The anesthetic management of ventricular septal defect (VSD) repair in a child with mitochondrial cytopathy.

Purpose: To 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 Features: An 11-yr-old girl with GAII manifested as severe hypoglycemia since she was a newborn and generalized muscle weakness.