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Catecholaminergic Polymorphic Ventricular Tachycardia.

Abbas, M; Miles, C; Behr, E (2022) Catecholaminergic Polymorphic Ventricular Tachycardia. Arrhythm Electrophysiol Rev, 11. e20. ISSN 2050-3369 https://doi.org/10.15420/aer.2022.09
SGUL Authors: Behr, Elijah Raphael

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Abstract

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited arrhythmia syndrome characterised by adenergically mediated bidirectional and/or polymorphic ventricular tachycardia. CPVT is a significant cause of autopsy-negative sudden death in children and adolescents, although it can also affect adults. It is often caused by pathogenic variants in the cardiac ryanodine receptor gene as well as other rarer genes. Early identification and risk stratification is of major importance. β-blockers are the cornerstone of therapy. Sodium channel blockers, specifically flecainide, have an additive role. Left cardiac sympathetic denervation is playing an increasing role in suppression of arrhythmia and symptoms. Concerns have been raised, however, about the efficacy of implantable cardioverter defibrillator therapy and the risk of catecholamine driven proarrhythmic storms. In this review, we summarise the clinical characteristics, genetics, and diagnostic and therapeutic strategies for CPVT and describe recent advances and challenges.

Item Type: Article
Keywords: catecholaminergic polymorphic ventricular tachycardia, flecainide, left cardiac sympathetic denervation, ryanodine receptor mutation, sudden cardiac death, β-blockers
SGUL Research Institute / Research Centre: Academic Structure > Molecular and Clinical Sciences Research Institute (MCS)
Journal or Publication Title: Arrhythm Electrophysiol Rev
ISSN: 2050-3369
Language: eng
Dates:
DateEvent
April 2022Published
21 October 2022Published Online
2 July 2022Accepted
Publisher License: Creative Commons: Attribution-Noncommercial 4.0
PubMed ID: 36644199
Go to PubMed abstract
URI: https://openaccess.sgul.ac.uk/id/eprint/115141
Publisher's version: https://doi.org/10.15420/aer.2022.09

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