Beyond the label: Long-term outcomes after surgical management of Ebstein anomaly

Abstract

Ebstein anomaly is a rare congenital malformation of the tricuspid valve and right ventricle characterized by marked heterogeneity in anatomy, physiology, and clinical presentation. This broad phenotypic spectrum strongly influences the natural history of the disease, the timing and type of intervention, and ultimately long-term outcomes. Patients range from critically ill neonates with severe hemodynamic compromise and circular shunts to asymptomatic children and adults with progressive tricuspid regurgitation, right ventricular dysfunction, or arrhythmias, as well as survivors of previous surgical interventions. Accordingly, long-term outcomes are best understood within the context of the presenting phenotype and its associated management pathway rather than as a single aggregate measure of survival. Contemporary management therefore requires phenotype-specific decision-making. This review summarizes long-term outcomes across the major clinical and therapeutic subgroups, including critically ill neonates managed with the Starnes procedure, stable neonates and infants undergoing biventricular repair, patients treated with historical repair techniques, contemporary cone reconstruction, and tricuspid valve replacement. Particular emphasis is placed on transplant-free survival, valve durability, ventricular remodeling, arrhythmia burden, functional status, quality of life, and the need for reintervention.