is down syndrome related to pulmonary arterial hypertension? a comparative study

Down syndrome (ds) is a genetic impairment associated with comorbidities such as congenital heart disease (chd). pulmonary arterial hypertension (pah) is a complication of chd in most patients. due to insufficient documents about the prevalence of pah in ds with chds compared to non-ds (nds)+chd patients, this study aimed to compare the prevalence of pah between ds-chd and nds-chd patients. this is a cross-sectional study conducted on ds-chd patients referred to the pediatric and congenital cardiology division at imam reza training hospital in mashhad, iran, between april 2015 and february 2016. the comparison group included nds-chd children matched in terms of age and gender. a comprehensive echocardiography was run for all patients to determine the types of chd and pulmonary arterial pressure. seventy-seven patients were enrolled in the study (47 in the ds-chd group and 30 in the nds-chd group). 48.9% of the ds-chd patients and 23.3% of the nds-chd group developed pah, which revealed a significantly higher rate of ph among ds-chd patients (p=0.025). our findings denote a higher prevalence of pah among ds-chd patients compared to nds-chd patients. such an observation is a meaningful warning for ds patients to take early necessary medical or corrective therapies for chd in order to prevent complications and irreversible pulmonary vascular disease