Bentall Operation in a Patient with a Unicommissural Unicuspid Aortic Valve

A unicuspid aortic valve is a rare congenital malformation that frequently presents with valvular dysfunction and dilatation or aortic aneurysm, requiring combined aortic valve surgery and aortic repair. Some patients show severe valve calcification extending into the interventricular septum, possibly resulting in damage to the conduction system during debridement for valve replacement. We present a rare case of severe aortic stenosis with a unicommissural unicuspid aortic valve diagnosed by preoperative transesophageal echocardiography in a 36-year-old man. After composite graft replacement of the aortic valve, aortic root, and ascending aorta, a permanent pacemaker was placed because of postoperative complete heart block.