Tricuspid valve repair for Ebstein's anomaly in young children: a 30-year experience.

BACKGROUND: The purpose of this study was to examine early and late outcome of tricuspid valve repair for Ebstein's anomaly in young children.

METHODS: Between October 1974 and November 2003, 52 children (25 boys) underwent tricuspid valve repair and annuloplasty for Ebstein's anomaly. Mean age was 7.1 +/- 3.9 years (range, 5 months to 12 years). Concomitant procedures included atrial septal defect closure (n = 46), division of accessory conduction pathways (n = 4), ventricular septal defect closure (n = 3), and other (n = 7).

RESULTS: Early mortality was 5.8% (3 of 52 patients; no mortality since 1984, n = 31). Risk factors were age younger than 2.5 years (p = 0.03) and weight less than 10.7 kg (p = 0.03). Morbidity included transient atrial (n = 11) and ventricular arrhythmias (n = 5), and early reoperation in 3 patients. There was no need for a permanent pacemaker. Mean follow-up was 12.2 +/- 7.4 years (maximum, 24.3 years). Actuarial survival at 5, 10, and 15 years was 92.3% +/- 3.7%, 89.9% +/- 4.3%, and 89.9% +/- 4.3%, respectively. Freedom from all reoperations at 5, 10, and 15 years was 91.0% +/- 4.3%, 76.9% +/- 6.8%, and 61.4% +/- 8.8%, respectively. Moderate (grade II) or more tricuspid regurgitation on dismissal echocardiogram was the only risk factor for reoperation (p = 0.04). Tricuspid stenosis did not occur in any patient. At late follow-up, 89% of patients were in New York Heart Association class I or II.

CONCLUSIONS: Ebstein's anomaly in young children can now be repaired with low mortality and good tricuspid valve durability. Tricuspid regurgitation at the completion of operation should be mild or less to minimize need for reoperation. Tricuspid valve repair and annuloplasty did not result in stenosis despite somatic growth. Most patients enjoy an excellent quality of life.