Impairment of mitochondrial respiratory chain enzyme activities in tetralogy of fallot

• Published in: Clinica chimica acta 2007-02, Vol.377 (1-2), p.138-143
• Main Authors: Shinde, Santosh B., Save, Vipul C., Patil, Neela D., Mishra, Kaushala P., Tendolkar, Anil G.
• Format: Article
• Language: English
• Subjects: Adenosine Triphosphatases - metabolism; Cell Respiration; Child; Cytochromes - metabolism; Electron Transport; Female; Humans; Male; Mitochondria, Heart - enzymology; Mitochondria, Heart - metabolism; Mitochondria, Heart - pathology; Mitochondrial respiratory chain; Oxidative phosphorylation; RVOT; Tetralogy of fallot; Tetralogy of Fallot - enzymology; Tetralogy of Fallot - metabolism; Tetralogy of Fallot - pathology
• ISSN: 0009-8981; 1873-3492
• DOI: 10.1016/j.cca.2006.09.011

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. 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. In the presence of glutamate and succinate as substrates, the rate of mitochondrial oxygen consumption was significantly lower in RVOT muscles (p