Unicuspid Unicommissural Aortic Valve in Young Adult: Rare Congenital Abnormality Presenting as Symptomatic and Severe Aortic Stenosis

Isolated aortic valve stenosis in adults with or without aortic regurgitation is almost always secondary to a congenital valvular malformation. These congenital malformations usually present as a bicuspid, quadricuspid, or unicuspid aortic valve. The unicuspid acommissural valve has no commissures or lateral attachments to the aorta at the level of the orifice and appears as a pinhole on imaging. The unicommissural UAV has one lateral commissural attachment to the aorta at the level of the orifice and appears as a slit-shaped structure. We report the case of a 21-year-old man who presented with dyspnea, palpitations and one episode of syncope on exertion. Transesophageal echocardiography confirmed a stenotic, unicuspid, unicommissural aortic valve with dilated ascending aorta. He underwent successful aortic valve replacement since the ascending aorta was only mildly dilated (40 mm). Postoperative course was uneventful and discharge from the hospital in stable condition.