Rapid progression from hypertrophic cardiomyopathy to heart failure in a patient with Becker's muscular dystrophy.
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.
Park
Ok Young
OY
Seonam University College of Medicine, Namwon, South Korea.
Ahn
Youngkeun
Y
Park
Woo Seok
WS
Lim
Ji Hyun
JH
Park
Hyung Wook
HW
Kim
Ju Han
JH
Hong
Young Joon
YJ
Kim
Weon
W
Jeong
Myung Ho
MH
Cho
Jeong Gwan
JG
Park
Jong Chun
JC
Lee
Min Cheol
MC
Kang
Jung Chaee
JC
D000293:Adolescent; D002312:Cardiomyopathy, Hypertrophic / Q000453:epidemiology*; D015897:Comorbidity; D018450:Disease Progression; D016189:Dystrophin / Q000378:metabolism; D006333:Heart Failure / Q000453:epidemiology*; D006801:Humans; D006984:Hypertrophy; D007150:Immunohistochemistry; D008297:Male; D018482:Muscle, Skeletal / Q000473:pathology; D020388:Muscular Dystrophy, Duchenne / Q000453:epidemiology*; D009206:Myocardium / Q000473:pathology; D013997:Time Factors
D016189:Dystrophin