Cone Repair Combined with Bidirectional Cavopulmonary Shunt for Ebstein’s Anomaly: A Single-Center Analysis

All articles of this category (opens in new window) Background: To analyze the outcomes of Cone repair for Ebstein’s anomaly with concomitant bidirectional cavopulmonary connection (BCPC). Methods: Retrospective single-center study of patients with Ebstein’s anomaly who underwent Cone repair between August 2012 and February 2025. Results: Sixty-four patients with a median age of 8 (IQR: 2–18) years underwent Cone repair. Of these, 34 patients (53.1%) with a median age of 12 (IQR: 5–29) years had Cone repair alone, while 30 patients (46.9%) with a median age of 5.5 (IQR: 1–11) years underwent Cone repair with BCPC. The 30-day mortality for the entire cohort was 3.1% (2/64), with both deaths occurring in the Cone-alone group ( p = 0.177). There was no significant difference between groups in hospital length of stay (8.5 [IQR: 7–13] vs. 8.5 [IQR: 7–10] days, p = 0.5) or ICU length of stay (2 [IQR: 2–3] vs. 3 [IQR: 1–3] days, p = 0.8). However, ventilation time was significantly shorter in the Cone and BCPC group (13 [IQR: 8–22] vs. 19.5 [IQR: 16–41] hours, p = 0.02). Independent predictors for BCPC addition included Carpentier classification type C and D (OR 6.1, 95% CI: 1.3–28.9, p = 0.005), preoperative cyanosis (OR 5.4, 95% CI: 1.1–27.8, p = 0.04), and severe tricuspid valve (TV) regurgitation (OR 7.3, 95% CI: 1.6–33.3, p = 0.01). At complete follow-up, overall survival was 96.9% (95% CI: 88.1–99.2%) at 5 years and beyond. Freedom from TV reintervention was 82.1% (95% CI: 66.3–91%) at 5 years and 70.3% (95% CI: 40.1–87%) at 9 years. No TV replacements were required. There were no significant differences in survival or reintervention rates between patients with and without BCPC. Conclusion: Cone repair demonstrates excellent survival and freedom from TV replacement regardless of BCPC addition. BCPC was associated with shorter ventilation time and was more frequently added in patients with Carpentier class C and D, and preoperative cyanosis. Publication History Article published online: 17 February 2026 © 2026. Thieme. All rights reserved. Georg Thieme Verlag KG Oswald-Hesse-Straße 50, 70469 Stuttgart, Germany