Key result
Lower 4D CMR basal circumferential and surface area strain links to faster DMD cardiomyopathy progression.
Why the study?
Does 4D CMR regional strain analysis predict cardiomyopathy progression in pediatric patients with Duchenne muscular dystrophy?
Cohort (n=66)
Does 4D CMR regional strain analysis predict cardiomyopathy progression in pediatric patients with Duchenne muscular dystrophy?
p-value: p=<0.01
Novel 4D CMR regional strain metrics, including peak systolic strain and late diastolic strain rate, can identify pediatric DMD patients at high risk for rapid cardiomyopathy progression before changes in standard LVEF or LGE occur.
Basal 4D CMR strain metrics may enable early risk stratification in pediatric DMD; leaves open prospective validation before clinical adoption.
Cardiomyopathy (CMP) is the leading cause of death in Duchenne muscular dystrophy (DMD). Characterization of disease trajectory can be challenging, especially in early stages of CMP where onset and progression may vary. Traditional metrics from cardiovascular magnetic resonance (CMR) imaging such as LVEF (left ventricular ejection fraction) and LGE (late gadolinium enhancement) are often insufficient for assessing the pace of disease progression. We hypothesized that strain patterns from a novel 4D (3D+time) CMR regional strain analysis method can be used to predict DMD CMP progression. We compiled 190 short-axis cine CMR image stacks for n=66 pediatric DMD patients (13.3 [10.8-16.5] years; median [interquartile range]) imaged for 3 consecutive years and computed regional strain metrics using custom-built feature tracking software. We measured regional strain parameters from the generated 4D endocardial surface mesh. Using LVEF decrease, measured two years following the initial scan, we classified patients into slow (ΔLVEF%<5; n=35) or fast (ΔLVEF%≥5; n=30) progressing groups. There was no statistical difference between the slow and fast-progressing groups in terms of standard metrics such as age, LVEF, or LGE status. However, peak basal circumferential strain (E cc ) and surface area strain (E a ) magnitudes were decreased in fast progressors ( p <0.01 for all). Basal E cc late diastolic strain rate and basal E a late diastolic strain rate magnitude were also significantly decreased in fast progressors ( p <0.01 for all). Regional strain metrics from 4D CMR can be used to differentiate between slow or fast CMP progression in a longitudinal DMD cohort. Clinical Perspective: Cardiomyopathy is the number one cause of death in Duchenne muscular dystrophy (DMD), but the onset and progression of the disease are variable and heterogeneous. Currently, there are no reliable methods used clinically that can identify DMD patients at higher risk of cardiomyopathy progression. In this study, we used a novel 4D cardiovascular magnetic resonance regional strain analysis method to evaluate longitudinal cardiomyopathy progression in 66 pediatric DMD patients over three consecutive annual visits. From our analysis, we found that peak systolic strain and late diastolic strain rate from 4D analysis were early indicators of cardiomyopathy progression. This method offers promise for early detection and monitoring, potentially improving patient outcomes through timely intervention and management.
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Earl et al. (2025) conducted a cohort in Duchenne muscular dystrophy cardiomyopathy (n=66). 4D CMR regional strain analysis vs. Standard CMR metrics was evaluated on Cardiomyopathy progression (ΔLVEF% ≥5 over two years) (p=<0.01). Peak basal circumferential strain and surface area strain magnitudes from 4D CMR were significantly decreased in fast-progressing DMD cardiomyopathy patients compared to slow progressors (p<0.01).
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