Li, Zhuo

Objective To review the long-term functional outcomes of patients with Ebstein anomaly who had cardiac surgery at our institution. Methods From April 1, 1972 to January 1, 2006, 539 patients with Ebstein anomaly had 604 cardiac operations at Mayo Clinic Rochester. The 506 late survivors form the basis of this report. Patient records were reviewed and all patients were mailed a medical questionnaire or contacted by telephone. Patients who had associated complex congenital heart disease were excluded (pulmonary atresia, conotruncal abnormalities, and atrioventricular discordance and ventriculoarterial discordance). Results At the initial operation at our institution, the mean age was 24 years (range 8 days to 79 years) and 53% were female. Survival at 5, 10, 15, and 20 years was 94%, 90%, 86%, and 76%. Survival free of late reoperation was 86%, 74%, 62%, and 46% at 5, 10, 15, and 20 years respectively. Surveys were returned by 285 patients. Two hundred and thirty-seven (83%) of patients were in NYHA class I or II and 34% were taking no cardiac medication. Five patients (2%) reported having endocarditis, 1 patient (<1%) reported a stroke, and 103 patients (36%) had atrial fibrillation or flutter. There were a total of 202 pregnancies among 82 women. Recurrence of congenital heart disease was low. Only 9/232 (0.04%) of liveborn children had congenital heart disease. Conclusions Patients have good long term survival and functional outcomes, after operation for Ebstein anomaly. Atrial arrhythmias are relatively common both before and after operation. Pregnancy appears to be well tolerated with low recurrence risk of congenital heart disease.