Department of Cardiology, Heart Centre, Amsterdam UMC Location Academic Medical Centre, University of Amsterdam, Meibergdreef 9, Amsterdam, the Netherlands; Amsterdam Cardiovascular Sciences, Heart Failure and Arrhythmias, Amsterdam, the Netherlands.
Department of Cardiology, Heart Centre, Amsterdam UMC Location Academic Medical Centre, University of Amsterdam, Meibergdreef 9, Amsterdam, the Netherlands; Amsterdam Cardiovascular Sciences, Heart Failure and Arrhythmias, Amsterdam, the Netherlands.
Card Electrophysiol Clin. 2023 Sep;15(3):293-305. doi: 10.1016/j.ccep.2023.04.002. Epub 2023 Jun 20.
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.
儿茶酚胺多形性室性心动过速（CPVT）是一种遗传性心律失常综合征，其特征为运动或情绪诱发双向或多形性室性心律失常。大多数病例由编码心脏兰尼碱受体（RYR2）的基因突变引起。CPVT 患者的治疗选择在这些年中有所增加，并且有证据表明这些治疗选择降低了心律失常事件的发生率。此外，还有许多潜在的新疗法正在研究中。在这篇综述中，我们总结了 CPVT 患者的既定和潜在未来治疗策略的知识现状，并描述了我们对其管理的方法。
