Myocardial Strain Assessment for Early Duchenne Muscular Dystrophy Diagnosis in Pediatric Patients Using Cardiac MRI

Abstract

Assessing myocardial strain remains challenging, particularly in the pediatric population, due to the smaller heart sizes, higher heart rates, and variability in strain parameters compared to adult populations. This study aimed to investigate the utility of myocardial strain measurements using cardiac magnetic resonance-feature tracking (CMR-FT) for early diagnosis of Duchenne muscular dystrophy (DMD) in pediatric patients. Twenty-eight DMD patients and 20 healthy controls were involved in this study. Global circumferential, longitudinal, and radial strain (GCS, GLS, and GRS) were measured for the left ventricle (LV) using CMR-FT. Segmental strain values only of the inferolateral and anterolateral LV segments in DMD patients without late gadolinium enhancement (LGE) and DMD patients with LGE were compared to the healthy controls. Strain measurements using CMR-FT in DMD patients were considerably lower than those of healthy controls, with all p-values lower than 0.001. DMD patients without LGE showed decreased inferolateral and anterolateral segmental values only relative to healthy controls. The same behavior was maintained for the LV geometry. Multivariable linear regression demonstrated that the end-systole (ES) wall thicknesses and thickening were associated with decreased GCS and GLS. CMR-FT is crucial in detecting cardiac abnormalities in patients with DMD. It represents an innovative imaging biomarker that can detect initial myocardial alterations in DMD cardiomyopathy without relying on gadolinium.