Anemia, Sickle Cell – ABC CARDIOL

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Editorial

Sickle Cell Anemia as the First in the Hierarchy for Cardiac Alterations, Drives Attention to the Whole Spectrum of Hemolytic Anemias

Ana Paula Marte Chacra et al. Anita L. R. Saldanha Tania Leme da Rocha Martinez

The focus on the cardiac repercussions of Hemolytic Anemias has been greatly emphasized by publications issued by academic and official government documents that are easily accessed by all health professionals, mainly articles that aim at Sickle Cell Anemia^, and Thalassemia.^,

Sickle cell disease is the most frequent genetic hemoglobinopathy worldwide. Thanks to the improvement of the medical management of these patients, their life expectancy has improved in recent years. However, cardiopulmonary complications remain one of the main causes of death in adult patients with sickle cell disease.

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Original Article

Prevalence of Cardiovascular Complications in Individuals with Sickle Cell Anemia and Other Hemoglobinopathies: A Systematic Review

Andressa Lopes et al. Marina Tejo Dantas Ana Marice Teixeira Ladeia

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.

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Original Article

Association Between Lipid Profile and Clinical Manifestations in Sickle Cell Anemia: A Systematic Review

Marina Tejo Dantas et al. Andressa Lopes Ana Marice Teixeira Ladeia

Abstract

Introduction

Sickle cell anemia (SCA) is a genetic disease associated with frequent episodes of acute illness. Changes in the lipid profile and a chronic inflammatory process make up the molecular aspects observed in this disease. Associations between these mechanisms and clinical manifestations could thus define severity profiles and therapeutic strategies.

Objectives

To verify whether there is an association between lipid profile and clinical manifestations in patients with SCA and if there is a correlation between lipid profile and laboratory markers in this disease.

Methodology

According to the PRISMA guidelines, a systematic review of the literature was conducted by searching the MEDLINE/PubMed, LILACS, SciELO, Scopus, and Cochrane databases. Articles were screened by reading the titles and abstracts, reaching those selected for full-text reading. The included studies were published between 2010 and 2020, were fully available in the databases, and addressed the proposed theme. The risk of individual bias was assessed by using the Joanna Briggs Institute checklist and the Newcastle-Ottawa scale.

Results

Out of the 144 identified articles, 15 were selected for analysis, resulting in a sample size of 2,230 individuals. HDL-C, LDL-C, total cholesterol , and triglycerides were the main variables analyzed in the lipid profiles. A correlation was observed between these variables and some of the most relevant clinical events in the disease, including vaso-occlusive seizures and acute thoracic syndrome.

Conclusion

Lipid metabolism disorders, especially hypocholesterolemia and hypertriglyceridemia, are linked to clinical events observed in SCA, suggesting they play a relevant role in the multifactorial pathogenesis of this disease.

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Original Article

Exercise Testing In Patients with Sickle Cell Disease: Safety, Feasibility and Potential Prognostic Implication

Christiano Gonçalves de Araújo et al. Maria Betânia Solis Resende Julia Teixeira Tupinambás Rebeca Coeli Teodoro Maciel Dias … Flávio Coelho Barros Maria Carmen Melo Vasconcelos José Nelio Januário Antonio Luiz Pinho Ribeiro Maria Carmo P. Nunes

Abstract

Background

Patients with sickle cell disease (SCD) are at increased risk for cardiovascular complications. Exercise testing is used as a prognostic marker in a variety of cardiovascular diseases. However, there is a lack of evidence on exercise in SCD patients, particularly regarding its safety, feasibility, and possible prognostic role.

Objectives

We used the maximal treadmill test to determine safety and feasibility of the exercise testing in SCD patients. Additionally, the factors associated with exercise duration, as well as the impact of exercise-induced changes on clinical outcome, were also assessed.

Methods

One-hundred thirteen patients with SCD, who underwent exercise testing, were prospectively enrolled. A comprehensive cardiovascular evaluation, including echocardiography and B-type natriuretic peptide (BNP) levels, were obtained. The long-term outcome was a composite endpoint of death, severe acute painful episodes, acute chest syndrome, or hospitalization for other SCD-related complications. Cox regression analysis was performed to identify the variables associated with the outcome. A p-value<0.05 was considered to be statistically significant.

Results

The mean age was 36 ± 12 years (range, 18-65 years), and 62 patients were women (52%). Ischemic electrocardiogram and abnormal blood pressure (BP) response to exercise were detected in 17% and 9%, respectively. Two patients experienced pain crises within 48 hours that required hospitalization. Factors associated with exercise duration were age, sex, tricuspid regurgitation (TR) maximal velocity, and E/e’ ratio, after adjustment for markers of disease severity. During the mean follow-up of 10.1 months (ranging from 1.2 to 26), the endpoint was reached in 27 patients (23%). Independent predictors of adverse events were hemoglobin concentration, late transmitral flow velocity (A wave), and BP response to exercise.

Conclusions

Exercise testing in SCD patients who were clinically stable is feasible. Exercise duration was associated with diastolic function and pulmonary artery pressure. Abnormal BP response was an independent predictor of adverse events.

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Case Report

Sickle-cell Anemia and Latent Diastolic Dysfunction: Echocardiographic Alterations

André Fattori et al. Daniela Camargo Oliveira Roger Frigério Castilho Otávio Rizzi Coelho

Introduction

Sickle-cell anemia (SCA) is a disease that can cause systemic complications, such as multiple organ dysfunction due to vaso-occlusion and endothelial activation. The genetic cause of the disease is a substitution of the amino acid glutamic acid for valine in the position 6 of the beta globin chain. Stress factors in the vascular microenvironment (cellular dehydration, hypoxemia, increased corpuscular hemoglobin concentration, decreased red blood cell transit time in the microcirculation, and decreased blood pH) trigger intracellular hemoglobin polymerization, forming paracrystalline structures that cause sickling of erythrocytes and increased blood viscosity, hemolysis, and vaso-occlusion^,. Simultaneously, free hemoglobin in the plasma sequesters nitric oxide (NO), leading to decreased NO bioavailability and increased endothelial adhesion (in physiological conditions, NO inhibits of platelet aggregation, platelet activation, transcription of platelet adhesion proteins).

The increase in cardiac output (CO), the afterload reduction due to the peripheral vasodilation as a response to hypoxemia, the increase in blood viscosity secondary to the morphological alterations, and the loss of the deformability of the sickle red blood cells are factors involved with the systolic ventricular overload and progressive enlargement of the cardiac chambers^–.

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