932 www.anesthesia-analgesia.org October 2014 • Volume 119 • Number 4 L ong QT syndrome (LQTS) is the prototype cardiac ion channelopathy. Mutations in the genes encoding structural proteins that comprise or support ion chan- nels involved in myocardial action potential conduction result in channel dysfunction and consequent prolonged cardiac repolarization. The disease is essentially one of reduced myocardial repolarization reserve. 1 Clinically, it is highly heterogeneous, with a spectrum ranging from asymptomatic, through episodes of presyncope, syncope, and aborted cardiac arrest, to sudden cardiac death. Arrhythmias in LQTS, speciically the ventricular arrhythmia torsades de pointes (TdP), are often precipi- tated by changes in cardiac autonomic tone, which makes the perioperative period a time of potential high risk in patients with LQTS. Although there are 15 known sub- types of LQTS, the 3 most common subtypes, LQTS types 1, 2, and 3, account for more than 90% of genotype-positive patients. Intense adrenergic stimulation, classically exer- cise-induced, is a common trigger in LQTS 1 and, to a less predictable extent, LQTS 2, which is classically triggered by startle, fright, or emotion. Events in LQTS 3 frequently occur at rest or times of slow heart rate. It is important to bear in mind that there is overlap between each group. In addition, patients with latent LQTS may have their reduced repolarization reserve unmasked by perioperative factors, including electrolyte disturbance, drug administration, and hypothermia. Unfortunately, because LQTS is relatively uncommon (1 in 2500), 2 there is a lack of a robust evidence base for appropriate perioperative management; detailed Copyright © 2014 International Anesthesia Research Society DOI: 10.1213/ANE.0000000000000389 BACKGROUND: Patients with long QT syndrome (LQTS) may experience a clinical spectrum of symptoms, ranging from asymptomatic, through presyncope, syncope, and aborted cardiac arrest, to sudden cardiac death. Arrhythmias in LQTS are often precipitated by autonomic changes. This patient population is believed to be at high risk for perioperative arrhythmia, spe- ciically torsades de pointes (TdP), although this perception is largely based on limited literature that predates current anesthetic drugs and standards of perioperative monitoring. We present the largest multicenter review to date of anesthetic management in children with LQTS. METHODS: We conducted a multicentered retrospective chart review of perioperative manage- ment of children with clinically diagnosed LQTS, aged 18 years or younger, who received general anesthesia (GA) between January 2005 and January 2010. Data from 8 institutions were col- lated in an anonymized database. RESULTS: One hundred three patients with LQTS underwent a total of 158 episodes of GA. The median (interquartile range) age and weight of the patients at the time of GA was 9 (3–15) years and 30.3 (15.4–54) kg, respectively. Surgery was LQTS-related in 81 (51%) GA episodes (including pacemaker, implantable cardioverter-deibrillator, and loop recorder insertions and revisions and lead extractions) and incidental in 77 (49%). β-blocker therapy was administered to 76% of patients on the day of surgery and 47% received sedative premedication. Nineteen percent of patients received total IV anesthesia, 30% received total inhaled anesthesia, and the remaining 51% received a combination. No patient received droperidol. There were 5 periopera- tive episodes of TdP , all in neonates or infants, all in surgery that was LQTS-related, and none of which was overtly attributable to anesthetic regimen. Thus the incidence (95% conidence interval) of perioperative TdP in incidental versus LQTS-related surgery was 0/77 (0%; 0%–5%) vs 5/81 (6.2%; 2%–14%). CONCLUSIONS: With optimized perioperative management, modern anesthesia for inciden- tal surgery in patients with LQTS is safer than anecdotal case report literature might sug- gest. Our series suggests that the risk of perioperative TdP is concentrated in neonates and infants requiring urgent interventions after failed irst-line management of LQTS. (Anesth Analg 2014;119:932–8) From the *Department of Pediatric Anesthesia, BC Children’s Hospital and Department of Anesthesia, Pharmacology and Therapeutics, University of British Columbia, Vancouver, British Columbia, Canada; †Pediatric Anesthesia, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania; ‡Pediatric Cardiac Anesthesia, Monroe Carell Jr. Children’s Hospital at Vanderbilt University, Nashville, Tennessee; §Pediatric Cardiology, Vanderbilt University School of Medicine, Nashville, Tennessee; ∥Pediatric Cardiology, University of Utah School of Medicine and Primary Children’s Medical Center, Salt Lake City, Utah; ¶Montreal Heart Institute, Montreal, Quebec, Canada; #Cardiology, Children’s Hospital Colorado, Aurora, Colorado; **Division of Cardiology, University of Iowa Children’s Hospital, Iowa City, Iowa; ††Pediatric Anesthesia, The Hospital for Sick Children, Toronto, Ontario, Canada; and ‡‡Children’s Heart Centre, BC Children’s Hospital and Department of Pediatrics, University of British Columbia, Vancouver, British Columbia, Canada. Accepted for publication June 17, 2014. Funding: This study was supported by a Rare Disease Foundation Microgrant to SDW. The authors declare no conlicts of interest. Reprints will not be available from the authors. Address correspondence to Simon D. Whyte, MBBS, FRCA, Department of Pediatric Anesthesia, BC Children’s Hospital, 1L7-4480 Oak St.,Vancouver, British Columbia V6H 3V4, Canada. Address e-mail to sdwhyte@mac.com. The Safety of Modern Anesthesia for Children with Long QT Syndrome Simon D. Whyte, MBBS, FRCA,* Aruna Nathan, MBBS,† Dorothy Myers, MSc,* Scott C. Watkins, MD,‡ Prince J. Kannankeril, MD, MSCI,§ Susan P . Etheridge, MD,∥ Jason Andrade, MD,¶ Kathryn K. Collins, MD,# Ian H. Law, MD,** Jason Hayes, MD, FRCPC,†† and Shubhayan Sanatani, MD, FRCPC‡‡ CME