Document Detail


Cardiomyopathy and angiopathy in patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes.
MedLine Citation:
PMID:  7942444     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
In four patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes (MELAS) in which mutated mitochondrial deoxyribonucleic acid was seen, hypertrophic cardiomyopathy and angiopathy was demonstrated by echocardiography, dipyridamole stress scintigraphy, and cardiac catheterization. On stress scintigraphy with dipyridamole, three patients showed hypoperfusion in the early image and a "filling-in" pattern in the late image. However, coronary angiography did not demonstrate narrowing of the large vessels in these patients. Light and electron microscopy of endomyocardial biopsy specimens indicated abnormal mitochondria, with marked increase in the number and size of mitochondria in endothelium. Modified Gomori's trichrome staining in biopsied endomyocardial specimens revealed a red-purple deposit similar in appearance of the ragged-red fibers in skeletal muscle, a characteristic finding of mitochondrial disease. Deterioration of complex I in the mitochondrial electron transfer system, which is widely observed in various mitochondrial diseases, appeared in biopsied skeletal muscle of our patients, indicating deficiency of some subunits of complex I. These results indicate that mitochondrial diseases such as MELAS show not only cardiomyopathy but also angiopathy. We speculate that proliferation of mitochondria leads to narrowing of the lumen of arterioles, which might be responsible for the ischemic findings observed scintigraphically.
Authors:
W Sato; M Tanaka; S Sugiyama; T Nemoto; K Harada; Y Miura; Y Kobayashi; A Goto; G Takada; T Ozawa
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Publication Detail:
Type:  Comparative Study; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  American heart journal     Volume:  128     ISSN:  0002-8703     ISO Abbreviation:  Am. Heart J.     Publication Date:  1994 Oct 
Date Detail:
Created Date:  1994-10-27     Completed Date:  1994-10-27     Revised Date:  2008-11-21    
Medline Journal Info:
Nlm Unique ID:  0370465     Medline TA:  Am Heart J     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  733-41     Citation Subset:  AIM; IM    
Affiliation:
Department of Pediatrics, Akita University School of Medicine, Japan.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Biochemical Phenomena
Biochemistry
Biopsy
Blotting, Western
Cardiomyopathies / diagnosis*
Child
Coronary Angiography
Coronary Disease / diagnosis*
Dipyridamole / diagnostic use
Echocardiography
Endocardium / pathology
Female
Heart Catheterization
Humans
MELAS Syndrome / complications*,  diagnosis,  pathology
Male
Mitochondria / enzymology,  pathology
Myocardium / pathology
Tomography, Emission-Computed, Single-Photon
Chemical
Reg. No./Substance:
58-32-2/Dipyridamole

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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