Ebstein’s anomaly is a congenital heart disease that results from failure of delamination of the tricuspid valve with resulting apical displacement of the septal and posterior leaflets of the tricuspid valve. Age at presentation can vary greatly but neonatal presentation is associated with extraordinary high mortality rates. Comprehensive multispecialty care is required starting at the time of fetal diagnosis. Fetal echocardiography is vital in monitoring progression of the disease in utero. Fetal echocardiogram can evaluate for complications such as arrhythmias, pericardial effusion, or fetal hydrops. Post-natal evaluation should include evaluation of functional pulmonary atresia or circular shunt. Despite advances in surgical technique for Ebstein’s anomaly, mortality for it remains high with early surgical intervention. Aggressive medical management should be used to support patients with Ebstein’s anomaly during the neonatal period. Surgical procedures for neonatal Ebstein’s vary widely from systemic to pulmonary shunts with or without tricuspid valve closure to tricuspid valve repair.
Part of the book: Congenital Anomalies