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Current topics in catecholaminergic polymorphic ventricular tachycardia

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is induced by emotions or exercise in patients without organic heart disease and may be polymorphic or bidirectional in nature. The prognosis of CPVT is not good, and therefore prevention of sudden death is of utmost importance. Genetic va...

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Autor principal: Sumitomo, Naokata
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5063269/
https://www.ncbi.nlm.nih.gov/pubmed/27761157
http://dx.doi.org/10.1016/j.joa.2015.09.008
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author Sumitomo, Naokata
author_facet Sumitomo, Naokata
author_sort Sumitomo, Naokata
collection PubMed
description Catecholaminergic polymorphic ventricular tachycardia (CPVT) is induced by emotions or exercise in patients without organic heart disease and may be polymorphic or bidirectional in nature. The prognosis of CPVT is not good, and therefore prevention of sudden death is of utmost importance. Genetic variants of CPVT include RyR2, CASQ2, CALM2, TRD, and possibly KCNJ2 and ANK2 gene mutations. Hypotheses that suggest the causes of CPVT include weakened binding of FKBP12.6 and RyR2, a store overload-induced Ca(2+) release (SOICR), unzipping of intramolecular domain interactions in RyR2, and molecular and functional abnormalities caused by mutations in the CASQ2 gene. The incidence of an RyR2 anomaly in CPVTs is about 35–79%, whereas anomalies in the CASQ2 gene account for 3–5% CPVTs. The ping-pong theory, suggesting that reciprocating delayed after depolarization induces bigeminy of the right and left bundle branches, may explain the pathogenesis of bidirectional ventricular tachycardia. Flecainide, carvedilol, left sympathetic nerve denervation, and catheter ablation of the PVC may serve as new therapeutic strategies for CPVT while gene-therapy may be applied to some types of CPVT in the future. Although, not all sudden cardiac deaths in CPVT patients are currently preventable, new medical and interventional therapies may improve CPVT prognosis.
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spelling pubmed-50632692016-10-19 Current topics in catecholaminergic polymorphic ventricular tachycardia Sumitomo, Naokata J Arrhythm Review Catecholaminergic polymorphic ventricular tachycardia (CPVT) is induced by emotions or exercise in patients without organic heart disease and may be polymorphic or bidirectional in nature. The prognosis of CPVT is not good, and therefore prevention of sudden death is of utmost importance. Genetic variants of CPVT include RyR2, CASQ2, CALM2, TRD, and possibly KCNJ2 and ANK2 gene mutations. Hypotheses that suggest the causes of CPVT include weakened binding of FKBP12.6 and RyR2, a store overload-induced Ca(2+) release (SOICR), unzipping of intramolecular domain interactions in RyR2, and molecular and functional abnormalities caused by mutations in the CASQ2 gene. The incidence of an RyR2 anomaly in CPVTs is about 35–79%, whereas anomalies in the CASQ2 gene account for 3–5% CPVTs. The ping-pong theory, suggesting that reciprocating delayed after depolarization induces bigeminy of the right and left bundle branches, may explain the pathogenesis of bidirectional ventricular tachycardia. Flecainide, carvedilol, left sympathetic nerve denervation, and catheter ablation of the PVC may serve as new therapeutic strategies for CPVT while gene-therapy may be applied to some types of CPVT in the future. Although, not all sudden cardiac deaths in CPVT patients are currently preventable, new medical and interventional therapies may improve CPVT prognosis. Elsevier 2016-10 2015-11-24 /pmc/articles/PMC5063269/ /pubmed/27761157 http://dx.doi.org/10.1016/j.joa.2015.09.008 Text en © 2015 Japanese Heart Rhythm Society. Published by Elsevier B.V. http://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Review
Sumitomo, Naokata
Current topics in catecholaminergic polymorphic ventricular tachycardia
title Current topics in catecholaminergic polymorphic ventricular tachycardia
title_full Current topics in catecholaminergic polymorphic ventricular tachycardia
title_fullStr Current topics in catecholaminergic polymorphic ventricular tachycardia
title_full_unstemmed Current topics in catecholaminergic polymorphic ventricular tachycardia
title_short Current topics in catecholaminergic polymorphic ventricular tachycardia
title_sort current topics in catecholaminergic polymorphic ventricular tachycardia
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5063269/
https://www.ncbi.nlm.nih.gov/pubmed/27761157
http://dx.doi.org/10.1016/j.joa.2015.09.008
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