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. 2013 Jul;69(3):241-5.
doi: 10.1016/j.mjafi.2012.11.013. Epub 2013 Feb 23.

A study to determine the prevalence of pulmonary arterial hypertension in children with Down syndrome and congenital heart disease

Mukti Sharma  1 , Sanjeev Khera  2 , Vishal Sondhi  3 , Amit Devgan  3
Affiliations

A study to determine the prevalence of pulmonary arterial hypertension in children with Down syndrome and congenital heart disease

Mukti Sharma et al. Med J Armed Forces India. 2013 Jul.

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.

Conclusion: DS children with CHD have a higher predisposition to develop PAH, and the likelihood is highest with underlying AVSD.

Keywords: Atrioventricular septal defects; Congenital heart disease; Down syndrome; Left-to-right shunt; Pulmonary arterial hypertension; Ventricular septal defect.

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