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Rate of Change in Cardiac Magnetic Resonance Imaging Measures Is Associated With Death in Duchenne Muscular Dystrophy

Joseph R Starnes1, Meng Xu2, Kristen George-Durrett1

  • 1Division of Cardiology, Department of Pediatrics Vanderbilt University Medical Center Nashville TN USA.

Journal of the American Heart Association
|April 30, 2024
PubMed

Insights

Cardiovascular disease worsens faster in Duchenne muscular dystrophy patients who die. Tracking changes in ejection fraction and ventricular volumes can identify high-risk individuals and serve as trial outcomes.

Area of Science:

  • Cardiology
  • Genetics
  • Neurology

Background:

  • Cardiovascular disease is the primary cause of mortality in Duchenne muscular dystrophy (DMD).
  • Early identification of patients at high risk for mortality is crucial for timely intervention and intensive management.
  • Identifying predictive markers can establish surrogate outcomes for clinical trials in DMD.

Purpose of the Study:

  • To investigate cardiac magnetic resonance imaging (CMR) parameters that predict mortality in Duchenne muscular dystrophy patients.
  • To identify specific rates of change in cardiac function and structure associated with increased mortality risk.
  • To evaluate the potential of these changes as surrogate endpoints in therapeutic trials.

Main Methods:

  • Prospective enrollment of Duchenne muscular dystrophy subjects in observational studies.
  • Utilized generalized least squares models to compare CMR measurements between deceased and surviving participants.
  • Analyzed data from 63 participants with multiple CMR assessments over a median follow-up of 5 years.

Main Results:

  • Twelve participants (19.1%) died during the follow-up period.
  • Deceased subjects exhibited a faster rate of decline in left ventricular ejection fraction (P<0.0001).
  • Increased rates of left ventricular end-diastolic (P=0.0132) and systolic (P<0.0001) volumes were observed in deceased subjects.
  • Faster progression of mid-circumferential strain was noted in deceased individuals (P=0.049), but global circumferential strain showed no significant difference.
  • Rates of change in late gadolinium enhancement, base T1, and mid T1 did not differ between the groups.

Conclusions:

  • The rate of change in left ventricular ejection fraction, mid-circumferential strain, and ventricular volumes are significantly associated with mortality in Duchenne muscular dystrophy.
  • Intensified medical therapies aimed at slowing disease progression may reduce mortality rates in DMD patients.
  • A reduced rate of cardiac progression could serve as a valid surrogate outcome measure in clinical trials for Duchenne muscular dystrophy.
Abstract

Keywords:
follow‐up studiesgadoliniumglobal longitudinal strainmagnetic resonance imagingmuscular dystrophies

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