Long Term Follow-Up of Ebstein’s Anomaly—What to Expect in Adult Life?

Introduction: Due to the low prevalence and wide variation of severity of Ebstein’s Anomaly (EA), long-term follow-up data are scarce. The aim was to evaluate the long-term outcome of an adult population with EA. Methods: Retrospective analysis of EA adults followed in the past 42 years in a tertiary congenital heart disease outpatient clinic. Predictors of complications and mortality were assessed. Results: We studied 53 patients: 53% females, mean age 46 ± 19 years, 36% cyanotic, 55% diagnosed at adult age. Tricuspid regurgitation was moderate or severe in 33% and 46%, respectively, and during follow-up (mean 12 ± 10 years) 11 patients had right ventricular dysfunction. We found an association between New York Heart Association class and cyanosis (p = 0.041) and severity of tricuspid regurgitation (p = 0.02). The most frequent symptom was palpitations (57%), with 29 patients exhibiting rhythm disturbances (62% supraventricular tachycardia). Thromboembolic events were found in 23% and were associated with atrial septal defect or patent foramen ovale (p = 0.017) and arrhythmia diagnosis (p = 0.011). Nine patients required tricuspid valve surgery and two underwent cardiac transplantation. In 25 pregnancies, 48% developed fetal complications. Total of 14 deaths (2.4 deaths per 10 patients-years) occurring at a mean age of 49 ± 18 years, of cardiac cause in more than half of the cases and 29% of sudden death. No significant differences were found in the mortality rate of patients presenting with severe tricuspid regurgitation, with or without surgical management. Conclusion: Ebstein’s Anomaly is often diagnosed in adulthood. It is accompanied by high morbidity, especially arrhythmias, and non-negligible mortality with relevance in assessing the risk of sudden death.