Ebstein's anomaly in children: A single-center study in Angola

Introduction and ObjectiveEbstein's anomaly is a rare complex congenital heart defect of the tricuspid valve. We aimed to describe the frequency, clinical profile, and early and short-term post-operative results in patients under the age of 18 years operated for this anomaly in a tertiary center in Angola.MethodsA retrospective cross-sectional study was conducted over a period of 37 months. We analyzed all patients diagnosed with congenital heart defects.ResultsOf the 1362 patients studied, eight (0.6%) had Ebstein's anomaly; six patients (75%) were female. Mean age was 69±59 months. Five patients were in NYHA functional class III or IV. Mean cardiothoracic index was 0.72. Seven patients (87.5%) had severe tricuspid regurgitation and five (62.5%) had another associated congenital heart defect. All patients were operated: two had complications and one (12.5%) died in the early post-operative period. The mean follow-up time was 1.22±0.6 years, and mortality during follow-up was 12.5% (n=1). At the end of the study, of the five patients in whom cone reconstruction was performed, four (80%) were in functional class I. Mean cardiothoracic index decreased to 0.64. Three patients had mild and two had moderate tricuspid regurgitation. The patient who underwent cone reconstruction and a Glenn procedure was in functional class I.ConclusionThe frequency of Ebstein's anomaly was similar to that in other centers. Cone reconstruction was viable in the majority of patients, with good early and short-term results.

ResumoIntrodução e ObjectivosA anomalia de Ebstein é uma cardiopatia congénita complexa e rara da válvula tricúspide. Descrever a frequência, o perfil clínico e os resultados pós operatórios imediatos desta anomalia em crianças e adolescentes.MétodosEstudo transversal retrospetivo no único centro com tratamento integrado em cardiopatias congénitas em recém nascidos, crianças e adolescentes (<18 anos de idade) em Angola, num período de 37 meses. Foram analisados todos os doentes com diagnóstico de cardiopatia congénita. O diagnóstico foi feito com base no exame clínico e dados da ecocardiografia transtorácica e Doppler. Os ecocardiogramas foram realizados pelos Cardiologistas Pediátricos numa máquina Vivid 7 G.E.ResultadosForam analisados 1362 ecocardiogramas sequenciais. Oito doentes (0,6%) tinham anomalia de Ebstein, destes 6 (75%) eram do género feminino. As idades variaram entre 4 a 168 meses, a média foi de 69 ± 59 meses. Cinco estavam em classe funcional III ou IV da NYHA. A média do índice cardiotorácico foi de 0,72. Sete doentes (87,5%) tinham regurgitação tricúspide grave e 5 (62,5%) tinham outra cardiopatia congénita associada. Todos os doentes foram submetidos a intervenção cirúrgica. Dois tiveram complicações no pós-operatório imediato. A mortalidade pós operatória foi de 12,5% (n=1).ConclusãoA frequência da Anomalia de Ebstein encontrada foi semelhante a de outros Centros. A técnica de Cone foi viável na maior parte dos pacientes. A mortalidade pós operatória imediata foi baixa.