Sickle cell disease is a hereditary, autosomal recessive pathology caused by the replacement of adenine by thymin in position 6 of the beta globin gene, producing anomalous hemoglobin (hemoglobin S- HbS). It is characterized by a chronic inflammatory state with hemolytic anemia and vaso-occlusive phenomena and occurs when the HbS gene is in homozygosis (SS) or heterozygosis, in association with other variant hemoglobins (SD, SC, SE) or thalassemia (). Sickle cell disease is recognized by the World Health Organization (WHO) as a serious public health problem, and, despite advances in treatment, patients with sickle cell disease have a life expectancy of nearly 30 years lower than the general population.

About 4% of the Brazilian population has a sickle cell trait, and between 60,000 and 100,000 Brazilians have the disease, which translates into an economic burden of more than US$ 400,000,000/year for the Brazilian society. The number of patients affected by this pathology is increasing due to the evolution of treatment and improvement in early diagnosis, as a result of the institution of neonatal screening. Early identification of the disease is important in reducing its morbidity and mortality, especially with the prophylaxis of infections, which are a significant cause of death in children. This improvement in survival leads to the development of chronic complications resulting from vasculopathy and to more frequent cases of chronic ischemia.

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Abstract

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.