ML Brown, JA Dearani, DJ Driscoll, F Cetta, HM Connolly, CA Warnes, GK Danielson
Objective: Our objective was to review the long-term outcomes of patients with Ebstein’s anomaly who had cardiac surgery at our institution.Methods: Patient records were reviewed and all patients were mailed a survey or contacted by telephone. Patients who had pulmonary atresia with an intact ventricular septum, complex conotruncal abnormalities, and atrioventricular discordance with ventriculoarterial discordance were excluded.
Results: From 1963 to 2005, 539 patients with Ebstein anomaly had 603 cardiac operations. The mean age of the initial surgery at our institution was 24.6 years (range 2 days to 79 years). Three hundred and eighteen of the patients were female. Two hundred and nine patients (35%) had a previous cardiac surgical procedure. There were 182 tricuspid valve repairs (TVrep) and 401 tricuspid valve replacements (TVR) (48 mechanical valves and 353 bioprostheses). Only 4 patients had modified Fontan procedures, 11 patients had 1.5 ventricle repairs, and 4 patients had cardiac transplantation. At late follow-up, of those with initial TVrep, 1 patient had later re-repair and 32 patients had TVR at our institution. Twenty patients who had initial TVR had a second TVR. The 30 day mortality was 5.6% for the entire cohort (3.2% after 1999). Survival was 84% (95% CI 81-88%) at 10 years and 70.7% (CI 64-78%) at 20 years. For patients <18 years of age, 10 year survival was 92% (CI 86-98%) after TVrep and 82% (CI 76-89%) after TVR (p<0.01). For patients ≥18 years of age, there was no statistically significant difference in 10 year survival with either TVrep or TVR. Patients rated their health as excellent or good (NYHA Class I or II) in 84% of surveys returned.
Conclusion: Ebstein anomaly can be surgically treated with low perioperative mortality. Both tricuspid valve repair and replacement are associated with good long term survival.
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