Bicuspid aortic valve (BAV) is the most frequently occurring congenital cardiac anomaly, affecting 1% to 2% of the population. BAV disease is increasingly recognized as a disease of the entire proximal aorta up to the level of the ligamentum arteriosum. The recent unfolding of the genetic and biologic background of the disease and the accumulating data regarding the natural history of BAV-associated aortic dilatation have accrued multiple levels of evidence strongly supporting early surgical intervention.
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