Exercise Performance and Right Ventricular Remodeling After the Cone Repair in Patients With Different Severity of Ebstein Anomaly.

Abstract

Although the cone repair has shown good outcome regarding valve competence, its effect on exercise performance and right ventricular remodeling are unclear. Between January 2010 and June 2021, 60 patients underwent a cone repair at our center. We excluded patients with previous operation (n = 6), age <5 years (n = 3), in-hospital death (n = 2), replacement before discharge (n = 1), and missing follow-up (n = 2), resulting in a study population of 46 patients. Median age was 27 years (interquartile range, 5.6-68.2 years), and 28 (61%) were female. Patients were divided based on indication: Group A, patients in New York Heart Association (NYHA) Functional Classification I or II without findings of severe/progressed disease; group B, patients in NYHA I or II and findings of severe/progressed disease on magnetic resonance imaging or spiroergometry; and group C, patients in NYHA III or IV. Follow-up was 6.7 (SD, 3.7) years. Tricuspid regurgitation at the last follow-up was none-mild in 40 patients, moderate in 5, and severe in 1 patient, who underwent reoperation after 5.7 years. NYHA class improved in 18 patients, stayed equal in 24, and worsened in 4 (P = .002). Results of cardiopulmonary exercise testing showed no difference between preoperative and postoperative exercise capacity (preoperative: 72% of predicted vs postoperative: 71%, P = 1.0). Exercise-capacity improved only in group C. Indexed right ventricular-end-diastolic volume decreased from 166 mL/m² to 114 mL/m² (P < .001), and the reduction was smaller in group A (-13%) compared with groups B (-23%) and C (-53%). Antegrade stroke volume increased in every group (preoperative: 55 mL, postoperative: 72 mL; P = .005). The cone repair results in a substantial and sustained decrease in tricuspid valve regurgitation. Although objective exercise capacity did not improve, NYHA class improved, right ventricle size decreased, and antegrade stroke volume increased.