Prevalence of pulmonary hypertension among children with Down syndrome: A systematic review and meta-analysis

Abstract

BACKGROUND

Pulmonary hypertension (PH) has serious short- and long-term consequences. PH is gaining increasing importance in high risk groups such as Down syndrome (DS) as it influences their overall survival and prognosis. Hence, there is a dire need to collate the prevalence rates of PH in order to undertake definitive measures for early diagnosis and management.

AIM

To determine the prevalence of PH in children with DS.

METHODS

The authors individually conducted a search of electronic databases manually (Cochrane library, PubMed, EMBASE, Scopus, Web of Science). Data extraction and quality control were independently performed by two reviewers and a third reviewer resolved any conflicts of opinion. The words used in the literature search were “pulmonary hypertension” and “pulmonary arterial hypertension”; “Down syndrome” and “trisomy 21” and “prevalence”. The data were analyzed by Comprehensive Meta-Analysis Software Version 2. Risk of bias assessment and STROBE checklist were used for quality assessment.

RESULTS

Of 1578 articles identified, 17 were selected for final analysis. The pooled prevalence of PH in these studies was 25.5%. Subgroup analysis was carried out for age, gender, region, year of publication, risk of bias and etiology of PH.

CONCLUSION

This review highlights the increasing prevalence of PH in children with DS. It is crucial for pediatricians to be aware of this morbid disease and channel their efforts towards earlier diagnosis and successful management. Community-based studies with a larger sample size of children with DS should be carried out to better characterize the epidemiology and underlying etiology of PH in DS.

Keywords: Down syndrome; Pulmonary hypertension; Prevalence; Trisomy 21; Persistent pulmonary hypertension; Congenital heart disease

Core Tip: The objective of this review is to provide quantitative data on the prevalence of pulmonary hypertension (PH) in pediatric patients with Down syndrome (DS). In addition, we also wish to address the lack of consensus on screening guidelines for PH in DS, as it is frequently missed unless associated with an underlying congenital heart disease. We conclude that children with DS require early echocardiography irrespective of an underlying congenital heart disease. We, therefore, by means of this systematic review would like to increase the vigilance for PH in DS, with the ultimate goal of reducing the morbidity due to PH in these children.