title: [Congenital Heart Disease in Children with Down Syndrome: What Has Changed in the Last Three Decades?]
abstract: BACKGROUND
  The prevalence of Down syndrome has increased in the last 30 years; 55% of these children have congenital heart disease.
  
  
  METHODS
  A retrospective longitudinal cohort study; clinical data from 1982 to 2013 databases with the diagnosis of Down syndrome or trisomy 21 in a reference hospital in pediatric cardiology and cardiac surgery.
  
  
  OBJECTIVE
  to assess the progress in the last three decades of cardiological care given to children with Down syndrome and congenital heart disease.
  
  
  RESULTS
  We studied 102 patients with Down syndrome and congenital heart disease subjected to invasive therapy: corrective or palliative cardiac surgery and therapeutic catheterization. The referral age was progressively earlier in patients referred in the first year of life. The most frequent diagnosis was complete atrioventricular sptal defect (41%). There was a trend towards increasingly early corrective surgery in patients under 12 months (p < 0.001). Since 2000, the large majority of patients were operated before reaching six months of age. The main cardiac complications were rhythm dysfunction and low output. More frequent noncardiac complications were pulmonary and infectious. The 30-day mortality rate was 3/102 cases (2.9%). Of patients in follow-up, 89% are in NYHA class I.
  
  
  CONCLUSIONS
  The early surgical correction seen over the past 15 years follows the approach suggested in the literature. The observed 30-day mortality rate is overlapping international results. Patients with Down syndrome subjected to corrective surgery of congenital heart disease have an excellent long-term functional capacity.
authors.affiliation: Serviço de Pediatria. Unidade de Faro. Centro Hospitalar do Algarve. Faro. Portugal.
authors.forename: Filipa Mestre
authors.initials: FM
authors.lastname: Dias
authors.affiliation: Serviço de Cardiologia Pediátrica. Hospital de Santa Cruz. Centro Hospitalar de Lisboa Ocidental. Lisboa. Portugal.
authors.forename: Susana
authors.initials: S
authors.lastname: Cordeiro
authors.affiliation: Serviço de Cardiologia Pediátrica. Hospital de Santa Cruz. Centro Hospitalar de Lisboa Ocidental. Lisboa. Portugal.
authors.forename: Isabel
authors.initials: I
authors.lastname: Menezes
authors.affiliation: Serviço de Cardiologia Pediátrica. Hospital de Santa Cruz. Centro Hospitalar de Lisboa Ocidental. Lisboa. Portugal.
authors.forename: Graça
authors.initials: G
authors.lastname: Nogueira
authors.affiliation: Serviço de Cardiologia Pediátrica. Hospital de Santa Cruz. Centro Hospitalar de Lisboa Ocidental. Lisboa. Portugal.
authors.forename: Ana
authors.initials: A
authors.lastname: Teixeira
authors.affiliation: Serviço de Cirurgia Cardiotorácica. Hospital de Santa Cruz. Centro Hospitalar de Lisboa Ocidental. Lisboa. Portugal.
authors.forename: Marta
authors.initials: M
authors.lastname: Marques
authors.affiliation: Serviço de Cirurgia Cardiotorácica. Hospital de Santa Cruz. Centro Hospitalar de Lisboa Ocidental. Lisboa. Portugal.
authors.forename: Miguel
authors.initials: M
authors.lastname: Abecasis
authors.affiliation: Serviço de Cardiologia Pediátrica. Hospital de Santa Cruz. Centro Hospitalar de Lisboa Ocidental. Lisboa. Portugal.
authors.forename: Rui
authors.initials: R
authors.lastname: Anjos
mesh_terms: D002675:Child, Preschool; D015331:Cohort Studies; D004314:Down Syndrome / Q000150:complications / Q000601:surgery*; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications / Q000601:surgery*; D006801:Humans; D007223:Infant; D008137:Longitudinal Studies; D008297:Male; D012189:Retrospective Studies; D013997:Time Factors