Premium
Rapid progression from hypertrophic cardiomyopathy to heart failure in a patient with Becker's muscular dystrophy
Author(s) -
Park Ok Young,
Ahn Youngkeun,
Park Woo Seok,
Lim Ji Hyun,
Park Hyung Wook,
Kim Ju Han,
Hong Young Joon,
Kim Weon,
Jeong Myung Ho,
Cho Jeong Gwan,
Park Jong Chun,
Lee Min Cheol,
Kang Jung Chaee
Publication year - 2005
Publication title -
european journal of heart failure
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 5.149
H-Index - 133
eISSN - 1879-0844
pISSN - 1388-9842
DOI - 10.1016/j.ejheart.2004.07.019
Subject(s) - medicine , cardiology , heart failure , ventricle , hypertrophic cardiomyopathy , muscle hypertrophy , restrictive cardiomyopathy , interventricular septum , muscular dystrophy , biceps , cardiomyopathy , dystrophin , anatomy
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.