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Pediatric Catecholaminergic Polymorphic Ventricular Tachycardia: A Translational Perspective for the Clinician-Scientist Kallas, Dania; Lamba, Avani; Roston, Thomas; Arslanova, Alia; Franciosi, Sonia; Tibbits, Glen; Sanatani, Shubhayan

Abstract

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare and potentially lethal inherited arrhythmia disease characterized by exercise or emotion-induced bidirectional or polymorphic ventricular tachyarrhythmias. The median age of disease onset is reported to be approximately 10 years of age. The majority of CPVT patients have pathogenic variants in the gene encoding the cardiac ryanodine receptor, or calsequestrin 2. These lead to mishandling of calcium in cardiomyocytes resulting in after-depolarizations, and ventricular arrhythmias. Disease severity is particularly pronounced in younger individuals who usually present with cardiac arrest and arrhythmic syncope. Risk stratification is imprecise and long-term prognosis on therapy is unknown despite decades of research focused on pediatric CPVT populations. The purpose of this review is to summarize contemporary data on pediatric CPVT, highlight knowledge gaps and present future research directions for the clinician-scientist to address.

Item Metadata

Title
Pediatric Catecholaminergic Polymorphic Ventricular Tachycardia: A Translational Perspective for the Clinician-Scientist
Creator
Kallas, Dania; Lamba, Avani; Roston, Thomas; Arslanova, Alia; Franciosi, Sonia; Tibbits, Glen; Sanatani, Shubhayan
Contributor
Children's Hospital (Vancouver, B.C.). Children's Heart Centre; University of British Columbia. Clinical Investigator Program; Children's Hospital (Vancouver, B.C.). Research Institute
Publisher
Multidisciplinary Digital Publishing Institute
Date Issued
2021-08-27
Description
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare and potentially lethal inherited arrhythmia disease characterized by exercise or emotion-induced bidirectional or polymorphic ventricular tachyarrhythmias. The median age of disease onset is reported to be approximately 10 years of age. The majority of CPVT patients have pathogenic variants in the gene encoding the cardiac ryanodine receptor, or calsequestrin 2. These lead to mishandling of calcium in cardiomyocytes resulting in after-depolarizations, and ventricular arrhythmias. Disease severity is particularly pronounced in younger individuals who usually present with cardiac arrest and arrhythmic syncope. Risk stratification is imprecise and long-term prognosis on therapy is unknown despite decades of research focused on pediatric CPVT populations. The purpose of this review is to summarize contemporary data on pediatric CPVT, highlight knowledge gaps and present future research directions for the clinician-scientist to address.
Subject
ventricular tachycardia; sudden cardiac death; pediatric electrophysiology; catecholaminergic polymorphic ventricular tachycardia; inherited arrhythmia; primary electrical disease; ryanodine receptor
Genre
Article
Type
Text
Language
eng
Date Available
2021-10-06
Provider
Vancouver : University of British Columbia Library
Rights
CC BY 4.0
DOI
10.14288/1.0402448
URI
http://hdl.handle.net/2429/79895
Affiliation
Medicine, Faculty of; Other UBC; Non UBC; Cellular and Physiological Sciences, Department of
Citation
International Journal of Molecular Sciences 22 (17): 9293 (2021)
Publisher DOI
10.3390/ijms22179293
Peer Review Status
Reviewed
Scholarly Level
Faculty; Researcher
Rights URI
https://creativecommons.org/licenses/by/4.0/
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CC BY 4.0