Speckle tracking evaluation of right ventricular functions in children with sickle cell disease

Tolba, Osama A.; El-Shanshory, Mohamed; El-Gamasy, Mohamed Abdelaziz; El-Shehaby, Walid Ahmed

doi:10.4103/apc.apc_140_16

Cited by 5 publications

(1 citation statement)

References 14 publications

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“…Our findings were consistent with Tolba et al who reported significant difference in diastolic parameters (E,A, E/A) by TDI of SCD asymptomatic children versus control [35]. Similarly, Charaf El Dein et al [36].…”

Section: Discussionsupporting

confidence: 92%

Evaluation of Cardiac Fibrosis and Subclinical Cardiac Changes in Sickle Cell Children by Magnetic Resonance Imaging, Echocardiography and Serum Galectin-3

Wagdy

Fathy

Elnekidy

et al. 2023

Preprint

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Cardiovascular complications account for about one third of the premature mortality rate in patients with sickle cell disease (SCD). Underlying this cardiac risk is a progressive cardiomyopathy which is multifactorial. Recently myocardial fibrosis is assumed to be a novel mechanism for cardiac dysfunction. This study aimed to detect cardiac fibrosis and subclinical cardiac changes in sickle cell children by; cardiac magnetic resonance imaging (CMR), Tissue Doppler Echocardiography (TDE) and Galectin-3. Children with SCD were subjected to assessment of cardiac function by TDE and had estimated serum level of Gal-3. Cardiac MRI was used to assess volumes and function by cine sequence, LGE for detection of focal myocardial fibrosis, native T1 mapping and extracellular volume (ECV) for quantification of diffuse myocardial fibrosis as well as CMR T2* for myocardial iron load. Thirty-four childrenwith SCD (mean age 13.32 ± 3.24 years) were enrolled in our study, another thirty-four healthy children served as controls. Although myocardial iron load by T2* was normal, ECV was increased among all cases with mean level 35.41 ± 5.02 %. The mean of ECV was significantly associated with the frequency of VOCs (P= 0.017) and negatively correlated to hemoglobin level (P=0.005). In consistent with ECV, Galectin-3 level was significantly higher among cases when compared to controls (7.75 ± 1.86 ng/ml versus 6.04 ±1.64 ng/ml, p<0.001) with cutoff value over the ROC curve 6.5ng/ml, sensitivity; 82.5 %, specificity; 72.8%. ECV were significantly higher for cases with Tie index > 0.4 by TDI subgroup when compared to ECV of subgroup with Tie index < 0.4 (38.17 ± 4.25 versus 34.43 ± 5.02, P: 0.03*). In conclusion: ECV is a validated CMR parameter to detect and quantify diffuse interstitial myocardial fibrosis in asymptomatic children with SCD. Cardiac fibrosis in SCD children is associated with frequent attacks of ischemic re-perfusion injury and correlated to the severity of anemia rather than myocardial iron load. Tie index by TDE and serum Gal-3 are recommended screening tools.

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Section: Discussionsupporting

confidence: 92%

Evaluation of Cardiac Fibrosis and Subclinical Cardiac Changes in Sickle Cell Children by Magnetic Resonance Imaging, Echocardiography and Serum Galectin-3

Wagdy

Fathy

Elnekidy

et al. 2023

Preprint

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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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Evaluation of cardiac fibrosis and subclinical cardiac changes in children with sickle cell disease using magnetic resonance imaging, echocardiography, and serum galectin-3

Wagdy,

Fathy,

Elnekidy

et al. 2023

Pediatr Radiol

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Background Myocardial fibrosis has recently been proposed as one of the contributing factors to the diverse pathogenicity of cardiomyopathy in sickle cell disease. Objective In this study, cardiac fibrosis and subclinical cardiac changes in children with sickle cell disease were evaluated using cardiac magnetic resonance imaging (MRI), tissue Doppler echocardiography and serum galectin-3. Materials and methods The study included 34 children with sickle cell disease who were compared with a similar number of healthy controls. Cardiac MRI was used to evaluate late gadolinium enhancement, native T1 mapping, extracellular volume, and T2* for estimation of iron load. Cardiac function and myocardial performance index (MPI, evaluated by tissue Doppler echocardiography) and serum galectin-3 were compared to controls. Results The mean age of the included patients was 13.3 ± 3.2 years. Myocardial iron load by T2* was normal. The mean level of extracellular volume (35.41 ± 5.02%) was significantly associated with the frequency of vaso-occlusive crises (P = 0.017) and negatively correlated with hemoglobin levels (P = 0.005). Galectin-3 levels were significantly higher among cases than controls (P = 0.00), at a cutoff value on the receiver operating characteristic curve of 6.5 ng/ml, sensitivity of 82.5% and specificity of 72.8%. The extracellular volume was significantly higher in cases, with a MPI > 0.4. Conclusion Diffuse interstitial myocardial fibrosis can be detected early in children with sickle cell disease using T1 mapping and is associated with a high frequency of vaso-occlusive crisis. MPI of the left ventricle and serum galectin-3 are recommended screening tools for subclinical cardiac abnormalities. Graphical abstract

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Speckle tracking evaluation of right ventricular functions in children with sickle cell disease

Cited by 5 publications

References 14 publications

Evaluation of Cardiac Fibrosis and Subclinical Cardiac Changes in Sickle Cell Children by Magnetic Resonance Imaging, Echocardiography and Serum Galectin-3

Evaluation of Cardiac Fibrosis and Subclinical Cardiac Changes in Sickle Cell Children by Magnetic Resonance Imaging, Echocardiography and Serum Galectin-3

Sickle cell disease and ventricular myocardial strain: A systematic review

Evaluation of cardiac fibrosis and subclinical cardiac changes in children with sickle cell disease using magnetic resonance imaging, echocardiography, and serum galectin-3

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