Natasha Kaweme, Sahar Buttress, Arthemon Nguweneza, Cynthia Phiri, Takudzwa Mtisi, Pauline Sambo, Gwendoline Kandawasvika, Hamakwa Mantina, Patience Kuona, Catherine Chunda-Liyoka
University Teaching Hospitals. University of Cape Town. University of Zimbabwe.
Zambia, South Africa and Zimbabwe
Systematic Reviews
Syst Rev 2026;
DOI: 10.1186/s13643-026-03077-1
Abstract
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.
Category
Class V. Pulmonary Hypertension Associated with Hematological, Systemic, Metabolic, Nutritional and Other Disorders
Review Articles Concerning Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease or Adult Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes