Ebstein’s Anomaly

Ebstein’s anomalyEbstein’s anomaly is a rare congenital heart diseaseCongenital heart disease characterized by downward displacement and failure of delamination of the septal and posterior leaflets of the tricuspid valveTricuspid valve. There is a wide spectrum of anatomy regarding the degree of downward displacement, severity of tricuspid regurgitation, size of the atrialized and functional portion of the right ventricleVentricles, and presence of right ventricular outflow tract obstructionRight ventricular outflow tract obstruction. Physiology and clinical presentation vary substantially from neonatal presentation with profound low cardiac output, often in the context of circular shunting, to a stable biventricular circulation with minimal symptoms. Echocardiography is essential for diagnosis, and MRI is increasingly used in older patients. The natural history, indication for and type of surgical treatment strategy depend on the age and physiology at presentation. Neonatal management includes conservative medical management, ligation of the patent ductus arteriosus, or single ventricleSingle ventricle palliation in the form of right ventricular exclusion (Starnes ProcedureStarnes procedure) depending on the severity of the lesion. Tricuspid valveTricuspid valve repair (cone reconstructionCone reconstruction) in later childhood or adult life is the mainstay of management in stable cases—but exact indications and timing for surgery are still controversial. Mortality is highest in the neonatal period at approximately 25%, with or without surgical intervention. The Starnes procedureStarnes procedure and subsequent single ventricleSingle ventricle palliation yields 10-year survivalSurvival of approximately 80%. The cone reconstructionCone reconstruction, which allows 360 degrees of tricuspid valveTricuspid valve tissue in the anatomic, non-displaced tricuspid valveTricuspid valve annulus, has become the first line surgical technique in childhood and young adults (beyond the neonatal period) and is associated with favorable short and medium-term clinical outcomes. The cone reconstructionCone reconstruction can be done with very low operative mortality (< 2%) with 5-year freedom from tricuspid valveTricuspid valve reoperation of 90–95%.