Advancements in the Diagnosis and Surgical Management of Ebstein's Anomaly: A Comprehensive Review

Ebstein’s anomaly is a rare congenital heart defect characterized by the apical displacement of the septal and posterior leaflets of the tricuspid valve, resulting in atrialization of the right ventricle and significant hemodynamic disturbances. This anomaly often presents with a spectrum of clinical manifestations, ranging from asymptomatic cases to severe heart failure and cyanosis in neonatal life. The diagnostic process has been significantly enhanced by advancements in echocardiography, magnetic resonance imaging (MRI), and electrocardiographic techniques, enabling early detection and detailed assessment of structural abnormalities. Treatment strategies for Ebstein’s anomaly are tailored according to the severity of the condition and patient age. While mild cases may be managed conservatively, severe forms necessitate surgical interventions, including tricuspid valve repair or replacement, bidirectional Glenn shunt, and occasionally the Fontan procedure. Novel approaches, such as cone repair, have shown promising outcomes in improving valve function and overall cardiac performance. This review article provides a detailed exploration of the pathophysiology, clinical manifestations, diagnostic advancements, and therapeutic options for Ebstein’s anomaly, with an emphasis on surgical innovations.