Meta-Analysis
Copyright ©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Pediatr. Nov 9, 2021; 10(6): 177-191
Published online Nov 9, 2021. doi: 10.5409/wjcp.v10.i6.177
Prevalence of pulmonary hypertension among children with Down syndrome: A systematic review and meta-analysis
Amar Taksande, Divya Pujari, Patel Zeeshan Jameel, Bharati Taksande, Revat Meshram
Amar Taksande, Divya Pujari, Patel Zeeshan Jameel, Revat Meshram, Department of Pediatrics, Jawaharlal Nehru Medical College, Wardha 442004, Maharashtra, India
Bharati Taksande, Department of Medicine, Mahatma Gandhi Institute of Medical Sciences, Wardha 442102, Maharashtra, India
Author contributions: Taksande A contributed to the conception and design of this study; Pujari D, Jameel PZ and Meshram R contributed to the identification of studies and analysis of the data; Taksande B solved disagreements; Taksande B, Taksande A and Meshram R interpreted the data; Pujari D and Jameel PZ drafted the article; Taksande A, Taksande B and Meshram R critically revised the article for important intellectual content; All authors have approved the final version; Taksande A shall act as guarantor of the paper and he/she should take the responsibility for the integrity of the work as a whole, from its inception to published article.
Conflict-of-interest statement: All the authors declare that they have no competing interests.
PRISMA 2009 Checklist statement: The authors have read the PRISMA 2009 Checklist and the manuscript was prepared and revised according to the PRISMA 2009 Checklist.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Amar Taksande, MBBS, MD, Professor, Department of Pediatrics, Jawaharlal Nehru Medical College, Sawangi Meghe, Wardha 442004, Maharashtra, India. amar.taksande@gmail.com
Received: January 19, 2021
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
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.