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

Abstract

Purpose: 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. Method: 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). Results: 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). Conclusions: 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.

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