Contemporary outcomes and mortality risks of Ebstein anomaly: A single‐center experience in Thailand

Background: The increasing number of patients with Ebstein anomaly (EA) survivinginto adulthood implies improvements in the treatments for the complex lesion. Werevisited the clinical outcomes of patients with EA to demonstrate their “real world”survival.
Objectives: To identify the survival and predictors of mortality in patients with EAwho underwent medical or surgical management in the present era.
Methods: All patients who had EA with atrioventricular concordance between 1994and 2016 were retrospectively reviewed. Baseline characteristics, initial echocardio‐graphic findings, treatments, and outcomes were explored. The survival analysis wasperformed at the end of 2017. A multivariate analysis was used to assess mortalityrisks.
Results: A total of 153 patients (25.4 ± 20.4 years, 60% female) were analyzed. Ofthese, 89 patients had been diagnosed with EA in childhood. During the follow‐up[median time of 5.2 years (3 days‐23.5 years)], 32 patients (20.9%) died due to majorcardiac adverse events. The overall survival at 1, 5, and 10 years were 89%, 82.2%,and 79%, respectively. Of the total 153 patients, 64 patients underwent at least onesurgical intervention [median age of 17 years (1 day‐64.4 years)]. The survival at 1, 5,and 10 years were 87.5%, 82.4%, and 77.7%, respectively, in patients with EA sur‐gery. This survival is comparable to the survival of 89 nonoperated patients with EA:89.9%, 87.5%, and 81.8%, at 1, 5, and 10 years, respectively. The significant predic‐tors of mortality were: age at diagnosis ≤2 years, tricuspid valve (TV) z‐score >3.80,TV displacement >19.5 mm/m2, presence of severe tricuspid regurgitation, and ab‐sence of forward flow across the pulmonic valve at the initial diagnosis.
Conclusion: Patients with EA had a moderately good survival in this era. In this paper,we report five simple predictors of death in this patient population.