A case of severe aortic stenosis caused by unicuspid unicommissural aortic valve.

Unicuspid aortic valve (UAV) is an extremely rare congenital malformation that frequently presents with valvular dysfunction or aortic aneurysm. Here we report the case of a 49-year-old man with severe aortic stenosis caused by UAV requiring the Bentall procedure. Two- and three-dimensional transesophageal echocardiography revealed an eccentric opening in an aortic valve and a lateral attachment to the aorta at the orifice level, suggestive of which is consistent with unicommissural UAV as confirmed by surgical findings. < We report a rare case of unicuspid aortic valve (UAV) requiring the Bentall procedure. This case demonstrates the utility of two- and three-dimensional transesophageal echocardiography in revealing the morphology of UAV, as confirmed by surgical findings.>.