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European Journal of Heart Failure 2005 7(4):684-688; doi:10.1016/j.ejheart.2004.07.019
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© 2005 European Society of Cardiology

Rapid progression from hypertrophic cardiomyopathy to heart failure in a patient with Becker's muscular dystrophy

Ok Young Parka, Youngkeun Ahnb,*, Woo Seok Parkb, Ji Hyun Limb, Hyung Wook Parkb, Ju Han Kimb, Young Joon Hongb, Weon Kimb, Myung Ho Jeongb, Jeong Gwan Chob, Jong Chun Parkb, Min Cheol Leeb and Jung Chaee Kangb

a Seonam University College of Medicine Namwon, South Korea
b Department of Cardiology and Pathology, Chonnam National University Hospital 8 Hakdong, Dongku, Gwangju 501-757, South Korea

* Corresponding author. Department of Cardiology, Chonnam National University Hospital, 8 Hakdong, Dongku, Gwangju 501-757, South Korea. Tel.: +82 62 220 4764; fax: +82 62 227 7174. E-mail address: cecilyk{at}hanmail.net


  Abstract

We describe the case of a 17-year-old boy with Becker*s muscular dystrophy (BMD) presenting with rapid progression from hypertrophic cardiomyopathy to heart failure within 2 years. Initial echocardiogram showed severe hypertrophy of left ventricle (LV) and right ventricle (RV) with normal chamber size, and preserved LV systolic function. Microscopic study of cardiac muscle obtained by endomyocardial biopsy of the interventricular septum showed severe hypertrophy of the muscle fibers and interstitial fibrosis. Follow-up echocardiogram 2 years after the first examination exhibited marked dilated LV and RV with severe LV global hypokinesia. Follow-up endomyocardial biopsy demonstrated increased interstitial cellular matrix. Immunohistochemical staining for dystrophin revealed significant loss of dystrophin along the sarcoplasmic membrane of the right biceps brachii muscle, compatible with BMD.

Key Words: Becker's muscular dystrophy • Hypertrophic cardiomyopathy • Heart failure

Received November 6, 2003; Revised May 28, 2004; Accepted July 14, 2004


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