Congenital quadricuspid aortic valve associated with severe regurgitation.

A 56-year-old man was referred because of severe aortic regurgitation. He had a quadricuspid aortic valve with a small accessory cusp between the right coronary and noncoronary cusps. The ostium of the right coronary artery was deviated toward the accessory cusp commissure. Aortic valve replacement was performed with a bioprosthesis. The resected cusps showed fibrotic thickening with calcification and fenestration.