Anaesthesia for patients with hereditary arrhythmias; part 2: congenital long QT syndrome and arrhythmogenic right ventricular cardiomyopathy

Abstract

Congenital long QT syndrome Congenital long QT syndrome (LQTS) is an inherited disorder affecting cardiac ion channels resulting in prolonged action potential duration and propensity to sudden cardiac death (SCD). Two classical genetic variants are RomanoeWard Learning objectives By reading this article, you should be able to: Describe the commonest forms of long QT syndrome and how they present. Recognise the disease course of arrhythmogenic right ventricular cardiomyopathy. Explain the diagnostic criteria for long QT syndrome and arrhythmogenic right ventricular cardiomyopathy. Discuss standard cardiology treatment strategies and perioperative management, and have some insight into management in intensive care.

Anaesthetic management of a patient with Jervell and Langee Nielson Syndrome (a long QT variant) for cochlear implantation

“…1 Diagnostic evaluation for LQTS can be done using Schwartz score. 2 Score ≥3.5 indicates high probability of LQTS. Score 1.5 to 3.5; intermediate probability and a score ≤ 1.5 -low probability.…”

“…Score 1.5 to 3.5; intermediate probability and a score ≤ 1.5 -low probability. 2 LQTS can be inherited or acquired. Prevalence of congenital LQTS is 1 in 2500 live births.…”

“…Avoidance of "noncompliance" or "medication failure" by concurrent use of drugs that prolong QT time can provoke life threatening arrhythmias. 2 Almost always medication lists should be assessed. Treatment options would be implantation of cardioverter defibrillator (ICD) or left cardiac sympathetic denervation (LCSD) if the patients cannot tolerate beta blockers.…”

“…All possible efforts were taken to alleviate the anxiety during the time of entrance to the theatre. QTc (best monitored in lead II) 2 was added with AAGBI monitoring. All the measures were taken to avoid hypothermia, because it can provoke QT prolongation.…”

“…Depolarising neuromuscular agent (succinylcholine) and non-depolarising blocker pancuronium would prolong the QT interval. 2 Except for the effects of histamine release, atracurium can be used. Vecuronium and rocuronium are also safe but cis-atracurium would be the ideal.…”