Improvements in exercise performance after surgery for Ebstein anomaly

Objective: The purpose of this longitudinal study was to assess improvements in exercise performance and quality of life in patients with Ebstein anomaly after surgical intervention. Methods: In 21 patients with Ebstein anomaly (between 6 and 59 years of age; 16 female, 5 male) who underwent surgery for tricuspid regurgitation and, if present, closure of an interatrial shunt, a cardiopulmonary exercise test and a quality-of-life assessment by the Medical Outcomes Study 36 item short form was performed prior to and 6 to 18 months after surgery. Results: After surgery, peak oxygen uptake increased from 68.4% of predicted to 77.3% of predicted (P = .009), and ventilatory efficiency (Ve/Vco₂ slope) improved from 32.5 to 29.3 (P = .001). In 14 patients with additional interatrial shunt closure, oxygen saturation improved from 95% to 99% at rest (P = .003) and from 88% to 99% under peak exercise (P = .003). Improvements in Ve/Vco₂ slope were similar in patients who had undergone primary surgery (P = .005) or reoperation (P = .018). Increase in exercise capacity was also similar in both groups but failed significance in both (primary surgery, P = .064; reoperation, P = .063). There was no difference between tricuspid valve repair and replacement in the short-term follow-up. Self-estimated quality of life was fairly good prior to and after surgery. Only in the single question about health transition at follow-up did the patients confirm an improved situation after surgery. Conclusions: Patients with Ebstein anomaly and severe tricuspid regurgitation draw clinical benefit from surgical intervention as measured on exercise testing. This holds true for primary surgery and for reoperation.