title: [Epidemiology and registry of congenital heart disease in Costa Rica].
abstract: OBJECTIVE
  Characterize the population of children born with congenital heart disease (CHD) in Costa Rica and evaluate the country's registry processes.
  
  
  METHODS
  Exploratory observational study that included all children with CHD diagnosed at the National Children's Hospital between 1 May 2006 and 1 May 2007. Considering children under 1 year of age and their respective birth cohort, prevalence was estimated by sex, type of heart disease, age at diagnosis, maternal age, habitual residence, and associated extracardiac malformations, with 95% confidence intervals (95% CI). The data was compared with those of the Congenital Disease Registry Center (CREC).
  
  
  RESULTS
  During the period studied, 534 cases with CHD were diagnosed. There were 473 cases in children under 1 year of age in a birth cohort of 77 140 children. Prevalence was 0.6% (95% CI: 0.5-0.7). Based on CREC data, it was demonstrated that 71% of the cases were not detected at birth. The average age of diagnosis in infants under 1 year of age was 46.6 days. There were no differences by sex. Prevalence of CHD in children of mothers aged 35 years or over was significantly higher. However, when chromosomal abnormalities were excluded, the risk was no longer statistically significant. The provinces in the country with maritime ports were the areas with the highest risk in children of adolescent mothers. The most common CHDs were ventricular and atrial septal defects, patent ductus arteriosus, pulmonary valve stenosis, atrioventricular septal defects, coarctation of the aorta, and tetralogy of Fallot. Thirty-four percent of the cases of CHD were multiple, 11.2% were associated with chromosomal abnormalities, and 19% had associated congenital malformations.
  
  
  CONCLUSIONS
  CHD prevalence in Costa Rica is within the range reported globally. Significant underreporting of CHD was found in the CREC, primarily due to the age criteria applied. The results suggest that maternal age (under 20 and over 34) is a factor associated with CHD.
authors.affiliation: Unidad de Enfermedades Congénitas, Instituto Costarricense de Investigación y Enseñanza en Nutrición y Salud, Cartago, Costa Rica. abenavides@inciensa.sa.cr
authors.forename: Adriana
authors.initials: A
authors.lastname: Benavides-Lara
authors.forename: Jorge Enrique
authors.initials: JE
authors.lastname: Faerron Ángel
authors.forename: Lila
authors.initials: L
authors.lastname: Umaña Solís
authors.forename: Juan José
authors.initials: JJ
authors.lastname: Romero Zúñiga
mesh_terms: D000015:Abnormalities, Multiple / Q000453:epidemiology; D000328:Adult; D003364:Costa Rica / Q000453:epidemiology; D003430:Cross-Sectional Studies; D057210:Delayed Diagnosis; D005260:Female; D006330:Heart Defects, Congenital / Q000145:classification / Q000175:diagnosis / Q000453:epidemiology*; D006776:Hospitals, Pediatric / Q000706:statistics & numerical data; D006783:Hospitals, State / Q000706:statistics & numerical data; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008423:Maternal Age; D008875:Middle Aged; D011247:Pregnancy; D015995:Prevalence; D012042:Registries; D012189:Retrospective Studies; D012959:Socioeconomic Factors; D055815:Young Adult