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ARTICLE
Long Term Follow-Up of Ebstein’s Anomaly—What to Expect in Adult Life?
1 Department of Cardiology, Hospital de Santa Marta, Centro Hospitalar Universitário de Lisboa Central, Lisbon, 1169-024, Portugal
2 Department of Cardiothoracic Surgery, Hospital de Santa Marta, Centro Hospitalar Universitário de Lisboa Central, Lisbon, 1169-024, Portugal
3 Department of Paediatric Cardiology, Hospital de Santa Marta, Centro Hospitalar Universitário de Lisboa Central, Lisbon, 1169-024, Portugal
* Corresponding Author: Tânia Branco Mano. Email:
Congenital Heart Disease 2020, 15(3), 153-162. https://doi.org/10.32604/CHD.2020.011500
Received 14 May 2020; Accepted 28 May 2020; Issue published 15 July 2020
Abstract
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.Keywords
Cite This Article
Mano, T. B., Reis, J. F., Agapito, A. F., Monteiro, A., Oliveira, M. et al. (2020). Long Term Follow-Up of Ebstein’s Anomaly—What to Expect in Adult Life?. Congenital Heart Disease, 15(3), 153–162.