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Updated: Aug 20, 2025
A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
Prevalence of Cardiovascular Complications in Individuals with Sickle Cell Anemia and Other Hemoglobinopathies: A
Andressa Lopes1, Marina Tejo Dantas1, Ana Marice Teixeira Ladeia1
1Escola Bahiana de Medicina e Saúde Pública, Salvador, BA - Brasil.
Insights
Sickle cell anemia (SCA) patients experience more cardiovascular complications than those with other hemoglobinopathies. Early detection of these heart issues is crucial for better patient outcomes.
Area of Science:
- Cardiology
- Hematology
- Genetics
Background:
- Sickle cell anemia (SCA) is a hereditary blood disorder.
- Cardiovascular complications are the primary cause of mortality in SCA and other hemoglobinopathies.
- Early identification of cardiac changes can improve disease management.
Conclusions:
- Individuals with SCA exhibit greater cardiovascular impairment compared to those with other hemoglobinopathies.
- Regular cardiovascular monitoring is essential for patients with sickle cell anemia.
Background:
Sickle cell anemia (SCA) is a hereditary disease whose cardiovascular complications are the main cause of death, the same being observed in other hemoglobinopathies. Early identification of these changes can favorably modify the course of the disease.
Objective:
To compare the prevalence of cardiovascular complications between individuals with SCA and individuals with other hemoglobinopathies.
Method:
Following the recommendations of the PRISMA protocol, a systematic literature review was carried out with searches in PubMed/Medline databases, associated with a manual search. Studies that analyzed the prevalence of cardiovascular alterations in hemoglobinopathies (SCA, sickle cell trait, SC hemoglobinopathy, alpha-thalassemia and beta-thalassemia) were included. The methodological quality of the articles was assessed using the Newcastle-Ottawa scale.
Results:
Four studies were selected for analysis, resulting in a sample size of 582 participants: 289 with SCA, 133 with SC hemoglobinopathy, 40 with beta-thalassemia, 100 healthy individuals and none with alpha-thalassemia or sickle cell trait. Dilatation of the cardiac chambers, left and right ventricular hypertrophy, pulmonary hypertension, diastolic dysfunction, mitral regurgitation and tricuspid regurgitation are more prevalent in SCA than in the other hemoglobinopathies considered. Myocardial iron overload is more frequent in thalassemia major than in sickle cell anemia. Systolic function is similar between different hemoglobinopathies.
Conclusion:
There is greater cardiovascular impairment in individuals with SCA than in those with other hemoglobinopathies, reinforcing the necessity for regular cardiovascular follow-up in sickle cell patients.
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