Early and Mid-term Results for Repair of Ebstein's Anomaly

Lee, Cheul; Lee, Chang Ha; Hwang, Seong Wook; Lim, Hong Gook; Kim, Woong Han; Kim, Chong Whan

Abstract

BACKGROUND: We evaluated the early and mid-term results for repair of Ebstein's anomaly. MATERIAL AND METHOD: Between January 1989 and June 2004, 29 patients underwent repair of Ebstein's anomaly. The median age was 11.4 years (4 days-50 years). Tricuspid insufficiency equal to or greater than grade 3 was present in 21 (72.4%) patients. Surgical techniques included tricuspid valve repair with vertical plication of the atrialized ventricle (n=14), Carpentier's technique (n=7), tricuspid valve replacement (n=4), systemic-to-pulmonary arterial shunt (n=2), tricuspid valve repair (n=1), and Fontan operation (n=1). Bi-directional cavopulmonary shunt (BCPS) was required in 5 patients. Among the 2 neonates, one patient underwent successful biventricular repair, and the other patient underwent systemic-to-pulmonary arterial shunt. Follow-up was possible in 21 patients (75%), and the average follow-up was 37.6 months (3 months~11.3 years). RESULT: There were 1(3.4%) early and 1 late deaths. Reoperation was required in 4 patients. Two patients underwent tricuspid valve re-replacement, and the other 2 tricuspid valve repair. At recent follow-up, only 2 patients showed tricuspid insufficiency equal to or greater than grade 3, and most patients showed clinical improvement. Excluding the patients who underwent tricuspid valve replacement, the actuarial rate of freedom from reoperation at 1 and 5 years were 94.7% and 79.0%, respectively.
CONCLUSION
Tricuspid valve repair was possible in most patients with good mid-term outcome. Most patients showed clinical and hemodynamic improvement. Indications for the BCPS should be clarified.