cardiomyopathies

Cardiomyopathies are diseases of the myocardium associated with cardiac dysfunction. They are classified as dilated cardiomyopathy, hypertrophic cardiomyopathy, restrictive cardiomyopathy, and arrhythmogeníc right ventricular cardiomyopathy.

Classification

 dilated cardiomyopathy

• idiopathic
• familial/genetic
• viral
• immune
• alcoholic/toxic
• associated with recognized cardiovascular disease

 hypertrophic cardiomyopathy (familial disease with autosomal dominant inheritance)

• mutations in sarcomeric contractile protein genes

 restrictive cardiomyopathy

• isolated
  • desmin mutations
  • alpha-B crystallin mutations

• associated
  • amyloidosis
  • endomyocardial disease with or without hypereosinophilia

 arrhythmogenic right ventricular cardiomyopathy (ARVD)

 unclassified cardiomyopathies

specific cardiomyopathies

The term specific cardiomyopathies is now used to describe heart muscle diseases that are associated with specific cardiac or systemic disorders. These were previously defined as specific heart muscle diseases.

 ischemic cardiomyopathy
 valvular cardiomyopathy
 hypertensive cardiomyopathy
 inflammatory cardiomyopathy
 metabolic cardiomyopathies

• Endocrine
  • thyrotoxicosis
  • hypothyroidism
  • adrenal cortical insufficiency
  • pheochromocytoma
  • acromegaly
  • diabetes mellitus

• familial storage disease and infiltrations
  • hemochromatosis
  • glycogen storage disease
  • Hurler’s syndrome
  • Refsum’s syndrome
  • Niemann-Pick disease
  • Hand-Schüller-Christian disease
  • Fabry-Anderson disease
  • Morquio-Ullrich disease

• deficiency
  • disturbances of potassium metabolism
  • magnesium deficiency

• nutritional disorders
  • kwashiorkor
  • anemia
  • beri-beri
  • selenium deficiency

 cardiac amyloidosis

• primary
• secondary
• familial (hereditary cardiac amyloidoses, familial Mediterranean fever, and senile amyloidosis)

 systemic dysimmune diseases

• connective tissue disorders
  • systemic lupus erythematosus
  • polyarteritis nodosa
  • rheumatoid arthritis
  • scleroderma
  • dermatomyositis

 myocardic infiltrations

• cardiac granulomatosis
• sarcoidosis
• leukemia

Muscular dystrophies

Duchenne
Becker-type
myotonic dystrophies

Neuromuscular disorders
Friedreich’s ataxia
Noonan’s syndrome
lentiginosis

Sensitivity and toxic reactions

alcohol
catecholamines
anthracyclines
irradiation
miscellaneous

Peripartal cardiomyopathy

References

 Seidman, J. G.; Seidman, C. The genetic basis for cardiomyopathy: from mutation identification to mechanistic paradigms. Cell 104: 557-567, 2001. PubMed ID : #11239412#

 Circulation 1996; 93:841-842