title: Prevalence and profile of congenital heart disease and pulmonary hypertension in Down syndrome in a pediatric cardiology service.
abstract: OBJECTIVE
  To determine the frequence and profile of congenital heart defects in Down syndrome patients referred to a pediatric cardiologic center, considering the age of referral, gender, type of heart disease diagnosed by transthoracic echocardiography and its association with pulmonary hypertension at the initial diagnosis.
  
  
  METHODS
  Cross-sectional study with retrospective data collection of 138 patients with Down syndrome from a total of 17,873 records. Descriptive analysis of the data was performed, using Epi-Info version 7.
  
  
  RESULTS
  Among the 138 patients with Down syndrome, females prevailed (56.1%) and 112 (81.2%) were diagnosed with congenital heart disease. The most common lesion was ostium secundum atrial septal defect, present in 51.8%, followed by atrioventricular septal defect, in 46.4%. Ventricular septal defects were present in 27.7%, while tetralogy of Fallot represented 6.3% of the cases. Other cardiac malformations corresponded to 12.5%. Pulmonary hypertension was associated with 37.5% of the heart diseases. Only 35.5% of the patients were referred before six months of age.
  
  
  CONCLUSIONS
  The low percentage of referral until six months of age highlights the need for a better tracking of patients with Down syndrome in the context of congenital heart disease, due to the high frequency and progression of pulmonary hypertension.
authors.affiliation: Unidade de Cardiologia Materno-Fetal, Recife, PE, Brasil.
authors.forename: Felipe Alves
authors.initials: FA
authors.lastname: Mourato
authors.affiliation: Unidade de Cardiologia Materno-Fetal, Recife, PE, Brasil.
authors.forename: Lúcia Roberta R
authors.initials: LR
authors.lastname: Villachan
authors.affiliation: Unidade de Cardiologia Materno-Fetal, Recife, PE, Brasil.
authors.forename: Sandra da Silva
authors.initials: Sda S
authors.lastname: Mattos
mesh_terms: D002300:Cardiac Care Facilities; D003430:Cross-Sectional Studies; D004314:Down Syndrome / Q000150:complications*; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications* / Q000175:diagnosis / Q000453:epidemiology*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000175:diagnosis / Q000453:epidemiology*; D007223:Infant; D008297:Male; D015995:Prevalence; D012189:Retrospective Studies