Bicuspid aortic valve and aortopathy: see the first, then look at the second.

t a h a a ( p c H u e c D n c o c b d a Bicuspid aortic valve (BAV) disease is the most common congenital cardiac anomaly, with a prevalence in the general population between 0.5% and 2% (1). There is significant cardiac morbidity associated with BAV disease, predominantly due to progressive valve dysfunction (stenosis or regurgitation) that requires surgical intervention for symptom relief or prevention of left ventricular dysfunction, or less commonly, for complications of endocarditis (2,3). We now understand that BAV disease is more than simply having 2, instead of 3, aortic valve leaflets. BAV disease encompasses a spectrum of phenotypic manifestations that not only includes valve dysfunction, but also abnormalities of the ascending aorta. Less common cardiovascular abnormalities may also occur, such as aortic coarctation, atrial septal defects, and ventricular septal defects.