title: Ebstein's anomaly in children: a single-center study in Angola.
abstract: OBJECTIVE
  Ebstein'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.
  
  
  METHODS
  A retrospective cross-sectional study was conducted over a period of 37 months. We analyzed all patients diagnosed with congenital heart defects.
  
  
  RESULTS
  Of 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.
  
  
  CONCLUSIONS
  The 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.
authors.affiliation: Cardio-Thoracic Center, Clinica Girassol, Luanda, Angola. Electronic address: valdanympub@gmail.com.
authors.forename: Valdano
authors.initials: V
authors.lastname: Manuel
authors.affiliation: Cardiology Department, Hospital Militar Principal/Instituto Superior, Luanda, Angola.
authors.forename: Humberto
authors.initials: H
authors.lastname: Morais
authors.affiliation: Cardio-Thoracic Center, Clinica Girassol, Luanda, Angola; Hospital Cruz Vermelha, Lisbon, Portugal.
authors.forename: Manuel Pedro
authors.initials: MP
authors.lastname: Magalhães
authors.affiliation: Cardio-Thoracic Center, Clinica Girassol, Luanda, Angola.
authors.forename: Maria Ana Sampaio
authors.initials: MA
authors.lastname: Nunes
authors.affiliation: Cardio-Thoracic Center, Clinica Girassol, Luanda, Angola.
authors.forename: Gilberto
authors.initials: G
authors.lastname: Leon
authors.affiliation: Cardio-Thoracic Center, Clinica Girassol, Luanda, Angola; Hospital Cruz Vermelha, Lisbon, Portugal.
authors.forename: Manuel
authors.initials: M
authors.lastname: Ferreira
authors.affiliation: Cardio-Thoracic Center, Clinica Girassol, Luanda, Angola.
authors.forename: António Pedro
authors.initials: AP
authors.lastname: Filipe Júnior
mesh_terms: D000293:Adolescent; D000810:Angola; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D004437:Ebstein Anomaly / Q000175:diagnosis / Q000601:surgery; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D012189:Retrospective Studies
keywords: Cardiopatia congénita; Cirurgia cardíaca congénita; Cone reconstruction; Congenital heart disease; Congenital heart surgery; Lesão valvular; Reconstrução em cone; Tricuspid valve; Valve lesion; Válvula tricúspide