Progress in mechanisms, diagnosis and therapeutic management of catecholaminergic polymorphic ventricular tachycardia
Journal Title: Journal of Clinical Pediatrics - Year 2025, Vol 43, Issue 3
Abstract
Catecholaminergic polymorphic ventricular tachycardia is a hereditary cardiac channelopathy. Most cases are related to mutations in the RYR2 and CASQ2 genes, which severely disrupt the calcium homeostasis in cardiac cells. Excessive calcium release leads to delayed depolarization, ultimately leading to arrhythmia. This disease is seen in patients who experience syncope after intense exercise or stress-related emotions, as well as in patients with sudden cardiac arrest or even sudden cardiac death. It is mainly diagnosed through exercise stress testing and genetic testing. Standard treatment for CPVT relies on beta-blockers, while flucainide and left ventricular sympathetic nerve denervation are second-line treatments. Implantation of cardioverter defibrillators is suitable for patients at a high risk of sudden death, and some potential therapeutic interventions have also been identified. This review summarizes the genetics, pathophysiology, clinical features, diagnosis, and treatment strategies of CPVT, with the aim of providing clinical guidance.
Authors and Affiliations
Ge YAN, Cuilan HOU, Tingting XIAO
Keywords
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- EP ID EP763267
- DOI 10.12372/jcp.2025.24e0677
- Views 12
- Downloads 0
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