Functional status after operation for Ebstein anomaly: the Mayo Clinic experience

Area of Science:

• Cardiology
• Cardiac Surgery
• Congenital Heart Disease

Background:

• Ebstein anomaly is a spectrum of tricuspid valve and right ventricular dysplasia.
• Surgical intervention is often necessary to improve patient quality of life.

Purpose of the Study:

• To review the long-term functional outcomes of patients with Ebstein anomaly who underwent cardiac operations.
• To assess survival rates, reoperation status, and functional class post-surgery.

Main Methods:

• Retrospective review of 604 cardiac operations in 539 patients with Ebstein anomaly (1972-2006).
• Follow-up via medical questionnaires or telephone contact for surviving patients.
• Analysis of survival, reoperation-free survival, functional class, medication use, and pregnancy outcomes.

Main Results:

• Long-term survival at 5, 10, 15, and 20 years was 94%, 90%, 86%, and 76%, respectively.
• Survival free of late reoperation was 46% at 20 years; 83% of surveyed patients were in NYHA functional class I or II.
• Atrial fibrillation/flutter occurred in 36% of patients; 3.9% of liveborn children had congenital heart disease.

Conclusions:

• Cardiac surgery for Ebstein anomaly offers good long-term survival and functional outcomes.
• Atrial arrhythmias are a common complication, both pre- and post-operatively.
• Successful pregnancies are frequent, with a low risk of congenital heart disease recurrence in offspring.