title: Impairment of mitochondrial respiratory chain enzyme activities in tetralogy of Fallot.
abstract: BACKGROUND
  During the last decade, disorders of the respiratory chain, so-called mitochondrial disorders, have emerged as a major clinical entity. Tetralogy of fallot (TOF) children>2 month of age are at risk for postoperative myocardial contractile failure. Myocardial ischemia is associated with a reduction in mitochondrial enzyme activity and have impaired metabolism resulting in decreased postoperative myocardial adenosine triphosphate (ATP) concentrations and increased lactate levels. With this in view, we measured the mitochondrial energy system (respiration and OXPHOS) and to study morphological changes from the right ventricular outflow tract (RVOT) muscle of patients with TOF.
  
  
  METHODS
  30 infants with TOF were studied with age-matched control group consisted of 12 normal patients who died due to extracardiac causes. Mitochondrial respiratory chain complexes, OXPHOS, cytochrome content and ATPase activity were measured by documented standard procedure. Morphological changes examined with a transmission electron microscope.
  
  
  RESULTS
  In the presence of glutamate and succinate as substrates, the rate of mitochondrial oxygen consumption was significantly lower in RVOT muscles (p<0.001) by using with and without addition of ADP. The ADP/O ratio indices for glutamate and succinate were not significantly affected. The activities of rotenone-sensitive NADH cytochrome c reductase (complexes I+III), cytochrome c oxidase (complex IV) and the ratio of I and III to II and III complexes (complex I) were significantly lower in TOF (p<0.001). A significant reduction of total cytochrome content and ATPase activity (p<0.001) was noted in study group. Morphological changes were also seen in study group as compared with control.
  
  
  CONCLUSIONS
  OXPHOS, mitochondrial respiratory chain complex I, I+III and IV, cytochrome content and ATPase activity are more impaired in RVOT muscles in patients with TOF.
authors.affiliation: Department of Biochemistry, L.T.M.M.C and L.T.M.G.H., Mumbai 400025, India. 1976.santosh@gmail.com
authors.forename: Santosh B
authors.initials: SB
authors.lastname: Shinde
authors.forename: Vipul C
authors.initials: VC
authors.lastname: Save
authors.forename: Neela D
authors.initials: ND
authors.lastname: Patil
authors.forename: Kaushala P
authors.initials: KP
authors.lastname: Mishra
authors.forename: Anil G
authors.initials: AG
authors.lastname: Tendolkar
mesh_terms: D000251:Adenosine Triphosphatases / Q000378:metabolism; D019069:Cell Respiration; D002648:Child; D003580:Cytochromes / Q000378:metabolism; D004579:Electron Transport; D005260:Female; D006801:Humans; D008297:Male; D008929:Mitochondria, Heart / Q000201:enzymology* / Q000378:metabolism / Q000473:pathology; D013771:Tetralogy of Fallot / Q000201:enzymology* / Q000378:metabolism / Q000473:pathology
chemical_list: D003580:Cytochromes; D000251:Adenosine Triphosphatases