title: The safety of modern anesthesia for children with long QT syndrome.
abstract: 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, specifically 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 management 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 collated 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-defibrillator, 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 perioperative 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% confidence 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 incidental surgery in patients with LQTS is safer than anecdotal case report literature might suggest. Our series suggests that the risk of perioperative TdP is concentrated in neonates and infants requiring urgent interventions after failed first-line management of LQTS.
authors: 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.
authors: Simon D
authors: SD
authors: Whyte
authors: Aruna
authors: A
authors: Nathan
authors: Dorothy
authors: D
authors: Myers
authors: Scott C
authors: SC
authors: Watkins
authors: Prince J
authors: PJ
authors: Kannankeril
authors: Susan P
authors: SP
authors: Etheridge
authors: Jason
authors: J
authors: Andrade
authors: Kathryn K
authors: KK
authors: Collins
authors: Ian H
authors: IH
authors: Law
authors: Jason
authors: J
authors: Hayes
authors: Shubhayan
authors: S
authors: Sanatani
mesh_terms: D000293:Adolescent; D000768:Anesthesia, General / Q000009:adverse effects / Q000379:methods* / Q000592:standards; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008133:Long QT Syndrome / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery*; D008297:Male; D061214:Patient Safety / Q000592:standards; D019990:Perioperative Care / Q000379:methods* / Q000592:standards; D012189:Retrospective Studies; D012307:Risk Factors