Update on Bicuspid Aortic Valve Syndrome: Patient Selection and Therapies in 2020

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(2020) 22:51

Valvular Heart Disease (J Dal-Bianco, Section Editor)

Update on Bicuspid Aortic Valve Syndrome: Patient Selection and Therapies in 2020 David Anderson, MD* Aaron Grober, MD Vaikom Mahadevan, MD Kirsten Tolstrup, MD Address * San Francisco, USA

* Springer Science+Business Media, LLC, part of Springer Nature 2020

This article is part of the Topical Collection on Valvular Heart Disease Keywords Bicuspid aortic valve I Aortopathy I Transcatheter therapy I Aortic valve replacement

Abstract Purpose of review This article highlights our current understanding of bicuspid aortic valve (BAV) syndrome and how genetics, pathology, imaging features, and clinical symptoms direct the clinical management, surveillance, and intervention of those with BAV. Recent findings While randomized data and guidelines are lacking, transcatheter therapies are routinely used for BAV stenosis in medical centers throughout the world. Guidelines suggest that younger patients age less than 50 years undergo surgical aortic valve replacement (AVR) with a mechanical valve and the elderly 9 70 years of age receive a bioprosthetic valve. For the age group 50–70 years, either option should be considered. Decision on type of intervention depends on specific features such as presence of stenosis or regurgitation, if there is any associated aortopathy, comorbid illnesses, contraindications, and patient preference. We have learned that the decision to intervene and mode of intervention is best achieved by a multidisciplinary valve team. Summary Despite improvement in our understanding of BAV and the advancement of novel therapies particularly for BAV stenosis, much is still to be learned about the optimal treatment of BAV disease in the current era.

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Curr Treat Options Cardio Med

(2020) 22:51

Introduction Bicuspid aortic valve (BAV) is the most common congenital cardiac anomaly occurring in approximately 1– 2% of the general population [1]. There is a male predominance with a male to female ratio of 2–3:1 with most cases being spontaneous; however, familial inheritance occurs in which case the ratio is 1:1 [2, 3]. BAV disease has a wide range of presentations, from severe disease detected at an early age to asymptomatic disease of the elderly. Potential complications of BAV include endocarditis, severe aortic stenosis (AS), aortic regurgitation (AR), aortic aneurysm, and/or dissection. Although complications contribute to morbidity, survival of patients with BAV is similar to age-matched controls [4]. Given that ~ 2–6% of those with severe AS and up to 20% of octo-nonagenarians undergoing aortic valve surgery have BAV, clinicians must understand the clinical and surgical management of these patients. Making decisions surrounding surgery and

surveillance is nuanced in BAV disease. We have learned that it is not only a disorder of valvulogenesis [5] but also a genetic disorder of cardiac development that falls into the spectrum of left ventricular outflow tract obstructive defects (LVOTO) and variably affects the aorta [