Bicuspid aortic valve and coarctation: two villains part of a diffuse problem.

Bicuspid aortic valve is the most common congenital cardiovascular anomaly, occurring in 1–2% of the population. It has a male predominance and often occurs in multiple members of the same family, suggesting that it may have an autosomal dominant inheritance, perhaps with variable penetrance. 2 Much of the data relating to its complications derive from necropsy studies since reliable diagnosis has only been possible for the last 15 years or so by two dimensional echocardiography. The most common complication is aortic stenosis and it is the most frequent cause of patients under 60 years of age requiring aortic valve replacement. Aortic stenosis appears to result from an active disease process reminiscent of atherosclerosis. 4 It has features similar to the degenerative calcification that occurs on tricuspid aortic valves, but tends to occur at an earlier age, perhaps because of different mechanical or shear stresses on the bicuspid valve. Other complications include aortic regurgitation and infective endocarditis.