Chinese Journal of Practical Internal Medicine

›› 2013, Vol. 33 ›› Issue (1): 17-20.

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Diagnosis and treatment of short QT syndrome

HONG Kui   

  1. Department of Cardiovascular Medicine，the Second Affiliated Hospital of Nanchang University，Nanchang 330006，China
  • Online:2013-01-01 Published:2013-01-05

短QT综合征诊断与治疗

洪葵   

  1. 南昌大学第二附属医院心内科，南昌 330006
  • 作者简介:洪葵，教授、主任医师、博士生导师。历任留日医学博士、留美博士后及助理教授。现任南昌大学第二附属医院心内科副主任，教育部创新团队带头人。发表论文近100余篇，SCI收录40篇。曾获江西省自然科学一等奖。
  • 基金资助:

    教育部创新团队基金(IRT1141)；国家自然科学基金(81070148)

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Abstract:

Abstract：Short QT syndrome (SQTS)，an ion channel abnormalityrelated disease with normal cardiac structures，is clinically characterized of a shortened QT interval on electrocardiogram (ECG) and a high incidence of familial atrial fibrillation and (or) ventricular fibrillation that is linked to sudden cardiac death (SCD).Mutations in the genes encoding potassium ion channel (KCNH2，KCNQ1 and KCNJ2)，calcium ion channel (CACNAIC，CACNB2B and CACNA2D1) and sodium ion channel (SCN5A) subunits have been identified.Increased variation in repolarization and shortened refractory period have been linked to the pathogenesis of cardiac arrhythmia in SQTS.Intracardiac defibrillator has been suggested to be the firstline therapy for prevention of grade 1 and 2 SCD，for which quinidine is considered as an effective medication.

Key words: short QT syndrome, sudden cardiac death, atrial fibrillation, ventricular fibrillation, ion channel, QT interval

摘要：

摘要：短QT综合征(SQTS)是以QT间期缩短为特征、与心源性猝死(SCD)相关、常伴有家族性心房颤动和(或)心室颤动，而心脏结构正常的离子通道疾病。目前已知7个致病基因，包括KCNH2、KCNQ1、KCNJ2、CACNAIC、CACNB2B、CACNA2D1及SCN5A基因，分别编码于钾通道、钙通道和钠通道亚单位。复极离散度增加和不应期的缩短被认为是SQTS心律失常的分子机制。SCD的一级和二级预防均推荐植入心脏复律除颤器。奎尼丁是有效的治疗药物。
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