美西律可缩短一个相关长QT综合征家系中的QT间期。

Mexiletine shortens the QT interval in a pedigree of related long QT syndrome.

作者信息

Fujisawa Taishi, Aizawa Yoshiyasu, Katsumata Yoshinori, Kimura Kensuke, Hashimoto Kenji, Yamashita Terumasa, Miyama Hiroshi, Kimura Takehiro, Kosaki Kenjiro, Takatsuki Seiji, Shimizu Wataru, Fukuda Keiichi

机构信息

Department of Cardiology Keio University School of Medicine Tokyo Japan.

Department of Cardiovascular Medicine International University of Health and Welfare Chiba Japan.

出版信息

J Arrhythm. 2020 Jan 8;36(1):193-196. doi: 10.1002/joa3.12300. eCollection 2020 Feb.

DOI:10.1002/joa3.12300

PMID:32071644

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7011830/

Abstract

A 23-year-old female had been suffering from recurrent syncopal episodes during sleep since her childhood. She had a family history of sudden death and her QTc interval was remarkably prolonged to 537 ms A Holter ECG revealed torsade de pointes, corresponding to syncope. She was started on mexiletine and her QTc interval shortened. Her symptoms were controlled after β-blockers and Ca-blockers were added. A genetic analysis with a next generation sequencer identified a frameshift mutation at the C terminus of the gene. Here we present a type 2 long QT syndrome case in which mexiletine was effective.

摘要

一名23岁女性自童年起就一直遭受睡眠期间反复出现的晕厥发作。她有猝死家族史，其QTc间期显著延长至537毫秒。动态心电图显示尖端扭转型室速，与晕厥相符。她开始服用美西律，QTc间期缩短。在加用β受体阻滞剂和钙通道阻滞剂后，她的症状得到控制。使用下一代测序仪进行的基因分析在该基因的C末端发现了一个移码突变。在此，我们报告一例美西律有效的2型长QT综合征病例。