Complete repair of Ebstein anomaly in neonates and young infants: a 16-year follow-up.

OBJECTIVE: The purpose of this study was to review the long-term outcome of patients with Ebstein anomaly who underwent complete repair as neonates and young infants.

METHODS: Between March 1994 and May 2010, 32 patients (23 neonates and 9 young infants) underwent surgery for Ebstein anomaly. Mean weight was 3.9 ± 2.0 kg (range, 1.9-8.6 kg). The Great Ormond Street Echocardiography score was greater than 1.5 in 22 of the 23 neonates and greater than 1.0 in all infants. All associated cardiac defects were repaired including pulmonary atresia in 15 and ventricular septal defect in 4. Primary outcome measures included (1) early and late survival, (2) freedom from reoperation, (3) durability of tricuspid valve repair, and (4) functional status.

RESULTS: Early survival was 78.1% (25/32). There was 1 late death. Fifteen-year survival estimate was 74% ± 8%. For patients with pulmonary atresia, early and late survival was 60% ± 12% (9/15) versus 94.1% (16/17) (P < .05), respectively; for those without pulmonary atresia, early and late survival was 60% ± 12% versus 85% ± 10 (P = .06), respectively. The mean follow-up was 5.9 ± 4.5 years (0.1-16 years). A biventricular repair was achieved in 90.6% (29/32) patients. Median preoperative tricuspid regurgitation was 4/4 and at late follow-up, 1/4. Freedom from reoperation at 15 years was 74% ± 10% for patients undergoing biventricular repair. All survivors are in New York Heart Association class I or II.

CONCLUSIONS: Biventricular repair of Ebstein anomaly in symptomatic neonates is feasible with good early and late survival, especially in those without pulmonary atresia. Tricuspid valve repair is durable, and functional status is excellent.