Surgical treatment of Ebstein's malformation: report of 108 cases

OBJECTIVE: To review retrospectively the experience of surgical treatment of Ebstein's malformation in 108 patients. METHODS: One hundred and eight patients with Ebsteins malformation underwent surgical correction, including tricuspid valve annuloplasty in 83 patients (73.4%) and tricuspid valve replacement in 30 (5 with redo-operation). Concomitant procedure included division of the abnormal accessory conduction pathway in 8 patients. RESULTS: The overall hospital mortality was 8.8%. The hospital mortality rate was 10.4% before 1990 and 5.5% after that time. There was a significant difference between the two periods (P < 0.05). The main causes of death were low cardiac output, arrhythmias and right heart failure. The mean follow-up time was 6.3 years. The heart function improved to be NYHA class I or II in 92% of the patients. CONCLUSIONS: Early surgical intervention is recommended for patients with Ebstein's malformation, especially for those with symptoms and cardiac enlargement. Tricuspid valve repair should be performed if anterior leaflet of the tricuspid valve well developed. However, tricuspid valve replacement is advocated if anterior leaflet is dysplastic or its origin displaced.