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Management of adults with bicuspid aortic valve disease

Author
Alan C Braverman, MD
Section Editor
Candice Silversides, MD, MS, FRCPC
Deputy Editor
Susan B Yeon, MD, JD, FACC

INTRODUCTION

Adults with bicuspid aortic valves require surveillance for aortic valve dysfunction and for disease of the aortic root and/or ascending aorta. Aortic valve replacement is generally required to treat symptomatic aortic stenosis except in selected young adults who may benefit from percutaneous balloon valvuloplasty. Aortic valve replacement is generally the definitive therapy for severe aortic regurgitation. Progressive aortic root and/or ascending aortic dilation (or ascending aortic dissection) is an indication for replacement of the aortic root and/or ascending aorta.

Treatment of adults with bicuspid aortic valve disease will be discussed here. The natural history and clinical manifestations of bicuspid aortic valve disease in adults, management of pregnancy in women with bicuspid aortic valve, and clinical manifestations and management of aortic stenosis in children are discussed separately. (See "Clinical manifestations and diagnosis of bicuspid aortic valve in adults" and "Pregnancy in women with a bicuspid aortic valve" and "Valvar aortic stenosis in children".)

SURVEILLANCE

Who and how frequently to monitor — Patients with bicuspid aortic valves should be monitored for progressive aortic valve dysfunction (stenosis and/or regurgitation) as well as for aortic dilation with risk of aneurysm formation and aortic dissection. There are many different phenotypes of bicuspid aortic valve aortic enlargement (bicuspid valve "aortopathy") [1]. The most common site of aortic dilation is at the ascending aorta and monitoring by transthoracic echocardiography is often inadequate at this level unless high right parasternal views are obtained. Echocardiography should be performed at intervals, based upon the lesion requiring greatest frequency of surveillance among aortic stenosis, aortic regurgitation, and dilation of the aorta. The rate of aortic growth in bicuspid aortic valve patients is variable, ranging from 0.2 to 0.9 mm/year, depending upon patient characteristics [1,2]. Higher rates of growth are observed in older adults and those with larger aneurysms [2,3]. Development of significant aortic stenosis is much more common than development of significant aortic regurgitation.

Earlier re-evaluation (beyond the below recommended surveillance) is indicated for changes in symptoms or examination and for hemodynamic assessment related to pregnancy. (See "Pregnancy in women with a bicuspid aortic valve".) Of note, although surveillance of the aorta is based upon aortic diameter, other factors (eg, genetic factors, hemodynamic effects) likely contribute to the risk of acute aortic events (eg, aortic dissection and aortic rupture) [2].

The following recommendations for surveillance and management for aortic stenosis, aortic regurgitation, and aortic dilatation are based upon the 2014 American Heart Association/American College of Cardiology (AHA/ACC) valve guidelines, the 2008 ACC/AHA adult congenital heart disease guidelines, and the ACC/AHA scientific statement on eligibility and disqualification recommendations for competitive athletes with cardiovascular abnormalities (table 1A-C) [4,5] and are consistent with the 2011 appropriate use criteria for echocardiography [6-8]:

                    

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Literature review current through: Nov 2016. | This topic last updated: Tue Jul 19 00:00:00 GMT 2016.
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