关键词: Catecholaminergic polymorphic ventricular tachycardia Flecainide RYR2-specific compounds Sudden cardiac death Therapeutic management β-blockers

Mesh : Humans Anti-Arrhythmia Agents Tachycardia, Ventricular / genetics therapy Exercise Death, Sudden, Cardiac / etiology Ryanodine Receptor Calcium Release Channel / genetics Mutation

来  源:   DOI:10.1016/j.ccep.2023.04.002

Abstract:
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited arrhythmia syndrome characterized by bidirectional or polymorphic ventricular arrhythmia provoked by exercise or emotion. Most cases are caused by pathogenic variants in the gene encoding the cardiac ryanodine receptor (RYR2). The options for treating patients with CPVT have increased during the years, and evidence suggests that these have led to lower arrhythmic event rates. In addition, numerous potential new therapies are being investigated. In this review, we summarize the state of knowledge on both established and potential future treatment strategies for patients with CPVT and describe our approach to their management.
摘要:
儿茶酚胺能多形性室性心动过速(CPVT)是一种遗传性心律失常综合征,其特征是由运动或情绪引起的双向或多形性室性心律失常。大多数病例是由编码心脏ryanodine受体(RYR2)的基因中的致病变体引起的。多年来,治疗CPVT患者的选择有所增加,和证据表明,这些导致较低的心律失常事件率。此外,许多潜在的新疗法正在研究中。在这次审查中,我们总结了关于CPVT患者既定和潜在未来治疗策略的知识状况,并描述了我们的治疗方法.
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