Unicuspid Aortic Valve: A Review of Literature

Abstract

Unicuspid aortic valve (UAV) is a rare congenital heart disease that leads to aortic valve dysfunction, most commonly stenosis, but also regurgitation. It typically presents in the third to fourth decade of life. Because of its rarity, UAV is less well-characterized than other congenital valve disorders, such as the bicuspid aortic valve (BAV). Furthermore, its similarities to BAV often lead to misdiagnosis, making accurate differentiation critical for management and prognosis. This article reviews the literature on this rare condition regarding its epidemiology, embryology, genetics, clinical presentation, diagnosis, and management.