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© The European Society of Cardiology 2007. All rights reserved. For Permissions, please e-mail: journals.permissions@oxfordjournals.org

Perindopril preserves left ventricular function in X-linked Duchenne muscular dystrophy

Denis Duboc1,*, Christophe Meune1, Bertrand Pierre1, Karim Wahbi1,2, Bruno Eymard2, Annick Toutain3, Carole Berard4, Guy Vaksmann5 and Henri-Marc Bécane2

1 Department of Cardiology, Cochin Hospital, APHP, Paris V René Descartes University, 27 rue du Fg St-Jacques, Paris 75014, France
2 Myology Institute, Pitié-Salpétrière Hospital, Paris, France
3 Department of Genetics, Bretonneau University Hospital, Tours, France
4 Department of Pediatric Rehabilitation, Lyon-Sud Hospital, Lyon, France
5 Department of Pediatrics, Cardiology Hospital, Lille, France

* Corresponding author. Tel: +33 1 58 41 16 56; fax: +33 1 58 41 16 05. E-mail address: denis.duboc{at}cch.aphp.fr

Duchenne muscular dystrophy (DMD), an X-linked disorder due to a lack of dystrophin, is associated with muscle weakness and myocardial dysfunction. DMD children between the ages of 9.5 and 13 years with normal left ventricular ejection fraction were included in this prospective study. They were randomly assigned for 3 years to perindopril 2–4 mg (group 1) or placebo (group 2) in a double-blind protocol, followed by open-label treatment with perindopril for all patients. Left ventricular function was compared between the two groups at 5 years. A total of 28 patients were assigned to group 1 and 29 patients were assigned to group 2. Baseline characteristics were similar in both groups. At the end of the 5-year follow-up period, eight patients had an ejection fraction under 45% in the placebo group vs. one patient in the perindopril group (P < 0.02). All patients were alive in the perindopril group and three had died in the placebo group (P = 0.07). Early initiation of treatment with perindopril is associated with a preservation of left ventricular function in DMD children and with a trend towards a lower mortality.

Key Words: Duchenne muscular dystrophy • Cardiac dysfunction • Cardiomyopathy • Perindopril • Angiotensin-converting enzyme inhibition


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Eur. Heart J. Suppl., September 1, 2007; 9(suppl_E): E1 - E1.
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