JCA-07580; No of Pages 2 Journal of Clinical Anesthesia xxx (2013) xxx–xxx
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Correspondence
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Trigger-free anesthetic management in congenital long QT syndrome☆
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To the Editor:
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Congenital long QT syndrome (LQTS) is a genetic disorder characterized by cardiac repolarization abnormalities, manifested as QT interval prolongation on an electrocardiogram (ECG). These individuals are susceptible to life-threatening ventricular arrhythmias and sudden cardiac death. The anesthetic management of these patients is complicated by the tendency of many commonly used pharmacologic agents and sympathetic stimulation to prolong the QT interval and increase the risk of malignant tachyarrhythmias perioperatively [1]. The case of a patient with LQTS and worsening arrhythmias who underwent an elective procedure using a triggerfree anesthetic strategy is presented. The patient was a 23 year old, ASA physical status 2, 85 kg, 163 cm, woman with a body mass index of 32 kg/m 2, who presented for dilatation and curettage at 15 weeks’ gestation. In addition to her well-controlled asthma, the patient had a history of long QT syndrome type 1 (LQT1), diagnosed after a syncopal episode at the age of 15 years, which required placement of an implantable cardioverterdefibrillator (ICD). During a prenatal visit, her ICD was interrogated; she was having increasing episodes of ventricular tachycardia. However, there were no episodes requiring shock. The patient had been maintained on nadolol for beta blockade, and the dosage was increased at that time. Due to the increasing frequency of the arrhythmias, the patient opted for termination of pregnancy. Preoperatively, the corrected QT (QTc) interval was 470 milliseconds (msec). An echocardiogram showed normal left ventricular size and systolic function without evidence of structural cardiac disease. On the day of surgery, potassium supplementation was given with the goal of maintaining a level in the high-normal (4.5 - 5.0 mEq/L) range. Magnesium sulfate and an emergency cardiac defibrillator were also immediately available, and defibrillator pads were placed on the patient prior to induction as a precautionary measure. Standard ASA monitors including continuous dual-lead ECG monitoring were used throughout the case. Total intravenous anesthesia was used for the procedure. The patient was administered fentanyl and propofol for induction, followed by an infusion of propofol and remifentanil for maintenance. A phenylephrine infusion was added in order to maintain desired hemodynamics. A low dose of a neuromuscular blocker, vecuronium, was given to decrease the need for reversal of neuromuscular blockade with anticholinesterase-anticholinergic administration; neuromuscular monitoring was also utilized. After airway topicalization with a local anesthetic, the patient was intubated with no increase in heart rate or blood pressure. Reversal agents were not given at the end of the case, and prophylactic antiemetics were also ☆ Funded by department sources only.
withheld. The patient had a stable intraoperative course, was extubated without complications, and transported to the Postanesthesia Care Unit (PACU). The postoperative course was uneventful. An ECG showed a QTc of 489 msec. The patient was discharged after a 24-four hour period of inpatient telemetry monitoring. The perioperative management of patients with long QT syndrome presents unique challenges to the anesthesiologist, as any inciting event that increases the QT interval potentially increases the risk of arrhythmias. Volatile anesthetics prolong the QT interval, although the clinical significance of these findings has not been prospectively studied in patients with LQTS [2–5]. A combination of a propofolopioid anesthetic seems to have a favorable safety profile in these patients and has been suggested by some authors [6–10]. Sympathetic stimulation, physical and emotional stress, and a variety of routinely used pharmacologic agents such as reversal agents and several classes of antiemetics may acutely exacerbate the underlying electrophysiologic cardiac deficits in LQTS and contribute to the development of ventricular arrhythmias in high-risk patients [11–13]. The Valsalva maneuver also prolongs the QTc interval [14]. The preoperative management of these patients should include a comprehensive electrophysiologic evaluation with attention to any cardiac rhythm management devices, continuation of beta-blocker maintenance therapy, and correction of electrolyte imbalances, as hypokalemia, hypocalcemia, and hypomagnesemia increase the potential for arrhythmias. Premedication with benzodiazepines may be useful in patients with anxiety or emotional stress in order to avoid a catecholamine surge. Judicious use of muscle relaxants together with neuromuscular monitoring allows for the decreased need to administer reversal agents. Avoidance of triggering factors, particularly during periods of increased sympathetic stimulation including induction, intubation, and emergence, and vigilant monitoring throughout the perioperative period are important considerations when formulating an anesthetic plan for these individuals.
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Emmanuel Edson MD Resident Linda Chen MD (Associate Professor) John G.T. Augoustides MD, FASE, FAHA (Associate Professor) Department of Anesthesiology and Critical Care University of Pennsylvania Health System Hospital of the University of Pennsylvania Philadelphia, PA19104, USA E-mail address:
[email protected]
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http://dx.doi.org/10.1016/j.jclinane.2013.01.004
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References
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[1] Kies SJ, Pabelick CM, Hurley HA, White RD, Ackerman MJ. Anesthesia for patients 94 with congenital long QT syndrome. Anesthesiology 2005;102:204–10. 95
0952-8180/$ – see front matter © 2013 Published by Elsevier Inc.
Please cite this article as: Edson E, et al. Trigger-free anesthetic management in congenital long QT syndrome. J Clin Anesth (2013), http:// dx.doi.org/10.1016/j.jclinane.2013.01.004
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Please cite this article as: Edson E, et al. Trigger-free anesthetic management in congenital long QT syndrome. J Clin Anesth (2013), http:// dx.doi.org/10.1016/j.jclinane.2013.01.004
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