Bicuspid and Unicuspid Aortic Valves: Development, Genetics, and Lifelong Management

Bicuspid and unicuspid aortic valves represent the most common congenital aortic valve malformations and pose unique challenges in clinical management across the lifespan. These anomalies are associated with progressive valvular dysfunction and aortopathy, often necessitating early intervention. Multiple publications have described life-long management of aortic stenosis (AS), affecting tricuspid valve. This review outlines the embryologic basis, natural history, and clinical spectrum of uni- and bicuspid aortic valve, highlighting diagnostic strategies, surveillance protocols, and surgical - transcatheter interventions. Emphasis is placed on longitudinal care, including transition from pediatric to adult congenital cardiology, multimodality imaging, and timing of surgical or transcatheter interventions. In conclusion, the article aims to provide a framework for evidence-informed, individualized management of these complex valvulo-aortic disorders.