Key result
Becker muscular dystrophy presents with severe LVH and rapid progression to DCM within ~2 years.
Case Report (n=1)
No
Alerts clinicians to consider Becker muscular dystrophy in unexplained severe LVH with elevated CK; extends phenotypic spectrum but leaves open prevalence.
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.
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Park et al. (2004) conducted a case report in Becker muscular dystrophy with hypertrophic cardiomyopathy (n=1). Becker muscular dystrophy was evaluated on Progression of hypertrophic cardiomyopathy to dilated heart failure. Becker muscular dystrophy presented with rare severe left ventricular hypertrophy, elevated CK (4730 IU/L), and rapid progression to dilated cardiomyopathy within 2 years.
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