Surgical repair of Ebstein's anomaly: selection of patients and early and late operative results.

Between April 1972 and August 1984, 72 patients with Ebstein's malformation underwent surgery at a Mayo Clinic-affiliated hospital. In 58 (81%), plastic reconstruction of the tricuspid valve in which the anterior leaflet was used as a monocusp valve was possible. Eleven patients (15%) required a prosthetic valve (Hancock in 10; Starr-Edwards in one), and in three patients who had received a previous Glenn anastomosis, conversion to a Fontan type of arrangement was carried out. Age at operation ranged from 11 months to 64 years. There were five hospital deaths (6.9%) and three late, presumably arrhythmic, deaths. Follow-up of the initial 39 surgical survivors at least 2 years after the operation (mean, 5 years) revealed that 87% were in functional NYHA class I or II, a marked improvement over preoperative status. Surgical intervention is now recommended for all Ebstein's patients whose symptoms have deteriorated to NYHA class III or further. In addition, elective operation is recommended for less symptomatic patients in whom the cardiothoracic ratio has reached 0.65 or who are significantly cyanotic and polycythemic secondary to right-to-left shunting through an associated atrial septal defect.