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

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.