@Article{CHD.2020.011500,
AUTHOR = {Tânia Branco Mano, João Ferreira Reis, Ana Figueiredo Agapito, André Monteiro, Mário Oliveira, Luísa Moura Branco, José Fragata, Fátima Pinto, Rui Cruz Ferreira, Lídia de Sousa},
TITLE = {Long Term Follow-Up of Ebstein’s Anomaly—What to Expect in Adult Life?},
JOURNAL = {Structural and Congenital Heart Disease},
VOLUME = {15},
YEAR = {2020},
NUMBER = {3},
PAGES = {153--162},
URL = {http://www.techscience.com/schd/v15n3/39526},
ISSN = {3071-1738},
ABSTRACT = {<b>Introduction:</b> 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. <b>Methods: </b>
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. <b>Results:</b> 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 (<i>p</i> = 0.041) 
and severity of tricuspid regurgitation (<i>p</i> = 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 (<i>p</i> = 0.017) and 
arrhythmia diagnosis (<i>p</i> = 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. <b>Conclusion:</b> 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.},
DOI = {10.32604/CHD.2020.011500}
}