Discrepancy in patterns of myocardial involvement in beta-thalassaemia vs. sickle cell anaemia

AbdelMassih, Antoine; Salama, Khalid M.; Ghobrial, Carolyne; Haroun, Basma; Rahman, Mohamed Abdel

doi:10.1080/00015385.2019.1610836

Cited by 8 publications

(16 citation statements)

References 26 publications

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“…This contradicts the fixed cliché about non-reduced strains in patients with SCD and underlines the neglected discrepancy between different phenotypes. This might also explain the observed ventricular dysfunction in the study by AbdelMassih et al [ 18 ], whereby the HED phenotypes develop vascular stiffness due to intravascular hemolysis that can impair the subendocardial function of both ventricles. Moreover, RV dysfunction was marked in the HED group and no data are available that can be compared with our series.…”

Section: Resultsmentioning

confidence: 90%

“…Several studies, such as those by Barbosa et al and Ahmad et al [ 6 , 7 ], have concluded that ventricular strains are not reduced but might be increased in cases with SCD. A recent study by AbdelMassih et al [ 18 ] showed that left ventricular strains are reduced in patients with SCD mainly in the subendocardium with overall preserved left ventricular global longitudinal strain. AbdelMassih et al [ 18 ] compared the pattern of left ventricular dysfunction in thalassemia with SCD and proved that the patients with SCD experience early subendocardial involvement.…”

Section: Resultsmentioning

confidence: 99%

“…A recent study by AbdelMassih et al [ 18 ] showed that left ventricular strains are reduced in patients with SCD mainly in the subendocardium with overall preserved left ventricular global longitudinal strain. AbdelMassih et al [ 18 ] compared the pattern of left ventricular dysfunction in thalassemia with SCD and proved that the patients with SCD experience early subendocardial involvement. The latter finding raises the suspicion of possible lack of vascular relaxibility in SCD as an underlying pathogenesis for the observed dysfunction.…”

Section: Resultsmentioning

confidence: 99%

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Right Ventricular Functions in Subphenotypes of Sickle Cell Disease

Youssry¹,

Shaltout²,

AbdelMassih³

et al. 2020

Journal of the Saudi Heart Association

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Objectives Sickle cardiomyopathy is the most important cause of death in patients with sickle cell disease (SCD). Based on recent evidence, SCD can be divided into two subphenotypes, namely, the viscosity vasoocclusion (VVO) subphenotype and the hemolysis endothelial dysfunction (HED) subphenotype. The aim of our series is to study right ventricular (RV) functions in both subphenotypes. Methods Echocardiography including conventional and tissue Doppler imaging as well as speckle tracking echocardiography was performed in 50 patients (23 from the VVO subgroup and 27 from the HED subgroup) based on a serum lactate dehydrogenase (LDH) level below or above 270 U/L, respectively, and in 50 controls. Reticulocyte count and hemoglobin levels were assessed in different groups of patients. Results The HED subgroup showed RV dysfunction. Patients in this subgroup also showed systolic and diastolic functions similar to those seen in the VVO subgroup and controls. In addition, a tight correlation exists between LDH and both RV global longitudinal strain (−0.68) and RV E/E′ ratio (0.9), defined as the ratio of early diastolic tricuspid inflow velocity to tricuspid annular early diastolic velocity. Conclusions Results reveal a marked discrepancy in RV functions between HED and VVO subphenotypes of SCD, with patients in the former subgroup being more prone to RV dysfunction. This warrants early screening of such patients in daily practice.

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Section: Resultsmentioning

confidence: 90%

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

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Right Ventricular Functions in Subphenotypes of Sickle Cell Disease

Youssry¹,

Shaltout²,

AbdelMassih³

et al. 2020

Journal of the Saudi Heart Association

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“…was retrospective, and one (Mah et al.) was experimental 15,31–43,45,47 . Most studies obtained written consent and approval through an institutional review board or ethics committee.…”

Section: Resultsmentioning

confidence: 99%

“…A growing number of studies have evaluated myocardial strain in individuals with SCD, but collective results have not yet been summarized. This systematic review is warranted to synthesize reported findings and remaining gaps in knowledge 15,31–47 …”

Section: Introductionmentioning

confidence: 99%

Sickle cell disease and ventricular myocardial strain: A systematic review

Whipple

Joshi

Naik

et al. 2021

Pediatric Blood & Cancer

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Cardiac disease is the primary cause of death in sickle cell disease (SCD). Cardiac abnormalities begin in childhood and progress throughout life. Right and left ventricular (RV, LV) myocardial strain are early markers of systolic dysfunction but are not well investigated among individuals with SCD. The objectives of this review were to (1) identify all published studies that have evaluated ventricular myocardial strain, (2) summarize their values, and (3) compare findings with those obtained from controls. From search results of four electronic databases—Medline, Embase, Scopus, and Web of Science—42 potential articles were identified, of which 18 articles and 17 studies met eligibility criteria for inclusion. The evaluated studies demonstrate that RV and LV myocardial strain are generally abnormal in individuals with SCD compared with controls, despite having normal ejection/shortening fraction. Myocardial strain has been inconsistently evaluated in this population and should be considered any time an echocardiogram is performed.

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Speckle tracking echocardiography and β-thalassemia major. A systematic review

Patsourakos,

Aggeli,

Dimitroglou

et al. 2023

Ann Hematol

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Heart disease is among the primary causes of morbidity and mortality in β-thalassemia major (β-TM). Conventional echocardiography has failed to identify myocardial dysfunction at an early stage among these patients, thus speckle tracking echocardiography (STE) has been lately used. The objectives of this review were to 1) identify all published studies having evaluated myocardial strain among β-TM patients, 2) gather their results, 3) compare their findings and 4) propose recommendations based on these data. Literature search was conducted in PubMed, SCOPUS and Cohrane Library. Data regarding left ventricular global longitudinal (LV-GLS), circumferential (LV-GCS) and radial strain (LV-GRS), right ventricular longitudinal strain (RV-GLS), left and right atrial strain were extracted. Thirty-five studies (34 original articles and 1 meta-analysis) have met the inclusion criteria. LV-GLS has been reported being worse in patients compared to controls in 13 of 21 studies, LV-GCS in 7 of 11 studies, LV-GRS in 6 of 7 studies, RV-GLS in 2 of 3 studies and left atrial strain in all case–control studies. Myocardial iron overload (MIO) patient subgroups had worse LV-GLS in 6 of 15 studies, LV-GCS in 2 of 7 studies and LV-GRS in none of 7 studies. A small number of studies suggest left atrial strain correlation with electrical atrial ectopy and atrial fibrillation. It is suggested that STE should be applied supplementary to conventional echocardiography for early identification of myocardial dysfunction among β-TM patients. Potential myocardial strain utilities could be screening for myocardial iron overload, left ventricular diastolic dysfunction and atrial fibrillation.

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Discrepancy in patterns of myocardial involvement in beta-thalassaemia vs. sickle cell anaemia

Cited by 8 publications

References 26 publications

Right Ventricular Functions in Subphenotypes of Sickle Cell Disease

Right Ventricular Functions in Subphenotypes of Sickle Cell Disease

Sickle cell disease and ventricular myocardial strain: A systematic review

Speckle tracking echocardiography and β-thalassemia major. A systematic review

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