Couderc Jean-Philippe, Lopes Coeli M
Center for Quantitative Electrocardiography and Cardiac Safety, Department of Medicine, University of Rochester Medical Center, Rochester, NY 14642, USA.
J Electrocardiol. 2010 Sep-Oct;43(5):396-9. doi: 10.1016/j.jelectrocard.2010.07.009.
The short and long QT syndromes are inherited diseases associated with an increased risk for life-threatening arrhythmias. The first case of long QT syndrome (LQTS) was reported more than 150 years ago, and the study of this disease led to crucial advancement of our understanding of channelopathies and associated ventricular arrhythmias. Ten years ago, Gussak et al. reported four cases of idiopathic ventricular fibrillation in individuals from a family with a history of sudden cardiac death exhibited very short QT interval and labeled the disease: short QT syndrome (SQTS). Over this decade, the SQTS was found to be a rare inherited syndrome with the potential to provide novel insights into the main mechanisms of cardiac arrhythmogenicity. In this review, we discuss these mechanisms and provocatively question the role of the QT interval duration as a surrogate marker of increased risk for arrhythmia in both the LQTS and the SQTS.
短QT综合征和长QT综合征是与危及生命的心律失常风险增加相关的遗传性疾病。长QT综合征（LQTS）的首例病例在150多年前就有报道，对这种疾病的研究推动了我们对离子通道病及相关室性心律失常认识的关键进展。十年前，古萨克等人报告了来自一个有心脏性猝死家族史的家庭中的4例特发性室颤患者，这些患者表现出非常短的QT间期，并将这种疾病命名为：短QT综合征（SQTS）。在这十年间，短QT综合征被发现是一种罕见的遗传性综合征，有可能为心脏心律失常发生的主要机制提供新的见解。在这篇综述中，我们讨论了这些机制，并大胆质疑QT间期持续时间作为长QT综合征和短QT综合征中心律失常风险增加的替代标志物的作用。
