Book chapter
Ventricular arrhythmia in muscular dystrophies
The ESC Textbook of Cardiovascular Medicine
The European Society of Cardiology Series, Oxford University Press, Third edition
2019
DOI: 10.1093/med/9780198784906.003.0834
Abstract
Muscular dystrophies are a heterogeneous group of genetic disorders with a collective prevalence of 19–25 per 100,000. Most of these conditions have some degree of cardiac involvement, with phenotypes ranging from mild conduction abnormalities to progressive cardiomyopathy resulting in endstage heart failure. The type of muscular dystrophy determines the prevalence, clinical features, and severity of cardiac involvement. In general, dilated cardiomyopathy is a primary cardiac phenotype of dystrophinassociated muscular dystrophies (i.e. dystrophinopathies: Duchenne and Becker) and those affecting the dystrophin–glycoprotein complex (limbgirdle types 2C, 2F, and 2I). Conduction system defect is a characteristic and commonly initial cardiac presentation of myotonic dystrophy (MD), Emery–Dreifuss, and limbgirdle type 1B muscular dystrophies. Whereas most MD patients have no significant functional cardiac abnormalities, progressive dilated cardiomyopathy is common in Emery–Dreifuss and limbgirdle type 1B muscular dystrophies.
Details
- Title: Subtitle
- Ventricular arrhythmia in muscular dystrophies
- Creators
- Brian OlshanskyEdward M. PowersAlexander Mazur
- Resource Type
- Book chapter
- Publication Details
- The ESC Textbook of Cardiovascular Medicine
- Edition
- Third edition
- Publisher
- Oxford University Press; Oxford
- Series
- The European Society of Cardiology Series
- DOI
- 10.1093/med/9780198784906.003.0834
- Alternative title
- Section 42 Ventricular tachycardia
- Language
- English
- Date published
- 2019
- Academic Unit
- Cardiovascular Medicine; Internal Medicine
- Record Identifier
- 9984642340202771
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