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

Lopes, Andressa; Dantas, Marina Tejo; Ladeia, Ana Marice Teixeira

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Original Article • Arq. Bras. Cardiol. 119 (6) • Dec 2022 • https://doi.org/10.36660/abc.20220207 copy

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

Authorship SCIMAGO INSTITUTIONS RANKINGS

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.

Anemia, Sickle Cell; Pregnancy Complications, Cardiovascular; Hemoglobinopathies

Author	Year of publication	Title	Performance period	Place of performance	Study design	Sample size	Objective
Philippe M. Adjagba, Gaston Habib, Nancy Robitaille, et al	2016	Impact of sickle cell anaemia on cardiac chamber size in the paediatric population	Not mentioned	Canada	Retrospective cohort	n = 110	To describe the extent of myocardial abnormalities and determine hematologic indices that could primarily affect cardiac function in patients with sickle cell disease
Jamie K. Harrington, Usha Krishnan, Zhezhen Jin, et al	2017	Longitudinal Analysis of Echocardiographic Abnormalities in Children With Sickle Cell Disease	1994 - 2013	United States	Retrospective cohort	n = 172	To identify clinical and laboratory parameters associated with the development of cardiac abnormalities
Paul Guedeney, François Lionnet, Alexandre Ceccaldi, et al	2018	Cardiac manifestations in sickle cell disease varies with patient genotype	Maio 2008 Maio 2015	France	Retrospective cohort	n = 180	To describe cardiac remodeling and its correlations in patients with HbSC and to compare with patients with sickle cell anemia and with healthy individuals
Antoine Fakhry AbdelMassih, Khaled M. Salama, Carolyne Ghobrial, et al	2019	Discrepancy in patterns of myocardial involvement in beta-thalassaemia vs. sickle cell anaemia	April 2017 October 2018	Egypt	Case control	n = 120	To compare left ventricular mechanics in thalassemia and sickle cell patients using the latest 2D spot tracking analysis software

Author/year of publication	Population	Analyzed cardiovascular parameter	Cardiovascular alteration found	Conclusion
Philippe M. Adjagba et al. (2016)	110 patients with sickle cell disease (72 HbSS; 32 HbSC; 6 HbSβ – thalassemia)	RVV LVV LVM FSLV E/e´ relation MPI	RVD LVD Diastolic dysfunction Systolic dysfunction Abnormality in LVM	LVD was higher in patients with SCA (HbSS) than in patients with SC hemoglobinopathy (HbSC); LVH was observed only in patients with SCA (HbSS) and the abnormality in LVM was more prevalent in this group of patients; RVD, FSLV and E/e´ relation were similar between the patients with SCA (HbSS) and the individuals with SC hemoglobinopathy (HbSC).
Jamie K. Harrington et al. (2017)	172 patients with sickle cell disease (117 HbSS; 41 HbSC; 5 HbSβᴼ – thalassemia; 9 HbSβ⁺ – thalassemia)	LVM LVESD LVEDD FSLV TRV	LVH LVD at the end of the systole and at the end of the ao final da sístole e ao final da diastole ↓EF OF LV ↑TRV	Patients HbSS e HbSβ0-thalassemia were more likely to develop LVH, LVD at the end of the sistole and at the end of the diastole. The chance of developing increased TRV and decrease of FSLV were similar between all genotypes involved in this study.
Paul Guedeney et al. (2018)	120 patients with sickle cell disease (60 HbSS; 60 HbSC) and 60 healthy patients	LVEDD/BS LVM/BS LVM/H LVEDV/BS CI TRV EFLV EM wave A wave E/A relation DT e´ wave E/e´ relation LAV/BS	LVD at the end of diastole LAD LVH ↑CI ↑TRV ↑E/e´relation LVDD	LAD, LVD e CI were higer in HbSS patients than in HbSC patients and the controls; LVH, increase of TRV and diastolic dysfunction disfunção of LV were more frequent in HbSS patients than in HbSC patients and the controls (HbSS patients had higher: E wave, E/A relation, DT, e´wave, E/e´ relation); LAD, LVD, LVM/BS, LVM/H, E/e´ relation were higer in HbSC patients than in the control; e´ wave was smaller in HbSC patients than in controls; CI e TRV were similar between HbSC patients and the controls; EFLV was similar between the 3 groups.
Antoine Fakhry AbdelMassih et al. (2019)	40 patients with sickle cell anemia (HbSS), 40 patients with beta thalassemia major (β0/β0) and 40 healthy patients	EFLV FSLV E/e´ relation GLS GLS epicardial SGL endocardial T2* miocardyal	Myocardial iron overload ↓GLS Subendocardial dysfunction Subepicardial dysfunction	T2* myocardial was higher in patients with beta thalassemia major than in patients with SCA; GLS was similar between patients with beta thalassemia major and those with SCA, but both groups of patients had GLS reduced in comparision with healthy individuals; Epicardial GLS was lower in patients with beta thalassemia major than in patients with SCA; Endocardial GLS was lower in patients with SCA than in patients with beta thalassemia major; The systolic function and the diastolic function of left ventricle were normal and similar between the 3 groups.

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[1] Mailing Address: Andressa Lopes • Escola Bahiana de Medicina e Saúde Pública – Avenida Dom João VI, 275. Postal Code 40290-000, Salvador, BA – Brazil E-mail: andressalopes18.1@bahiana.edu.br