Surgical repair of Ebstein's anomaly.

Ebstein's anomaly is a complex malformation that has been treated by various surgical techniques, with variable results. We have developed a repair-by plication of the free wall of the atrialized portion of the right ventricle, posterior tricuspid annuloplasty, and right atrial reduction--that has been used since 1972 in 16 patients. The repair is based on the construction of a monocusp valve by the use of the anterior leaflet of the tricuspid valve, which is usually enlarged in this anomaly. There were 14 operative survivors and no instances of complete heart block. Two patients with refractory dysrhythmias due to the Wolff-Parkinson-White syndrome underwent successful intraoperative epicardial and endocardial mapping and surgical division of accessory pathways. This repair has resulted in improvement in the operative mortality and in gratifying early and late results in most patients.