title: A study to determine the prevalence of pulmonary arterial hypertension in children with Down syndrome and congenital heart disease.
abstract: BACKGROUND
  Down syndrome (DS) is a common chromosomal abnormality associated with congenital heart disease (CHD). These cardiac abnormalities are known to be associated with pulmonary arterial hypertension (PAH).
  
  
  METHODS
  The aim of this study was to assess the prevalence of PAH in DS children with CHD and to compare it with PAH prevalence in non-syndromic children with CHD. It was a cross-sectional study including all children presenting to Cardiology Department at a tertiary care center between Jan 2007 and Dec 2010.
  
  
  RESULTS
  Thirty-five DS children with CHD were compared with 38 non-syndromic children with CHD. Atrioventricular septal defect (AVSD, 13/35) was the commonest CHD among DS children while isolated VSD (26/38) accounted for maximum cases in non-syndromic CHD. PAH was more prevalent among DS children with CHD (18/42) than among the control group (7/38, p = 0.038). In addition, 14/19 patients with AVSD in the entire cohort developed PAH.
  
  
  CONCLUSIONS
  DS children with CHD have a higher predisposition to develop PAH, and the likelihood is highest with underlying AVSD.
authors.affiliation: Consultant (Paediatrics) Army Hosp (R&R), Delhi Cantt-10, India.
authors.forename: Mukti
authors.initials: M
authors.lastname: Sharma
authors.affiliation: Graded Specialist (Paediatrics), Mil Hosp Danapur, C/o 56 APO, India.
authors.forename: Sanjeev
authors.initials: S
authors.lastname: Khera
authors.affiliation: Associate Professor, Dept of Paediatrics, AFMC, Pune-40, India.
authors.forename: Vishal
authors.initials: V
authors.lastname: Sondhi
authors.affiliation: Associate Professor, Dept of Paediatrics, AFMC, Pune-40, India.
authors.forename: Amit
authors.initials: A
authors.lastname: Devgan
keywords: Atrioventricular septal defects; Congenital heart disease; Down syndrome; Left-to-right shunt; Pulmonary arterial hypertension; Ventricular septal defect