Cardiac Assessment in Duchenne and Becker Muscular Dystrophies
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Mutations in the dystrophin gene cause Duchenne and Becker muscular dystrophies. In addition to muscle disease, there nearly always is an associated cardiomyopathy in Duchenne or Becker muscular dystrophy. In these muscular dystrophies, the severity of cardiomyopathy and congestive heart failure may not parallel the severity of skeletal muscle disease. Loss of normal dystrophin function in the heart produces four-chamber dilation and reduction in left ventricular function that develop after the onset of muscle weakness. Arrhythmias affecting both atrial and ventricular rhythms occur and may be life threatening. The degree to which hypoventilation and pulmonary dysfunction are present also directly affect cardiac function in muscular dystrophy. Care guidelines recently were issued to outline surveillance and treatment strategies for the younger patient with Duchenne muscular dystrophy. Herein, we review those guidelines, and additionally, provide recommendations for monitoring and treating cardiac disease in the populations of advanced Duchenne and Becker muscular dystrophies.
KeywordsCardiomyopathy Muscular dystrophy Arrhythmia Congestive heart failure Dystrophin Duchenne muscular dystrophy Becker muscular dystrophy Cardiac magnetic resonance Late gadolinium enhancement
Dr. Elizabeth M. McNally has received a grant from the Doris Duke Charitable Foundation. No other potential conflicts of interest relevant to this article were reported.
Papers of particular interest, published recently, have been highlighted as: •• Of major importance
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