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Canadian Journal of Anesthesia 49:958-962 (2002)
© Canadian Anesthesiologists' Society, 2002
Obstetrical and Pediatric Anesthesia

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

[La démarche anesthésique adoptée pendant la réparation d’une communication interventriculaire (CIV) chez une enfant présentant une cytopathie mitochondriale]

Ehab Farag, MD FRCA, Maged Argalious, MD, Samer Narouze, MD, Glenn E. DeBoer, MD and Julie Tome, MD

From Sections of Pediatrics and Congenital Heart Disease Anesthesia, The Cleveland Clinic Foundation, Cleveland, Ohio, USA.

Address correspondence to: Dr. Julie Tome, Staff Anesthesiologist, The Cleveland Clinic Foundation, Division of Anesthesia and Critical Care Medicine, 9500 Euclid Ave., Cleveland, OH 44195, USA. Phone: 216-444-8389; Fax: 216-445-2536; E-mail: TOMEJ{at}CCF.ORG

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. 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.

Conclusion: The 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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Copyright © 2002 by the Canadian Anesthesiologists' Society.