Effects of angiotensin-converting enzyme inhibitors and/or beta blockers on the cardiomyopathy in Duchenne muscular dystrophy.

Authors:
Laurence Viollet
Laurence Viollet
Nationwide Children's Hospital
Philip T Thrush
Philip T Thrush
Nationwide Children's Hospital
United States
Kevin M Flanigan
Kevin M Flanigan
Nationwide Children's Hospital
United States
Jerry R Mendell
Jerry R Mendell
Center for Gene Therapy
Houston | United States
Hugh D Allen
Hugh D Allen
The Ohio State University
United States

Am J Cardiol 2012 Jul 29;110(1):98-102. Epub 2012 Mar 29.

Nationwide Children's Hospital, Columbus, OH, USA.

Cardiomyopathy is a consequence of Duchenne muscular dystrophy (DMD). Suggested treatments include angiotensin-converting enzyme (ACE) inhibitors and/or β blockers (BBs), but few large series have been reported. We 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 (r(2) = 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. 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. In conclusion, treatment with ACE inhibitor or ACE inhibitor plus BB can delay progression of cardiomyopathy.

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http://dx.doi.org/10.1016/j.amjcard.2012.02.064DOI Listing

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July 2012
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