A case of late presenting unicuspid aortic valve in a 62-year-old female.

We describe the case of a 62-year-old female presenting with new onset progressive dyspnea on exertion and a new diagnosis of aortic stenosis. Transesophageal echocardiography showed fusion of the aortic valve leaflets suspicious for bicuspid aortic valve with dilated ascending aorta. Surgical replacement of the valve revealed a unicuspid aortic valve with one true commissure at the level of the left and noncoronary cusps. The case is supported by clinical, echocardiographic and angiographic imaging and discussion of current imaging modalities used in diagnosis of aortic valve pathology.