Incidence of Aortic Complications in Patients With Bicuspid Aortic Valves

Michelena, Héctor I.; Khanna, Amber; Mahoney, Douglas W.; Margaryan, Edit; Topilsky, Yan; Suri, Rakesh M.; Eidem, Ben; Edwards, William D.; Sundt, Thoralf M.; Enríquez-Sarano, Maurice

doi:10.1001/jama.2011.1286

Cited by 725 publications

(614 citation statements)

References 40 publications

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“…Evidence strongly implicates genetic mechanisms for selected aortic diseases such as Marfan syndrome, but the underlying cause of ascending aortic aneurysm formation in other aortic diseases is less well understood 1, 2. For example, many patients with congenital bicuspid aortic valve (BAV) develop ascending aortic dilatation,3, 4 in whom the underlying causative mechanisms are not clear 5. Genetic variants may contribute to BAV mediated aortopathy, but no common or unifying genetic pattern has emerged.…”

Section: Introductionmentioning

confidence: 99%

Aortic Valve Stenosis Alters Expression of Regional Aortic Wall Shear Stress: New Insights From a 4‐Dimensional Flow Magnetic Resonance Imaging Study of 571 Subjects

Ooij

Markl

Collins

et al. 2017

JAHA

131

104

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BackgroundWall shear stress (WSS) is a stimulus for vessel wall remodeling. Differences in ascending aorta (AAo) hemodynamics have been reported between bicuspid aortic valve (BAV) and tricuspid aortic valve patients with aortic dilatation, but the confounding impact of aortic valve stenosis (AS) is unknown.Methods and ResultsFive hundred seventy‐one subjects underwent 4‐dimensional flow magnetic resonance imaging in the thoracic aorta (210 right‐left BAV cusp fusions, 60 right‐noncoronary BAV cusp fusions, 245 tricuspid aortic valve patients with aortic dilatation, and 56 healthy controls). There were 166 of 515 (32%) patients with AS. WSS atlases were created to quantify group‐specific WSS patterns in the AAo as a function of AS severity. In BAV patients without AS, the different cusp fusion phenotypes resulted in distinct differences in eccentric WSS elevation: right‐left BAV patients exhibited increased WSS by 9% to 34% (P<0.001) at the aortic root and along the entire outer curvature of the AAo whereas right‐noncoronary BAV patients showed 30% WSS increase (P<0.001) at the distal portion of the AAo. WSS in tricuspid aortic valve patients with aortic dilatation patients with no AS was significantly reduced by 21% to 33% (P<0.01) in 4 of 6 AAo regions. In all patient groups, mild, moderate, and severe AS resulted in a marked increase in regional WSS (P<0.001). Moderate‐to‐severe AS further increased WSS magnitude and variability in the AAo. Differences between valve phenotypes were no longer apparent.Conclusions AS significantly alters aortic hemodynamics and WSS independent of aortic valve phenotype and over‐rides previously described flow patterns associated with BAV and tricuspid aortic valve with aortic dilatation. Severity of AS must be considered when investigating valve‐mediated aortopathy.

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Section: Introductionmentioning

confidence: 99%

Aortic Valve Stenosis Alters Expression of Regional Aortic Wall Shear Stress: New Insights From a 4‐Dimensional Flow Magnetic Resonance Imaging Study of 571 Subjects

Ooij

Markl

Collins

et al. 2017

JAHA

131

104

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“…ДАК и синдром Марфана несут жизнеугрожающие осложнения, такие как разрыв и расслоение, однако распространенность синдрома Марфана все еще остается достаточно низкой (0,01-0,02%) в сравнении с ДАК (0,5-1%) [5]. Как результат этого, частота встречаемости случаев аневризмы аорты в популяции среди пациентов с ДАК выше в сравнении с пациентами с синдромом Марфана [2]. Важно иметь в виду, что пациенты с большими размерами аорты имеют существенный риск смертности в течении 5 лет, особенно когда диаметр аорты достигает 6 см [6].…”

Section: аортопатия ассоциированная с дакunclassified

“…ДАК может сочетаться со значительной дисфункцией аортального клапана (АК), что проявляется в формировании стеноза или недостаточности, а также иметь повышенную предрасположенность к развитию инфекционного эндокардита. Дополнительно ДАК также может сочетаться с патологией стенки аорты, которая включает коарктацию аорты, расслоение аорты, дилятацию аорты с последующим формированием аневризмы [2]. У многих пациентов с ДАК могут развиваться осложнения в течение жизни, что требует продолжительного наблюдения и своевременного хирургического вмешательства.…”

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Aortopathy pathophysiology features in patients with bicuspid aortic valve.

et al. 2016

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This review is devoted to one of the vital topics in cardiovascular surgery -pathophysiologyof an aortopathy in case of bicuspid aortic valve. In the article reviewed the modern point of view on a problem considering new achievements in genetics, molecular biology and altered haemodynamic.Key words: aortic aneurysm-bicuspid aortic valve-metalloproteinase. ТұжырымдамаӘдебиетке болжамдалған шолу қантамырлық-жүрек хирургиясының өзекті тақырыптарының бірі -екі жақтаулы аорталық қақпашасы бар пациенттерде патофизиологиялық аортопатияның ерекшеліктеріне арналған. Мақалада генетикадағы, молекулярлық биологиядағы, гемодинамика патологиясындағы жаңа жетістіктер ескеріле отырып, проблемаға заманауи тұрғыдағы көзқарас берілен.Маңызды сөздер: аорта аневризмі -екі жақтаулы аорталық қақпаша -металлопротеиназдар.оСоБенноСти патофизиологии аортопатии у пациентов С двуСтворчатыМ аортальныМ клапаноМ. Сейдалин а.о. 1 ,туганбеков т.у. 2 , диколаев в.д. 2,3, айталиев С.е 3 .1 «Городской кардиологический центр», отделение кардиохирургии, Алматы, Казахстан 2 «Медицинский Университет Астана», кафедра хирургии, Астана, Казахстан 3«Национальный научный медицинский центр», отделение кардиохирургии, Астана, Казахстан Резюме.Предполагаемый обзор литературы посвящен одной из актуальных тем в сердечно-сосудистой хирургии -патофизиологии аортопатии при двустворчатом аортальном клапане. В статье дан современный взгляд на проблему с учетом новых достижений в генетике, молекулярной биологии, патологии гемодинамики.Ключевые слова: аневризма аорты -двустворчатый аортальный клапан -металлопротеиназы.

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“…34 Whereas the lifetime risk of aortic dissection in BAV patients was initially reported around 5%, recent studies show a lifetime risk of aortic dissection of less than 1% in BAV patients and a normal life expectancy. 4,6,35,36 This difference can potentially be explained by increased surveillance and timely surgical intervention in recent years. Aortic dissection is, in the majority of cases, preceded by slowly progressive asymptomatic aortic dilatation.…”

Section: Bicuspid Aortic Valve: Clinical and Genetic Aspectsmentioning

confidence: 99%

Clinical and Genetic Aspects of Bicuspid Aortic Valve: A Proposed Model for Family Screening Based on a Review of Literature

et al. 2015

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Bicuspid aortic valve (BAV) is the most common congenital cardiac defect causing serious morbidity including valvular dysfunction and thoracic aortic aneurysms (TAA) in around 30% of BAV patients. Cardiological screening of first-degree relatives is advised in recent guidelines given the observed familial clustering of BAV. However, guidelines regarding screening of family members and DNA testing are not unequivocal. The aim of this review is to provide an overview of the literature on echocardiographic screening in first-degree relatives of BAV patients and to propose a model for family screening. In addition, we provide a flowchart for DNA testing. We performed a PubMed search and included studies providing data on echocardiographic screening in asymptomatic relatives of BAV patients. Nine studies were included. In 5.8-47.4% of the families BAV was shown to be familial. Of the screened first-degree relatives 1.8-11% was found to be affected with BAV. Results regarding a potential risk of TAA in first-degree relatives with a tricuspid aortic valve (TAV) were conflicting. The reported familial clustering of BAV underlines the importance of cardiological screening in relatives. After reviewing the available family history, patient characteristics and the results of cardiological screening in relatives, follow-up in relatives with a TAV and/or DNA testing may be advised in a subset of families. In this study we propose a model for the clinical and genetic work-up in BAV families, based on the most extensive literature review on family screening performed until now.

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Incidence of Aortic Complications in Patients With Bicuspid Aortic Valves

Abstract: In the population of patients with BAV, the incidence of aortic dissection over a mean of 16 years of follow-up was low but significantly higher than in the general population.

Cited by 725 publications

References 40 publications

Aortic Valve Stenosis Alters Expression of Regional Aortic Wall Shear Stress: New Insights From a 4‐Dimensional Flow Magnetic Resonance Imaging Study of 571 Subjects

Aortic Valve Stenosis Alters Expression of Regional Aortic Wall Shear Stress: New Insights From a 4‐Dimensional Flow Magnetic Resonance Imaging Study of 571 Subjects

Aortopathy pathophysiology features in patients with bicuspid aortic valve.

Clinical and Genetic Aspects of Bicuspid Aortic Valve: A Proposed Model for Family Screening Based on a Review of Literature

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