妊娠合并长QT间期综合征的研究进展

doi:10.12280/gjfckx.20220290

摘要/Abstract

摘要：

长QT间期综合征（long QT syndrome,LQTS）是一种由基因突变导致离子通道结构或功能异常的遗传性心脏疾病,心电图以QT间期延长及T波改变为特征,易发生心悸、晕厥、心律失常、心脏骤停甚至猝死等。LQTS患者临床最常见的基因亚型为LQT1型、LQT2型和LQT3型,不同基因亚型的LQTS在突变基因、触发因素甚至心电图改变上均有差异,明确患者基因亚型,有助于临床诊断、治疗及对心脏事件发生的预防。有效预防心脏事件发生对LQTS患者是最重要的措施之一;合并LQTS孕产妇在妊娠、产褥期将经历激素水平、血流动力学改变等一系列生理性变化,均可能影响LQTS相关心脏事件的发生风险;其临床管理也不同于非LQTS孕产妇,有必要了解其发病机制、诊断标准、治疗方案及围生期管理的研究进展。

关键词: 妊娠, QT延长综合征, 围产期, 诊断, 治疗

Abstract:

Long QT syndrome (LQTS) is a genetic heart disease caused by mutations in genes that lead to abnormalities in the structure or function of ion channels, and is characterized by prolonged QT interval and T-wave changes in the electrocardiogram (ECG), and it is prone to palpitation, synocope, arrhythmia, cardiac arrest and even sudden death. The most common genetic subtypes of LQTS patients are LQT1, LQT2, and LQT3. Different Genetic subtypes of LQTS have different mutation genes, triggers, and even ECG changes, so clarifying the genetic subtypes of patients can help clinical diagnosis, treatment and prevention of cardiac events. Effective prevention of cardiac events is one of the most important measures for patients with LQTS. During pregnancy and puerperium, women with LQTS will experience a series of physiological changes such as hormonal levels and hemodynamic changes, which may affect the risk of cardiac events associated with LQTS; their clinical management is also different from that of non-LQTS women, and it is necessary to review the research progress of its pathogenesis, diagnostic criteria, treatment options and perinatal management.

Key words: Pregnancy, Long QT syndrome, Peripartum period, Diagnosis, Therapy

梁美育, 范卓然, 华绍芳. 妊娠合并长QT间期综合征的研究进展[J]. 国际妇产科学杂志, 2022, 49(5): 580-584.

LIANG Mei-Yu, FAN Zhuo-Ran, HUA Shao-Fang. Progress in the Study of Pregnancy Complicated with Long QT Syndrome[J]. Journal of International Obstetrics and Gynecology, 2022, 49(5): 580-584.

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