Improvements in Exercise Performance After Surgery for Ebstein Anomaly

Study Questions:

Do exercise performance and quality-of-life measures improve following surgical intervention for Ebstein anomaly?


This was a prospective study of 21 patients with Ebstein anomaly undergoing primary (n = 14) or reoperation (n = 7) for moderate to severe tricuspid regurgitation and, if present, closure of an interatrial shunt. Patients were assessed preoperatively and 6-18 months after surgery with a cardiopulmonary exercise test and quality-of-life assessment using the Medical Outcomes Study 36-item short form (SF-36).


After surgery, peak oxygen uptake increased significantly (68.4-77.3% predicted, p = 0.009) and ventilator efficiency (VE/VCO2) slope improved (32.5-29.3, p = 0.001). In the 14 primary operation patients, rest and peak exercise saturations significantly improved (95-99%, p = 0.003 and 88-99%, p = 0.005, respectively) due to closure of interatrial shunts. At baseline, patients overall reported good quality of life on the SF-36. The only question that demonstrated improvement was regarding health transition at follow-up.


The authors concluded that patients with Ebstein anomaly and moderate to severe tricuspid regurgitation demonstrate improved exercise tolerance following surgical intervention, with minimal change in already quite good baseline quality-of-life measures.


The best time to intervene on patients with Ebstein anomaly has not been well established. It is difficult to clearly define symptoms as well as decline in symptoms. Often, these patients require tricuspid valve replacement rather than repair, which leads many to wait as long as possible before intervening. This study demonstrates significant improvement in exercise capacity in Ebstein patients, even with relatively preserved preoperative function and quality of life. Therefore, earlier intervention prior to the progression of right ventricular dysfunction may be a more appropriate surgical management strategy for this diverse and challenging patient population.

Clinical Topics: Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Heart Failure and Cardiomyopathies, Invasive Cardiovascular Angiography and Intervention, Cardiac Surgery and CHD and Pediatrics, Cardiac Surgery and Heart Failure, Congenital Heart Disease, CHD and Pediatrics and Interventions, CHD and Pediatrics and Quality Improvement, Interventions and Structural Heart Disease

Keywords: Exercise Tolerance, Rest, Outcome Assessment (Health Care), Health Transition, Follow-Up Studies, Quality of Life, Tricuspid Valve Insufficiency, Ebstein Anomaly, Cardiac Surgical Procedures, Ventricular Dysfunction, Right

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