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Duchenne muscular dystrophy: how bad is the heart?

Correspondence to:
Dr Elizabeth M McNally, The University of Chicago, 5841 S. Maryland, MC6088, Chicago, IL 60637, USA; emcnally@medicine.bsd.uchicago.edu

The first 150 words of the full text of this article appear below.

Duchenne muscular dystrophy (DMD), and the associated cardiomyopathy, develops from mutations the dystrophin gene. Dystrophin supports the plasma membrane of skeletal myofibres and cardiomyocytes. Nearly all skeletal muscle groups are affected in DMD with preferential early involvement of the limbs and trunk, followed by respiratory muscle compromise and cardiomyopathy. Because of enhanced ventilatory support, DMD patients now survive longer, and cardiac involvement dominates the later stage management. Treatment using β adrenergic blockade and inhibition of angiotensin-converting enzymes (ACEs) has been shown to be effective in promoting favourable remodelling. Management of cardiac arrhythmias presents challenges in the DMD patient.

The majority of DMD-associated mutations result in the complete loss of dystrophin, and the loss of dystrophin protein interrupts the normal link between the actin cytoskeleton and the plasma membrane of muscle.¹ Dystrophin associates with a series of membrane-spanning proteins to complete the connection to the extracellular matrix. Disruption of dystrophin or . . . [Full text of this article]