Pulmonary Hypertension among Children and Adolescents with Sickle Cell Anemia: A Systematic Review and Meta-analysis

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 (I2 = 87.8%; P < 0.001). The presence of publication bias 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.