The presence of late gadolinium enhancement (LGE) on cardiac magnetic resonance imaging (MRI) before the age of 14 years in patients with Duchenne muscular dystrophy (DMD) is associated with a faster rate of cardiac decline, according to a study published in Pediatric Cardiology.
Patients with an earlier onset of LGE had a more rapid reduction in left ventricular fractional shortening per year than patients without LGE before the age of 14 years (-1.11% vs -0.57%; P =.004). This correlated with a lower mean fractional shortening value after age 14 in the early-onset LGE group than that in the control DMD group (24.89% vs 29.78%; P =.028).
Treatment initiation was also earlier in the early-onset LGE group than in the control DMD group. Angiotensin-converting enzyme (ACE) inhibitors were started at a median age of 8.4 years compared to 10.56 years for controls (P =.025). The median age at initiation was also lower for mineralocorticoid receptor antagonists (13.4 years vs 19.1 years; P =.0024) and beta blockers (13.4 years vs 19.1 years; P =.0017).
“Aggressive augmentation of cardioprotective drugs in these patients who demonstrate early LGE does not prevent development of cardiac dysfunction but may potentially slow the course of cardiomyopathy,” the authors said. “Early assessment by cMRI provides valuable prognostic information to help counsel families and guide medical decisions for young patients with DMD.”
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Other clinical factors that might affect the severity of cardiac disease were not found to be significantly different between the early LGE group and the control DMD group, including the age at loss of ambulation (P =.31), presence of obesity (P =.32), and heart rate (P >.8).
Forty-one patients were included in the study, 15 of whom had LGE prior to the age of 14 years. This indicates that this risk factor may be present in roughly one-third of DMD patients. Because of this, the authors recommend that at least 1 cardiac MRI scan is performed before 14 years of age to help in the risk stratification of patients.
James L, Menteer J, Moss LC, et al. Early-onset late gadolinium enhancement is a prognostic factor for Duchenne cardiomyopathy. Pediatr Cardiol. Published online September 3, 2022. doi:10.1007/s00246-022-02989-8