Diagnosis and treatment of catecholaminergic polymorphic ventricular tachycardia
10.3760/cma.j.issn.1673-4408.2021.02.003
- VernacularTitle:儿茶酚胺敏感性多形性室性心动过速的诊治进展
- Author:
Yan MA
;
Ji CHENG
- From:
International Journal of Pediatrics
2021;48(2):82-86
- CountryChina
- Language:Chinese
-
Abstract:
Catecholaminergic polymorphic ventricular tachycardia is a rare hereditary ion channel disease, which mainly affects children and young adults.Clinically, the pathogenic genes of CPVT in adolescents are mainly RYR2, CASQ2, TRDN, CALM1 and TRD.Currently, beta blockers remain the first-line treatment for CPVT, and moderate exercise training is considered as an effective potential synergism for CPVT patients.Implantable cardioverter defibrillators are commonly used in the treatment of CPVT, but inappropriate shocks and device-related complications need to be carefully considered before implantation.Flecainide has been shown to be effective therapeutic agent, especially for the treatment of resistant CPVT cases with beta blockers.Left cardiac sympathetic denervation has also shown to be highly effective in preventing CPVT symtoms and cardiac events, and a combination of beta blockers can precede implantable cardioverter defibrillator implantation.
