A Rare Case of Unicuspid Aortic Valve with Postoperative Heart Block

Unicuspid aortic valve (UAV) is an extremely rare cause of aortic stenosis, accounting for about 0.02% of the adult population. We present a rare case of UAV in a young woman who presented with dyspnea on exertion. She underwent extensive work-up, including a transthoracic echocardiogram, which was notable for critical aortic stenosis with significant pulmonary hypertension. A subsequent transesophageal echocardiogram confirmed the severe degree of aortic stenosis and revealed the possibility of a UAV. She was referred to a cardiothoracic surgeon and underwent bioprosthetic aortic valve replacement. Intraoperative evaluation confirmed the rare occurrence of a UAV. Postoperative course was complicated by complete heart block necessitating pacemaker placement.