Abstract
BackgroundIn 2005, the American Academy of Pediatrics (AAP) published a policy statement on cardiovascular health supervision for Duchenne Muscular Dystrophy (DMD). Data is lacking on its effect on cardiovascular care. MethodsUsing the infrastructure of the Muscular Dystrophy Surveillance, Tracking and Research Network, we examined a population-based sample of individuals with DMD, focusing on cardiac testing and prescription of cardiac medication. Comparisons were made pre- (2000–2005) and post- (2006–2011) publication Results731 cases were studied; mean age of diagnosis 3.8 years and mean duration of follow-up 9.3 years. There was a significant decrease in time from diagnosis to first electrocardiogram (ECG) and echocardiogram (ECHO) from pre- to post-publication; 1047 (95% CI: 905–1188) days to 411 (95% CI: 324–498) days for ECG (p < 0.01) and 1153 (95% CI: 1022–1284) days to 352 (95% CI: 280–424) days for ECHO (p < 0.01). Only 4.5% and 8.2% of those ≥10 years of age met the recommendation for yearly ECG and ECHO respectively. Prescription of a cardiac medication with an abnormal ECHO increased post-publication 91/125, 72.8% versus pre- 47/77, 61.0% (p = 0.08). After 2005, cases with a normal ECHO were 2.3 (95% CI 1.23–4.31) times more likely to be prescribed a cardiac medication (p < 0.01). ConclusionsSince publication of the AAP statement, DMD patients are having cardiac testing sooner and cardiac medication is prescribed more often. However, the majority are not having testing with recommended frequency. Overcoming barriers to recommended cardiac care may lead to a better understanding of the natural history of cardiomyopathy in DMD and better treatment strategies.
Published Version
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