ArticleInternational journal of surgery (London, England)2026
Timely surgical intervention and risk stratification in patients with Ebstein anomaly: a 20-year retrospective cohort study.
Article in International journal of surgery (London, England), 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
backgroundThe 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
methodsWe 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.
resultsThe 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).
conclusionTimely 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.
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