Abnormalties in cardiac mitochondrial respiratory enzymes and mitochon
drial DNA have been found in an increasing number of pediatric cases o
f both dilated and hypertrophic cardiomyopathy, giving rise to the ent
ity known as mitochondrial cardiomyopathy. Histochemical, biochemical,
and molecular findings are described in this review of mitochondrial
cardiomyopathy, which should provide assistance in its diagnostic iden
tification.