Ebstein’s Anomaly: Contemporary Medical and Surgical Management Strategies

Ebstein’s anomaly is an uncommon congenital defect of the tricuspid valve, marked by the apical displacement of the septal and posterior leaflets, leading to the “atrialization” of the right ventricle and subsequent dysfunction on the right side. Clinical manifestations vary from asymptomatic adults to neonates exhibiting severe cyanosis and heart failure. Advancements in diagnostic imaging, therapeutic interventions, and surgical techniques have significantly enhanced the prognosis for individuals afflicted by this historically serious condition. This review provides an in-depth examination of Ebstein’s anomaly, discussing its embryological origins, underlying pathophysiology, clinical manifestations, diagnostic assessment, medical treatment protocols, and surgical interventions. Particular emphasis is placed on contemporary surgical options, including cone reconstruction, the Starnes procedure, one-and-a-half ventricle repair, and valve replacement.