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Review
. 2009 May;21(5):241-9.
doi: 10.1111/j.1745-7599.2009.00404.x.

Current understanding and management of dilated cardiomyopathy in Duchenne and Becker muscular dystrophy

Rita Wen Kaspar  1 , Hugh D Allen, Federica Montanaro
Affiliations
Review

Current understanding and management of dilated cardiomyopathy in Duchenne and Becker muscular dystrophy

Rita Wen Kaspar et al. J Am Acad Nurse Pract. 2009 May.

Abstract

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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Figures

Figure 1
Figure 1
Known pathways through which abnormal dystrophin leads to cardiomyocyte death.
Figure 2
Figure 2
Progression from cardiomyocyte death to dilated cardiomyopathy.
Figure 3
Figure 3
Electrocardiogram of a patient with Becker muscular dystrophy. Note the short PR interval (98 ms, black horizontal bar, normal range: 120–200 ms), Q waves in leads 2, 3, aVF, V5, V6 (arrows), and tall R wave in V1 (asterisks). The EKG finding in patients with Duchenne muscular dystrophy is similar.
See this image and copyright information in PMC

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