Timely surgical intervention and risk stratification in patients with Ebstein anomaly: a 20-year retrospective cohort study

BACKGROUND: The optimal timing and choice of surgery for Ebstein anomaly (EA), a complex congenital heart defect, remain challenging due to the heterogeneity and lack of robust long-term data on EA. In this study, we aimed to evaluate the long-term outcomes of surgical management in patients with EA, identify the prognostic risk factors, and develop a predictive model. MATERIALS AND METHODS: We conducted a retrospective analysis of data from 332 patients with EA who were treated at a tertiary center between January 2000 and December 2021. Among them, 269 underwent surgery: tricuspid valve repairs, 150; replacements, 77; and isolated bidirectional Glenn procedures, 42. Additionally, 70 patients received a concomitant Glenn shunt during valve surgery, resulting in a total of 112 Glenn procedures. The median follow-up was 10.12 years. The primary outcomes were freedom from reoperation and medical interventions. A predictive nomogram was developed using least absolute shrinkage and selection operator regression and internally validated. RESULTS: The early surgical mortality rate was 2.60%. Postoperative complications occurred in 15.24% of the patients, with renal failure (4.83%) and arrhythmias (2.23%) being the most common. During a median follow-up of 10.12 years, the freedom from operation rates were 97.95, 92.48, 87.04, and 83.22% at 5, 10, 15, and 20 years, respectively. However, freedom from medical intervention showed a progressive decline (94.34% at 5 years vs. 62.31% at 20). Multivariable Cox regression analysis identified preoperative hepatic congestion [hazard ratio (HR) = 3.042], Wolff-Parkinson-White (WPW) syndrome (HR = 3.463), and elevated alanine aminotransferase (ALT) level (HR = 1.023) as independent risk factors for surgery. The concomitant bidirectional Glenn procedure was associated with a significantly reduced risk of both reoperation (HR = 0.160) and medical intervention (HR = 0.259). Patients requiring interventions showed significantly worse physical and emotional quality-of-life scores than did those who were event-free (P < 0.05). CONCLUSION: Timely surgical intervention guided by preoperative risk stratification optimizes the long-term outcomes of EA. The proposed nomogram was a practical tool for individualized risk assessment, supporting clinical decision making in patients with this complex condition.