British Journal of Anaesthesia 108 (5): 730–44 (2012) doi:10.1093/bja/aes105

REVIEW ARTICLE Perioperative management of hereditary arrhythmogenic syndromes

C. Staikou*, K. Chondrogiannis and A. Mani Department of Anaesthesia, Aretaieio Hospital, Medical School, University of Athens, 76 Vassilissis Sophias Ave., 11528 Athens, Greece * Corresponding author. E-mail: [email protected]

Summary. Patients with inherited cardiac channel disorders are at high risk of perioperative Editor’s key points lethal . Preoperative control of symptoms and a multidisciplinary approach are † Patients with inherited required for a well-planned management. Good haemodynamic , adequate cardiac channel disorders anaesthesia and analgesia, perioperative maintenance of normocarbia, normothermia, and are at high risk of severe normovolaemia are important. In congenital long QT syndrome, torsades de pointes should perioperative be prevented with magnesium sulphate infusion and avoidance of drugs such as droperidol, arrhythmias. succinylcholine, , and ondansetron. and epidural anaesthesia represent safe choices, while caution is needed with volatile agents. In , b-blockers, † Agents used for a-agonists, and drugs should be avoided, while isoproterenol reverses the ECG treatment or to be changes. Propofol, thiopental, and volatiles have been used uneventfully. In congenital sick avoided vary in the sinus syndrome, severe bradycardia resistant to atropine may require isoproterenol or various syndromes and epinephrine. Anaesthetics with vagolytic properties are preferable, while propofol and their subtypes. vecuronium should be given with caution due to risk of inducing bradyarrhythmias. Neuraxial † Understanding of the anaesthesia should produce the least autonomic imbalance. Arrhythmogenic right ventricular potential autonomic dysplasia/cardiomyopathy induces ventricular tachyarrhythmias, which should be treated actions of anaesthetic with b-blockers. Generally, b- stimulation and release should be agents on the different avoided. and pancuronium are contraindicated, while large doses of local conditions is required. anaesthetics and epinephrine should be avoided in neuraxial blocks. In catecholaminergic † Preoperative optimization polymorphic ventricular , b-blocker treatment should be continued and preparation of the perioperatively. Catecholamine release and b-agonists, such as isoproterenol, should be appropriate emergency avoided. Propofol and remifentanil are probably safe, while halothane and pancuronium are drugs is essential. contraindicated. Regional anaesthesia, without epinephrine, is relatively safe. In suspicious cardiac deaths, postmortem examination and familial screening are recommended. Keywords: anaesthesia; arrhythmogenic right ventricular dysplasia; Brugada syndrome; ; congenital long QT syndrome; congenital sick sinus syndrome; polymorphic catecholaminergic

Hereditary arrhythmias comprise a heterogeneous group of Nevertheless, they represent the most common cause of cardiac channel disorders occurring in patients with appar- sudden cardiac death in a young population.13Anaesthesia ently normal hearts.1 Subtype 3 of congenital long QT and surgery may unmask these syndromes, which usually syndrome (LQTS), Brugada syndrome, and congenital sick present as life-threatening arrhythmias in patients with an sinus syndrome (S