Eslam E Elhawary1, Osama A Tolba2, Asmaa A Elkaffas3, Ahmed H Shabana2. 1. Hematology and Bone Marrow Transplantation Unit, Pediatrics Department, Faculty of Medicine, Tanta University, Tanta, Egypt. eslam.elhawary@med.tanta.edu.eg. 2. Cardiology Unit, Pediatrics Department, Faculty of Medicine, Tanta University, Tanta, Egypt. 3. Hematology and Bone Marrow Transplantation Unit, Pediatrics Department, Faculty of Medicine, Tanta University, Tanta, Egypt.
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.
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.