title: 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.affiliation: Seonam University College of Medicine, Namwon, South Korea.
authors.forename: Ok Young
authors.initials: OY
authors.lastname: Park
authors.forename: Youngkeun
authors.initials: Y
authors.lastname: Ahn
authors.forename: Woo Seok
authors.initials: WS
authors.lastname: Park
authors.forename: Ji Hyun
authors.initials: JH
authors.lastname: Lim
authors.forename: Hyung Wook
authors.initials: HW
authors.lastname: Park
authors.forename: Ju Han
authors.initials: JH
authors.lastname: Kim
authors.forename: Young Joon
authors.initials: YJ
authors.lastname: Hong
authors.forename: Weon
authors.initials: W
authors.lastname: Kim
authors.forename: Myung Ho
authors.initials: MH
authors.lastname: Jeong
authors.forename: Jeong Gwan
authors.initials: JG
authors.lastname: Cho
authors.forename: Jong Chun
authors.initials: JC
authors.lastname: Park
authors.forename: Min Cheol
authors.initials: MC
authors.lastname: Lee
authors.forename: Jung Chaee
authors.initials: JC
authors.lastname: Kang
mesh_terms: 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
chemical_list: D016189:Dystrophin