Natural History of Bicuspid and Aneurysm-Associated Aortic Valve Disease

The most common congenital heart defect, congenital BAV, affects 1% of the population (2% in men, 0.5% in women), with a male-to-female ratio of 2:1 to 3:1. It's characterized by diverse phenotypic expressions and outcomes, falling into two prognostic groups: 

1. Typical valvulo-aortopathy: Most common, with progressive BAV dysfunction and/or aorta dilatation but long-term survival similar to the general population. 

2. Complex valvulo-aortopathy: Associated with significant concomitant disorders and/or accelerated valvulo-aortopathy, leading to inferior long-term survival. 

BAV is a lifelong clinical condition with a morbidity burden exceeding 80%. Common complications include progression to ≥ moderate AS or AR, native aortic valve surgery, aortic aneurysm, surgery for aortic aneurysm, surgery for coarctation of the aorta, infective endocarditis, and aortic dissection.

In this interview, Hector I. Michelena, MD, FACC and W. Douglas Weaver MD, MACC discuss the history of and complications associated with bicuspid valvuloaortopathy. 

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