Abstract
ObjectiveThis study aimed to evaluate Ebstein’s anomaly surgical correction and its early and long-term outcomes.MethodsA retrospective analysis of 62 consecutive patients who underwent surgical repair of Ebstein’s anomaly in our institution from January 2000 to July 2016. The following long-term outcomes were evaluated: survival, reoperations, tricuspid regurgitation, and postoperative right ventricular dysfunction.ResultsValve repair was performed in 46 (74.2%) patients - 12 of them using the Da Silva cone reconstruction; tricuspid valve replacement was performed in 11 (17.7%) patients; univentricular palliation in one (1.6%) patient; and the one and a half ventricle repair in four (6.5%) patients. The patients’ mean age at the time of surgery was 20.5±14.9 years, and 46.8% of them were male. The mean follow-up time was 8.8±6 years. The 30-day mortality rate was 8.06% and the one and 10-year survival rates were 91.9% both. Eleven (17.7%) of the 62 patients required late reoperation due to tricuspid regurgitation, in an average time of 7.1±4.9 years after the first procedure.ConclusionIn our experience, the long-term results of the surgical treatment of Ebstein's anomaly demonstrate an acceptable survival rate and a low incidence of reinterventions.
Highlights
In 1866, Wilhelm Ebstein described the anatomical findings related to the heart of a 19-year-old man with cyanosis, palpitations, and dyspnea
Valve repair was performed in 46 (74.2%) patients – 12 of them using the Da Silva cone reconstruction; tricuspid valve replacement was performed in 11 (17.7%) patients; univentricular palliation in one (1.6%) patient; and the one and a half ventricle repair in four (6.5%) patients
Eleven (17.7%) of the 62 patients required late reoperation due to tricuspid regurgitation, in an average time of 7.1±4.9 years after the first procedure
Summary
In 1866, Wilhelm Ebstein described the anatomical findings related to the heart of a 19-year-old man with cyanosis, palpitations, and dyspnea. The postmortem findings were tricuspid valve (TV) anomaly with dilation of the right ventricle (RV) and patent foramen ovale. Ebstein’s anomaly (EA) is a primary disorder of TV and accounts for approximately 1% of congenital heart defects[1,2]. The EA is characterized by various degrees of adherence and displacement of the septal and posterior leaflet into the RV, resulting in a rotational and apical displacement of TV, an. This study was carried out at Instituto do Coração do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (InCor-HCFMUSP), São Paulo, SP, Brazil.
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