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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

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.

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

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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
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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
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