Perioperative management of hereditary arrhythmogenic syndromes

Abstract

Patients with inherited cardiac channel disorders are at high risk of perioperative lethal arrhythmias. Preoperative control of symptoms and a multidisciplinary approach are required for a well-planned management. Good haemodynamic monitoring, adequate anaesthesia and analgesia, perioperative maintenance of normocarbia, normothermia, and normovolaemia are important. In congenital long QT syndrome, torsades de pointes should be prevented with magnesium sulphate infusion and avoidance of drugs such as droperidol, succinylcholine, ketamine, and ondansetron. Propofol and epidural anaesthesia represent safe choices, while caution is needed with volatile agents. In Brugada syndrome, β-blockers, α-agonists, and cholinergic drugs should be avoided, while isoproterenol reverses the ECG changes. Propofol, thiopental, and volatiles have been used uneventfully. In congenital sick sinus syndrome, severe bradycardia resistant to atropine may require isoproterenol or epinephrine. Anaesthetics with vagolytic properties are preferable, while propofol and vecuronium should be given with caution due to risk of inducing bradyarrhythmias. Neuraxial anaesthesia should produce the least autonomic imbalance. Arrhythmogenic right ventricular dysplasia/cardiomyopathy induces ventricular tachyarrhythmias, which should be treated with β-blockers. Generally, β-adrenergic stimulation and catecholamine release should be avoided. Halothane and pancuronium are contraindicated, while large doses of local anaesthetics and epinephrine should be avoided in neuraxial blocks. In catecholaminergic polymorphic ventricular tachycardia, β-blocker treatment should be continued perioperatively. Catecholamine release and β-agonists, such as isoproterenol, should be avoided. Propofol and remifentanil are probably safe, while halothane and pancuronium are contraindicated. Regional anaesthesia, without epinephrine, is relatively safe. In suspicious cardiac deaths, postmortem examination and familial screening are recommended.

Sevoflurane in patients at risk of ventricular dysrhythmias

“…

Terao et al In these patients, propofol should be preferred, without sevoflurane, for both induction and maintenance of anesthesia [5], especially as the present work confirms the good electrophysiological tolerance of propofol.

…”

Torsade de pointes in liver transplantation recipient after induction of general anesthesia: a case report

“…Long QT syndrome is a congenital or acquired disorder of cardiac ion channels, characterized by heterogeneity in cellular repolarization and precipitation of tachyarrhythmias [2]. The character of this syndrome is a marked prolongation of ventricular repolarization, including QT interval prolongation and T wave abnormalities leading to ventricular tachycardia, such as TdP and ventricular fibrillation [3].…”

Synergistic Restoring Effects of Isoproterenol and Magnesium on KCNQ1-Inhibited Bradycardia Cell Models Cultured in Microelectrode Array

“…In human studies, MgSO 4 is the drug of choice in preventing and treating bradycardia [30] and LQTS [31]. Internal perfusion of atrial cells in vitro with 0.3-1 m M Mg 2+ did not activate the full amplitude of Ik and shifted the steady-state activation curve by increasing Mg 2+ from 0.3 to 1 m M .…”