P = 0.030) was a significant risk factor for bird-beak progression. Appropriate stent grafts need to be chosen carefully to prevent Type 1a endoleak and bird-beak configuration after landing Zone 1 and 2 TEVAR. Patients with bird-beak configuration on early postoperative multidetector computed tomography require closer follow-up to screen for Type 1a endoleak. © The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic

  5. Comparison of neurocognitive results after coronary artery bypass grafting and thoracic aortic surgery using retrograde cerebral perfusion.

    Science.gov (United States)

    Miyairi, Takeshi; Takamoto, Shinichi; Kotsuka, Yutaka; Takeuchi, Atsuko; Yamanaka, Katsuo; Sato, Hajime

    2005-07-01

    Retrograde cerebral perfusion (RCP) is used as an adjunctive method to hypothermic circulatory arrest to enhance cerebral protection in patients undergoing thoracic aortic surgery. It remains unclear whether RCP provides improved neurological and neuropsychological outcome. Forty-six patients undergoing thoracic aortic surgery using RCP, and 28 undergoing coronary artery bypass grafting (CABG; n = 28) with CPB, were enrolled in the study. Patients receiving RCP were subdivided into two groups, those with less than 60 min of RCP (S-RCP; n = 27) and with 60 min or more (L-RCP; n = 19). The patients' neurocognitive state was assessed by the revised Wechsler Adult Intelligence Scale a few days before operation, at 2-3 weeks and 4-6 months after operation. There were no stroke, seizure, and hospital mortality in either group. Significant decline between baseline and early scores were seen in three subtests (digit span, arithmetic, and picture completion) for S-RCP and four (digit span, arithmetic, picture completion, and picture arrangement) for L-RCP. Significant decline between baseline and late scores were seen in one subtest (arithmetic) for S-RCP, four (digit span, arithmetic, picture completion, and picture arrangement) for L-RCP, and one (object assembly) for CABG. The mean change of scores for one late test (digit symbol) was significantly lower in S-RCP than in CABG. The mean change of scores for three early tests (digit span, vocabulary, and picture arrangement) and four late tests (information, digit span, picture completion, and picture arrangement) were significantly lower in L-RCP than in CABG. Stepwise logistic regression analysis disclosed that, after considering the other variables, significant difference in test score changes were observed between CABG and L-RCP for two early tests (picture completion and digit symbol) as well as for three late tests (digit span, similarities, and picture completion). None of test score changes showed significant

  6. 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.

  7. 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.

  8. 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.

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

    Science.gov (United States)

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

    2016-04-01

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

  10. 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.)

  11. 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.)

  12. 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

  13. Retrograde type A dissection: a serious complication due to thoracic aortic endovascular stent-graft repair for Stanford type B aortic dissection

    International Nuclear Information System (INIS)

    Wang Guoquan; Zhai Shuiting; Li Tianxiao; Shi Shuaitao; Zhang Kewei; Li Kun

    2011-01-01

    Objective: to discuss the possible causes and prevention of retrograde type A dissection occurred after thoracic aortic endovascular stent-graft repair (TEVAR) for symptomatic type B dissection. Methods: During the period from January 2005 to January 2011, TEVAR was carried out in 189 patients (157 males and 32 females) with symptomatic type B dissection. The average age of the patients was (51.2±13.5) years, ranged from 26 to 78 years. A follow-up lasting for 3-63 months (mean 32 months) was conducted in 135 patients (71.43%). Fifty-four patients lost in touch with the authors (28.57%). The occurrence of retrograde type A dissection after TEVAR was calculated and the possible causes were analyzed. Results: After TEVAR retrograde type A dissection occurred in two patients (1.48%), and both were males. One patient developed retrograde type A dissection in perioperative period, and the patient refused to have surgery. Conservative treatment was employed for over three years and be was still alive so far. The other patient developed retrograde type A dissection one month after TEVAR, and emergency surgery was performed. The patient was followed up for three months and he was still alive. Conclusion: The retrograde type A dissection occurred after TEVAR may be closely related to the stent-graft device, to the interventional manipulations and to the vascular disorders. Close attention should be paid to the direct damage produced by the stent-graft device to the vascular wall. (authors)

  14. Isolated Cervical Rib Fracture: A Rare Etiology of Thoracic Outlet Syndrome

    Directory of Open Access Journals (Sweden)

    Rayees Ahmad Dar

    2011-01-01

    Full Text Available Isolated fracture of a cervical rib is a very rare entity and usually presents as a painless swelling or as thoracic outlet syndrome. We describe a case of a 45-year-old woman with history of fall two months back. She presented with symptoms of neurogenic thoracic outlet syndrome for one month. Isolated left cervical rib fracture was documented on X-ray cervical spine. Her fractured cervical rib was resected through a supraclavicular approach, and symptoms resolved completely in the postoperative period.

  15. 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...

  16. 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.

  17. 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.

  18. [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.

  19. 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

  20. 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

  1. Association of left subclavian artery coverage without revascularization and spinal cord ischemia in patients undergoing thoracic endovascular aortic repair: A Vascular Quality Initiative® analysis.

    Science.gov (United States)

    Teixeira, Pedro Gr; Woo, Karen; Beck, Adam W; Scali, Salvatore T; Weaver, Fred A

    2017-12-01

    Objectives Investigate the impact of left subclavian artery coverage without revascularization on spinal cord ischemia development in patients undergoing thoracic endovascular aortic repair. Methods The Vascular Quality Initiative thoracic endovascular aortic repair module (April 2011-July 2014) was analyzed. Patients undergoing left subclavian artery coverage were divided into two groups according to revascularization status. The association between left subclavian artery revascularization with the primary outcome of spinal cord ischemia and the secondary outcome of stroke was assessed with multivariable analysis adjusting for between-group baseline differences. Results The left subclavian artery was covered in 508 (24.6%) of the 2063 thoracic endovascular aortic repairs performed. Among patients with left subclavian artery coverage, 58.9% underwent revascularization. Spinal cord ischemia incidence was 12.1% in the group without revascularization compared to 8.5% in the group undergoing left subclavian artery revascularization (odds ratio (95%CI): 1.48(0.82-2.68), P = 0.189). Multivariable analysis adjustment identified an independent association between left subclavian artery coverage without revascularization and the incidence of spinal cord ischemia (adjusted odds ratio (95%CI): 2.29(1.03-5.14), P = 0.043). Although the incidence of stroke was also higher for the group with a covered and nonrevascularized left subclavian artery (12.1% versus 8.5%), this difference was not statistically significant after multivariable analysis (adjusted odds ratio (95%CI): 1.55(0.74-3.26), P = 0.244). Conclusion For patients undergoing left subclavian artery coverage during thoracic endovascular aortic repair, the addition of a revascularization procedure was associated with a significantly lower incidence of spinal cord ischemia.

  2. 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

  3. Identification of type IV collagen exposure as a molecular imaging target for early detection of thoracic aortic dissection

    Science.gov (United States)

    Xu, Ke; Xu, Chen; Zhang, Yanzhenzi; Qi, Feiran; Yu, Bingran; Li, Ping; Jia, Lixin; Li, Yulin; Xu, Fu-jian; Du, Jie

    2018-01-01

    Thoracic aortic dissection (TAD) is an aggressive and life-threatening vascular disease and there is no effective means of early diagnosis of dissection. Type IV collagen (Col-IV) is a major component of the sub-endothelial basement membrane, which is initially exposed followed by endothelial injury as early-stage event of TAD. So, we want to build a noninvasive diagnostic method to detect early dissection by identifying the exposed Col-IV via MRI. Methods: Col-IV-targeted magnetic resonance/ fluorescence dual probe (Col-IV-DOTA-Gd-rhodamine B; CDR) was synthesized by amide reaction and coordination reaction. Flow cytometry analysis was used to evaluate the cell viability of SMC treated with CDR and fluorescence assays were used to assess the Col-IV targeting ability of CDR in vitro. We then examined the sensitivity and specificity of CDR at different stages of TAD via MRI and bioluminescence imaging in vivo. Results: The localization of Col-IV (under the intima) was observed by histology images. CDR bound specifically to Col-IV-expressing vascular smooth muscle cells and BAPN-induced dissected aorta. The CDR signal was co-detected by magnetic resonance imaging (MRI) and bioluminescence imaging as early as 2 weeks after BAPN administration (pre-dissection stage). The ability to detect rupture of dissected aorta was indicated by a strong normalized signal enhancement (NSE) in vivo. Moreover, NSE was negatively correlated with the time of dissection rupture after BAPN administration (r2 = 0.8482). Conclusion: As confirmed by in vivo studies, the CDR can identify the exposed Col-IV in degenerated aorta to monitor the progress of aortic dissection from the early stage to the rupture via MRI. Thus, CDR-enhanced MRI proposes a potential method for dissection screening, and for monitoring disease progression and therapeutic response. PMID:29290819

  4. Retrograde Ascending Dissection After Thoracic Endovascular Aortic Repair Combined With the Chimney Technique and Successful Open Repair Using the Frozen Elephant Trunk Technique.

    Science.gov (United States)

    Hirano, Koji; Tokui, Toshiya; Nakamura, Bun; Inoue, Ryosai; Inagaki, Masahiro; Maze, Yasumi; Kato, Noriyuki

    2018-01-01

    The chimney technique can be combined with thoracic endovascular aortic repair (TEVAR) to both obtain an appropriate landing zone and maintain blood flow of the arch vessels. However, surgical repair becomes more complicated if retrograde type A aortic dissection occurs after TEVAR with the chimney technique. We herein report a case involving a 73-year-old woman who developed a retrograde ascending dissection 3 months after TEVAR for acute type B aortic dissection. To ensure an adequate proximal sealing distance, the proximal edge of the stent graft was located at the zone 2 level and an additional bare stent was placed at the left subclavian artery (the chimney technique) at the time of TEVAR. Enhanced computed tomography revealed an aortic dissection involving the ascending aorta and aortic arch. Surgical aortic repair using the frozen elephant trunk technique was urgently performed. The patient survived without stroke, paraplegia, renal failure, or other major complications. Retrograde ascending dissection can occur after TEVAR combined with the chimney technique. The frozen elephant trunk technique is useful for surgical repair in such complicated cases.

  5. MRI evaluation of the aortic disease

    International Nuclear Information System (INIS)

    Kapuscinski, O.; Polkowski, J.; Zaleska, T.; Walecki, J.; Biesiadko, M.

    1994-01-01

    The goal of our study was to establish the value of MRI in diagnosing aortic disease. During 3 years period 46 patients were examined (12 women aged from 6 to 62 years and 34 men aged from 8 to 72 years). In 28 cases the thoracic aorta was examined, in 6 cases the abdominal aorta and in 12 - both the thoracic and the abdominal aorta. MR images were performed on MRT 50 A Toshiba unit 0.5 T. MR images demonstrated aortic aneurysm (caused by atherosclerosis or trauma), aortic dissections, aortic anomalies (i.e. in Turner syndrome) and non-specific aortitis and vasculitis. We regard MRI as preferable noninvasive imaging technique in diagnosing aortic disease. (author)

  6. 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.

  7. 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.

  8. 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.

  9. 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.

  10. 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.

  11. 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.

  12. 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.

  13. 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.

  14. 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.

  15. Radiofrequency thermocoagulation of the thoracic splanchnic nerve in functional abdominal pain syndrome -A case report-.

    Science.gov (United States)

    Choi, Ji-Won; Joo, Eun-Young; Lee, Sang-Hyun; Lee, Chul-Joong; Kim, Tae-Hyeong; Sim, Woo-Seok

    2011-07-01

    The thoracic splanchnic nerve block has been used in managing abdominal pain, especially for pains arising from abdominal cancers. A 27-year-old male patient who had a constant abdominal pain was referred to our clinic for pain management but had no organic disease. The numeric rating scale (NRS) for pain scored 7/10. We applied a diagnostic thoracic splanchnic nerve block under the diagnosis of functional abdominal pain syndrome. Since the block reduced the pain, we applied a radiofrequency thermocoagulation at the T11 and T12 vertebral level. Thereafter, his symptoms improved markedly with pain decreasing to an NRS score of 2-3/10. Hereby, we report a successful management of functional abdominal pain via radiofrequency thermocoagulation of the thoracic splanchnic nerves.

  16. Aneurysm-Specific miR-221 and miR-146a Participates in Human Thoracic and Abdominal Aortic Aneurysms

    Directory of Open Access Journals (Sweden)

    Premakumari Venkatesh

    2017-04-01

    Full Text Available Altered microRNA expression is implicated in cardiovascular diseases. Our objective was to determine microRNA signatures in thoracic aortic aneurysms (TAAs and abdominal aortic aneurysms (AAAs compared with control non-aneurysmal aortic specimens. We evaluated the expression of fifteen selected microRNA in human TAA and AAA operative specimens compared to controls. We observed significant upregulation of miR-221 and downregulation of miR-1 and -133 in TAA specimens. In contrast, upregulation of miR-146a and downregulation of miR-145 and -331-3p were found only for AAA specimens. Upregulation of miR-126 and -486-5p and downregulation of miR-30c-2*, -155, and -204 were observed in specimens of TAAs and AAAs. The data reveal microRNA expression signatures unique to aneurysm location and common to both thoracic and abdominal pathologies. Thus, changes in miR-1, -29a, -133a, and -221 are involved in TAAs and miR-145, -146, and -331-3p impact AAAs. This work validates prior studies on microRNA expression in aneurysmal diseases.

  17. Mid-term results of zone 0 thoracic endovascular aneurysm repair after ascending aorta wrapping and supra-aortic debranching in high-risk patients.

    Science.gov (United States)

    Pecoraro, Felice; Lachat, Mario; Hofmann, Michael; Cayne, Neal S; Chaykovska, Lyubov; Rancic, Zoran; Puippe, Gilbert; Pfammatter, Thomas; Mangialardi, Nicola; Veith, Frank J; Bettex, Dominique; Maisano, Francesco; Neff, Thomas A

    2017-06-01

    Surgical repair of aneurysmal disease involving the ascending aorta, aortic arch and eventually the descending aorta is generally associated with significant morbidity and mortality. A less invasive approach with the ascending wrapping technique (WT), supra-aortic vessel debranching (SADB) and thoracic endovascular aneurysm repair (TEVAR) in zone 0 was developed to reduce the associated risk in these patients. During a 10-year period, consecutive patients treated by the ascending WT, SADB and TEVAR in zone 0 were included. All patients were considered at high risk for conventional surgery. Measured outcomes included perioperative deaths and morbidity, maximal aortic transverse diameter (TD) and its postoperative evolution, endoleak, survival, freedom from cardiovascular reinterventions, SADB freedom from occlusion and aortic valve function during follow-up. Median follow-up was 37.4 [mean = 34; range, 0-65; standard deviation (SD) = 20] months. Twenty-six cases were included with a mean age of 71.88 ( r  = 56-87; SD = 8) years. A mean of 2.9 supra-aortic vessels (75) per patient was debranched from the ascending aorta. The mean time interval from WT/SADB and TEVAR was 29 ( r  = 0-204; SD = 48) days. TEVAR was associated with chimney and/or periscope grafts in 6 (23%) patients, and extra-anatomical supra-aortic bypasses were performed in 6 (23%) patients. Perioperative mortality was 7.7% (2/26). Neurological events were registered in 3 (11.5%) cases, and a reintervention was required in 3 (11.5%) cases. After the WT, the ascending diameter remained stable during the follow-up period in all cases. At mean follow-up, significant shrinkage of the arch/descending aorta diameter was observed. A type I/III endoleak occurred in 3 cases. At 5 years, the rates of survival, freedom from cardiovascular reinterventions and SADB freedom from occlusion were 71.7, 82.3 and 96%, respectively. The use of the ascending WT, SADB and TEVAR in selected patients

  18. Is chest x-ray an adequate screening tool for the diagnosis of blunt thoracic aortic injury?

    Science.gov (United States)

    Ekeh, Akpofure Peter; Peterson, Wylan; Woods, Randy J; Walusimbi, Mbaga; Nwuneli, Nancy; Saxe, Jonathan M; McCarthy, Mary C

    2008-11-01

    Blunt thoracic aortic injuries (BTAI) have a high mortality rate. For survivors, chest X-ray (CXR) findings are used to determine the need for further diagnostic testing with chest computerized tomography with angiography (CTA) or conventional angiography. We set to determine the adequacy of utilizing CXR alone as a screening tool for BTAI. All patients diagnosed with BTAI at a level I trauma-center during a 7-year-period were identified. CXRs of these patients and those of a control group of blunt trauma patients with an injury severity score >15 were reviewed by four trauma surgeons blinded to the diagnosis. Based on each CXR viewed, the surgeons decided if they would have proceeded to chest CTA, angiography, or required no further studies to rule out BTAI. In the 7-year-period, 83 patients had BTAI. CXRs were available in 45 patients. The four surgeons viewed 96 CXRs including those of 51 controls. Based on the CXR appearance in patients with BTAI, the surgeons chose to proceed to chest CTA in 38 patients (84.4%), conventional aortography in two patients (4.4%), and no further testing in five patients (11.2%). A widened mediastinum (75%) and loss of the aorto-pulmonary window (40%) were the most frequent CXR abnormalities. Patients with BTAI were more likely to have an abnormal CXR-40 of 45 (88.8%) patients when compared with the controls-25 of 51 (49%)patients-p < 0.001. Although CXR is a sensitive screening modality, it failed to identify the possibility of BTAI in 11% of patients. The liberal use of chest CTA after high speed motor vehicle crashes is recommended to minimize the incidence of missed BTAI.

  19. 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.)

  20. 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.

  1. 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

  2. Case of sudden death following rupture of thoracic aortic aneurysm during “a manual therapy” procedure in the spinal area

    Directory of Open Access Journals (Sweden)

    Aleksandra Kaczorowska

    2015-03-01

    Full Text Available The reported case concerns the rupture of an aneurysm located in the descending part of the thoracic aorta during the procedure of the so-called “spinal adjustment” performed by a chiropractor. A 45-year-old patient with no significant medical history of cardiovascular diseases visited, along with his wife, one of “manual therapy” practices because of back pain. During the procedure, the patient suddenly lost consciousness. Despite cardiopulmonary resuscitation and emergency assistance given by an ambulance team, the patient died. An autopsy revealed that the cause of death was increasing cardiorespiratory failure secondary to bleeding into the lumen of the left pleural cavity following the rupture of pathologically altered thoracic aortic wall.

  3. Risk model of thoracic aortic surgery in 4707 cases from a nationwide single-race population through a web-based data entry system: the first report of 30-day and 30-day operative outcome risk models for thoracic aortic surgery.

    Science.gov (United States)

    Motomura, Noboru; Miyata, Hiroaki; Tsukihara, Hiroyuki; Takamoto, Shinichi

    2008-09-30

    The objective of this study was to collect integrated data from nationwide hospitals using a web-based national database system to build up our own risk model for the outcome from thoracic aortic surgery. The Japan Adult Cardiovascular Surgery Database was used; this involved approximately 180 hospitals throughout Japan through a web-based data entry system. Variables and definitions are almost identical to the STS National Database. After data cleanup, 4707 records were analyzed from 97 hospitals (between January 1, 2000, and December 31, 2005). Mean age was 66.5 years. Preoperatively, the incidence of chronic lung disease was 11%, renal failure was 9%, and rupture or malperfusion was 10%. The incidence of the location along the aorta requiring replacement surgery (including overlapping areas) was: aortic root, 10%; ascending aorta, 47%; aortic arch, 44%; distal arch, 21%; descending aorta, 27%; and thoracoabdominal aorta, 8%. Raw 30-day and 30-day operative mortality rates were 6.7% and 8.6%, respectively. Postoperative incidence of permanent stroke was 6.1%, and renal failure requiring dialysis was 6.7%. OR for 30-day operative mortality was as follows: emergency or salvage, 3.7; creatinine >3.0 mg/dL, 3.0; and unexpected coronary artery bypass graft, 2.6. As a performance metric of the risk model, C-index of 30-day and 30-day operative mortality was 0.79 and 0.78, respectively. This is the first report of risk stratification on thoracic aortic surgery using a nationwide surgical database. Although condition of these patients undergoing thoracic aortic surgery was much more serious than other procedures, the result of this series was excellent.

  4. 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

  5. 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.

  6. 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...

  7. Utilization of Cupping Therapy in the Treatment of Vascular Thoracic Outlet Syndrome in a Collegiate Pitcher: A Case Study

    Directory of Open Access Journals (Sweden)

    Stephen A. Cage

    2017-12-01

    Full Text Available Objective: Present a clinical case detailing the effectiveness of dry cupping therapy in treating thoracic outlet syndrome. The utilization of dry cupping therapy on a 20-year-old collegiate baseball pitcher with diagnosed thoracic outlet syndrome is presented. Background: Thoracic outlet syndrome is a relatively rare musculoskeletal condition affecting 1/100,000 patients annually. Dry cupping therapy is an ancient therapeutic modality that utilizes various means of suction with the goal of decompressing myofascial layers. Treatment: Following diagnosis, patient was successfully treated in two weeks using dry cupping therapy. The patient experienced no further incidence of thoracic outlet syndrome symptoms and was able to complete the remainder of his competitive season. Uniqueness: The patient’s thoracic outlet syndrome was diagnosed at an early stage, leading to the need of clearance from a vascular specialist before returning to competition. To the author’s knowledge, there are currently no published case reports detailing the use of cupping therapy to treat thoracic outlet syndrome. Conclusion: Cupping therapy may be a viable treatment option when seeking to address tight musculature. Further research needs to be conducted to determine optimal parameters for cupping therapy as a therapeutic modality.

  8. Tratamento endovascular de ruptura traumática da aorta torácica descendente Endovascular treatment of traumatic descending thoracic aortic rupture

    Directory of Open Access Journals (Sweden)

    João Roberto Breda

    2007-06-01

    Full Text Available Paciente do sexo feminino, 55 anos de idade, vítima de atropelamento, foi admitida em unidade de emergência, onde se realizou o diagnóstico clínico, radiológico e tomográfico de ruptura traumática da aorta torácica descendente. Diante do achado, a paciente foi encaminhada para tratamento endovascular com colocação de endoprótese auto-expansível (stent pela artéria femoral. O tratamento obteve sucesso, evidenciado pela exclusão da lesão localizada previamente no istmo aórtico. O tratamento endovascular tem sido indicado nas afecções de aorta torácica descendente com bons resultados iniciais. Na ruptura traumática de aorta, a terapêutica endovascular representa uma alternativa aceitável, especialmente devido aos riscos do tratamento operatório convencional.A 55-year-old, female patient who was run over by a motor vehicle was admitted at an emergency room. Clinical, radiological and tomographic diagnosis of traumatic descending aortic thoracic rupture was performed. The patient was referred for endovascular treatment with placement of a self-expandable stent through the femoral artery. Treatment was successful, with exclusion of the lesion previously located in the aortic isthmus. Endovascular treatment has been indicated in the treatment of descending thoracic aortic diseases, with good initial results. In case of traumatic aortic rupture, endovascular treatment is a feasible alternative, especially due to risks offered by the conventional surgical treatment.

  9. 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.

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

    Science.gov (United States)

    Miyahara, Shunsuke; Okita, Yutaka

    2016-09-01

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

  11. [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.

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

    Science.gov (United States)

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

    2016-02-01

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

  14. 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.

  15. 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.

  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. Lung parenchymal analysis on dynamic MRI in thoracic insufficiency syndrome to assess changes following surgical intervention

    Science.gov (United States)

    Jagadale, Basavaraj N.; Udupa, Jayaram K.; Tong, Yubing; Wu, Caiyun; McDonough, Joseph; Torigian, Drew A.; Campbell, Robert M.

    2018-02-01

    General surgeons, orthopedists, and pulmonologists individually treat patients with thoracic insufficiency syndrome (TIS). The benefits of growth-sparing procedures such as Vertical Expandable Prosthetic Titanium Rib (VEPTR)insertionfor treating patients with TIS have been demonstrated. However, at present there is no objective assessment metricto examine different thoracic structural components individually as to their roles in the syndrome, in contributing to dynamics and function, and in influencing treatment outcome. Using thoracic dynamic MRI (dMRI), we have been developing a methodology to overcome this problem. In this paper, we extend this methodology from our previous structural analysis approaches to examining lung tissue properties. We process the T2-weighted dMRI images through a series of steps involving 4D image construction of the acquired dMRI images, intensity non-uniformity correction and standardization of the 4D image, lung segmentation, and estimation of the parameters describing lung tissue intensity distributions in the 4D image. Based on pre- and post-operative dMRI data sets from 25 TIS patients (predominantly neuromuscular and congenital conditions), we demonstrate how lung tissue can be characterized by the estimated distribution parameters. Our results show that standardized T2-weighted image intensity values decrease from the pre- to post-operative condition, likely reflecting improved lung aeration post-operatively. In both pre- and post-operative conditions, the intensity values decrease also from end-expiration to end-inspiration, supporting the basic premise of our results.

  18. Endovascular treatment of aortic pathologies - State of the art. Part 2 - Pathologies of thoracic aorta and other applications

    International Nuclear Information System (INIS)

    Uribe, Carlos E; Calderon, Luis L; Gomez, German S; Castro, Pablo; Hurtado, Edgar F; Estrada, Gilberto

    2007-01-01

    Endovascular treatment of aortic pathologies is actually an alternative to open surgery. It has proven to be safe, showing similar or better results to those achieved by surgery. In this article, treatment of aortic pathologies by means of endoprosthesis is presented, as well as its indications, contraindications and future treatment with this kind of devices

  19. Continuous Thoracic Sympathetic Ganglion Block in Complex Regional Pain Syndrome Patients with Spinal Cord Stimulation Implantation

    Directory of Open Access Journals (Sweden)

    EungDon Kim

    2016-01-01

    Full Text Available The sympathetic block is widely used for treating neuropathic pain such as complex regional pain syndrome (CRPS. However, single sympathetic block often provides only short-term effect. Moreover, frequent procedures for sympathetic block may increase the risk of complications. The use of epidural route may be limited by concern of infection in case of previous implantation of the spinal cord stimulation (SCS. In contrast, a continuous sympathetic block can be administered without such concerns. The continuous thoracic sympathetic block (TSGB has been used to treat the ischemic disease and other neuropathic conditions such as postherpetic neuralgia. We administered continuous thoracic sympathetic block using catheter in CRPS patients who underwent SCS implantations and achieved desirable outcomes. We believe a continuous sympathetic block is a considerable option before performing neurolysis or radiofrequency rhizotomy and even after SCS implantation.

  20. 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

  1. 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.

  2. Thoracic Malignancies and Pulmonary Nodules in Patients under Evaluation for Transcatheter Aortic Valve Implantation (TAVI): Incidence, Follow Up and Possible Impact on Treatment Decision

    Science.gov (United States)

    Kaleschke, Gerrit; Schülke, Christoph; Görlich, Dennis; Schliemann, Christoph; Kessler, Torsten; Schulze, Arik Bernard; Buerke, Boris; Kuemmel, Andreas; Thrull, Michael; Wiewrodt, Rainer; Baumgartner, Helmut; Berdel, Wolfgang E.; Mohr, Michael

    2016-01-01

    Background Transcatheter aortic valve implantation (TAVI) has become the treatment of choice in patients with severe aortic valve stenosis who are not eligible for operative replacement and an alternative for those with high surgical risk. Due to high age and smoking history in a high proportion of TAVI patients, suspicious findings are frequently observed in pre-procedural chest computer tomography (CCT). Methods CCT scans of 484 consecutive patients undergoing TAVI were evaluated for incidentally discovered solitary pulmonary nodules (SPN). Results In the entire study population, SPN ≥ 5 mm were found in 87 patients (18%). These patients were compared to 150 patients who were incidentally collected from the 397 patients without SPN or with SPN 8 mm (p = 0.328) were significant predictors of overall survival. Conclusions Despite the high prevalence of SPNs in this single center TAVI cohort lung cancer incidence at midterm follow-up seems to be low. Thus, aggressive diagnostic approaches for incidentally discovered SPN during TAVI evaluation should not delay the treatment of aortic stenosis. Unless advanced thoracic malignancy is obvious, the well documented reduction of morbidity and mortality by TAVI outweighs potentially harmful delays regarding further diagnostics. Standard guideline-approved procedure for SPN can be safely performed after TAVI. PMID:27171441

  3. Whole-exome sequencing links TMCO1 defect syndrome with cerebro-facio-thoracic dysplasia.

    Science.gov (United States)

    Pehlivan, Davut; Karaca, Ender; Aydin, Hatip; Beck, Christine R; Gambin, Tomasz; Muzny, Donna M; Bilge Geckinli, B; Karaman, Ali; Jhangiani, Shalini N; Gibbs, Richard A; Lupski, James R

    2014-09-01

    Whole-exome sequencing (WES) is a type of disruptive technology that has tremendous influence on human and clinical genetics research. An efficient and cost-effective method, WES is now widely used as a diagnostic tool for identifying the molecular basis of genetic syndromes that are often challenging to diagnose. Here we report a patient with a clinical diagnosis of cerebro-facio-thoracic dysplasia (CFTD; MIM#213980) in whom we identified a homozygous splice-site mutation in the transmembrane and coiled-coil domains 1 (TMCO1) gene using WES. TMCO1 mutations cause craniofacial dysmorphism, skeletal anomalies characterized by multiple malformations of the vertebrae and ribs, and intellectual disability (MIM#614132). A retrospective review revealed that clinical manifestations of both syndromes are very similar and overlap remarkably. We propose that mutations of TMCO1 are not only responsible for craniofacial dysmorphism, skeletal anomalies and mental retardation syndrome but also for CFTD.

  4. [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.

  5. 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.

  6. 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.

  7. 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...

  8. 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.

  9. Transcatheter valve implantation for patients with aortic stenosis: A position statement from the European Association of Cardio-Thoracic Surgery (EACTS) and the European Society of Cardiology (ESC), in collaboration with the European Association of Percutaneous Cardiovascular Interventions (EAPCI)

    NARCIS (Netherlands)

    A. Vahanian (Alec); O. Alfieri (Ottavio); N. Al-Attar (Nawwar); M. Antunes (Manuel); J.J. Bax (Jeroen); B. Cormier (Bertrand); A. Cribier (Alain); P.P.T. de Jaegere (Peter); G. Fournial (Gerard); A.P. Kappetein (Arie Pieter); J. Kovac (Jan); S. Ludgate (Susanne); F. Maisano (Francesco); N. Moat (Neil); F.W. Mohr (Friedrich); P. Nataf (Patrick); L. Pierard (Luc); J.L. Pomar (Jose); J. Schofer (Joachim); P. Tornos (Pilar); M. Tuzcu (Murat); B.A. van Hout (Ben); L.K. von Segesser (Ludwig); T. Walther (Thomas)

    2008-01-01

    textabstractAims: To critically review the available transcatheter aortic valve implantation techniques and their results, as well as propose recommendations for their use and development. Methods and results: A committee of experts including European Association of Cardio-Thoracic Surgery and

  10. Thoracic Outlet Syndrome in a Volleyball Player Due to Nonunion of the First Rib Fracture.

    Science.gov (United States)

    Puttmann, Kathleen T; Satiani, Bhagwan; Vaccaro, Patrick

    2016-11-01

    Fracture of the first rib with ensuing callus formation is a rare cause of thoracic outlet syndrome. We report a case of a 17-year-old female volleyball player who presented with months of chronic arm pain. Radiographic imaging demonstrated nonunion fracture of the first rib. Physical therapy had been unsuccessful in relieving the pain, and surgical management was performed with resection of the first rib through a transaxillary approach with complete resolution of symptoms. Inflammation surrounding such fractures may destroy tissue planes, making dissection more technically difficult.

  11. Thoracic Outlet Syndrome Caused by Hydatid Cyst of the First Rib-Rare But Important

    OpenAIRE

    Levy Faber, Dan; Best, Lael-Anson; Militianu, Daniela; Ben Nun, Alon

    2010-01-01

    Hydatid cysts are usually located in the liver and lungs. Skeletal echinococcosis is relatively rare and that of the rib is exceptional. Less than 50 cases of costal echinococcosis have been reported in the literature so far. To our knowledge, only one case report of thoracic outlet syndrome due to echinococcal cyst in the first rib was described in 1995. Accurate pre-operative diagnosis is important but may be challenging in some cases. Reported here is a case of echinococcosis of the first ...

  12. Ranking of conventional X-ray diagnosis and phlebography in the thoracic outlet syndrome (TOS)

    International Nuclear Information System (INIS)

    Vahlensieck, M.; Beltz, L.

    1991-01-01

    The compression of the neurovascular bundle of the upper limb (thoracic outlet syndrome, TOS) can be caused by osseous, muscular, fibrous, tumorous and habitual abnormalities of the cervicothoracic junction. Osseous causes can be shown in a conventional x-ray of the cervi cothoracic junction. In about 40% of the cases there is a venous stenosis which can be proved by means of phlebography in a special patient position (provocation position). The type of stenosis and location provides information on the cause of it. We examined 34 patients. (orig.) [de

  13. Effect of cannula shape on aortic wall and flow turbulence: hydrodynamic study during extracorporeal circulation in mock thoracic aorta.

    Science.gov (United States)

    Minakawa, Masahito; Fukuda, Ikuo; Yamazaki, Junichi; Fukui, Kozo; Yanaoka, Hideki; Inamura, Takao

    2007-12-01

    This study was designed to analyze flow pattern, velocity, and strain on the aortic wall of a glass aortic model during extracorporeal circulation, and to elucidate the characteristics of flow pattern in four aortic cannulas. Different patterns of large vortices and helical flow were made by each cannula. The high-velocity flow (0.6 m/s) was observed in end-hole cannula, causing high strain rate tensor (0.3~0.4 without unit) on the aortic arch. In dispersion cannula, a decreased strain rate tensor (less than 0.1) was found on the outer curvature of the aortic arch. In Soft-flow cannula (3M Cardiovascular, Ann Arbor, MI, USA), further decreased flow velocity (0.2 m/s) and strain (less than 0.2) were observed. In Select 3D cannula (Medtronic, Inc., Minneapolis, MN, USA), a high strain (0.4~0.5) was observed along the inner curvature of the aortic arch. In conclusion, end-hole cannula should not be used in atherosclerotic aorta. Particular attention should be paid both for selection of cannulas and cannulation site based on this result.

  14. 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.

  15. [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.

  16. 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.

  17. 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.

  18. 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.

  19. New Technique for the Preservation of the Left Common Carotid Artery in Zone 2a Endovascular Repair of Thoracic Aortic Aneurysm

    International Nuclear Information System (INIS)

    Juszkat, Robert; Kulesza, Jerzy; Zarzecka, Anna; Jemielity, Marek; Staniszewski, Ryszard; Majewski, Wacław

    2011-01-01

    To describe a technique for the preservation of the left common carotid artery (CCA) in zone 2 endovascular repair of thoracic aortic aneurysm. This technique involves the placement of a guide wire into the left CCA via the right brachial artery before stent graft deployment to enable precise visualization and protection of the left CCA during the whole procedure. Of the 107 patients with thoracic endovascular aortic repair in our study, 32 (30%) had the left subclavian artery intentionally covered (landing zone 2). Eight (25%) of those 32 had landing zone 2a—the segment distally the origin of the left CCA, halfway between the origin of the left CCA and the left subclavian artery. In all patients, a guide wire was positioned into the left CCA via the right brachial artery before stent graft deployment. It is a retrospective study in design. In seven patients, stent grafts were positioned precisely. In the remaining patient, the positioning was imprecise; the origin of the left CCA was partially covered by the graft. A stent was implanted into the left CCA to restore the flow into the vessel. All procedures were performed successfully. The technique of placing a guide wire into the left CCA via the right brachial artery before stent graft deployment is a safe and effective method that enables the precise visualization of the left CCA during the whole procedure. Moreover, in case of inadvertent complete or partial coverage of the origin of the left CCA, it supplies safe and quick access to the artery for stent implantation.

  20. 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.

  1. Ruptured thoracic aortic aneurysm in patient with systemic lupus erythematosus Aneurisma roto da aorta descendente em paciente com lúpus eritematoso sistêmico

    Directory of Open Access Journals (Sweden)

    Daniel Oliveira De Conti

    2011-03-01

    Full Text Available It is reported a ruptured descending thoracic aortic aneurysm in a 25-year-old systemic lupus erythematosus woman who underwent 19 years steroid therapy. She was treated with 2 endovascular stent-grafts, discharged from hospital 13 days after the procedure in good health. Three months later she returned with hemorrhagic shock due to high digestive hemorrhage secondary to an aortic-esophageal fistula. She underwent to an open emergency surgery, and died during the post-operative period.Paciente de 25 anos, do sexo feminino, portadora de lúpus eritematoso sistêmico, fazendo uso de corticoesteroide havia 19 anos, deu entrada em unidade de emergência com aneurisma roto de aorta torácica descendente. Foi submetida a tratamento endovascular com 2 stents, recebeu alta hospitalar no 13º dia de pós-operatório, em boas condições de saúde. Três meses depois, retornou em choque hemorrágico secundário a hemorragia digestiva alta. Feito o diagnóstico de fístula aorto-esofágica, foi submetida à cirurgia aberta de emergência, indo a óbito durante o período pós-operatório.

  2. A Review of Thoracic Outlet Syndrome and the Possible Role of Botulinum Toxin in the Treatment of This Syndrome

    Directory of Open Access Journals (Sweden)

    Andrew Travlos

    2012-11-01

    Full Text Available The objective of this paper is to discuss the classification, diagnosis, pathophysiology and management of Thoracic outlet syndrome (TOS. Thoracic outlet syndrome (TOS is a complex entity that is characterized by different neurovascular signs and symptoms involving the upper limb. TOS is defined as upper extremity symptoms due to compression of the neurovascular bundle in the area of the neck just above the first rib. Compression is thought to occur at one or more of the three anatomical compartments: the interscalene triangle, the costoclavicular space and the retropectoralis minor spaces. The clinical presentation can include both neurogenic and vascular symptoms. TOS can be difficult to diagnose because there is no standardized objective test that can be used and the clinician must rely on history and several positive findings on physical exam. The medial antebrachial cutaneous nerve conduction may be a sensitive way to detect pathology in the lower trunks of the brachial plexus which is promising for future research. Treatment options continue to be conservative and surgical. However, for those who have failed physical therapy there is research to suggest that botulinum toxin may help with symptom relief. However, given that there has been conflicting evidence, further research is required using randomized controlled trials.

  3. What is the optimal myocardial preservation strategy at re-operation for aortic valve replacement in the presence of a patent internal thoracic artery?

    Science.gov (United States)

    Park, Chan B; Suri, Rakesh M; Burkhart, Harold M; Greason, Kevin L; Dearani, Joseph A; Schaff, Hartzell V; Sundt, Thoralf M

    2011-06-01

    The optimal myocardial preservation strategy at re-operation for aortic valve replacement (AVR) after prior coronary artery bypass grafting (CABG) in the presence of a patent internal thoracic artery (ITA) remains undefined. Patients undergoing AVR after prior CABG at our institution between 1 January 1996 and 31 December 2007 were identified; operative notes and outcomes were reviewed. Of 628 patients with prior CABG undergoing AVR with or without concomitant procedures, 427 patients had a patent ITA. In 390, management of the ITA was detailed in the operative note, including 251 in whom it was clamped and 139 in whom it was left uncontrolled. Groups were demographically similar, although re-operative CABG was more frequent in the clamped group (42% vs 23%, poptimal perfusion temperature when the ITA was left uncontrolled. Efforts to control the patent ITA at re-operation for AVR after prior CABG increase risk of injury and may actually increase operative mortality rate compared with leaving this critical graft open and perfusing the heart. Copyright © 2010 European Association for Cardio-Thoracic Surgery. All rights reserved.

  4. 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.

  5. Health-related quality of life in children with thoracic insufficiency syndrome.

    Science.gov (United States)

    Vitale, Michael G; Matsumoto, Hiroko; Roye, David P; Gomez, Jaime A; Betz, Randal R; Emans, John B; Skaggs, David L; Smith, John T; Song, Kit M; Campbell, Robert M

    2008-03-01

    The traditional techniques to treat thoracic insufficiency syndrome (TIS) are not able to stabilize or improve chest wall size or pulmonary function while allowing spine growth. To this end, vertical expandable prosthetic titanium rib (VEPTR) was specifically designed to treat TIS by allowing growth of the thoracic cavity and control/correction of spine deformity. The purpose of this study was to determine quality of life (QOL) of children with TIS and its impact on their parents before and after implantation of the VEPTR and also compare these results to those of healthy children. As part of the original multicenter evaluation of the VEPTR, a Child Health Questionnaire (CHQ) was collected preoperatively on 45 patients who were subsequently treated with expansion thoracoplasty using the VEPTR. The average age was 8.2 +/- 2.6 years, and the parent form of the CHQ was filled out by the primary caretaker. Patients were divided into 3 diagnostic categories: rib fusion (n = 15), hypoplastic thorax syndromes (n = 17), and progressive spinal deformity (n = 13). There were significant differences between the study patients and healthy children in physical domains. Compared with parents of healthy children, parents of children with TIS experienced more limitations on their time and emotional lives due to their children's health problems. There were no significant differences in CHQ before and after the surgery except for a significant decrease in the self-esteem among a subgroup of patients with hypoplastic thorax syndromes. There were no significant differences in postoperative QOL between patients who had VEPTR-related complications and patients who did not have the complications. The children with TIS had lower physical scores and higher caregiver burden scores than healthy children. However, the scores in psychosocial domains were similar to those in healthy children. Our study demonstrated that QOL of children and burden of care in their parents remained the same after

  6. Association of Tricuspid Regurgitation With Transcatheter Aortic Valve Replacement Outcomes: A Report From The Society of Thoracic Surgeons/American College of Cardiology Transcatheter Valve Therapy Registry.

    Science.gov (United States)

    McCarthy, Fenton H; Vemulapalli, Sreekanth; Li, Zhuokai; Thourani, Vinod; Matsouaka, Roland A; Desai, Nimesh D; Kirtane, Ajay; Anwaruddin, Saif; Williams, Matthew L; Giri, Jay; Vallabhajosyula, Prashanth; Li, Robert H; Herrmann, Howard C; Bavaria, Joseph E; Szeto, Wilson Y

    2018-04-01

    The purpose of this study is to evaluate the association of tricuspid regurgitation (TR) severity with outcomes after transcatheter aortic valve replacement (TAVR). We analyzed data from 34,576 patients who underwent TAVR at 365 US hospitals from November 2011 through March 2015 submitted to The Society of Thoracic Surgeon/American College of Cardiology Transcatheter Valve Therapy Registry. We examined unadjusted mortality and heart failure readmission stratified by degree of preoperative TR and used multivariable models for 1-year mortality and heart failure readmission. Tricuspid regurgitation was present in 80% (n = 27,804) of TAVR patients, with mild TR in 56% (n = 19,393), moderate TR in 19% (n = 6687), and severe TR in 5% (n = 1,724). Increasing TR severity was associated with a number of comorbidities and The Society of Thoracic Surgeons predicted risk of mortality increased (p < 0.001): no TR (7.3 ± 5.4); mild TR (8.0 ± 5.7); moderate TR (9.6 ± 6.8); and severe TR (10.7 ± 7.4). In unadjusted analysis, moderate and severe TR were associated with increased use of cardiopulmonary bypass, longer intensive care unit and hospital stays, new dialysis, inhospital major adverse cardiac event, inhospital mortality, observed-to-expected inhospital mortality ratio, long-term heart failure readmission, and mortality (p < 0.001). Adjusted mortality at 1 year was significantly worse for patients with severe TR when left ventricular ejection fraction greater than 30% (hazard ratio 1.29, 95% confidence interval: 1.11 to 1.50) as was heart failure readmission (hazard ratio 1.27, 95% confidence interval: 1.04 to 1.54). Tricuspid regurgitation was common among patients undergoing TAVR. Increasing TR severity was associated with higher risk patients and increased mortality and readmission-particularly for patients with severe TR and left ventricular ejection fraction greater than 30%. The effectiveness of TAVR alone in patients with aortic stenosis and concomitant

  7. Anatomic factors associated with acute endograft collapse after Gore TAG treatment of thoracic aortic dissection or traumatic rupture.

    NARCIS (Netherlands)

    Muhs, B.E.; Balm, R.; White, G.H.; Verhagen, H.J.M.

    2007-01-01

    OBJECTIVE: The potentially devastating complication of total or near total thoracic endoprosthesis collapse has been described with the TAG device (W. L. Gore & Associates, Flagstaff, Ariz). This rare complication has resulted in a warning to clinicians and speculation about the etiology of this

  8. Anatomic factors associated with acute endograft collapse after Gore TAG treatment of thoracic aortic dissection or traumatic rupture

    NARCIS (Netherlands)

    Muhs, Bart E.; Balm, Ron; White, Geoffrey H.; Verhagen, Hence J. M.

    2007-01-01

    OBJECTIVE: The potentially devastating complication of total or near total thoracic endoprosthesis collapse has been described with the TAG device (W. L. Gore & Associates, Flagstaff, Ariz). This rare complication has resulted in a warning to clinicians and speculation about the etiology of this

  9. Dose reduction in spiral CT angiography of thoracic outlet syndrome by anatomically adapted tube current modulation

    International Nuclear Information System (INIS)

    Mastora, I.; Remy-Jardin, M.; Remy, J.; Suess, C.; Scherf, C.; Guillot, J.P.

    2001-01-01

    The aim of this study was to evaluate dose reduction in spiral CT angiography of the thoracic outlet by on-line tube-current control. Prospectively, 114 patients undergoing spiral CT angiography of the subclavian artery for thoracic outlet arterial syndromes were evaluated with and without tube-current modulation at the same session (scanning parameters for the two successive angiograms, one in the neutral position and one after the postural maneuver): 140 kV; 206 mA; scan time 0.75 s; collimation 3 mm; pitch = (1). The dose reduction system was applied in the neutral position in the first 92 consecutive patients and after postural maneuver in the remaining 22 consecutive patients. Dose reduction and image quality were analyzed in the overall study group (group 1; n = 114). The influence of the arm position was assessed in 44 of the 114 patients (group 2), matched by the transverse diameter of the upper thorax. The mean dose reduction was 33 % in group 1 (range 22-40 %) and 34 % in group 2 (range 26-40 %). In group 2 the only difference in image quality was a significantly higher frequency of graininess on low-dose scans compared with reference scans whatever the patient's arm position, graded as minimal in 38 of the 44 patients (86 %). When the low-dose technique was applied after postural maneuver in group 2: (a) the mean dose reduction was significantly higher (35 vs 32 % in the neutral position; p = 0.006); (b) graininess was less frequent (82 vs 91 % in the neutral position); and (c) the percentage of graininess graded as minimal was significantly higher (83 vs 70 % in the neutral position; p = 0.2027). On-line tube-current modulation enables dose reduction on high-quality, diagnostic spiral CT angiograms of the thoracic outlet and should be applied during data acquisition in the neutral position and after postural maneuver for optimal use. (orig.)

  10. Dose reduction in spiral CT angiography of thoracic outlet syndrome by anatomically adapted tube current modulation

    Energy Technology Data Exchange (ETDEWEB)

    Mastora, I.; Remy-Jardin, M.; Remy, J. [Dept. of Radiology, University Center Hospital Calmette, Lille (France); Medical Research Group, Lille (France); Suess, C.; Scherf, C. [Siemens Medical Systems, Forcheim (Germany); Guillot, J.P. [Dept. of Radiology, University Center Hospital Calmette, Lille (France)

    2001-04-01

    The aim of this study was to evaluate dose reduction in spiral CT angiography of the thoracic outlet by on-line tube-current control. Prospectively, 114 patients undergoing spiral CT angiography of the subclavian artery for thoracic outlet arterial syndromes were evaluated with and without tube-current modulation at the same session (scanning parameters for the two successive angiograms, one in the neutral position and one after the postural maneuver): 140 kV; 206 mA; scan time 0.75 s; collimation 3 mm; pitch = (1). The dose reduction system was applied in the neutral position in the first 92 consecutive patients and after postural maneuver in the remaining 22 consecutive patients. Dose reduction and image quality were analyzed in the overall study group (group 1; n = 114). The influence of the arm position was assessed in 44 of the 114 patients (group 2), matched by the transverse diameter of the upper thorax. The mean dose reduction was 33 % in group 1 (range 22-40 %) and 34 % in group 2 (range 26-40 %). In group 2 the only difference in image quality was a significantly higher frequency of graininess on low-dose scans compared with reference scans whatever the patient's arm position, graded as minimal in 38 of the 44 patients (86 %). When the low-dose technique was applied after postural maneuver in group 2: (a) the mean dose reduction was significantly higher (35 vs 32 % in the neutral position; p = 0.006); (b) graininess was less frequent (82 vs 91 % in the neutral position); and (c) the percentage of graininess graded as minimal was significantly higher (83 vs 70 % in the neutral position; p = 0.2027). On-line tube-current modulation enables dose reduction on high-quality, diagnostic spiral CT angiograms of the thoracic outlet and should be applied during data acquisition in the neutral position and after postural maneuver for optimal use. (orig.)

  11. Antenatal Diagnosis of Jeune Syndrome (Asphyxiating Thoracic Dysplasia) with Micromelia and Facial Dysmorphism on Second-Trimester Ultrasound

    International Nuclear Information System (INIS)

    Mistry, Kewal A.; Suthar, Pokhraj P.; Bhesania, Siddharth R.; Patel, Ankitkumar

    2015-01-01

    Jeune syndrome is a rare congenital malformation with a reported incidence of 1 in 100,000–130,000 live births. Thoracic hypoplasia is the most striking abnormality of this disorder. Here we report a case of Jeune syndrome with marked thoracic hypoplasia, micromelia and facial dysmorphism, which was diagnosed on a second-trimester antenatal real-time three-dimensional ultrasound. A 24-year-old primigravida came for routine anomaly scan at 19 weeks of gestation. Transabdominal grey scale and real time 3D ultrasound (US) was done with GE Logiq P5 with curvilinear array transducers (4C and 4D3C-L). US findings were consistent with the diagnosis of Jeune syndrome (Asphyxiating thoracic dysplasia). Jeune syndrome is an extremely rare congenital disorder with a spectrum of abnormalities of which thoracic hypoplasia is the most striking. It can be diagnosed on early antenatal US by its characteristic skeletal and morphological features which can guide further management of pregnancy in form of termination or preparation for surgical correction of the deformity

  12. 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...

  13. Bilateral Upper Extremity DVT in a 43-Year-Old Man: Is It Thoracic Outlet Syndrome?!

    Directory of Open Access Journals (Sweden)

    Hadoun Jabri

    2014-01-01

    Full Text Available Recurrent deep venous thrombosis, involving bilateral upper extremities, is an extremely rare phenomenon. We are presenting a 43-year-old man who was diagnosed with left upper extremity deep vein thrombosis (UEDVT and was treated with anticoagulation and surgical decompression in 2004. 9 years later, he presented with right arm swelling and was diagnosed with right UEDVT using US venous Doppler. Venogram showed compression of the subclavian vein by the first rib, diagnosing thoracic outlet syndrome (TOS. He was treated with anticoagulation and local venolysis and later by surgical decompression of the subclavian vein. Bilateral UEDVT, as mentioned above, is an extremely rare condition that is uncommonly caused by TOS. To our knowledge, we are reporting the first case of bilateral UEDVT due to TOS. Diagnosis usually starts with US venous Doppler to detect the thrombosis, followed by the gold standard venogram to locate the area of obstruction and lyse the thrombus if needed. The ultimate treatment for TOS remains surgical decompression of the vascular bundle at the thoracic outlet.

  14. 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.

  15. 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.

  16. [Commentary by the German Society for Thoracic and Cardiovascular Surgery on the positions statement by the German Cardiology Society on quality criteria for transcatheter aortic valve implantation (TAVI)].

    Science.gov (United States)

    Cremer, Jochen; Heinemann, Markus K; Mohr, Friedrich Wilhelm; Diegeler, Anno; Beyersdorf, Friedhelm; Niehaus, Heidi; Ensminger, Stephan; Schlensak, Christian; Reichenspurner, Hermann; Rastan, Ardawan; Trummer, Georg; Walther, Thomas; Lange, Rüdiger; Falk, Volkmar; Beckmann, Andreas; Welz, Armin

    2014-12-01

    Surgical aortic valve replacement is still considered the first-line treatment for patients suffering from severe aortic valve stenosis. In recent years, transcatheter aortic valve implantation (TAVI) has emerged as an alternative for selected high-risk patients. According to the latest results of the German external quality assurance program, mandatory by law, the initially very high mortality and procedural morbidity have now decreased to approximately 6 and 12%, respectively. Especially in Germany, the number of patients treated by TAVI has increased exponentially. In 2013, a total of 10.602 TAVI procedures were performed. TAVI is claimed to be minimally invasive. This is true concerning the access, but it does not describe the genuine complexity of the procedure, defined by the close neighborhood of the aortic valve to delicate intracardiac structures. Hence, significant numbers of life-threatening complications may occur and have been reported. Owing to the complexity of TAVI, there is a unanimous concordance between cardiologists and cardiac surgeons in the Western world demanding a close heart team approach for patient selection, intervention, handling of complications, and pre- as well as postprocedural care, respectively. The prerequisite is that TAVI should not be performed in centers with no cardiac surgery on site. This is emphasized in all international joint guidelines and expert consensus statements. Today, a small number of patients undergo TAVI procedures in German hospitals without a department of cardiac surgery on site. To be noted, most of these hospitals perform less than 20 cases per year. Recently, the German Cardiac Society (DGK) published a position paper supporting this practice pattern. Contrary to this statement and concerned about the safety of patients treated this way, the German Society for Thoracic and Cardiovascular Surgery (DGTHG) still fully endorses the European (ESC/EACTS) and other actual international guidelines and

  17. Preoperative Duplex Scanning is a Helpful Diagnostic Tool in Neurogenic Thoracic Outlet Syndrome.

    Science.gov (United States)

    Orlando, Megan S; Likes, Kendall C; Mirza, Serene; Cao, Yue; Cohen, Anne; Lum, Ying Wei; Freischlag, Julie A

    2016-01-01

    To evaluate the diagnostic role of venous and arterial duplex scanning in neurogenic thoracic outlet syndrome (NTOS). Retrospective review of patients who underwent duplex ultrasonography prior to first rib resection and scalenectomy (FRRS) for NTOS from 2005 to 2013. Abnormal scans included ipsilateral compression (IC) with abduction of the symptomatic extremity (>50% change in subclavian vessel flow), contralateral (asymptomatic side) compression (CC) or bilateral compression (BC). A total of 143 patients (76% female, average age 34, range 13-59) underwent bilateral preoperative duplex scanning. Ipsilateral compression was seen in 44 (31%), CC in 12 (8%), and BC in 14 (10%). Seventy-three (51%) patients demonstrated no compression. Patients with IC more often experienced intraoperative pneumothoraces (49% vs. 25%, P duplex ultrasonography can assist in NTOS diagnosis. Ipsilateral compression on abduction often correlates with Adson testing. © The Author(s) 2016.

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

    Science.gov (United States)

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

    2014-01-01

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

  19. 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.

  20. 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).

  1. 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.

  2. 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.

  3. 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.

  4. MRI evaluation of the aortic disease; Zastosowanie tomografii rezonansu magnetycznego w rozpoznawaniu chorob aorty

    Energy Technology Data Exchange (ETDEWEB)

    Kapuscinski, O; Polkowski, J [Zaklad Radiologii, Inst. Kardiologii, Warsaw-Anin (Poland); Zaleska, T [Oddzial Kardiologii, CSK, Warsaw-Miedzylesie (Poland); Walecki, J; Biesiadko, M [Zaklad Diagnostyki Obrazowej, CMKP and CSK, Warsaw-Miedzylesie (Poland)

    1994-12-31

    The goal of our study was to establish the value of MRI in diagnosing aortic disease. During 3 years period 46 patients were examined (12 women aged from 6 to 62 years and 34 men aged from 8 to 72 years). In 28 cases the thoracic aorta was examined, in 6 cases the abdominal aorta and in 12 - both the thoracic and the abdominal aorta. MR images were performed on MRT 50 A Toshiba unit 0.5 T. MR images demonstrated aortic aneurysm (caused by atherosclerosis or trauma), aortic dissections, aortic anomalies (i.e. in Turner syndrome) and non-specific aortitis and vasculitis. We regard MRI as preferable noninvasive imaging technique in diagnosing aortic disease. (author) 13 refs, 3 figs

  5. 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

  6. 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.

  7. Asphyxiating thoracic dysplasia

    International Nuclear Information System (INIS)

    Franzcr, J.; Kozlowski, K.

    2008-01-01

    Asphyxiating Thoracic Dysplasia is the most frequent form of Small Thorax - Short Rib Syndromes. Asphyxiating Thoracic Dysplasia in two patients with different clinical course is reported. Radiographic examination is the only method to diagnose Asphyxiating Thoracic Dysplasia with certainty. The correct diagnosis is important for prognostication and genetic counseling. It also excludes the necessity of further, often expensive investigations. (author)

  8. Sodium bicarbonate use and the risk of hypernatremia in thoracic aortic surgical patients with metabolic acidosis following deep hypothermic circulatory arrest

    Directory of Open Access Journals (Sweden)

    Kamrouz Ghadimi

    2016-01-01

    Full Text Available Objective: Metabolic acidosis after deep hypothermic circulatory arrest (DHCA for thoracic aortic operations is commonly managed with sodium bicarbonate (NaHCO 3 . The purpose of this study was to determine the relationships between total NaHCO 3 dose and the severity of metabolic acidosis, duration of mechanical ventilation, duration of vasoactive infusions, and Intensive Care Unit (ICU or hospital length of stay (LOS. Methods: In a single center, retrospective study, 87 consecutive elective thoracic aortic operations utilizing DHCA, were studied. Linear regression analysis was used to test for the relationships between the total NaHCO 3 dose administered through postoperative day 2, clinical variables, arterial blood gas values, and short-term clinical outcomes. Results: Seventy-five patients (86% received NaHCO 3 . Total NaHCO 3 dose averaged 136 ± 112 mEq (range: 0.0-535 mEq per patient. Total NaHCO 3 dose correlated with minimum pH (r = 0.41, P < 0.0001, minimum serum bicarbonate (r = −0.40, P < 0.001, maximum serum lactate (r = 0.46, P = 0.007, duration of metabolic acidosis (r = 0.33, P = 0.002, and maximum serum sodium concentrations (r = 0.29, P = 0.007. Postoperative hypernatremia was present in 67% of patients and peaked at 12 h following DHCA. Eight percent of patients had a serum sodium ≥ 150 mEq/L. Total NaHCO 3 dose did not correlate with anion gap, serum chloride, not the duration of mechanical ventilator support, vasoactive infusions, ICU or hospital LOS. Conclusion: Routine administration of NaHCO 3 was common for the management of metabolic acidosis after DHCA. Total dose of NaHCO 3 was a function of the severity and duration of metabolic acidosis. NaHCO 3 administration contributed to postoperative hypernatremia that was often severe. The total NaHCO 3 dose administered was unrelated to short-term clinical outcomes.

  9. 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.

  10. Adverse cardiac events in children with Williams syndrome undergoing cardiovascular surgery: An analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database.

    Science.gov (United States)

    Hornik, Christoph P; Collins, Ronnie Thomas; Jaquiss, Robert D B; Jacobs, Jeffrey P; Jacobs, Marshall L; Pasquali, Sara K; Wallace, Amelia S; Hill, Kevin D

    2015-06-01

    Patients with Williams syndrome (WS) undergoing cardiac surgery are at risk for major adverse cardiac events (MACE). Prevalence and risk factors for such events have not been well described. We sought to define frequency and risk of MACE in patients with WS using a multicenter clinical registry. We identified cardiac operations performed in patients with WS using the Society of Thoracic Surgeons Congenital Heart Surgery Database (2000-2012). Operations were divided into 4 groups: isolated supravalvular aortic stenosis, complex left ventricular outflow tract (LVOT), isolated right ventricular outflow tract (RVOT), and combined LVOT/RVOT procedures. The proportion of patients with MACE (in-hospital mortality, cardiac arrest, or postoperative mechanical circulatory support) was described and the association with preoperative factors was examined. Of 447 index operations (87 centers), median (interquartile range) age and weight at surgery were 2.4 years (0.6-7.4 years) and 10.6 kg (6.5-21.5 kg), respectively. Mortality occurred in 20 patients (5%). MACE occurred in 41 patients (9%), most commonly after combined LVOT/RVOT (18 out of 87; 21%) and complex LVOT (12 out of 131; 9%) procedures, but not after isolated RVOT procedures. Odds of MACE decreased with age (odds ratio [OR], 0.99; 95% confidence interval [CI], 0.98-0.99), weight (OR, 0.97; 95% CI, 0.93-0.99), but increased in the presence of any preoperative risk factor (OR, 2.08; 95% CI, 1.06-4.00), and in procedures involving coronary artery repair (OR, 5.37; 95% CI, 2.05-14.06). In this multicenter analysis, MACE occurred in 9% of patients with WS undergoing cardiac surgery. Demographic and operative characteristics were associated with risk. Further study is needed to elucidate mechanisms of MACE in this high-risk population. Copyright © 2015 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.

  11. 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.

  12. 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...

  13. 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

  14. 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

  15. 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...

  16. [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.

  17. 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.)

  18. 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 ...

  19. 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

  20. 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.

  1. 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.

  2. Ultrasound Guided Transversus Thoracic Plane block, Parasternal block and fascial planes hydrodissection for internal mammary post thoracotomy pain syndrome.

    Science.gov (United States)

    Piraccini, E; Biondi, G; Byrne, H; Calli, M; Bellantonio, D; Musetti, G; Maitan, S

    2018-05-16

    Pectoral Nerves Block (PECS) and Serratus Plane Block (SPB) have been used to treat persistent post-surgical pain after breast and thoracic surgery; however, they cannot block the internal mammary region, so a residual pain may occur in that region. Parasternal block (PSB) and Thoracic Transversus Plane Block (TTP) anaesthetize the anterior branches of T2-6 intercostal nerves thus they can provide analgesia to the internal mammary region. We describe a 60-year-old man suffering from right post-thoracotomy pain syndrome with residual pain located in the internal mammary region after a successful treatment with PECS and SPB. We performed a PSB and TTP and hydrodissection of fascial planes with triamcinolone and Ropivacaine. Pain disappeared and the result was maintained 3 months later. This report suggests that PSB and TTP with local anaesthetic and corticosteroid with hydrodissection of fascial planes might be useful to treat a post thoracotomy pain syndrome located in the internal mammary region. The use of Transversus Thoracic Plane and Parasternal Blocks and fascial planes hydrodissection as a novel therapeutic approach to treat a residual post thoracotomy pain syndrome even when already treated with Pectoral Nerves Block and Serratus Plane Block. © 2018 European Pain Federation - EFIC®.

  3. 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.

  4. [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.

  5. Cognitive and Affective Uses of a Thoracic Outlet Syndrome Facebook Support Group.

    Science.gov (United States)

    Walker, Kimberly K

    2014-09-01

    There are currently many disease-specific groups on Facebook in which patients may take an active part (Greene, Choudhry, Kilabuk, & Shrank, 2011). Although uses and gratifications of patient-disease groups have begun to be identified for chronic diseases, rare diseases have been omitted, even though they collectively affect roughly 30 million people in the United States and 350 million people worldwide. This study is a content analysis of one Facebook rare disease patient group, the Thoracic Outlet Syndrome (TOS) Awareness group. All wall posts were recorded and content analyzed for cognitive and affective categories and subcategories between October 9, 2011 (date of site origin), and May 1, 2012. Analysis of cognitive needs indicated TOS patients used the site more to share information about their own TOS symptoms and journey with diagnosis than to seek information. Analysis of affective needs found patients were more likely to use the site to give support and encouragement to others than to express concerns and complaints. The complaints they did express were primarily related to their frustration with the general medical community's perceived inability to diagnose and understand their disease or to question a specific doctor's diagnosis/recommendation. Results point to needs specific to TOS patients that uses and gratifications research can help clarify.

  6. 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.

  7. Digital subtraction angiography of the thoracic aorta

    International Nuclear Information System (INIS)

    Grossman, L.B.; Buonocore, E.; Modic, M.T.; Meaney, T.F.

    1984-01-01

    Forty-three patients with acquired and congenital abnormalities of the thoracic aorta were studied using digital subtraction angiography (DSA) after an intravenous bolus injection of 40 ml of contrast material. Abnormalities studied included coarctation, pseudocoarctation, Marfan syndrome, cervical aorta, double aortic arch, aneurysm, dissection, and tumor. Twenty-four patients also had conventional angiography. DSA was accurate in 95% of cases; in the other 5%, involving patients with acute type I dissection, the coronary arteries could not be seen. The authors concluded that in 92% of their patients, DSA could have replaced the standard aortogram

  8. Digital subtraction angiography of the thoracic aorta

    Energy Technology Data Exchange (ETDEWEB)

    Grossman, L.B.; Buonocore, E.; Modic, M.T.; Meaney, T.F.

    1984-02-01

    Forty-three patients with acquired and congenital abnormalities of the thoracic aorta were studied using digital subtraction angiography (DSA) after an intravenous bolus injection of 40 ml of contrast material. Abnormalities studied included coarctation, pseudocoarctation, Marfan syndrome, cervical aorta, double aortic arch, aneurysm, dissection, and tumor. Twenty-four patients also had conventional angiography. DSA was accurate in 95% of cases; in the other 5%, involving patients with acute type I dissection, the coronary arteries could not be seen. The authors concluded that in 92% of their patients, DSA could have replaced the standard aortogram.

  9. 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.

  10. Elsberg syndrome: A rarely recognized cause of cauda equina syndrome and lower thoracic myelitis.

    Science.gov (United States)

    Savoldi, Filippo; Kaufmann, Timothy J; Flanagan, Eoin P; Toledano, Michel; Weinshenker, Brian G

    2017-07-01

    Elsberg syndrome (ES) is an established but often unrecognized cause of acute lumbosacral radiculitis with myelitis related to recent herpes virus infection. We defined ES, determined its frequency in patients with cauda equina syndrome (CES) with myelitis, and evaluated its clinical, radiologic, and microbiologic features and outcomes. We searched the Mayo Clinic medical records for ES and subsequently for combinations of index terms to identify patients with suspected CES and myelitis. Our search yielded 30 patients, 2 diagnosed with ES and an additional 28 with clinical or radiologic evidence of CES retrospectively suspected of having ES. We classified patients in 5 groups according to diagnostic certainty. MRI and EMG confirmed that 2 had only myelitis, 5 only radiculitis, and 16 both. Two had preceding sacral herpes infection and 1 oral herpes simplex. Spinal cord lesions were commonly multiple, discontinuous, not expansile, and centrally or ventrally positioned. Lesions generally spared the distal conus. Nerve root enhancement was occasionally prominent and was smooth rather than nodular. Lymphocytic CSF pleocytosis was common. Thirteen patients (43%) had viral isolation studies, which were commonly delayed; the delay may have accounted for the low rate of viral detection. Acyclovir was administered to 6 patients. Most patients recovered with sequelae; 1 patient experienced encephalomyelitis and died. ES is a definable condition likely responsible for 10% of patients with combined CES and myelitis. Radiologic findings are not entirely specific but may help in differentiating ES from some competing diagnostic considerations. We propose criteria to facilitate diagnosis.

  11. 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.

  12. [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

  13. 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.

  14. 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

  15. 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.

  16. Pericardial and thoracic peri-aortic adipose tissues contribute to systemic inflammation and calcified coronary atherosclerosis independent of body fat composition, anthropometric measures and traditional cardiovascular risks

    International Nuclear Information System (INIS)

    Yun, Chun-Ho; Lin, Tin-Yu; Wu, Yih-Jer; Liu, Chuan-Chuan; Kuo, Jen-Yuan; Yeh, Hung-I.; Yang, Fei-Shih; Chen, Su-Chiu; Hou, Charles Jia-Yin; Bezerra, Hiram G.; Hung, Chung-Lieh; Cury, Ricardo C.

    2012-01-01

    Background: Coronary atherosclerosis has traditionally been proposed to be associated with several cardiovascular risk factors and anthropometric measures. However, clinical data regarding the independent value of visceral adipose tissue in addition to such traditional predictors remains obscure. Materials and methods: We subsequently studied 719 subjects (age: 48.1 ± 8.3 years, 25% females) who underwent multidetector computed tomography (MDCT) for coronary calcium score (CCS) quantification. Baseline demographic data and anthropometric measures were taken with simultaneous body fat composition estimated. Visceral adipose tissue of pericardial and thoracic peri-aortic fat was quantified by MDCT using TeraRecon Aquarius workstation (San Mateo, CA). Traditional cardiovascular risk stratification was calculated by metabolic (NCEP ATP III) and Framingham (FRS) scores and high-sensitivity CRP (Hs-CRP) was taken to represent systemic inflammation. The independent value of visceral adipose tissue to systemic inflammation and CCS was assessed by utilizing multivariable regression analysis. Results: Of all subjects enrolled in this study, the mean values for pericardial and peri-aortic adipose tissue were 74.23 ± 27.51 and 7.23 ± 3.69 ml, respectively. Higher visceral fat quartile groups were associated with graded increase of risks for cardiovascular diseases. Both adipose burdens strongly correlated with anthropometric measures including waist circumference, body weight and body mass index (all p < 0.001). In addition, both visceral amount correlates well with ATP and FRS scores, all lipid profiles and systemic inflammation marker in terms of Hs-CRP (all p < 0.001). After adjustment for baseline variables, both visceral fat were independently related to Hs-CRP levels (all p < 0.05), but only pericardial fat exerted independent role in coronary calcium deposit. Conclusion: Both visceral adipose tissues strongly correlated with systemic inflammation beyond traditional

  17. Significance and function of different spinal collateral compartments following thoracic aortic surgery: immediate versus long-term flow compensation.

    Science.gov (United States)

    Meffert, Philipp; Bischoff, Moritz S; Brenner, Robert; Siepe, Matthias; Beyersdorf, Friedhelm; Kari, Fabian A

    2014-05-01

    Iatrogenic paraplegia has been accompanying cardiovascular surgery since its beginning. As a result, surgeons have been developing many theories about the exact mechanisms of this devastating complication. Thus, the impact of single arteries that contribute to the spinal perfusion is one of the most discussed subjects in modern surgery. The subsequent decision of reattachment or the permanent disconnection of these intercostal arteries divides the surgical community. On the one hand, the anatomical or vascular approach pleads for the immediate reimplantation to reconstruct the anatomical situation. On the other hand, the decision of the permanent disconnection aims at avoiding stealing phenomenon away from the spinal vascular network. This spinal collateral network can be described as consisting of three components-the intraspinal and two paraspinal compartments-that feed the nutrient arteries of the spinal cord. The exact functional impact of the different compartments of the collateral network remains poorly understood. In this review, the function of the intraspinal compartment in the context of collateral network principle as an immediate emergency backup system is described. The exact structure and architectural principles of the intraspinal compartment are described. The critical parameters with regard to the risk of postoperative spinal cord ischaemia are the number of anterior radiculomedullary arteries (ARMAs) and the distance between them in relation to the longitudinal extent of aortic disease. The paraspinal network as a sleeping reserve is proposed as the long-term backup system. This sleeping reserve has to be activated by arteriogenic stimuli. These are presented briefly, and prior findings regarding arteriogenesis are discussed in the light of the collateral network concept. Finally, the role of preoperative visualization of the ARMAs in order to evaluate the risk of postoperative paraplegia is emphasized.

  18. Aortic valve replacement for aortic stenosis caused by alkaptonuria.

    Science.gov (United States)

    Hiroyoshi, Junko; Saito, Aya; Panthee, Nirmal; Imai, Yasushi; Kawashima, Dai; Motomura, Noboru; Ono, Minoru

    2013-03-01

    We report a case of aortic stenosis associated with ochronosis in a 70-year-old man who underwent biologic aortic valve replacement. Intraoperative findings included ochronosis of a severely calcified pigmented aortic valve along with pigmentation of the intima of the aorta. Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  19. 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)

  20. The Complement C3a-C3aR Axis Promotes Development of Thoracic Aortic Dissection via Regulation of MMP2 Expression.

    Science.gov (United States)

    Ren, Weihong; Liu, Yan; Wang, Xuerui; Piao, Chunmei; Ma, Youcai; Qiu, Shulan; Jia, Lixin; Chen, Boya; Wang, Yuan; Jiang, Wenjian; Zheng, Shuai; Liu, Chang; Dai, Nan; Lan, Feng; Zhang, Hongjia; Song, Wen-Chao; Du, Jie

    2018-03-01

    Thoracic aortic dissection (TAD), once ruptured, is devastating to patients, and no effective pharmaceutical therapy is available. Anaphylatoxins released by complement activation are involved in a variety of diseases. However, the role of the complement system in TAD is unknown. We found that plasma levels of C3a, C4a, and C5a were significantly increased in patients with TAD. Elevated circulating C3a levels were also detected in the developmental process of mouse TAD, which was induced by β-aminopropionitrile monofumarate (BAPN) treatment, with enhanced expression of C1q and properdin in mouse dissected aortas. These findings indicated activation of classical and alternative complement pathways. Further, expression of C3aR was obviously increased in smooth muscle cells of human and mouse dissected aortas, and knockout of C3aR notably inhibited BAPN-induced formation and rupture of TAD in mice. C3aR antagonist administered pre- and post-BAPN treatment attenuated the development of TAD. We found that C3aR knockout decreased matrix metalloproteinase 2 (MMP2) expression in BAPN-treated mice. Additionally, recombinant C3a stimulation enhanced MMP2 expression and activation in smooth muscle cells that were subjected to mechanical stretch. Finally, we generated MMP2-knockdown mice by in vivo MMP2 short hairpin RNA delivery using recombinant adeno-associated virus and found that MMP2 deficiency significantly reduced the formation of TAD. Therefore, our study suggests that the C3a - C3aR axis contributes to the development of TAD via regulation of MMP2 expression. Targeting the C3a-C3aR axis may represent a strategy for inhibiting the formation of TAD. Copyright © 2018 by The American Association of Immunologists, Inc.

  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. 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.

  5. 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.

  6. 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.

  7. Chondrosarcoma of right 1st rib presenting as neurogenic thoracic outlet syndrome; A 13th case report in world literature

    Directory of Open Access Journals (Sweden)

    Ravisagar Patel

    2016-07-01

    Full Text Available Thoracic outlet syndrome [TOS] caused by a tumor of the rib is rare and has been reported only 12 times in the literature over the past one and one-half centuries, with the majority of cases due to osteochondroma. We report a case of chondrosarcoma of right 1st rib causing neurogenic TOS that was resected via posterolateral thoracotomy and biopsy confirmed a grade I chondrosarcoma. In the treatment of chondrosarcoma, chemotherapy and radiotherapy are less effective, and appropriate surgery is needed.

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

    Science.gov (United States)

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

    2017-09-01

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

  9. 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.

  10. Retrograde Embolization of the Left Vertebral Artery in a Type II Endoleak After Endovascular Treatment of Aortic Thoracic Rupture: Technical Note

    International Nuclear Information System (INIS)

    Rabellino, Martin; Garcia Nielsen, L.; Baldi, S.; Zander, T.; Arnaiz, L.; Llorens, R.; Zerolo, I.; Maynar, M.

    2009-01-01

    Endoleak is a frequent complication after endovascular repair of aortic rupture. We describe the case of a female patient with traumatic aortic injury, treated with endograft, who developed a type II endoleak through the left subclavian and vertebral arteries. Both arteries originated independently from the aortic arch, and were managed with coil embolization of each vessel. We also report our experience with treating the left vertebral artery by placing a microcatheter through the right vertebral one.

  11. 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.

  12. 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.

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

    Directory of Open Access Journals (Sweden)

    Ibrahim Akin

    2008-02-01

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

  14. [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.

  15. [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.

  16. 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.

  17. Extended replacement of the thoracic aorta.

    Science.gov (United States)

    Hino, Yutaka; Okada, Kenji; Oka, Takanori; Inoue, Takeshi; Tanaka, Akiko; Omura, Atsushi; Kano, Hiroya; Okita, Yutaka

    2013-01-01

    We present our experience of total aortic arch replacement. Twenty-nine patients (21 males and 8 females; mean age 63.3 ± 13.3 years) with extended thoracic aortic aneurysms underwent graft replacement. The pathology of the diseased aorta was non-dissecting aneurysm in 11 patients, including one aortitis and aortic dissection in 18 patients (acute type A: one, chronic type A: 11, chronic type B: six). Five patients had Marfan syndrome. In their previous operation, two patients had undergone the Bentall procedure, three had endovascular stenting, one had aortic root replacement with valve sparing and 12 had hemi-arch replacement for acute type A dissection. Approaches to the aneurysm were as follows: posterolateral thoracotomy with rib-cross incision in 16, posterolateral thoracotomy extended to the retroperitoneal abdominal aorta in seven, mid-sternotomy and left pleurotomy in three, anterolateral thoracotomy with partial lower sternotomy in two and clam-shell incision in one patient. Extension of aortic replacement was performed from the aortic root to the descending aorta in 4, from the ascending aorta to the descending aorta in 17 and from the ascending to the abdominal aorta in eight patients. Arterial inflow for cardiopulmonary bypass consisted of the femoral artery in 15 patients, ascending aorta and femoral artery in seven, descending or abdominal aorta in five and ascending aorta in two. Venous drainage site was the femoral vein in 10, pulmonary artery in eight, right atrium in five, femoral artery with right atrium/pulmonary artery in four and pulmonary artery with right atrium in two patients. The operative mortality, 30-day mortality and hospital mortality was one (cardiac arrest due to aneurysm rupture), one (rupture of infected aneurysm) and one (brain contusion), respectively. Late mortality occurred in three patients due to pneumonia, ruptured residual aneurysm and intracranial bleeding. Actuarial survival at 5 years after the operations was 80.6

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

    Science.gov (United States)

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

    2011-11-01

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

  19. 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.

  20. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

  2. Comparing American, European and Asian practice guidelines for aortic diseases.

    Science.gov (United States)

    Rozado, Jose; Martin, Maria; Pascual, Isaac; Hernandez-Vaquero, Daniel; Moris, Cesar

    2017-05-01

    The aortic disease comprises a group of different pathologies of high prevalence, seriousness and ever changing by the medical and surgical investigations. Therefore cardiovascular scientific societies in USA, Europe and Asia have created Task Force on practice guidelines (PG) to develop, update and revise PG for aortic diseases. These documents issue recommendations on the diagnosis and management of different aortic diseases. The three societies agree on the recommendations about diagnostic tests and on the value of computed tomography and magnetic resonance as the main tools for the diagnosis and follow-up of aortic disease. Concerning to acute aortic syndromes (AAS), American and European GPs recognize intramural hematoma (IMH) as a type of AAS with surgery indication; however Asian guidelines consider IMH a pathological process different from AAS and indicate medical treatment. In thoracic aortic aneurysms (TAA), all express the need for an adequate control of cardiovascular risk factors, emphasizing strict control of blood pressure, smoking cessation and recommend the use of beta-blockers and statins. The threshold for asymptomatic repair is 5.5 cm in European and American and 6 cm for Asian PG, with lower thresholds in Marfan and bicuspid aortic valve (BAV). As regards the abdominal aortic aneurysms (AAA), the PGs recognize the adequate control of cardiovascular risk factors, but there are differences in class of recommendation on statins, angiotensin-converting enzyme inhibitors or beta-blockers to prevent progression of AAA. For intervention, the threshold diameter in asymptomatic is 5.5 cm but can be reduced to 5 cm in women as recommended by Asian PG. Moreover the specific diseases such as Marfan, BAV, pregnancy or atherosclerosis aortic present specific recommendations with small differences between PGs. In conclusion, PGs are interesting and appropriate documents at present. They issue recommendations based on evidence that help the clinician and

  3. Spontaneous aortic dissecting hematoma in two dogs.

    Science.gov (United States)

    Boulineau, Theresa Marie; Andrews-Jones, Lydia; Van Alstine, William

    2005-09-01

    This report describes 2 cases of spontaneous aortic dissecting hematoma in young Border Collie and Border Collie crossbred dogs. Histology was performed in one of the cases involving an unusual splitting of the elastin present within the wall of the aorta, consistent with elastin dysplasia as described in Marfan syndrome in humans. The first case involved a young purebred Border Collie that died suddenly and the second case involved a Border Collie crossbred dog that died after a 1-month history of seizures. Gross lesions included pericardial tamponade with dissection of the ascending aorta in the former case and thoracic cavity hemorrhage, mediastinal hematoma, and aortic dissection in the latter. Histologic lesions in the case of the Border Collie crossbred dog included a dissecting hematoma of the ascending aorta with elastin dysplasia and right axillary arterial intimal proliferation.

  4. 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.

  5. 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.

  6. 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

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

    Science.gov (United States)

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

    2013-03-01

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

  8. Cardiovascular evaluation in Turner syndrome: utility of MR imaging

    International Nuclear Information System (INIS)

    Dawson-Falk, K.; Bakker, B.; Rosenfeld, R.G.

    1992-01-01

    Forty patients with karyotypically proven Turner syndrome were prospectively studied using magnetic resonance imaging (MRI) and echocardiography in order to determine the frequency of cardiovascular anomalies and to assess the utility of both imaging modalities as methods for cardiovascular evaluation in Turner syndrome. Cardiovascular anomalies were found in 45% of patients. A high absolute prevalence of bicuspid aortic valve (17.5%) and aortic coarctation (12.5%) were observed relative to comparable series. Of clinically significant abnormalities, three of five aortic coarctations and four of five ascending aortic dilatations were solely MRI detected and not evident at echocardiographic examination. MRI is thus seen as a valuable adjunct to echocardiography in the cardiovascular evaluation of Turner syndrome patients. The usefulness of MRI primarily relates to its ability to provide excellent visualisation of the entire thoracic aorta where a large proportion of clinically significant anomalies occur in Turner syndrome. 23 refs., 2 tabs., 5 figs

  9. Cardiovascular evaluation in Turner syndrome: utility of MR imaging

    Energy Technology Data Exchange (ETDEWEB)

    Dawson-Falk, K; Bakker, B; Rosenfeld, R G [Stanford Univ., CA (United States). School of Medicine

    1992-08-01

    Forty patients with karyotypically proven Turner syndrome were prospectively studied using magnetic resonance imaging (MRI) and echocardiography in order to determine the frequency of cardiovascular anomalies and to assess the utility of both imaging modalities as methods for cardiovascular evaluation in Turner syndrome. Cardiovascular anomalies were found in 45% of patients. A high absolute prevalence of bicuspid aortic valve (17.5%) and aortic coarctation (12.5%) were observed relative to comparable series. Of clinically significant abnormalities, three of five aortic coarctations and four of five ascending aortic dilatations were solely MRI detected and not evident at echocardiographic examination. MRI is thus seen as a valuable adjunct to echocardiography in the cardiovascular evaluation of Turner syndrome patients. The usefulness of MRI primarily relates to its ability to provide excellent visualisation of the entire thoracic aorta where a large proportion of clinically significant anomalies occur in Turner syndrome. 23 refs., 2 tabs., 5 figs.

  10. 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

  11. Thoracic spine pain

    Directory of Open Access Journals (Sweden)

    Aleksey Ivanovich Isaikin

    2013-01-01

    Full Text Available Thoracic spine pain, or thoracalgia, is one of the common reasons for seeking for medical advice. The epidemiology and semiotics of pain in the thoracic spine unlike in those in the cervical and lumbar spine have not been inadequately studied. The causes of thoracic spine pain are varied: diseases of the cardiovascular, gastrointestinal, pulmonary, and renal systems, injuries to the musculoskeletal structures of the cervical and thoracic portions, which require a thorough differential diagnosis. Facet, costotransverse, and costovertebral joint injuries and myofascial syndrome are the most common causes of musculoskeletal (nonspecific pain in the thoracic spine. True radicular pain is rarely encountered. Traditionally, treatment for thoracalgia includes a combination of non-drug and drug therapies. The cyclooxygenase 2 inhibitor meloxicam (movalis may be the drug of choice in the treatment of musculoskeletal pain.

  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. Thoracic meningocele in lumbo-costo-vertebral syndrome in a child: possible enlargement with repeated motion by anchoring to the diaphragm.

    Science.gov (United States)

    Wataya, Takafumi; Horikawa, Kyohei; Kitagawa, Masashi; Tashiro, Yuzuru

    2016-08-01

    Lumbo-costo-vertebral syndrome (LCVS) is a rare disorder in children that is characterized by hemivertebrae, congenital absence of ribs, meningocele, and hypoplasia of the truncal and abdominal wall presenting as a congenital lumbar hernia. An otherwise healthy 12-month-old girl was referred to the authors' hospital with soft swelling on her left middle back; scoliosis had been present since birth. Imaging revealed a thoracic meningocele, ectopia of the spleen suggesting lumbar hernia, multiple anomalies of the thoracic vertebral columns, and defects of the ribs; thus, LCVS was diagnosed. Surgical observation revealed that the meningocele was firmly anchored to part of the diaphragm, which created stretching tension in the meningocele continuously with exhalation. Once detached, the meningocele shrank spontaneously and never developed again after cauterization. In this case, continuous or pulsatile pressure in the presence of a vertebral defect was thus considered to be an important factor for formation of the thoracic meningocele.

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

    Science.gov (United States)

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

    2011-11-01

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

  15. Co-occurrence of outlet impingement syndrome of the shoulder and restricted range of motion in the thoracic spine - a prospective study with ultrasound-based motion analysis

    Directory of Open Access Journals (Sweden)

    Fuchs-Winkelmann Susanne

    2010-06-01

    Full Text Available Abstract Background Shoulder complaints, and especially the outlet-impingement syndrome, are a common condition. Among other things, poor posture has been discussed as a cause. A correlation between impingement syndrome and restricted mobility of the thoracic spine (T has been described earlier, but there has been no motion analysis of the thoracic spine to show these correlations. In the present prospective study, we intended to find out whether there is a significant difference in the thoracic sagittal range of motion (ROM between patients with a shoulder outlet impingement syndrome and a group of patients who had no shoulder pathology. Secondly, we wanted to clarify whether Ott's sign correlates with ultrasound topometric measurements. Methods Two sex- and age-matched groups (2 × n = 39 underwent a clinical and an ultrasound topometric examination. The postures examined were sitting up straight, sitting in maximal flexion and sitting in maximal extension. The disabilities of the arm, shoulder and hand (DASH score (obtained by means of a self-assessment questionnaire and the Constant score were calculated. Lengthening and shortening of the dorsal projections of the spine in functional positions was measured by tape with Ott's sign. Results On examination of the thoracic kyphosis in the erect seated posture there were no significant differences between the two groups (p = 0.66. With ultrasound topometric measurement it was possible to show a significantly restricted segmental mobility of the thoracic spine in the study group compared with the control group (p = 0.01. An in-depth look at the mobility of the subsegments T1-4, T5-8 and T9-12 revealed that differences between the groups in the mobility in the lower two sections of the thoracic spine were significant (T5-8: p = 0.03; T9-12: p = 0.02. The study group had an average Constant score of 35.1 points and the control group, 85.5 (p Conclusion The mobility of the thoracic spine should

  16. Aortic distensibility as a surrogate for intertwin pulse pressure differences in monochorionic pregnancies with and without twin-twin transfusion syndrome.

    Science.gov (United States)

    Wohlmuth, C; Osei, F A; Moise, K J; Johnson, A; Papanna, R; Bebbington, M; Gardiner, H M

    2016-08-01

    Twin-twin transfusion syndrome (TTTS) complicates 10-15% of monochorionic diamniotic (MCDA) twin pregnancies. The donor response to hypovolemia allows the transfer of vasoactive mediators to the recipient, causing increased recipient afterload and hypertension. Our objective was to apply a novel speckle-tracking technique to measure the aortic fractional area change (AFAC) during the cardiac cycle in MCDA twins with and without TTTS, and identify intertwin differences in AFAC and parameters of cardiac function. High-frame rate four-chamber views of the fetal heart, including the mid-thoracic aorta, were collected prospectively in MCDA twin pairs referred to our center between June 2014 and April 2015. Using speckle-tracking software, the endovascular border of the aorta was traced manually during systole, with guidance on cardiac cycle timing by anatomical M-mode. AFAC, defined as the difference between maximum and minimum area divided by minimum area, expressed as a percentage, was calculated offline and averaged over three cardiac cycles. Tissue Doppler and displacement were used to measure long-axis cardiac function. Intra- and interclass correlation coefficients were used to test observer variability. Fifty-one MCDA twin pregnancies were included, comprising uncomplicated MCDA (n = 14), TTTS Stages 1/2 (n = 21) and TTTS Stages 3/4 (n = 16). Median gestational age was 20.4 (range, 16.2-27.5) weeks. Mean ± SD heart rate was 142.6 ± 7.2 bpm with no significant intertwin pair differences. AFAC was significantly higher in recipients than in donors of TTTS pairs (Stages 1/2: 72.3 ± 29.9% vs 43.7 ± 19.3%, P pregnancies with TTTS. Copyright © 2015 ISUOG. Published by John Wiley & Sons Ltd. Copyright © 2015 ISUOG. Published by John Wiley & Sons Ltd.

  17. 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.

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

    Science.gov (United States)

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

    2015-04-01

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

  19. 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.

  20. One-stage surgery in combination with thoracic endovascular grafting and resection of T4 lung cancer invading the thoracic aorta and spine

    OpenAIRE

    Sato, Seijiro; Goto, Tatsuya; Koike, Terumoto; Okamoto, Takeshi; Shoji, Hirokazu; Ohashi, Masayuki; Watanabe, Kei; Tsuchida, Masanori

    2017-01-01

    A novel strategy of one-stage surgery in combination with thoracic endovascular grafting and resection for T4 lung cancer invading the thoracic aorta and spine is described. A 56-year-old man with locally advanced lung cancer infiltrating the aortic wall and spine underwent neoadjuvant chemotherapy and thoracic irradiation, followed by en bloc resection of the aortic wall and spine with thoracic endovascular grafting. He developed postoperative chylothorax, but there were no stent graft-relat...

  1. Thoracic CT

    Science.gov (United States)

    ... through a vein (IV) in your hand or forearm. It may be given through the rectum using ... CT scan Vertebra, thoracic (mid back) Normal lung anatomy Thoracic organs References Gotway MB, Panse PM, Gruden ...

  2. Online network of subspecialty aortic disease experts: Impact of "cloud" technology on management of acute aortic emergencies.

    Science.gov (United States)

    Schoenhagen, Paul; Roselli, Eric E; Harris, C Martin; Eagleton, Matthew; Menon, Venu

    2016-07-01

    For the management of acute aortic syndromes, regional treatment networks have been established to coordinate diagnosis and treatment between local emergency rooms and central specialized centers. Triage of acute aortic syndromes requires definitive imaging, resulting in complex data files. Modern information technology network structures, specifically "cloud" technology, coupled with mobile communication, increasingly support sharing of these data in a network of experts using mobile, online access and communication. Although this network is technically complex, the potential benefit of online sharing of data files between professionals at multiple locations within a treatment network appear obvious; however, clinical experience is limited, and further evaluation is needed. Copyright © 2016 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.

  3. Risk factors of significant pain syndrome 90 days after minor thoracic injury: trajectory analysis.

    Science.gov (United States)

    Daoust, Raoul; Emond, Marcel; Bergeron, Eric; LeSage, Natalie; Camden, Stéphanie; Guimont, Chantal; Vanier, Laurent; Chauny, Jean-Marc

    2013-11-01

    The objective was to identify the risk factors of clinically significant pain at 90 days in patients with minor thoracic injury (MTI) discharged from the emergency department (ED). A prospective, multicenter, cohort study was conducted in four Canadian EDs from November 2006 to November 2010. All consecutive patients aged 16 years or older with MTI were eligible at discharge from EDs. They underwent standardized clinical and radiologic evaluations at 1 and 2 weeks, followed by standardized telephone interviews at 30 and 90 days. A pain trajectory model characterized groups of patients with different pain evolutions and ascertained specific risk factors in each group through multivariate analysis. In this cohort of 1,132 patients, 734 were eligible for study inclusion. The authors identified a pain trajectory that characterized 18.2% of the study population experiencing clinically significant pain (>3 of 10) at 90 days after a MTI. Multivariate modeling found two or more rib fractures, smoking, and initial oxygen saturation below 95% to be predictors of this group of patients. To the authors' knowledge, this is the first prospective study of trajectory modeling to detect risk factors associated with significant pain at 90 days after MTI. These factors may help in planning specific treatment strategies and should be validated in another prospective cohort. © 2013 by the Society for Academic Emergency Medicine.

  4. 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.

  5. 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.

  6. 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.)

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

    Science.gov (United States)

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

    2013-02-01

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

  8. 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

  9. Venous Thoracic Outlet Compression and the Paget-Schroetter Syndrome: A Review and Recommendations for Management

    International Nuclear Information System (INIS)

    Thompson, J. F.; Winterborn, R. J.; Bays, S.; White, H.; Kinsella, D. C.; Watkinson, A. F.

    2011-01-01

    Paget Schroetter syndrome, or effort thrombosis of the axillosubclavian venous system, is distinct from other forms of upper limb deep vein thrombosis. It occurs in younger patients and often is secondary to competitive sport, music, or strenuous occupation. If untreated, there is a higher incidence of disabling venous hypertension than was previously appreciated. Anticoagulation alone or in combination with thrombolysis leads to a high rate of rethrombosis. We have established a multidisciplinary protocol over 15 years, based on careful patient selection and a combination of lysis, decompressive surgery, and postoperative percutaneous venoplasty. During the past 10 years, a total of 232 decompression procedures have been performed. This article reviews the literature and presents the Exeter Protocol along with practical recommendations for management.

  10. Prevalence and Outcomes of Mitral Stenosis in Patients Undergoing Transcatheter Aortic Valve Replacement: Findings From the Society of Thoracic Surgeons/American College of Cardiology Transcatheter Valve Therapies Registry.

    Science.gov (United States)

    Joseph, Lee; Bashir, Mohammad; Xiang, Qun; Yerokun, Babatunde A; Matsouaka, Roland Albert; Vemulapalli, Sreekanth; Kapadia, Samir; Cigarroa, Joaquin E; Zahr, Firas

    2018-04-09

    This study sought to examine the prevalence of mitral stenosis (MS) and its impact on in-hospital and 1-year clinical outcomes among patients undergoing transcatheter aortic valve replacement (TAVR). Patients with coexisting severe aortic stenosis and MS are increasingly being considered for TAVR. The study cohort included 44,755 patients (age ≥18 years) who underwent TAVR during November 1, 2011, to September 30, 2015, and were registered in Society of Thoracic Surgeons/American College of Cardiology Transcatheter Valve Therapies (TVT) Registry. One-year outcomes were assessed by linking TVT registry data of this cohort to patient-specific Centers for Medicare & Medicaid Services administrative claims data (n = 31,453). The primary outcome was the composite of death, stroke, heart failure-related hospitalization, and mitral valve intervention at 1 year. MS was present in 11.6% of cohort (mean age, 82 years; 52% males), being severe in 2.7%. Severe MS was associated with higher in-hospital mortality rates (5.6% vs. 3.9% for nonsevere MS and 4.1% for no MS; p = 0.02). In contrast to those without MS, severe MS group had significantly higher risk for the primary outcome, mortality (1 year), and heart failure-related hospitalization (1 year) (adjusted hazard ratio: 1.2 [95% confidence interval (CI): 1.1 to 1.4], 1.2 [95% CI: 1.0 to 1.4], and 1.3 [95% CI: 1.1 to 1.5], respectively; p < 0.05 for all). Approximately one-tenth of patients undergoing TAVR have concomitant MS. Severe MS is an independent predictor of 1-year adverse clinical outcomes following TAVR. The higher risk for long-term adverse events must be considered when evaluating patients with combined aortic stenosis and MS for TAVR. Copyright © 2018 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

  11. Thoracic Outlet Syndrome and Volkmann's Contracture in a Field Hockey Player

    Directory of Open Access Journals (Sweden)

    Morgan Sawyer

    2016-05-01

    Full Text Available Background: A 19-year-old female field hockey player presents with bilateral upper trapezius and rhomboid tightness and spasm beginning in January of 2016. She has no previous history of upper back pain or injury. The student first reported the injury immediately after running sprints. She stated that she could not move or feel her fingers or hands. She presents with a visible hump on her left upper trapezius. She also has forward-rounded shoulders. She has regular and equal radial pulses. She has decreased sensation upon palpation of her fingers, hands and forearms. After extraneous exercise, the athlete’s hands, wrists and forearms go into contracture and flexion and she is unable to move from this position until manual extension is applied, or 10 or more minutes of rest occurs. She is TTP over her upper trapezius musculature. She had a positive Military Brace test. Differential Diagnosis: Based on evaluation, the athlete could simply just have upper trapezius musculature spasms. The diagnosis could also be shoulder impingement syndrome, due to the neurological signs and symptoms. Compartment syndrome could produce similar s/s that the athlete experiences, as well. Treatment: X-rays show no bony abnormalities and no extra ribs. The athlete’s injury has been treated with ice post-practice, heat pre-practice and upper body stretching exercises. Three days a week the athlete works on postural exercises that strengthen her back musculature. The athlete also receives combination therapy over her upper trapezius trigger points, along with massage and Hawkgrip technique over the same areas. Uniqueness: The uniqueness in this case is that the athlete has both TOS and Volkmann’s contracture. Volkmann’s contractures typically occur in pediatrics, which is another reason why this particular case is unique. Conclusion: This case allows other clinicians to understand the causes of TOS and the secondary s/s that TOS can potentially cause.

  12. The novel EuroSCORE II algorithm predicts the hospital mortality of thoracic aortic surgery in 461 consecutive Japanese patients better than both the original additive and logistic EuroSCORE algorithms.

    Science.gov (United States)

    Nishida, Takahiro; Sonoda, Hiromichi; Oishi, Yasuhisa; Tanoue, Yoshihisa; Nakashima, Atsuhiro; Shiokawa, Yuichi; Tominaga, Ryuji

    2014-04-01

    The European System for Cardiac Operative Risk Evaluation (EuroSCORE) II was developed to improve the overestimation of surgical risk associated with the original (additive and logistic) EuroSCOREs. The purpose of this study was to evaluate the significance of the EuroSCORE II by comparing its performance with that of the original EuroSCOREs in Japanese patients undergoing surgery on the thoracic aorta. We have calculated the predicted mortalities according to the additive EuroSCORE, logistic EuroSCORE and EuroSCORE II algorithms in 461 patients who underwent surgery on the thoracic aorta during a period of 20 years (1993-2013). The actual in-hospital mortality rates in the low- (additive EuroSCORE of 3-6), moderate- (7-11) and high-risk (≥11) groups (followed by overall mortality) were 1.3, 6.2 and 14.4% (7.2% overall), respectively. Among the three different risk groups, the expected mortality rates were 5.5 ± 0.6, 9.1 ± 0.7 and 13.5 ± 0.2% (9.5 ± 0.1% overall) by the additive EuroSCORE algorithm, 5.3 ± 0.1, 16 ± 0.4 and 42.4 ± 1.3% (19.9 ± 0.7% overall) by the logistic EuroSCORE algorithm and 1.6 ± 0.1, 5.2 ± 0.2 and 18.5 ± 1.3% (7.4 ± 0.4% overall) by the EuroSCORE II algorithm, indicating poor prediction (P algorithms were 0.6937, 0.7169 and 0.7697, respectively. Thus, the mortality expected by the EuroSCORE II more closely matched the actual mortality in all three risk groups. In contrast, the mortality expected by the logistic EuroSCORE overestimated the risks in the moderate- (P = 0.0002) and high-risk (P < 0.0001) patient groups. Although all of the original EuroSCOREs and EuroSCORE II appreciably predicted the surgical mortality for thoracic aortic surgery in Japanese patients, the EuroSCORE II best predicted the mortalities in all risk groups.

  13. 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.

  14. A deleterious gene-by-environment interaction imposed by calcium channel blockers in Marfan syndrome.

    Science.gov (United States)

    Doyle, Jefferson J; Doyle, Alexander J; Wilson, Nicole K; Habashi, Jennifer P; Bedja, Djahida; Whitworth, Ryan E; Lindsay, Mark E; Schoenhoff, Florian; Myers, Loretha; Huso, Nick; Bachir, Suha; Squires, Oliver; Rusholme, Benjamin; Ehsan, Hamid; Huso, David; Thomas, Craig J; Caulfield, Mark J; Van Eyk, Jennifer E; Judge, Daniel P; Dietz, Harry C

    2015-10-27

    Calcium channel blockers (CCBs) are prescribed to patients with Marfan syndrome for prophylaxis against aortic aneurysm progression, despite limited evidence for their efficacy and safety in the disorder. Unexpectedly, Marfan mice treated with CCBs show accelerated aneurysm expansion, rupture, and premature lethality. This effect is both extracellular signal-regulated kinase (ERK1/2) dependent and angiotensin-II type 1 receptor (AT1R) dependent. We have identified protein kinase C beta (PKCβ) as a critical mediator of this pathway and demonstrate that the PKCβ inhibitor enzastaurin, and the clinically available anti-hypertensive agent hydralazine, both normalize aortic growth in Marfan mice, in association with reduced PKCβ and ERK1/2 activation. Furthermore, patients with Marfan syndrome and other forms of inherited thoracic aortic aneurysm taking CCBs display increased risk of aortic dissection and need for aortic surgery, compared to patients on other antihypertensive agents.

  15. 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. ...

  16. Aortic and Cardiac Structure and Function Using High-Resolution