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Chapter 49 - Long QT Syndrome

from Section 7 - Miscellaneous Lesions and Syndromes

Published online by Cambridge University Press:  09 September 2021

By
Kelly L. Grogan  and
Susan C. Nicolson
Edited by
Laura K. Berenstain  and
James P. Spaeth
Laura K. Berenstain
Affiliation:
Cincinnati Children's Hospital Medical Center
James P. Spaeth
Affiliation:
Cincinnati Children's Hospital Medical Center
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Summary

Congenital long QT syndrome is a group of genetically transmitted disorders characterized by abnormal cardiac repolarization resulting in QT interval prolongation that predisposes patients to the acute onset of ventricular arrhythmias, most notably torsades de pointes, which may cause syncope or sudden cardiac death. Long QT syndrome is usually transmitted in an autosomal dominant pattern. Diagnosis remains challenging, as roughly 40% of patients with genotype-positive LQTS do not demonstrate QT prolongation on resting ECG. Clinical manifestations are heterogenous and include presyncope, syncope, aborted cardiac arrest, cardiac arrest, and sudden cardiac death but many patients are completely asymptomatic. Many of the medications administered during an anesthetic affect the QT interval. Additionally, patients with long QT syndrome may require the placement of pacemakers, implantable cardioverter-defibrillators and/or cardiovascular implantable electronic devices. This chapter discusses the perioperative management of patients with long QT syndrome and appropriate management of implanted devices during the perioperative period.

Keywords

cardiovascular Implantable electronic devicecongenital long QT syndromeleft cardiac sympathetic denervationtorsades de pointes
Type
Chapter
Information
Congenital Cardiac Anesthesia
A Case-based Approach
 , pp. 374 - 385
Publisher: Cambridge University Press
Print publication year: 2021

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References

References

Schwartz, P. J., Crotti, L., and Insolia, R.. Long-QT syndrome: from genetics to management. Circ Arrhythm Electrophysiol 2012; 5: 868–77.CrossRefGoogle ScholarPubMed
Barsheshet, A., Dotshenko, O., and Goldenberg, I.. Genotype-specific risk stratification and management of patients with long QT syndrome. Ann Noninvasive Electrocardiol 2013; 18: 499–509.Google Scholar
Ackerman, M. J., Priori, S. G., Willems, S., et al. HRS/EHRA expert consensus statement on the state of genetic testing for the channelopathies and cardiomyopathies: this document was developed as a partnership between the Heart Rhythm Society (HRS) and the European Heart Rhythm Association (EHRA). Heart Rhythm 2011; 10: e85–108.Google Scholar
Priori, S. G., Wilde, A. A., Horie, M., et al. HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes: document endorsed by HRS, EHRA, and APHRS in May 2013 and by ACCF, AHA, Paces and AEPC in June 2013. Heart Rhythm 2013; 10: 1932–63.CrossRefGoogle ScholarPubMed
Giudicessi, J. R. and Ackerman, M. J.. Genotype- and phenotype-guided management of congenital long QT syndrome. Curr Probl Cardiol 2013; 38: 417–55.CrossRefGoogle ScholarPubMed
Priori, S. G., Schwartz, P. J., Napolitano, C., et al. Risk stratification in the long-QT syndrome. N Engl J Med 2003; 348: 1866–74.CrossRefGoogle ScholarPubMed
Ban, J-E. Neonatal arrhythmias: diagnosis, treatment, and clinical outcome. Korean J Pediatr 2017; 60: 344–52.CrossRefGoogle ScholarPubMed
Schwartz, P. J. and Ackerman, M. J.. The long QT syndrome: a transatlantic clinical approach to diagnosis and therapy. Eur Heart J 2013; 34: 3109–16.CrossRefGoogle Scholar
Kaufman, E. S., McNitt, S., Moss, A. J., et al. Risk of death in the long QT syndrome when a sibling has died. Heart Rhythm 2008; 5: 831–6.CrossRefGoogle Scholar
Nguyen, H. L., Pieper, G. H., and Wilders, R.. Andersen-Tawil syndrome: clinical and molecular aspects. Int J Cardiol 2013; 170: 1–16.Google Scholar
Schwartz, P. J., Spazzolini, C., Priori, S. G., et al. Who are the long-QT syndrome patients who receive an implantable cardioverter-defibrillator and what happens to them? Data from the European Long-QT Syndrome Implantable Cardioverter-Defibrillator (LQTS ICD) Registry. Circulation 2010; 122: 1272–82.CrossRefGoogle ScholarPubMed
Spazzolini, C., Mullaly, J., Moss, A. J., et al. Clinical implications for patients with long QT syndrome who experience a cardiac event during infancy. J Am Coll Cardiol 2009; 54: 832–7.Google Scholar
Schneider, H. E., Steinmetz, M., Krause, U., et al. Left cardiac sympathetic denervation for the management of life-threatening ventricular tachyarrhythmias in young patients with catecholaminergic polymorphic ventricular tachycardia and long QT syndrome. Clin Res Cardiol 2013; 102: 33–42.Google Scholar
American Society of Anesthesiologists. Practice advisory for the perioperative management of patients with cardiac implantable electronic devices: pacemakers and implantable cardioverter-defibrillator: an updated report by the American Society of Anesthesiologists Task Force on Perioperative Management of Patients with Cardiac Implantable Electronic Devices. Anesthesiology 2011; 114: 247–61.Google Scholar
Crossley, G. H., Poole, J. E., Rozner, M. A., et al. The Rhythm Society (HRS)/ American Society of Anesthesiologists (ASA) Expert Consensus Statement on the perioperative management of patients with implantable defibrillators, pacemakers and arrhythmia monitors: facilities and patient management: executive summary. Heart Rhythm 2011; 8: 1114.Google Scholar
Zareba, W., Moss, A. J., Daubert, J. P., et al. Implantable cardioverter defibrillator in high-risk long QT syndrome patients. J Cardiovasc Electrophysiol 2003; 14: 337–41.CrossRefGoogle ScholarPubMed
Kies, S. J., Pabelick, C. M., Hurley, H. A., et al. Anesthesia for patients with congenital long QT syndrome. Anesthesiology 2005; 102: 204–10.CrossRefGoogle ScholarPubMed
Priori, S. G., Blomström-Lundqvist, C., Mazzanti, A., et al. Task Force for the Management of Patients with Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death for the European Society of Cardiology. 2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Europace 2015; 17: 1601–87.Google Scholar
Staudt, G. E. and Watkins, S. C.. Anesthetic considerations for pediatric patients with congenital long QT syndrome. J Cardiothorac Vasc Anesth 2019; 33: 2030–8.CrossRefGoogle ScholarPubMed
Hammer, G. B., Drover, D. R., Cao, H., et al. The effects of dexmedetomidine on cardiac electrophysiology in children. Anesth Analg 2008; 106: 79–83.CrossRefGoogle ScholarPubMed
Owczuk, R., Wujtewicz, M. A., Zienciuk-Krajka, A., et al. The influence of anesthesia on cardiac repolarization. Minerva Anesthesiol 2012; 78: 483–95.Google ScholarPubMed
Kumakura, M., Hara, K., and Sata, T.. Sevoflorane-associated torsade de pointes in a patient with congenital long QT syndrome genotype 2. J Clin Anesth 2016; 33: 81–5.Google Scholar
Naguib, M., Samarkandi, A. H., Bakhamees, H. S., et al. Histamine-release haemodynamic changes produced by rocuronium, vecuronium, mivacurium, atracurium and tubocurarine. Br J Anaesth 1995; 75: 588–92.CrossRefGoogle ScholarPubMed
de Kam, P-J, Grobara, P., Dennie, J., et al. Effect of sugammadex on QT/QTc interval prolongation when combined with QTc-prolonging sevoflurane or propofol anaesthesia. Clin Drug Invest 2013; 33: 545–51.CrossRefGoogle ScholarPubMed
Odening, K. E., Koren, G., Hospital, R. I., et al. HHS public assess 2015; 11: 2107–15.Google Scholar
Shah, M. J.. Implantable cardioverter defibrillator-related complications in the pediatric population. Pacing Clin Electophysiol 2009; 32: S71–4.Google ScholarPubMed
Bernstein, A. D., Daubert, J-C, Fletcher, R. D., et al. NAPSE Position Statement. The revised NAPSE/BPEG generic code for antibradycardia, adaptive-rate, and multisite pacing. Pacing Clin Electophysiol 2002; 25: 260–4.CrossRefGoogle Scholar
Cronin, B. and Essandoh, M. K.. Update on cardiovascular implantable electronic devices for anesthesiologists. J Cardiothorac Vasc Anes 2018; 32: 1871–84.CrossRefGoogle ScholarPubMed

Suggested Reading

American Society of Anesthesiologists Task Force on Perioperative Management of Patients with Cardiac Implantable Electronic Devices. Practice advisory for the perioperative management of patients with cardiac implantable electronic devices: pacemakers and implantable cardioverter-defibrillators. An updated report. Anesthesiology 2011; 114: 247–61.Google Scholar
Cronin, B. and Essandoh, M. K. Update on cardiovascular implantable electronic devices for anesthesiologists. J Cardiothorac Vasc Anes 2018; 32: 1871–84.Google ScholarPubMed
Crossley, G. H., Poole, J. E., Rozner, M. A., et al. The Rhythm Society (HRS)/American Society of Anesthesiologists (ASA) Expert Consensus Statement on the Perioperative Management of Patients with Implantable Defibrillators, Pacemakers and Arrhythmia Monitors: Facilities and Patient Management: Executive Summary. Heart Rhythm 2011; 8: 1114.Google Scholar
Fazio, G., Vernuccio, F., Grutta, G., et al. Drugs to be avoided in patients with long QT syndrome: focus on the anaesthesiological management. World J Cardiol 2013; 26: 87–93.CrossRefGoogle Scholar
Navaratnam, M. and Dubin, A. Pediatric pacemakers and ICDs: how to optimize perioperative care. Pediatr Anesth 2011; 21: 512–21.Google Scholar
O’Hare, M., Maldonado, Y., Muro, J., et al. Perioperative management of patients with congenital or acquired disorder of the QT interval. BJA 2018; 120: 629–44.Google Scholar
Staudt, G. E. and Watkins, S. C. Anesthetic considerations for pediatric patients with congenital long QT syndrome. J Cardiothorac Vasc Anesth 2019; 33: 2030–8.Google Scholar

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