Mitochondrial cardiomyopathy: molecular and biochemical analysis

Pediatr Cardiol. 1997 Jul-Aug;18(4):251-60. doi: 10.1007/s002469900169.


Abnormalities in cardiac mitochondrial respiratory enzymes and mitochondrial DNA have been found in an increasing number of pediatric cases of both dilated and hypertrophic cardiomyopathy, giving rise to the entity known as mitochondrial cardiomyopathy. Histochemical, biochemical, and molecular findings are described in this review of mitochondrial cardiomyopathy, which should provide assistance in its diagnostic identification.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Cardiomyopathy, Dilated* / genetics
  • Cardiomyopathy, Dilated* / metabolism
  • Cardiomyopathy, Hypertrophic* / genetics
  • Cardiomyopathy, Hypertrophic* / metabolism
  • Child
  • DNA, Mitochondrial / genetics*
  • Genes / genetics
  • Humans
  • Mitochondria, Heart*
  • Mitochondrial Myopathies* / genetics
  • Mitochondrial Myopathies* / metabolism
  • Mutation
  • Oxidative Phosphorylation
  • RNA, Transfer / genetics


  • DNA, Mitochondrial
  • RNA, Transfer