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用于伴有2:1房室传导阻滞的新生儿长QT综合征的美西律输注激发试验。

Mexiletine infusion challenge test for neonatal long QT syndrome with 2:1 atrioventricular block.

作者信息

Okuwaki Hajime, Kato Yoshiaki, Lin Lisheng, Nozaki Yoshihiro, Takahashi-Igari Miho, Horigome Hitoshi

机构信息

Department of Child Health, Faculty of Medicine University of Tsukuba Tsukuba Japan.

Division of Pediatric Cardiology National Cerebral and Cardiovascular Center Suita Japan.

出版信息

J Arrhythm. 2019 Jun 17;35(4):685-688. doi: 10.1002/joa3.12209. eCollection 2019 Aug.

DOI:10.1002/joa3.12209
PMID:31410243
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6686300/
Abstract

For applying a genotype-based treatment in neonatal long QT syndrome (LQTS), early detection of the genotype becomes an important issue. We report a case of a neonate with LQTS type 3 that presented with 2:1 atrioventricular block and underwent a mexiletine infusion challenge test, and achieved shortening of the QTc and 1:1 atrioventricular conduction. The mexiletine infusion challenge test was helpful to make an early detection of the genotype of the LQTS and predicted the drug efficacy in a neonatal patient.

摘要

对于在新生儿长QT综合征（LQTS）中应用基于基因型的治疗，基因型的早期检测成为一个重要问题。我们报告了一例3型LQTS新生儿病例，该患儿表现为2:1房室传导阻滞，并接受了美西律输注激发试验，结果QTc缩短且房室传导转为1:1。美西律输注激发试验有助于早期检测LQTS的基因型，并预测新生儿患者的药物疗效。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0fe5/6686300/3527696a8444/JOA3-35-685-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0fe5/6686300/3e9c4416f2a9/JOA3-35-685-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0fe5/6686300/ffb777a6667d/JOA3-35-685-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0fe5/6686300/3527696a8444/JOA3-35-685-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0fe5/6686300/3e9c4416f2a9/JOA3-35-685-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0fe5/6686300/ffb777a6667d/JOA3-35-685-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0fe5/6686300/3527696a8444/JOA3-35-685-g003.jpg

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本文引用的文献

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Lidocaine attenuation testing: An in vivo investigation of putative LQT3-associated variants in the SCN5A-encoded sodium channel.利多卡因衰减测试：对SCN5A编码的钠通道中假定的LQT3相关变体的体内研究。
Heart Rhythm. 2017 Aug;14(8):1173-1179. doi: 10.1016/j.hrthm.2017.04.020. Epub 2017 Apr 13.
2
Gene-Specific Therapy With Mexiletine Reduces Arrhythmic Events in Patients With Long QT Syndrome Type 3.美西律基因特异性疗法可减少3型长QT综合征患者的心律失常事件。
J Am Coll Cardiol. 2016 Mar 8;67(9):1053-1058. doi: 10.1016/j.jacc.2015.12.033.
3
Pronounced Shortening of QT Interval With Mexiletine Infusion Test in Patients With Type 3 Congenital Long QT Syndrome.
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Korean Circ J. 2021 Sep;51(9):792-796. doi: 10.4070/kcj.2021.0202.
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4
Clinical characteristics and genetic background of congenital long-QT syndrome diagnosed in fetal, neonatal, and infantile life: a nationwide questionnaire survey in Japan.先天性长 QT 综合征在胎儿、新生儿和婴儿期的临床特征和遗传背景：日本全国问卷调查。
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All LQT3 patients need an ICD: true or false?所有长QT综合征3型（LQT3）患者都需要植入式心律转复除颤器（ICD）：是真还是假？
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