Di Diego José M, Antzelevitch Charles
Lankenau Institute for Medical Research, Wynnewood, PA, USA.
Lankenau Heart Institute, Wynnewood, PA, USA.
Pacing Clin Electrophysiol. 2018 Jul;41(7):684-699. doi: 10.1111/pace.13408. Epub 2018 Jun 30.
The J wave syndromes, including the Brugada (BrS) and early repolarization (ERS) syndromes, are characterized by the manifestation of prominent J waves in the electrocardiogram appearing as an ST segment elevation and the development of life-threatening cardiac arrhythmias. BrS and ERS differ with respect to the magnitude and lead location of abnormal J waves and are thought to represent a continuous spectrum of phenotypic expression termed J wave syndromes. Despite over 25 years of intensive research, risk stratification and the approach to therapy of these two inherited cardiac arrhythmia syndromes are still rapidly evolving. Our objective in this review is to provide an integrated synopsis of the clinical characteristics, risk stratifiers, as well as the molecular, ionic, cellular, and genetic mechanisms underlying these two syndromes that have captured the interest and attention of the cardiology community over the past two decades.
J波综合征,包括Brugada综合征(BrS)和早期复极综合征(ERS),其特征是心电图上出现明显的J波,表现为ST段抬高,并伴有危及生命的心律失常。BrS和ERS在异常J波的幅度和导联位置方面存在差异,被认为代表了一种称为J波综合征的连续表型表达谱。尽管经过了25年多的深入研究,但这两种遗传性心律失常综合征的风险分层和治疗方法仍在迅速发展。我们撰写这篇综述的目的是综合概述这两种综合征的临床特征、风险分层指标,以及在过去二十年中引起心脏病学界兴趣和关注的分子、离子、细胞和遗传机制。