Burden and outcomes of cardiovascular and pulmonary complications in adolescents and adults with sickle cell disease: a systematic review and meta-analysis

Background Sickle cell disease (SCD) is a genetic blood disorder associated with chronic cardiovascular and pulmonary complications, including pulmonary hypertension (PHT), venous thromboembolism (VTE), and cardiac involvement (CI), all of which contribute significantly to premature mortality. Despite their clinical importance, the burden of these conditions in adolescents and adults with SCD remains poorly characterized. Methods We conducted a systematic review and meta-analysis to evaluate the prevalence and impact of these complications in SCD patients aged ≥ 10 years. Following PRISMA guidelines, we searched MEDLINE/PubMed, Web of Science, and Scopus for observational studies reporting prevalence or incidence. We did not apply any date or time restrictions to the literature search. Pooled estimates were calculated using random-effects meta-analysis. Results The studies yielded from the search were published between January 1, 1974, and October 12, 2023. Seventy-nine studies met the inclusion criteria, of which fifty-nine were included in the meta-analysis. The pooled prevalence estimates were as follows: PHT 30% (95% CI 26–34), VTE 13% (9–18), and CI 24% (17–33), with substantial heterogeneity (I2 > 90%) among the studies. PHT was primarily diagnosed via Doppler echocardiography, while VTE was identified through ultrasonography or CT angiography. CI manifestations included diastolic dysfunction and structural abnormalities. These complications were strongly associated with increased mortality and functional impairment. Conclusion Chronic cardiovascular and pulmonary complications are highly prevalent in adolescents and adults with SCD, leading to significant morbidity and mortality. Routine screening and early intervention are essential to mitigate disease progression and improve outcomes. Future research should focus on standardized diagnostic criteria and targeted therapies to reduce the burden of these conditions in SCD. Systematic review registration PROSPERO CRD42023474623