Bicuspid Aortic Valve
It is the most common congenital heart disease and is found in 0.9%-1.36% of the population.
by Warren Rosenblum, MD
Bicuspid aortic valve (BAV) involves the presence of only two leaflets in the aortic valve apparatus instead of the normal three leaflets. In this congenital condition, two leaflets are fused to create one. It is the most common congenital heart disease and is found in 0.9%-1.36% of the population. Most commonly, BAV involves fusion of the right coronary and left coronary cusps and, less often, of the right and noncoronary cusps. Fusion is associated with a dilated aortic root of the ascending aorta, aorticaneurysms, and aortic dissections. Over time, the valve may become stenotic and cause symptoms including congestive heart failure, hypertension, and syncope. Alternatively, BAV may result in regurgitation if the leaflets do not coapt correctly to cause dilation of the left ventricle. Patients with BAV also are at risk of developing endocarditis.
A bicuspid aortic valve often is discovered on physical exam. Auscultation of a systolic murmur best heard at the second right intercostal space is noted, prompting an echocardiographic examination. Confirmation can be obtained with transesophageal echocardiography or cardiac catheterization. It also may be evaluated by cardiac magnetic resonance imaging (MRI) or computed tomography (CT).
Treatment and Recommended Follow-up
Many patients survive long term without intervention. A significant percentage of patients require surgical repair if the valve is regurgitant or replacement if stenosis is present. If the aortic root is dilated or an aneurysm is found, the aorta may require repair or replacement.
Asymptomatic patients < 30 years of age who have low Doppler gradients require echocardiographic follow-up every 1-2 years; those who have elevated AV Doppler gradients require yearly echocardiographic follow-up. Older adults with aortic stenosis or regurgitation require follow-up based on the degree of stenosis or regurgitation; it may vary between every 6 months if severe, 1-2 years if moderate, and 3-5 years if mild.
Adults with an aortic root or ascending aorta > 4 cm should have a baseline CT or MRI; those with an aortic root or ascending aorta > 4.5 cm or who have a rapid change in aortic diameter should be imaged annually.
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