Investigation of high Prevalence of Pulmonary Arterial Hypertension in children with Down syndrome and congenital heart disease referred to Pediatric and Congenital Heart Disease department at Imam Reza Hospital, Mashhad-Iran

Down Syndrome (DS) is a genetic impairment has comorbidities such as Congenital Heart Disease (CHD). Pulmonary Atrial Hypertension (PAH) is a complication of CHD in these patients; however, there are no sufficient documents about the prevalence of PAH in CHD-DS patients in Iran. The purpose of this study is to investigate the prevalence of (PAH) in CHD-DS patients.

This is a cross-sectional study that conducted on CHD-DS patients referred to Imam Reza hospital, Mashhad, Iran (April 2015- February 2016). The comparison group includes non-DS (NDS) children with CHD. Both groups were same in terms of age and gender. Echocardiography was run for all patients to determine CHD types and pulmonary arterial pressure (PAP).

the cohort of 77 patients (including 47 in DS group and 30 in comparison group) was studied. It was observed that 66% of the DS group and 56.7% of the NDS group were female (P=0.412). The average age of case and comparison group were 15.1±25.83 and 36.6±44.17 month, respectively (p=0.108).Most of the CHD-DS patients (63.8%) had multiple heart disease. The most common impairments among the DS-CHD group were Ventricular Septal Defect (VSD), Atrial Septal Defect (ASD), and Patent Ductus Arteriosus (PDA).48.9% of the DS patients and 23.3% of NDS group had developed PAH which demonstrates a significant higher rate among DS patients (p=0.025).

Our findings denote higher prevalence of PAH among CHD-DS patients. Such an observation is a meaningful warning for the studied patients to take necessary medical therapies in order to prevent complications and irreversible pulmonary vascular disease.