Treatment Strategies in Ebstein's Anomaly: An Observational Study Over Three Decades.

OBJECTIVES

This study compared long-term outcomes of surgical management for Ebstein's anomaly before and after the introduction of an individualized surgical approach at our centre, including Cone repair.

METHODS

We conducted a retrospective, single-centre analysis of patients who underwent surgery for Ebstein's anomaly between 1988 and 2022. Since 2012, early intervention to preserve right ventricular (RV) function before severe deterioration was prioritized. Survival was characterized using Kaplan-Meier estimates, and restricted mean survival time (RMST) was computed up to a specified time horizon. Covariate-adjusted hazard ratios (HRs) were modelled using Cox proportional hazards regression with LASSO-based variable selection.

RESULTS

Out of 162 patients screened, 141 (median age: 14 years; IQR: 5-33 years) were included; 74 underwent surgery before 2012 and 67 after. Patients operated after 2012 were younger (median 7, IQR 1-14 vs 22, IQR 14-44 years; P < 0.001) and more frequently received Cone repair, either alone or combined with a bidirectional Glenn procedure (BDG). Five-year survival improved progressively over the analysed periods: from 81.7% (95% CI: 63.7%-91.3%) before January 2000, to 90% (95% CI: 75.4%-96.1%) between January 2000 and August 2012, reaching 98.5% (95% CI: 89.9%-99.8%) after August 2012. The survival difference before and after 2012 was significant (RMST-based log-rank test: P = 0.0094). Cox regression identified Carpentier classification above type B (HR: 6.19; 95% CI: 1.69-22.68; P = 0.006) and postoperative sepsis within 30 days (HR: 7.75; 95% CI: 2.09-28.75; P = 0.002) as risk factors for increased mortality. Cone repair itself was not associated with increased mortality (HR: 1.01; 95% CI: 0.11-9.57; P = 0.991), whereas BDG was associated with a lower hazard (HR: 0.11; 95% CI: 0.01-0.92; P = 0.041).

CONCLUSIONS

An individualized strategy that favoured earlier intervention, including Cone repair with or without BDG, was associated with improved survival. These findings highlight the importance of tailoring surgical strategies to anatomical and haemodynamic status in Ebstein's anomaly.