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患有与CASQ2相关的儿茶酚胺能多形性室性心动过速患者的左心交感神经去神经支配术

Left Cardiac Sympathetic Denervation in Patients with CASQ2-Associated Catecholaminergic Polymorphic Ventricular Tachycardia.

作者信息

Marai Ibrahim, Boulos Monther, Khoury Asaad

出版信息

Isr Med Assoc J. 2015 Sep;17(9):538-40.

Abstract

BACKGROUND

Left cardiac sympathetic denervation (LCSD) was reported to be effective in patients with intractable ryanodine receptor mutation-associated catecholaminergic polymorphic ventricular tachycardia (CPVT).

OBJECTIVES

To report our experience with LCSD in calsequestrin (CASQ2) mutation-associated CPVT.

METHODS

LCSD was performed in three patients with CASQ2 mutation-associated CPVT with symptoms and exercise-induced ventricular arrhythmia despite high dose beta-blocker

RESULTS

None of them experienced symptoms or exercise-induced ventricular arrhythmia after LCSD. However, all had recurrence of symptoms and/or exercise-induced ventricular arrhythmia after 6 months (6-18 months).

CONCLUSIONS

LCSD conferred short-term suppression but less than optimal long-term suppression of exercise-induced ventricular arrhythmia among CASQ2-associated CPVT patients.

摘要

背景

据报道,左心交感神经去神经支配术(LCSD)对患有难治性兰尼碱受体突变相关儿茶酚胺能多形性室性心动过速(CPVT)的患者有效。

目的

报告我们在肌集钙蛋白(CASQ2)突变相关CPVT患者中进行LCSD的经验。

方法

对3例患有CASQ2突变相关CPVT且尽管使用了高剂量β受体阻滞剂仍有症状和运动诱发室性心律失常的患者进行了LCSD。

结果

LCSD后,他们均未出现症状或运动诱发的室性心律失常。然而,6个月后(6 - 18个月),所有患者症状和/或运动诱发的室性心律失常均复发。

结论

在与CASQ2相关的CPVT患者中,LCSD可实现运动诱发室性心律失常的短期抑制,但长期抑制效果欠佳。

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