| Myocardial involvement is very frequent among patients affected with subclinical Becker's muscular dystrophy. | |
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MedLine Citation:
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PMID: 8989125 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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BACKGROUND: Several cases of Becker's muscular dystrophy (BMD) have been reported, which showed mild or subclinical skeletal muscle involvement with an overt dilated cardiomyopathy. Here, for the first time, a group of 28 patients with BMD who had a subclinical or benign myopathy have been studied through a thorough cardiological assessment. METHODS AND RESULTS: Each patient underwent ECG and echocardiographic examinations. Molecular analyses of the dystrophin gene and protein were performed. An unexpectedly high incidence of myocardial involvement was observed among patients affected with subclinical (72%) or benign (60%) BMD. The cardiac involvement appears to develop early from the right ventricle. Both the increase in left ventricular end-diastolic volume and the reduction in the ejection fraction appeared to be age related. Severe left ventricular dilation with reduced ejection fraction, which could be complicated by life-threatening arrhythmias, may occur. Contrary to previous reports, which indicated the involvement of 5'-end mutations in cardiomyopathies as a result of dystrophin gene alterations, this study shows that despite the apparent concentration of deletions in two regions (5'-end and exons 47 through 49), no general conclusions can be drawn regarding the involvement of specific gene mutations in the development of cardiomyopathy. CONCLUSIONS: Cardiomyopathy is the main clinical feature and complication in patients affected by subclinical or mild BMD. The cardiac manifestation is characterized by early right ventricular involvement and is later associated with left ventricular impairment. In mild BMD, myocardial damage may develop because the patients, who are unaware of a possible cardiac involvement, are still able to perform strenuous muscle exercise and, through pressure or volume overload, may induce mechanical stress, which is harmful for dystrophin-deficient myocardial cells. |
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Authors:
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P Melacini; M Fanin; G A Danieli; C Villanova; F Martinello; M Miorin; M P Freda; M Miorelli; M L Mostacciuolo; G Fasoli; C Angelini; S Dalla Volta |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: Circulation Volume: 94 ISSN: 0009-7322 ISO Abbreviation: Circulation Publication Date: 1996 Dec |
Date Detail:
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Created Date: 1997-02-03 Completed Date: 1997-02-03 Revised Date: 2012-04-16 |
Medline Journal Info:
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Nlm Unique ID: 0147763 Medline TA: Circulation Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 3168-75 Citation Subset: AIM; IM |
Affiliation:
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Department of Cardiology, University of Padua, Italy. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Adult Analysis of Variance Cardiomyopathy, Dilated / complications*, genetics, physiopathology Child Dystrophin / analysis, genetics* Echocardiography Electrocardiography Exons Female Humans Male Middle Aged Muscle, Skeletal / metabolism, pathology Muscular Dystrophies / complications, genetics, physiopathology* Pedigree Reference Values Regression Analysis Repetitive Sequences, Nucleic Acid Sequence Deletion Ventricular Dysfunction, Left Ventricular Function, Left |
| Grant Support | |
ID/Acronym/Agency:
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76//Telethon |
| Chemical | |
Reg. No./Substance:
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0/Dystrophin |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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