CPVT and Complete Atrio-Ventricular Block: The Flipside of the Same Coin

Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) is a rare electrical genetic disease characterized by ventricular polymorphic tachycardia and/or bidirectional ventricular tachycardia induced by the release of catecholamines caused by intense physical or emotional stress in structurally...

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Published inJournal of cardiovascular development and disease Vol. 10; no. 3; p. 97
Main Authors Petrungaro, Mattia, Scarà, Antonio, Borrelli, Alessio, Sciarra, Luigi
Format Journal Article
LanguageEnglish
Published Switzerland MDPI AG 23.02.2023
MDPI
Subjects
Age
Behavior modification
Beta blockers
Calcium-binding proteins
Cardiac arrhythmia
Cardiac stress tests
Cardiomyopathy
catecholaminergic polymorphic ventricular tachycardia
Catecholamines
Communication
CPVT
Epilepsy
Exercise
Families & family life
Genes
Genetic aspects
Magnetic resonance imaging
Mutation
Pacemakers
Patients
Physical fitness
Ryr2
Stress (Psychology)
sudden cardiac death
Tachycardia
TVNS
Ryr2
TVNS
catecholaminergic polymorphic ventricular tachycardia
sudden cardiac death
CPVT
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Summary:Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) is a rare electrical genetic disease characterized by ventricular polymorphic tachycardia and/or bidirectional ventricular tachycardia induced by the release of catecholamines caused by intense physical or emotional stress in structurally normal hearts. Mostly, it is caused by mutations in genes that are involved in calcium homeostasis, in particular in the gene encoding for cardiac ryanodine receptor (RyR2). Our observation is the first description of familial CPVT caused by mutation of the RyR2 gene, linked to the complete AV block.
Bibliography:ObjectType-Article-1
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ISSN:2308-3425
2308-3425
DOI:10.3390/jcdd10030097