Changes in Provision of Recommended Cardiac Care for Duchenne Muscular Dystrophy Using a Longitudinal Cohort

Cardiac disease remains the leading cause of mortality in Duchenne muscular dystrophy (DMD), spurring release of cardiovascular supervision guidelines in 2005, 2010, 2018 and most recently, 2025. Little is known about changes in cardiac care after the updates to cardiac care recommendations for DMD. This study compares provision of recommended cardiac care before and after publication of the 2010 DMD Care Considerations.

Data from MD STARnet for individuals with DMD with at least one visit between March 1, 2010, through March 31, 2016 were used to assess frequency of cardiac visits stratified by age interval, diagnosis age, or left ventricular function (LVF) severity status. Data compared care received from February 2005 through February 2010 and March 2010 through March 2016.

We assessed data for 922 individuals. Key findings overall demonstrate a significant increase in baseline assessments at diagnosis (39.5%-52.4%, p < 0.05), asymptomatic follow-up cardiac assessments every other year through 10 (22.1%-36.0%, p < 0.001) and annually age 11+ years (6.0%-18.1%, p < 0.001) and semi-annually for individuals with LVF abnormality (0.6%-1.4%, p < 0.01). There was no change in LVF assessment following new onset sinus tachycardia (ST). Arrhythmia, excluding ST, triggered investigation with a Holter in the post-guidelines period only (14.2%).

We show increases in timing and frequency of cardiac care following publication of the 2010 Care Considerations. Areas for future research include understanding the relationship between adherence and cardiac outcomes, and barriers to receiving guideline-based care to ensure equitable DMD management.