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  • the term is usually reserved for conditions in which severe myocardial disease leads to cardiac failure
  • they can be classified as:
    • “primary” or “intrinsic”
      • condition is primarily due to the cardiac muscle, although many of thee conditions now have identifiable extrinsic aetiologies
    • “secondary” or “extrinsic”
      • cardiac muscle dysfunction is due to a cause outside the cardiac muscle
  • they can also be classified as either:
    • dilated cardiomyopathy (DCM)
      • dilated cardiomyopathy is the most common form of non-ischaemic cardiomyopathy
      • accounts for 1/3rd of cases of congestive cardiac failure
      • most common in ages 20-60yr olds but may occur in children
      • 25-35% are familial
    • restrictive cardiomyopathy (RCM)
      • a form of cardiomyopathy in which the walls are rigid, and the heart is restricted from stretching and filling with blood properly
    • hypertrophic cardiomyopathy

primary cardiomyopathies

secondary cardiomyopathies

  • Metabolic/storage
  • Inflammatory
    • Chagas disease
  • Endocrine
  • Toxicity
    • chemotherapy
    • Alcoholic cardiomyopathy
  • Neuromuscular
    • muscular dystrophy
  • Nutritional diseases
    • Obesity-associated cardiomyopathy
  • Other
    • ischaemic cardiomyopathy

dilated cardiomyopathies

  • cardiac dilation is a transversely isotropic, irreversible process resulting from excess strains on the myocardium.
  • may be caused by:
    • ischaemic injury
    • alcoholic cardiomyopathy
    • chemotherapy - particularly doxorubicin (Adriamycin), and cobalt
    • chronic tachycardias or excess catecholamines
    • peri-partum cardiomyopathy
    • late sequelae of viral myocarditis
    • autoimmune disease
    • in the tropics, Chagas disease is the most common infectious cause
    • 25-35% are genetic such as:
      • gene mutations in the TTN gene (which codes for a protein called titin) are responsible for “approximately 25% of familial cases of idiopathic dilated cardiomyopathy and 18% of sporadic cases”
      • various autosomal dominant conditions
      • Alström syndrome (autosomal recessive)
      • Duchenne muscular dystrophy (X-linked)
  • many cases are “idiopathic” and thus regarded as “intrinsic”

restrictive cardiomyopathies

cardiomyopathy.txt · Last modified: 2013/04/24 18:22 (external edit)