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Effects of angiotensin-converting enzyme inhibitors and/or beta blockers on the cardiomyopathy in duchenne muscular dystrophy
The American Journal of Cardiology, 07/11/2012  Clinical Article

Viollet L et al. – Treatment with angiotensin–converting enzyme (ACE) inhibitor or ACE inhibitor plus β blocker (BB), can delay progression of cardiomyopathy.

Methods
  • Authors present 42 patients with DMD and cardiomyopathy treated with an ACE inhibitor or an ACE inhibitor plus a BB.
  • Serial echocardiograms were recorded. Adequate ejection fractions (EFs) were obtained at initiation of therapy (EF <55%).
  • ACE inhibitor dosage adjustments were made if a continued decrease in EF was noted.
  • BB therapy was initiated when average heart rate on Holter monitoring exceeded 100 beats/min.
  • Data were analyzed using paired t test and linear regression.
  • Before ACE inhibition, patients (n = 22) demonstrated decreased EF over time (r2 = 0.23).
  • At ACE inhibitor therapy initiation, mean age was 14.1 ± 4.6 years and mean EF was 44.2 ± 6.8%.
  • BB therapy was used in 24 of 42 patients.
  • Mean age for the ACE inhibitor + BB group was 15.7 ± 3.9 years.

Results
  • The 2 groups showed significant improvement (p <0.0001 for ACE inhibitor and ACE inhibitor plus BB) compared to the pretherapy group.
  • No significant differences were noted between treatment groups.
  • Patients with DMD demonstrated a gradual decrease in myocardial function.
  • Treatment with ACE inhibitor or ACE inhibitor plus BB resulted in significant improvement compared to pretherapy.
  • No significant difference occurred in EF improvement between treatment groups.

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