Replacement of Regurgitant Bicuspid Aortic Valve in a Dilated, Non-Compacted Left Ventricle.

Bicuspid aortic valve (BAV) is the most common form of congenital heart disease, with 20% of asymptomatic adults with BAV presenting with significant valve insufficiency. Yet, limited data exist regarding surgical indications and outcomes when BAV is accompanied by left ventricular dilation, systolic dysfunction, or left ventricle non-compaction (LVNC) syndrome. We present a case of dilated cardiomyopathy due to severe BAV regurgitation and partial LVNC syndrome and the decision to undergo aortic valve replacement. Our patient represents the most extreme documented case of regurgitant BAV with dilated, dysfunctional, and partially non-compacted left ventricle. Yet, surgical intervention provided improvement in systolic performance and ventricular dimensions.