Exercise performance and right ventricular remodeling following the Cone repair in patients with different severity of Ebstein´s anomaly.

Background: While the Cone repair has shown good outcome regarding valve competence, its effect on exercise performance and right ventricular remodelling are unclear.

Methods: Between 01/2010 and 06/2021, 60 patients underwent a Cone repair at our centre. 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 [range: 5.6-68.2], 28 (61%) were female. Patients were divided based on indication: (A) patients in NYHA I-II without findings of severe/progressed disease (B), patients in NYHA I-II and findings of severe/progressed disease in MRI or Spiroergometry, and (C) patients in NYHA III-IV.

Results: Follow-up was 6.7±3.7years. Tricuspid regurgitation at last follow-up was none-mild in 40 patients, moderate in five, and severe in one patient, who underwent reoperation after 5.7years. NYHA class improved in 18 patients, stayed equal in 24 and worsened in four (p=0.002). CPET 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. RV-EDVI decreased from 166ml/m2 to 114ml/m2 (p<0.001), the reduction was smaller in group A (-13%) compared to group B and C (-23% and -53%). Antegrade stroke-volume increased in every group (preoperative: 55ml, postoperative: 72ml, p=0.005).

Conclusions: The Cone repair results in a substantial and sustained decrease in tricuspid valve regurgitation. While objective exercise capacity did not improve, NYHA class improvement, right ventricle size decreased and antegrade stroke volume increased.