The unicuspid aortic valve

The unicuspid aortic valve is a very rare congenital anomaly, which usually presents as aortic stenosis, incompetence, or a combination of both. Other congenital disorders may accompany this phenomenon and aortic dilatation and left ventricular hypertrophy are frequent complications. We present a case report of a young, symptomatic patient with a unicuspid aortic valve, complicated by dilatation of the aortic root and ascending aorta, with left ventricular hypertrophy. The patient recovered fully after a Bentall procedure.