Abstract
A unicuspid aortic valve (UAV) in adults is a very rare form of aortic valve (AV) malformation. UAV has two distinct subtypes, acommissural UAV and unicommissural, and can be differentiated by anatomical features, imaging modalities, and clinical presentation. With the development of significant AV lesion (s), surgical or transcatheter intervention will be required. The first part is a summarized review of UAV (anatomical features, clinical presentation, diagnostic modalities, and management). In the second part, we present a series of four patients diagnosed with UAV (3 unicommissural and 1 acommissural). The first case underwent balloon aortic valvuloplasty during childhood and surgical AV replacement later, with the progression to severe aortic stenosis (AS). The second case underwent a Ross procedure. The third and fourth cases were asymptomatic with moderate AS and mild-to-moderate AR and were kept on follow-up. In all the cases, transesophageal echocardiography confirmed the diagnosis of UAV with detailed morphological and functional assessment of AV.