Obstetric management of a patient with Andersen?Tawil syndrome: A case report

Obstetric management of a patient with Andersen?Tawil syndrome: A case report
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安德森·塔维尔综合征患者的产科管理:病例报告

DOI:
10.1111/jog.14553
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发表时间:
2020
影响因子:
1.6
通讯作者:
Miyasaka Naoyuki
Miyasaka Naoyuki
中科院分区:
医学4区
文献类型:
--
作者:
Inagaki Mai;Tatsumi Takayuki;Yomogita Hiroshi;Hirose Asuka;Kubo Takuyuki;Sekiguchi Masaki;Miyasaka Naoyuki

文献摘要

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anderson - tawil综合征(ATS)是一种罕见的遗传性长QT综合征7型,由kcnj2基因错义突变引起。ATS的特点是室性心律失常,周期性肢体麻痹和轻微的外部畸形。尽管迄今为止只有3例Andersen-Tawil综合征的孕妇报告,但与怀孕前相比,所有病例从产前到分娩均未观察到室性心律失常加重,这表明从产前到分娩发生心律失常事件的风险并不高。与以往的报道不同,我们在此报告一例安德森-塔威尔综合征,在分娩过程中室性心律失常增加,室性心动过速持续发展。我们还建议谨慎的孕妇安德森-塔威尔综合征可能有不同时间的心律失常加重,室性心律失常可能与子宫收缩疼痛有关。
Andersen–Tawil syndrome (ATS) is a rare hereditary long QT syndrome type 7 caused by a missense mutation in theKCNJ2gene. ATS is characterized by ventricular arrhythmia, periodic limb paralysis and minor external malformations. Although only three reports of pregnant women with Andersen–Tawil syndrome have been reported to date, no exacerbation of ventricular arrhythmia was observed from pre‐partum to delivery in all cases compared to that before pregnancy, and it was suggested that the risk of arrhythmic events from pre‐partum to delivery is not high. Unlike these previous reports, we herein present a case of Andersen–Tawil syndrome in which ventricular arrhythmias increased and sustained ventricular tachycardia was developed during labor progression. We also advise caution that pregnant patients with Andersen–Tawil syndrome may have varying times of exacerbation of the arrhythmia, and ventricular arrhythmias may be associated with painful uterine contractions.