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Myocardial involvement is very frequent among patients affected with subclinical Becker's muscular dystrophy
- Source :
- Scopus-Elsevier
- Publication Year :
- 1996
-
Abstract
- 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.
- Subjects :
- Adult
Cardiomyopathy, Dilated
Male
medicine.medical_specialty
Adolescent
Becker's muscular dystrophy
Cardiomyopathy
Muscular Dystrophies
Ventricular Function, Left
Dystrophin
Electrocardiography
Ventricular Dysfunction, Left
Reference Values
Physiology (medical)
Internal medicine
medicine
molecular biology
Humans
Muscular dystrophy
Myopathy
Child
Muscle, Skeletal
Subclinical infection
Repetitive Sequences, Nucleic Acid
Sequence Deletion
Analysis of Variance
Ejection fraction
business.industry
Dilated cardiomyopathy
Exons
Middle Aged
medicine.disease
Surgery
Pedigree
medicine.anatomical_structure
Ventricle
Echocardiography
cardiomyopathy
echocardiography
muscles
Cardiology
Regression Analysis
Female
medicine.symptom
Cardiology and Cardiovascular Medicine
business
Subjects
Details
- ISSN :
- 00097322
- Volume :
- 94
- Issue :
- 12
- Database :
- OpenAIRE
- Journal :
- Circulation
- Accession number :
- edsair.doi.dedup.....44eeffdb962e74606abdf3e5c5dccb50