Bicuspid aortic valve and associated aortopathy: an update.

Bicuspid aortic valve (BAV) is the most common form of congenital heart disease and most such patients develop cardiovascular complications over time. Recent studies have shed light on one of the most common of these complications, BAV-associated aortopathy. Two distinct BAV phenotypes have been identified, which may have different causes of their associated aortopathy. Increasing evidence suggests that the BAV stenosis phenotype is predominantly secondary to hemodynamic perturbances in transvalvular flow and is associated with a more benign long-term prognosis once the stenotic BAV is replaced. In contrast, the root phenotype--which is associated with aortic insufficiency--appears to have a genetic origin and may be associated with a higher risk of adverse aortic complications, irrespective of the extent of valvular disease. Such observations may have implications for patient decision making. Future studies should be performed so as to better define phenotypes and risk factors for BAV-associated aortopathy.