Ebstein malformation of the tricuspid valve: current concepts in management and outcomes

Ebstein anomaly is a myopathy of the right ventricle that results in variable degrees of failure of delamination of the tricuspid valve leaflets from the underlying endocardium, leading to severe tricuspid valve regurgitation and some degree of right ventricular dysfunction. In neonates or infants who remain in congestive heart failure or profoundly cyanotic while receiving appropriate medical therapy, operation is required. Current strategies include biventricular or single-ventricle repair. In children and adults, medical management may be used, but most patients eventually require surgery. Tricuspid valve repair is preferred; we believe the cone repair is the most anatomic repair and is the operation of choice. Tricuspid valve replacement may be necessary in cases in which the valve is not repairable. A bidirectional cavopulmonary shunt is useful in patients with severe right ventricular dilatation and/or dysfunction. Transplantation rarely is necessary.