Cardiomyopathy in Duchenne muscular dystrophy: pathogenesis and therapeutics

Heart Fail Rev. 2010 Jan;15(1):103-7. doi: 10.1007/s10741-009-9156-8.

Abstract

Duchenne muscular dystrophy (DMD) is an X-linked recessive disorder caused by the absence of dystrophin, a sarcolemmal protein which links the cytoskeleton to the extracellular matrix by interacting with a large number of proteins. Heart failure is a classic complication of this disease. The authors review the pathogenesis and therapeutics of cardiac involvement in DMD.

Publication types

  • Review

MeSH terms

  • Adrenergic beta-Antagonists / therapeutic use
  • Angiotensin-Converting Enzyme Inhibitors / therapeutic use
  • Biomarkers / metabolism
  • Cardiac Pacing, Artificial / methods
  • Cardiomyopathies / etiology
  • Cardiomyopathies / genetics*
  • Cardiomyopathies / metabolism
  • Cardiomyopathies / therapy*
  • Drug Therapy, Combination
  • Dystrophin / biosynthesis
  • Dystrophin / genetics
  • Genetic Therapy / methods
  • Heart Failure / etiology
  • Heart Failure / genetics*
  • Heart Failure / metabolism
  • Heart Failure / therapy*
  • Humans
  • Muscular Dystrophy, Duchenne / complications
  • Muscular Dystrophy, Duchenne / genetics*
  • Muscular Dystrophy, Duchenne / metabolism
  • Muscular Dystrophy, Duchenne / therapy*
  • Practice Guidelines as Topic
  • Prognosis
  • Stem Cell Transplantation / methods
  • Ventricular Function, Left / drug effects

Substances

  • Adrenergic beta-Antagonists
  • Angiotensin-Converting Enzyme Inhibitors
  • Biomarkers
  • Dystrophin