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Dystrophin-Associated Cardiomyopathy Risk Index

  • Patient current age (years)
  • Most recent LVEF (%)
  • Age when LVEF first < 55% (years) (enter numeric age or leave blank if never <55%)
  • Age when LVEF first < 40% (years) (enter numeric age or leave blank if never <40%)
  • LGE present on CMR?
  • If LGE present, Global Severity Score (GSS) 0–4 (see reference figure)
  • Age when LGE first detected (years) (enter numeric age or leave blank if never)
  • Explanation — Dystrophin-Associated Cardiomyopathy Risk Index (imaging-based)
    This tool implements an imaging-based composite risk index developed to stratify patients with dystrophin-associated cardiomyopathy (principally Duchenne muscular dystrophy, DMD) by combining the timing and severity of left ventricular dysfunction with the timing and severity of myocardial fibrosis detected by late gadolinium enhancement (LGE) on cardiac magnetic resonance (CMR). The index assigns points for earlier onset of LV systolic dysfunction (<55% and <40% thresholds) and for earlier onset and greater extent of LGE (Global Severity Score, GSS). Points from the LVEF domain and LGE domain are summed to form the composite risk score; higher scores indicate earlier, more severe cardiac involvement and were associated with higher mortality in the original cohort. In the published cohort the median score was 3 (IQR 2–5), scores were stable over time for most subjects, and no deaths occurred in subjects with a score < 2. Clinically, the score is intended as an adjunct to guide surveillance intensity and therapeutic escalation (e.g., earlier initiation of guideline-directed heart failure therapy, closer follow-up, arrhythmia surveillance), not as an absolute rule for management decisions. Integrate with full clinical assessment including symptoms, ECG, biomarkers, ambulatory monitoring, and multi-disciplinary input.

    Reference:
    Starnes JR, Crum K, George-Durrett K, et al. Novel Cardiac Imaging Risk Score for Mortality Prediction in Duchenne Muscular Dystrophy. Pediatr Cardiol. 2024;45(6):1221–1231. doi:10.1007/s00246-022-03040-6. PMCID: PMC10151437.

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