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Right ventricular function in β-thalassemia children: comparing three-dimensional echocardiography with other functional parameters

Abstract

Background

Cardiomyopathy is a major cause of mortality and morbidity in beta-thalassemia major (β-TM), and its early detection is critical for prompt management. We aimed to evaluate right ventricle (RV) function in β-TM, in absence of cardiac symptoms, using 3D echocardiography, and compare it with other functional parameters.

Methods

Cross-sectional cohort study was conducted on 50 β-TM children with no cardiac manifestations and 50 healthy controls of matched age and sex. We evaluated RV function using; Fraction Area Change (2DE-RV FAC), Ejection Fraction (3D-RVEF), Tricuspid annular plane systolic excursion (TAPSE), Tissue Doppler imaging (TDI) systolic (S′) and diastolic (E′, A′), Myocardial performance index (MPI), and speckle tracking (2D-STE) of RV global longitudinal strain (LSS), systolic strain rate (SSR), early diastolic strain rate (DSR E), and late diastolic strain rate (DSR A).

Results

3D-RVEF, MPI, and 2D-STE showed significant differences between the two groups. ROC curve analysis measurements had an AUC above 0.7—which indicate at least a fair discriminatory power between the β-TM group with RV dysfunction and normal controls.

Conclusions

β-TM patients have decreased RV function indices at a pre-symptomatic stage. Early detection of RV dysfunction is feasible and can allow for closer follow-up to detect pre-clinical changes.

Impact

  • The key message of this article is to emphasize the importance of the evaluation of right ventricular function in children with beta-thalassemia major.

  • This article adds to the existing literature the use of recent echocardiographic modalities as three-dimensional echocardiography and two-dimensional speckle tracking in the evaluation of right ventricular dysfunction in beta-thalassemia children while cardiac affection is still subclinical.

  • Impact: Recent echocardiographic functional parameters can be effectively used to evaluate the right ventricle in beta-thalassemia and can detect dysfunction in asymptomatic patients allowing for early intervention.

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Fig. 1: Example of a three-dimensional echocardiographic reconstruction of the delineation of the right ventricle seen from the septal side.
Fig. 2: ROC curve analysis of right ventricular function using different echocardiographic parameters.

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Authors and Affiliations

Authors

Contributions

O.A.T. provided the research idea and initiated the study design and E.E.E. and A.A.E. helped with implementation. O.A.T. and A.H.S. were responsible for the technical part of the study. All authors substantially contributed to the acquisition, analysis, and interpretation of data for the manuscript and drafting, revising, and critically reviewing the manuscript for important intellectual content. All authors approved the final version of this manuscript to be published and agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.

Corresponding author

Correspondence to Eslam E. Elhawary.

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The authors declare no competing interests.

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Informed written consent was obtained from the guardian of every child enrolled in the study (both patients and controls).

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Elhawary, E.E., Tolba, O.A., Elkaffas, A.A. et al. Right ventricular function in β-thalassemia children: comparing three-dimensional echocardiography with other functional parameters. Pediatr Res 91, 1709–1714 (2022). https://doi.org/10.1038/s41390-021-01900-0

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