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

Abstract

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. <Learning objective: 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.>

One-leaf clover: a rare case of a unicuspid unicommissural aortic valve

“… 1 A UAV is an extremely rare type of malformation with an estimated annual prevalence of 0.02%. 2 It is often seen in adulthood, especially during the third to fifth decades of life, with presenting symptoms of severe aortic stenosis or aortic regurgitation. 1 , 3 We report a case of a 30-year-old male who presented with generalized fatigue, worsening dyspnoea on exertion, and palpitations who was found to have a unicuspid unicommissural aortic valve.…”

“…Unicuspid aortic valves can be divided into two distinct subtypes: acommissural and unicommissural. 2 Subtypes of UAV differ structurally and in time of symptomatic onset. An acommissural UAV is defined by a single leaflet, no commissure, and small central opening.…”