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INNOSC Theranostics and Pharmacological Sciences 2023 Vol. 6 (No. 1) pp: 35-45

INNOSC Theranostics and Pharmacological Sciences

Journal homepage: https://accscience.com/journal/ITPS

REVIEW ARTICLE
Pulmonary Hypertension among Children and Adolescents with

Sickle Cell Anemia: A Systematic Review and Meta-analysis

Vishnu Shankar Hariharan*

Department of Internal Medicine, Hindu Mission Hospital, Chennai, India
*Corresponding author: Vishnu Shankar Hariharan, Email: vishank91@gmail.com
Received: September 16, 2022; Accepted: April 7, 2023; Published: April 20, 2023 DOI: 10.36922/itps.198
Copyright: Author(s). This is an open-access article distributed under the terms of the Attribution Non-Commercial 4.0 International
4.0 (CC BY-NC 4.0), which permits all non-commercial use, distribution, and reproduction in any medium, provided the original
work is properly cited.

Abstract:

Pulmonary hypertension (PHT) is a major life-threatening complication associated with sickle cell anemia (SCA). However, there
is scarcity of evidence in pooling the knowledge regarding the prevalence of PHT in the pediatric SCA patients. Hence, this
systematic review was done to determine the pooled prevalence of PHT among SCA children and adolescents. Until January
2021, systematic searches were conducted in MEDLINE, SCOPUS, Web of Science, ScienceDirect, Cochrane library, and Google
Scholar. The listed studies’ caliber was evaluated using the Newcastle Ottawa scale. The results of a meta-analysis using a random-
effects model included a pooled prevalence and 95% confidence intervals (CIs). In total, 31 studies with 3686 participants were
included in the study. Majority of the included studies (26 out of 31 studies) had low risk of bias. The final pooled prevalence of
PHT among children and adolescents with SCA was 22% (95% CI: 18 – 26%). Maximum burden of PHT among SCA children
was reported in Europe (26%) and Eastern Mediterranean region, while the least burden was found in Africa (17%). There was
a significant heterogeneity found between the studies in our analysis (I = 87.8%; P < 0.001). The presence of publication bias
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indicated by an asymmetrical funnel plot was also found. About one in five children and adolescents with SCA suffer from PHTN.
The burden is maximum in Europe followed by Eastern Mediterranean region. Diagnostic and intervention packages targeting
these patients should be developed and implemented across the high-risk settings.
Keywords: Epidemiology, Meta-analysis, Pulmonary hypertension, Sickle cell anemia

1. Introduction is associated with several different complications
with varying severity.
Sickle cell anemia (SCA) is one of the most Pulmonary hypertension (PHT) is a major
common inherited genetic hematological life-threatening complication associated with
condition affecting children and adolescents [1]. SCA [3]. It is a hemodynamic illness characterized
It can be characterized by the red cell sickling by increased vascular resistance of pulmonary
accompanied by end organ damage due to vaso- circulation. The previous studies have shown that
occlusion and hemolytic anemia [1]. Almost 5% SCA is frequently associated with PHT among
of the world population were found to be healthy children and adolescents [3,4]. The occurrence
carriers of SCA or thalassemia gene [2]. About of PHT in SCA patients is caused by hemolytic
300,000 children are born with severe form of reaction and distorted nitric acid metabolism [5,6].
these conditions, with majority occurring in It may remain clinically silent for prolonged period
low- and low middle-income countries [2]. SCA and can be discovered late during illness. It has

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