Why the study?
Myocardial involvement is a leading cause of mortality in DMD and often progresses subclinically due to limited physical activity.
Multiparametric CMR, including quantitative mapping and strain analysis, allows for detailed myocardial characterization in DMD, potentially facilitating early therapeutic intervention.
Case illustrates subclinical LV dysfunction detectable by CMR strain in DMD; leaves open standardized screening protocols.
Duchenne muscular dystrophy (DMD) is a progressive X-linked disorder characterized by degeneration of both skeletal and cardiac muscles. Myocardial involvement is a leading cause of mortality and often progresses subclinically due to patients' limited physical activity. We report the case of a 15-year-old boy with DMD who exhibited asymptomatic left ventricular dysfunction. Cardiac magnetic resonance imaging (CMR) revealed diffuse systolic impairment with reduced circumferential strain. Multiparametric CMR demonstrated elevated native T1 and extracellular volume (ECV), indicative of diffuse myocardial fibrosis, along with prolonged T2 values suggestive of myocardial edema. Subepicardial fibrosis was identified on late gadolinium enhancement (LGE) imaging. These findings demonstrate the utility of advanced CMR techniques, including quantitative myocardial mapping and myocardial strain analysis, for comprehensive evaluation of DMD-associated cardiomyopathy. Multiparametric CMR allows for detailed myocardial characterization and may facilitate early therapeutic intervention prior to the onset of overt heart failure, thereby improving clinical management in patients with DMD.
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Takamure et al. (2025) studied this question.
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