Reviews

Sickle cell disease and cardiovascular health in children: a systematic review of early myocardial damage, biomarkers, and preventive strategies in low-resource settings

Publisher's note
All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.
Published: 23 October 2026
39
Views
26
Downloads

Authors

Sickle Cell Disease (SCD) is one of the most prevalent inherited hemoglobinopathies worldwide, with sub-Saharan Africa bearing the highest burden. In Nigeria, where an estimated 150,000 children are born annually with SCD, the cardiovascular consequences of chronic hemolytic anemia, recurrent vaso-occlusive episodes, and endothelial dysfunction remain underappreciated. Children with SCD are at heightened risk of myocardial ischemia, silent cardiac damage, and progressive ventricular dysfunction, yet routine surveillance protocols are absent in most Nigerian tertiary health facilities. This systematic review synthesizes current evidence on the prevalence of early myocardial injury in pediatric SCD populations, evaluates the utility of high-sensitivity troponin assays and advanced echocardiographic modalities (speckletracking imaging), and examines the potential role of prophylactic therapies such as hydroxyurea, chronic transfusion regimens, and targeted antioxidant interventions in mitigating silent cardiac events. Particular attention is directed toward knowledge gaps specific to the Nigerian health system, where diagnostic limitations, workforce shortages, and competing infectious disease priorities impede cardiovascular screening. The review highlights that while advanced imaging may not be universally accessible, simple preventive measures - hydroxyurea, L-glutamine, transfusion, aggressive hydration, and infection prevention - can be initiated at the first point of contact even in rural settings. The review concludes with a proposed interdisciplinary framework to guide future implementation research in resource-constrained environments.

Downloads

Download data is not yet available.

Citations

1.Rees DC, Williams TN, Gladwin MT. Sickle cell disease. Lancet 2010;376:201831.
2.Gladwin MT, Vichinsky E. Pulmonary complications of sickle cell disease. N Engl J Med 2008;359:225465.
3.Piel FB, Hay SI, Gupta S, et al. Global burden of sickle cell anemia in children under five, 2010-2050: modelling based on demographics, excess mortality, and interventions. PLoS Med 2013;10:e1001483.
4.Global Burden of disease (GBD) 2021 Sickle Cell disease Collaborators. Global, regional, and national prevalence and mortality burden of sickle cell disease, 2000-2021: a systematic analysis from the Global Burden of disease Study 2021. Lancet Hematol 2023;10:e58599.
5.Nwogoh B, Adewoyin AS, Iheanacho OE, Bolarinwa RA. Cardiovascular complications of sickle cell disease: a review of the literature. J Blood Med 2021;12:45970.
6.Manci EA, Culberson DE, Yang YM, et al. Causes of death in sickle cell disease: an autopsy study. Br J Hematol 2003;123:35965.
7.Mor-Avi V, Lang RM, Badano LP, et al. Current and evolving echocardiographic techniques for the quantitative evaluation of cardiac mechanics: ASE/Ee consensus statement on methodology and indications endorsed by the Japanese Society of Echocardiography. J Am Soc Echocardiogr 2011;24:277313.
8.Apple FS, Sandoval Y, Jaffe AS, et al. Cardiac troponin assays: guide to understanding analytical characteristics and their impact on clinical care. Clin Chem 2017;63:7381.
9.Wells GA, Shea B, O’Connell D, et al. The Newcastle-Ottawa Scale (NOS) for assessing the quality of non-randomized studies in meta-analyses. Ottawa Hospital Research Institute. Available from: https://www.ohri.ca/programs/clinical_epidemiology/oxford.asp.
10.Dosunmu A, Akinbami A, Uche E, et al. Electrocardiographic study in adult homozygous sickle cell disease patients in Lagos, Nigeria. J Trop Med 2016;2016:4214387.
11.Akkus NI, Rajpal S, Hilbun J, et al. Troponin elevation in sickle cell disease. Med Princ Pract 2021;30:43742.
12.Whipple NS, Naik RJ, Kang G, et al. Ventricular global longitudinal strain is altered in children with sickle cell disease. Br J Hematol 2018;183:796806.
13.Wagdy R, Assem H, Abd Elmohsen AM, et al. Altered ventricular longitudinal strain in children with sickle cell disease: role of TGF-β and IL-18. Pediatr Blood Cancer 2024;71:e30762.
14.AbdelMassih AF, El-Gamasy MA, El-Razek AA, et al. Speckle tracking evaluation of right ventricular functions in children with sickle cell disease. Ann Pediatr Cardiol 2017;10:2303.
15.Yildirim A, Buyukavci M, Keskin M, et al. Subclinical left ventricular dysfunction in children with sickle cell disease: a speckle tracking echocardiography study. J Pediatr Hematol Oncol 2019;41:e2149.
16.Chenik S, Noamen A, Bouslimi A, et al. Evaluation of left ventricular systolic function in children with sickle cell anemia: contribution of 2D strain. F1000Res 2022;11:1207.
17.Sabatini L, Chinali M, Franceschini A, et al. Echocardiographic evaluation in pediatric sickle cell disease patients: a pilot study. J Clin Med 2022;12:7.
18.Dhar A, Leung TM, Appiah-Kubi A, et al. Longitudinal analysis of cardiac abnormalities in pediatric patients with sickle cell anemia and effect of hydroxyurea therapy. Blood Adv 2021;5:467382.
19.Lee MT, Kavanagh PL, Hankins JS, et al. Hydroxyurea and cardiac sequele in children with sickle cell disease. Pediatr Blood Cancer 2019;66:e27692.
20.Yawn BP, Buchanan GR, Afenyi-Annan AN, et al. Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members. JAMA 2014;312:103348.
21.Colombatti R, Andemariam B. Microvasculopathy and biomarkers in sickle cell disease: the promise of non-invasive real-time in vivo tools. Br J Hematol 2020;190:4835.
22.Niss O, Quinn CT, Lane A, et al. Cardiomyopathy with restrictive physiology in sickle cell disease. JACC Cardiovasc Imaging 2016;9:24352.
23.Raghupathy R, Billett HH. The cardiovascular system in sickle cell disease. J Am Coll Cardiol 2009;53:107988.
24.Mekontso Dessap A, Le Roux G, Habibi A, et al. Risk factors for left ventricular dysfunction in patients with sickle cell disease. Am J Cardiol 2011;108:102934.
25.Ware RE. How I use hydroxyurea to treat young patients with sickle cell anemia. Blood 2010;115:530011.
26.Chou ST, Fasano RM. Management of patients with sickle cell disease using transfusion therapy: guidelines and complications. Hematol Oncol Clin North Am 2016;30:591608.
27.Mourão LT, Souza DB, Silveira DM, et al. Carvedilol improves left ventricular strain in adults with sickle cell disease: a pilot study. Am J Hematol 2016;91:E3123.
28.Almeida-Santos MA, Pinheiro AC, Santiago RP, et al. ACE inhibitors in sickle cell disease: a systematic review. Rev Bras Hematol Hemoter 2019;41:26471.
29.Okoromah CA, Ekure EN, Falase B, et al. Pediatric cardiology in Nigeria: current status and future directions. Pan Afr Med J 2017;27:112.
30.Galadanci NA, Abdullahi SU, Vance LD, et al. Health-seeking behaviour for children with sickle cell disease in Nigeria. J Glob Health 2019;9:010407.
31.Tubman VN, Makani J. Sickle cell disease in Africa: time to address the burden. Lancet Hematol 2016;3:e4034.
32.Covitz W, Espeland M, Gallagher D, et al. The heart in sickle cell anemia. The Cooperative Study of Sickle Cell disease (CSSCD). Chest 1995;108:12149.
33.Johnson CS. The heart in sickle cell disease. J Natl Med Assoc 1991;83:10328.
34.Kato GJ, Steinberg MH, Gladwin MT. Intravascular hemolysis and the pathophysiology of sickle cell disease. J Clin Invest 2017;127:75060.
35.Connes P, Alexy T, Detterich J, et al. The role of blood rheology in sickle cell disease. Blood Rev 2016;30:1118.
36.Westwood MA, Shah F, Anderson LJ, et al. Myocardial tissue characterization and the role of chronic anemia in sickle cell cardiomyopathy. J Magn Reson Imaging 2007;26:5648.
37.Ansa VO, Ekott JU, Essien IO, Bassey EO. Myocardial injury in patients with sickle cell anemia and myocardial ischemia in Calabar, Nigeria. Trop Doct 2014;44:20912.
38.Kavsak PA, Don-Wauchope AC, Hill SA, et al. Acceptable analytical performance of high-sensitivity cardiac troponin I and T assays in a pediatric setting. Clin Biochem 2014;47:31822.
39.Nir A, Lindinger A, Rauh M, et al. NT-pro-B-type natriuretic peptide in infants and children: reference values based on a combined data from four studies. Pediatr Cardiol 2009;30:38.
40.Niihara Y, Miller ST, Kanter J, et al. A phase 3 trial of L-glutamine in sickle cell disease. N Engl J Med 2018;379:22635.

How to Cite



“Sickle Cell Disease and Cardiovascular Health in Children: A Systematic Review of Early Myocardial Damage, Biomarkers, and Preventive Strategies in Low-Resource Settings”. 2026. Pyramid Journal of Medicine 9 (1). https://doi.org/10.4081/pjm.2026.634.