• Ehab Farag, Maged Argalious, Samer Narouze, Glenn E DeBoer, and Julie Tome.
• Sections of Pediatrics and Congenital Heart Disease Anesthesia, The Cleveland Clinic Foundation, Cleveland, Ohio, USA.
• Can J Anaesth. 2002 Nov 1;49(9):958-62.

PurposeTo present the anesthetic management for the correction of a ventricular septal defect (VSD) in a patient with multiple acyl CoA dehydrogenase deficiency (glutaric aciduria type II; GAII). A review of the literature about anesthetic management of patients with mitochondrial diseases undergoing cardiopulmonary bypass (CPB) is also included.Clinical FeaturesAn 11-yr-old girl with GAII manifested as severe hypoglycemia since she was a newborn and generalized muscle weakness. She underwent open-heart surgery for VSD correction with CPB. The anesthetic management avoided inhalational anesthetics, maintained the blood sugar within normal limits and continued normothermia during CPB in order to avoid the stress of hypothermia for her abnormal mitochondria. The patient tolerated the procedure well and experienced a good recovery.ConclusionThe anesthetic management of patients with any mitochondrial disease requires normoglycemia, normothermia and the avoidance of metabolic stress in order to preserve energy production by the diseased mitochondria.

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