TECRL gene in catecholaminergic polymorphic ventricular tachycardia
10.3760/cma.j.issn.1673-4408.2021.08.002
- VernacularTitle:TECRL基因与儿茶酚胺敏感性室性心动过速
- Author:
Junmin ZHENG
1
;
Cuilan HOU
;
Tingting XIAO
Author Information
1. 上海交通大学附属儿童医院心内科 200062
- Keywords:
Catecholaminergic polymorphic ventricular tachycardia;
TECRL gene
- From:
International Journal of Pediatrics
2021;48(8):511-514
- CountryChina
- Language:Chinese
-
Abstract:
Catecholaminergic polymorphic ventricular tachycardia(CPVT)is a highly fatal inherited arrhythmia induced by emotional stress or exercise.It can be triggered by rapid polymorphism of ventricular tachycardia and ventricular fibrillation, and may lead to syncope or sudden death, with a poor prognosis.Genetic testing is one way to diagnose the disease.It has been found that the disease is related to abnormalities of RyR2, CASQ2, TECRL and other genes, whose mutations affect calcium homeostasis and lead to abnormal electrophysiological activity of the heart, leading to delayed depolar(DADs), and subsequently to malignant arrhythmia.This paper reviewes the mutation of the new pathogenic gene TECRL gene in catecholamine sensitive ventricular tachycardia, through the understanding and learning of the mutation gene reported in the previous literature, in order to further explore the pathogenesis of the disease, learn to deal with the occurrence of malignant arrhythmia, and promote the clinical precise treatment of the disease.
