Mukti Sharma1, Sanjeev Khera2, Vishal Sondhi3, Amit Devgan3. 1. Consultant (Paediatrics) Army Hosp (R&R), Delhi Cantt-10, India. 2. Graded Specialist (Paediatrics), Mil Hosp Danapur, C/o 56 APO, India. 3. Associate Professor, Dept of Paediatrics, AFMC, Pune-40, India.
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.
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.
Authors: Michel Emile Weijerman; A Marceline van Furth; Maurike D van der Mooren; Miriam M van Weissenbruch; Lukas Rammeloo; Chantal J M Broers; Reinoud J B J Gemke Journal: Eur J Pediatr Date: 2010-04-23 Impact factor: 3.183
Authors: Sallie B Freeman; Lora H Bean; Emily G Allen; Stuart W Tinker; Adam E Locke; Charlotte Druschel; Charlotte A Hobbs; Paul A Romitti; Marjorie H Royle; Claudine P Torfs; Kenneth J Dooley; Stephanie L Sherman Journal: Genet Med Date: 2008-03 Impact factor: 8.822