Operative treatment of Ebstein's anomaly.

From April 1972 to February 12, 1991, 189 patients with Ebstein's anomaly underwent repair. Ages ranged from 11 months to 64 years (median 16 years, mean 19.1 years). In 58.2%, tricuspid valve reconstruction was possible, and in 36.5%, a prosthetic valve, usually a bioprosthesis, was inserted. In 5.3%, a modified Fontan or other procedure was performed. There were 12 hospital deaths (6.3%). All 28 patients who had accessory conduction pathways (Wolff-Parkinson-White syndrome) underwent successful ablation of the pathways as part of the operative treatment. Follow-up was obtained in 151 (85.3%) patients. Of those patients followed up more than 1 year after operation, 92.9% were in New York Heart Association class I or II. There were 10 late deaths: seven cardiac (four sudden), two noncardiac, and one of an unknown cause. Postoperative Doppler echocardiographic assessment showed the atrial septum was intact in all patients and tricuspid valve function was good to excellent in most patients. Four of the 110 patients (3.6%) who underwent valve reconstruction required reoperation 1.4 to 14.1 years later. Postoperative reduction in heart size was usual, atrial arrhythmias were reduced, and late postoperative exercise testing showed a significant improvement in performance: Maximal oxygen consumption increased from a mean of 47% of predicted value before the operation to a mean of 72% after the operation. Nine patients had a total of 12 successful pregnancies with deliveries of normal children.