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Published on: April 10, 2019
Current understanding and management of dilated cardiomyopathy in Duchenne and Becker muscular dystrophy
Rita Wen Kaspar1, Hugh D Allen, Federica Montanaro
1The Ohio State University College of Nursing, Columbus, Ohio, USA.
Insights
Dilated cardiomyopathy (DCM) is a leading cause of death in Duchenne and Becker muscular dystrophies. Early cardiac surveillance and treatment are crucial for managing heart health in these patients.
Area of Science:
- Cardiology
- Neuromuscular Disorders
- Genetics
Background:
- Duchenne and Becker muscular dystrophies stem from dystrophin gene mutations.
- Dilated cardiomyopathy (DCM) is a common and often fatal complication in these neuromuscular disorders.
Purpose of the Study:
- To review the pathophysiology of DCM in Duchenne and Becker muscular dystrophies.
- To assess cardiac dysfunction and evaluate pharmacological treatments for affected patients.
- To explore ongoing research in this field.
Main Methods:
- Literature review of scholarly articles and books.
- Analysis of current understanding of DCM in muscular dystrophy patients.
Main Results:
- DCM is the primary cause of mortality in Duchenne and Becker muscular dystrophies.
- Routine cardiac monitoring (ECG, echocardiograms, biomarkers) can detect early DCM.
- Early cardiac medication administration may delay DCM progression.
Conclusions:
- Current standard care often lacks emphasis on cardiac surveillance and timely DCM treatment.
- Proactive cardiac assessment and prompt pharmacological intervention are vital for optimizing cardiac health in these patients.
- Advanced practice nurses play a key role in aggressive and proactive patient care.
Purpose:
To review the current understanding of the pathophysiology of dilated cardiomyopathy (DCM) in patients with Duchenne and Becker muscular dystrophies, assessment of cardiac dysfunction for these patients, and the recommended pharmacological treatment options and ongoing research directions.
Data Sources:
Reviews and original research articles from scholarly journals and books.
Conclusions:
Duchenne and Becker muscular dystrophies are debilitating neuromuscular disorders, both caused by mutations in the dystrophin gene. Most patients develop DCM as part of the disease course; in fact, DCM is the leading cause of death among these patients. Cardiac surveillance, including routine monitoring of electrocardiograms, echocardiograms, and appropriate blood biomarkers, may detect early DCM development. Although previous studies have shown that early administration of cardiac medications may delay the development of DCM, current standard of care does not emphasize cardiac surveillance and timely treatment. This, in turn, limits clinicians, including advanced practice nurses, to be optimally engaged in providing the most aggressive and proactive patient care.
Implications For Practice:
Implementing a routine cardiac assessment and timely pharmacological treatment in primary or specialty care settings is highlighted as an important step to optimize cardiac health among patients with Duchenne and Becker muscular dystrophies.
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