Quadricuspid aortic valve with centrally fenestrated leaflets causing aortic insufficiency

The occurrence of a quadricuspid aortic valve with single, central leaflet fenestrations causing aortic insufficiency has not previously been reported. We recently encountered these features during aortic valve replacement for severe aortic regurgitation in a 20-year-old man who had had a history of aortic insufficiency since age 2 years. The patient's late increase in symptoms was probably due to the fact that 1 of the 4 leaflets was somewhat flail, allowing incomplete coaptation and producing regurgitation over and above that caused by the central fenestrations.