Ebstein's anomaly: Indications for repair and surgical technique

Ebstein's anomaly of the tricuspid valve is characterized by a regurgitant tricuspid valve with a large anterior leaflet and septal and tricuspid leaflets displaced into the right ventricle. Associated anomalies are common, especially atrial septal communication and right ventricular outflow tract obstruction. Clinical presentation includes a spectrum from minimal hemodynamic derangement and mild exercise intolerance to severe cardiopulmonary abnormalities and grave illness. The natural history of the disease is extremely variable; the worst prognosis is in the youngest patients, who often have associated cardiac abnormalities. Operative procedures range from ligation of a patent ductus arteriosus to cardiac transplantation. Tricuspid valvuloplasty has been advanced by several investigators and can be considered in nearly all cases. Repairs are based on the creation of a monocuspid or bicuspid valve with reduction of the enlarged annulus. While tricuspid valve repair or replacement is indicated for symptomatic children and adults, the management of the symptomatic neonate is difficult. Patients are stratified according to clinical status and morphology, then treated with observation, valve repair, conversion to single ventricle, or transplantation. We review the anatomy and pathophysiology of Ebstein's anomaly, the indications for surgery, and the appropriate timing of surgical intervention. Current techniques of tricuspid valvuloplasty are described and unresolved issues are discussed, including the role for superior cavopulmonary anastomosis, the management of symptomatic infants, and the necessity of ventricular plication in valve repair. Copyright 1999 by W.B. Saunders Company