Published online Nov 9, 2021. doi: 10.5409/wjcp.v10.i6.177
Peer-review started: January 19, 2021
First decision: May 6, 2021
Revised: May 13, 2021
Accepted: August 17, 2021
Article in press: August 17, 2021
Published online: November 9, 2021
Processing time: 293 Days and 23.7 Hours
Children with Down syndrome (DS) have an increased likelihood of developing pulmonary hypertension (PH) with serious short- and long-term consequences. Approximately 75% of all deaths in DS may be attributed to pneumonia and infectious lung disease, congenital heart disease (CHD) and circulatory disease (vascular diseases such PH). Despite the overwhelming evidence of morbidity, there have been no studies estimating the precise disease burden of PH in children with DS.
Additional information is required to collate the prevalence rates of PH in order to undertake definitive measures for early diagnosis and management.
The objective of this study is to determine the prevalence of PH in children with DS.
The electronic databases (PubMed, Cochrane library, EMBASE, Scopus, Web of Science) were searched. Any observational study which determined the prevalence of PH in DS was considered for the analysis. Data were extricated using a preconceived and standardized data abstraction form. The data were analyzed by Comprehensive Meta-Analysis Software Version 2.
Of 1578 articles identified, 17 were selected for final analysis. The pooled prevalence of PH in these studies was 25.5% (95%CI: 17.4%–35.8%). Subgroup analysis was carried out for age, gender, region, year of publication, risk of bias and etiology of PH.
This article highlights the increasing prevalence of PH in children with DS. This is accounted for by the high prevalence of underlying CHDs in these children. In order to improve the care given and reduce the disease burden, the attending pediatrician has a crucial role in being aware of this morbid disease and to channel his/her efforts towards routine screening of PH, earlier diagnosis and successful management. In addition, there should be early routine echocardiographic screening in children with DS even in the absence of CHDs. Community-based studies with a larger sample size of children with DS may be carried out to better characterize the epidemiology and underlying etiology of PH in DS. Our study reinforces what is already known and provides, in addition, reliable information about the prevalence of PH in DS.
Further studies are required to better characterize the epidemiology, underlying etiology, pathogenesis and risk factors of PH in children with DS.