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奎尼丁在猝死综合征中的应用面临的挑战和影响:一项全国性经验。

Challenge and Impact of Quinidine Access in Sudden Death Syndromes: A National Experience.

机构信息

University of British Columbia, Vancouver, British Columbia, Canada.

Western University, London, Ontario, Canada.

出版信息

JACC Clin Electrophysiol. 2019 Mar;5(3):376-382. doi: 10.1016/j.jacep.2018.10.007. Epub 2018 Nov 28.

DOI:10.1016/j.jacep.2018.10.007
PMID:30898241
Abstract

OBJECTIVES

This study sought to determine the nature of quinidine use and accessibility in a national network of inherited arrhythmia clinics.

BACKGROUND

Quinidine is an antiarrhythmic medication that has been shown to be beneficial in select patients with Brugada syndrome, early repolarization syndrome, and idiopathic ventricular fibrillation. Because of the low prevalence of these conditions and restricted access to quinidine through a single regulatory process, quinidine use is rare in Canada.

METHODS

Subjects prescribed quinidine were identified through the Hearts in Rhythm Organization that connects the network of inherited arrhythmia clinics across Canada. Cases were retrospectively reviewed for patient characteristics, indications for quinidine use, rate of recurrent ventricular arrhythmia, and issues with quinidine accessibility.

RESULTS

In a population of 36 million, 46 patients are currently prescribed quinidine (0.0000013%, age 48.1 ± 16.1 years, 25 are male). Brugada syndrome, early repolarization syndrome, and idiopathic ventricular fibrillation constituted a diagnosis in 13 subjects (28%), 6 (13%), and 21 (46%), respectively. Overall, 37 subjects (81%) had cardiac arrest as an index event. After initial presentation, subjects experienced 7.47 ± 12.3 implantable cardioverter-defibrillator shocks prior to quinidine use over 34.3 ± 45.9 months, versus 0.86 ± 1.69 implantable cardioverter-defibrillator shocks in 43.8 ± 41.8 months while on quinidine (risk ratio: 8.7, p < 0.001). Twenty-two patients access quinidine through routes external to Health Canada's Special Access Program.

CONCLUSIONS

Quinidine use is rare in Canada, but it is associated with a reduction in recurrent ventricular arrhythmias in patients with Brugada syndrome, early repolarization syndrome, and idiopathic ventricular fibrillation, with minimal toxicity necessitating discontinuation. Drug interruption is associated with frequent breakthrough events. Access to quinidine is important to deliver this potentially lifesaving therapy.

摘要

目的

本研究旨在确定在全国遗传性心律失常诊所网络中奎尼丁的使用和可及性。

背景

奎尼丁是一种抗心律失常药物,已被证明对 Brugada 综合征、早期复极综合征和特发性心室颤动的某些患者有益。由于这些疾病的患病率较低,并且通过单一监管程序获得奎尼丁的途径受限,因此在加拿大奎尼丁的使用非常罕见。

方法

通过连接加拿大遗传性心律失常诊所网络的 Hearts in Rhythm Organization 确定开奎尼丁处方的患者。对患者特征、奎尼丁使用的适应证、复发性室性心律失常的发生率以及奎尼丁可及性的问题进行回顾性审查。

结果

在 3600 万人口中,目前有 46 名患者开奎尼丁处方(0.0000013%,年龄 48.1±16.1 岁,25 名男性)。Brugada 综合征、早期复极综合征和特发性心室颤动分别构成 13 例(28%)、6 例(13%)和 21 例(46%)的诊断。总体而言,37 例(81%)患者以心脏骤停作为首发事件。初次就诊后,在使用奎尼丁前的 34.3±45.9 个月中,患者经历了 7.47±12.3 次植入式心律转复除颤器电击,而在使用奎尼丁后的 43.8±41.8 个月中则为 0.86±1.69 次(风险比:8.7,p<0.001)。22 名患者通过加拿大卫生部特殊准入计划以外的途径获得奎尼丁。

结论

奎尼丁在加拿大的使用很少见,但与 Brugada 综合征、早期复极综合征和特发性心室颤动患者的复发性室性心律失常减少有关,且需要停药的最小毒性。药物中断与频繁的突破性事件有关。获得奎尼丁对提供这种潜在的救生治疗很重要。

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