Pregnancy and the risk of torsades de pointes in congenital long-QT syndrome
Pregnancy and the risk of torsades de pointes in congenital long-QT syndrome
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DOI：
10.1007/bf03086191
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发表时间：
2008-12-01
影响因子：
2
通讯作者：
Wilde, A. A. M.
中科院分区：
文献类型：
--
作者：
Meregalli, P. G.;Westendorp, I. C. D.;Wilde, A. A. M.
先天性长QT综合征（LQTS）患者在应激状态下发生室性心律失常的风险增加。大规模的研究指出，受影响的人在怀孕后的时期（产后）特别危险。这尤其适用于患有LQTS 2型的女性。在这里，我们描述了两例年轻女性与LQTS 2型，都承认我们的机构与症状性尖端扭转型室性心动过速分娩后几周。两名患者都携带KCNH 2基因突变。一名患者为未产妇，另一名患者既往妊娠顺利。在这两种情况下，用P受体阻滞剂治疗并不能预防危及生命的心律失常。心律失常的风险被认为在分娩后的9个月内逐渐降低到怀孕前的水平。考虑到与持续监测患者如此长时间相关的困难以及这些患者希望在可预见的未来生育更多子女，进行了ICD植入。(Neth Heart J 2008; 16：422-5.）
Patients with congenital long-QT syndrome (LQTS) are at increased risk of ventricular arrhythmias during stressful situations. Large-scale studies have pointed out that affected individuals are particularly at risk in the period following pregnancy (post-partum). This is recognised especially for women with an LQTS type 2. Here, we describe two cases of young women with LQTS type 2, both admitted to our institution with symptomatic torsades de pointes a few weeks after delivery. Both patients carried a mutation in the KCNH2 gene. One patient was nullipara, while the other had had an uneventful previous pregnancy. In both cases treatment with a P-blocker did not prevent life-threatening cardiac arrhythmias. The risk of arrhythmias is thought to gradually decrease to pre-pregnancy values in the nine months after delivery. Considering the difficulties related to continuous monitoring of a patient for such a long period and the desire of these patients to have more children in the foreseeable future, ICD implantation,was performed. (Neth Heart J 2008; 16:422-5.)