Anesthetic management for cardioverter-defibrillator implantation in a patient with Kearns-Sayre syndrome

Kearns-Sayre syndrome (KSS) is a rare mitochondrial myopathy and often involves cardiac conduction abnormality and muscle weakness. We report a patient with KSS who had an implantable cardioverter-defibrillator (ICD) implanted during general anesthesia with propofol and nitrous oxide (N2O). These anesthetics did not show any effects on respiration, cardiac conduction, or neuromuscular transmission in this case. The surgery was performed successfully, and the postoperative period was uneventful. The authors recommend that propofol and N2O are suitable general anesthetics for ICD implantation in KSS patients. (C) 2004 by Elsevier Inc.