Exercise performance in Ebstein's anomaly in the course of time - Deterioration in native patients and preserved function after tricuspid valve surgery

Objective The purpose of this study was to assess the longitudinal follow-up of exercise performance in patients with native Ebstein anomaly and patients who had received previous tricuspid valve surgery. Patients and methods We identified 59 patients with Ebstein anomaly who had performed at least two cardiopulmonary exercise tests within a minimum of 6 months. 22 patients (15 female, 29.0 ± 13.4 years) with native conditions, 37 patients (21 female, 32.1 ± 15.3 years) had undergone surgery of their tricuspid valve before baseline testing. Results In the non-operated group peak workload (2.58 ± 0.7 to 2.38 ± 0.7 W/kg, p =.019) declined during a mean follow-up of 4.6 ± 2.6 years, whereas peak workload remained stable in the surgical group (2.02 ± 0.6 to 2.06 ± 0.7 W/kg, p =.229) throughout the follow-up of 3.4 ± 2.3 years. In the 18 patients who had their surgery less than one year prior to the first CPET the exercise performance even increased during the follow-up. Peak blood pressure as a marker of cardiac adaption to exercise increased in the follow-up examination only in the surgical group (159 ± 23 vs. 171 ± 30 mm Hg, p =.007). In the non-operated group blood pressure remained unchanged at follow-up (161 ± 26 vs. 166 ± 29, p =.358). Conclusion Exercise performance deteriorates in non-operated patients with Ebstein anomaly in contrast to patients after tricuspid valve surgery where exercise performance remains stable. These facts promote a surgical intervention latest at the time when exercise performance decreases.