Congenitally Bicuspid Aortic Valves: A Surgical Pathology Study of 542 Cases (1991 Through 1996) and a Literature Review of 2,715 Additional Cases

Sabet, Haideh Yazdani; Edwards, William D.; Tazelaar, Henry D.; Daly, Richard C.

doi:10.4065/74.1.14

Cited by 386 publications

(286 citation statements)

References 46 publications

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“…5,6,11 The lack of bicuspid valves in the female group is in accordance with earlier studies showing that isolated regurgitation of bicuspid valves occurs more often in men. 14 Our definitions excluded both concomitant aortic stenosis and AR secondary to aneurysm. Consequently, the number of patients was limited, which probably explains why we did not find a significant relation between preoperative LV function, gender, or age and survival, in contrast to the convincing relation of LV function to survival found in larger patient samples.…”

Section: Discussionmentioning

confidence: 99%

Are patients with isolated chronic aortic regurgitation operated in time? Analysis of survival data over a decade

Tamás

Nylander

Olin

2005

Clinical Cardiology

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SummaryBackground: Patients suffering from chronic isolated aortic regurgitation have a less favorable outcome than patients with aortic stenosis. According to international recommendations, these patients should undergo surgery as soon as left ventricular function begins to deteriorate, that is, surgery is not to be postponed until clinical symptoms become relevant.Hypothesis: The study was undertaken to evaluate how satisfactory our timing of surgery was, as reflected by survival data.Methods: Survival was studied retrospectively in a consecutive series of patients undergoing surgery for chronic isolated aortic regurgitation during a 10-year period in our institution. Results were compared with data from the literature. By excluding patients with aortic aneurysms and acute endocarditis, we formed a homogeneous patient group of 88 subjects.Results: Thirty-day mortality was 1% and late mortality after a mean follow-up period of 6 years was 11%. Compared with survival data from an earlier study in which the patient population was similar and resided in the same geographic area, the results in our patient group seem to be better. It is noteworthy that despite a strong effort to recommend surgery at an earlier stage of the disease than previously, 35% of the patients had moderate or severe left ventricular dysfunction preoperatively because of late referrals.Conclusion: This stresses the importance of early detection and careful preoperative follow-up with noninvasive methods in patients with aortic regurgitation.

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Section: Discussionmentioning

confidence: 99%

Are patients with isolated chronic aortic regurgitation operated in time? Analysis of survival data over a decade

Tamás

Nylander

Olin

2005

Clinical Cardiology

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“…(24)(25)(26)(27)(28) Despite this challenge, the many prognostic this information is implicit in most current classifications. (27) In addition to valve morphology, more complete phenotypic classifications also include typing of aortic dilation.…”

Section: Phenotypementioning

confidence: 99%

Bicuspid aortic valve disease

Lee

Otto

2017

SAHJ

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and pulmonary valves with the leaflets developing via a process of cavitation into three distinct layers (fibrosa, spongiosa, and ventricularis). (11) The development of the aorta is also a complex process in which the common truncus arteriosus is septated into the aorta and pulmonary arteries. Abnormalities in septation are the cause of common congenital heart defects such as tetraology of Fallot. This process is also likely related to the migration of cardiac neural crest cells from the pharyngeal arches and heart; (10) and these neuronal crest cells may be similar in origin to the cells involved in the development of the aortic outflow tract and aortic valve.

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“…10,14 A central raphe is absent in the less frequently occurring true bicuspid valve. [15][16][17] BAV can be an isolated congenital anomaly, but can also be associated with other abnormalities such as aortic coarctation, ventricular septal defects and hypoplastic left ventricle. 6,9,12 In most cases, BAV can be diagnosed and hemodynamically assessed using transthoracic echocardiography.…”

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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Congenitally Bicuspid Aortic Valves: A Surgical Pathology Study of 542 Cases (1991 Through 1996) and a Literature Review of 2,715 Additional Cases

Cited by 386 publications

References 46 publications

Are patients with isolated chronic aortic regurgitation operated in time? Analysis of survival data over a decade

Are patients with isolated chronic aortic regurgitation operated in time? Analysis of survival data over a decade

Bicuspid aortic valve disease

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

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