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

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.

• Authors: Ok Young Park, Youngkeun Ahn, Woo Seok Park, Ji Hyun Lim, Hyung Wook Park, Ju Han Kim, Young Joon Hong, Weon Kim, Myung Ho Jeong, Jeong Gwan Cho, Jong Chun Park, Min Cheol Lee, Jung Chaee Kang
• Journal: European journal of heart failure (Eur J Heart Fail) Vol. 7 Issue 4 Pg. 684-8 (Jun 2005) ISSN: 1388-9842 [Print] England
• PMID: 15921812 (Publication Type: Case Reports, Journal Article)

Chemical References

• Dystrophin

Topics

• Adolescent
• Cardiomyopathy, Hypertrophic (epidemiology)
• Comorbidity
• Disease Progression
• Dystrophin (metabolism)
• Heart Failure (epidemiology)
• Humans
• Hypertrophy
• Immunohistochemistry
• Male
• Muscle, Skeletal (pathology)
• Muscular Dystrophy, Duchenne (epidemiology)
• Myocardium (pathology)
• Time Factors