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World J Anesthesiol. Nov 27, 2016; 5(3): 44-53
Published online Nov 27, 2016. doi: 10.5313/wja.v5.i3.44
Anaesthesia for patients with arrhythmogenic right ventricular dysplasia
Ivett Blaskovics, Kamen Valchanov
Ivett Blaskovics, Kamen Valchanov, Department of Anaesthesia and Intensive Care, Papworth Hospital, Cambridge CB23 3RE, United Kingdom
Author contributions: Both authors contributed to this manuscript.
Conflict-of-interest statement: Authors declare no conflict of interests for this article.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Kamen Valchanov, MD, FRCA, FFICM, Department of Anaesthesia and Intensive Care, Papworth Hospital, Papworth Everard, Cambridge CB23 3RE, United Kingdom. kamen.valchanov@papworth.nhs.uk
Telephone: +44-1480-364406 Fax: +44-1480-364936
Received: July 20, 2016
Peer-review started: July 21 2016
First decision: September 5, 2016
Revised: October 13, 2016
Accepted: November 1, 2016
Article in press: November 2, 2016
Published online: November 27, 2016
Abstract

Arrhythmogenic right ventricular dysplasia (ARVD) is an inherited heart muscle disease. Myocyte apoptosis and fibro-fatty scar tissue predisposes patients to malignant ventricular arrhythmias. Patients may present to variety of surgical procedures with diagnosed ARVD. Surgical insult, catecholamine surge and physiological disturbance can be hazardous on the vulnerable myocardium and may result in life-threatening ventricular tachycardia or sudden cardiac death in the perioperative period. Anaesthetists have particular role in perioperative management of this patient population, meticulous perioperative planning, close haemodynamic monitoring and maintenance of physiological stability throughout helps to avoid devastating perioperative loss.

Keywords: Arrhythmogenic right ventricular dysplasia, Arrhytmogenic cardiomyopathy, Anaesthesia

Core tip: Arrhythmogenic right ventricular dysplasia (ARVD) is an inherited disease of the cardiac muscle, characterised by progressive myocyte death and scarring of the myocardium associated with ventricular tachycardia and sudden cardiac death. Electrical instability is exacerbated by physiological changes induced by surgical insult and may lead to unexpected perioperative death. Careful anaesthetic management can minimise stress response and reduce the incidence of malignant ventricular arrhythmias in the perioperative period. In this article we discuss the available literature with the aim to provide some guidance for the clinical anaesthetist encountering patient with ARVD.