Prevalence of pulmonary arterial hypertension in children with down syndrome attending new 500 bedded paediatric hospital, Government Medical College, Srinagar

Abstract

Background: Children with down syndrome are at increasing risk of developing pulmonary hypertension (PH) due to multiple factors. The high frequency of PH in this population has probable genetic, congenital, and environmental contributions. Methods: This prospective observation study was conducted in the Post Graduate Department of Pediatrics, New Pediatric Hospital GMC Srinagar, on all Indoor/Outdoor patients with karyotype documented cases of Down syndrome over a period of 2 years between December 2020 to December2022. After detailed history and examination routine baseline investigations both screening and diagnostic echocardiography was done. CT was done in selected patients. In some patients, whose history and examination was suggestive of obstructive sleep apnoea (OSA), polysomnography and OSA questionnaire was used to make the diagnosis. Results: This study included 306 children with karyotype documented Down syndrome. Out of them 72 were identified with PAH (Echocardiography documented) and the prevalence was 23.5%. Out of these 72 patients, 41 (57%) were males and 31 (43%) were females with male: female ratio of 1.3:1 and maximum number of patients were present in age group 1-6months. Out of them 60 (83%) patients had underlying echocardiographic documented CHD, 10 (14%) patients had OSA (Obstructive Sleep Apnea) and 2 (3%) had GERD (Gastrointestinal Reflux Disease). Conclusions: The PH is common in children with downs syndrome, is typically transient related to CHD or PPHN but can recur in setting of respiratory disease such as obstructive sleep apnea, intermittent hypoxia and recurrent pneumonia.