离子通道与长QT综合征的研究进展

Research progress on ion channels and long QT syndrome

长QT综合征患者临床上表现为心肌动作电位复极过程紊乱,心电图显示QT间期延长,常导致不定期反复性晕厥、癫痫、恶性心律失常甚至猝死等,而心脏结构没有任何异常。研究表明,长QT综合征与钙离子通道、钾离子通道、钠离子通道以及钙调蛋白等基因突变相关。本文综述了离子通道与长QT综合征的研究进展,总结了离子通道基因突变诱发长QT综合征的作用机制,以及临床上长QT综合征的治疗现状,为长QT综合征的防治提供一定的参考依据。

The clinical manifestations of patients with long QT syndrome are myocardial action potential repolarization disorder and electrocardiogram shows prolonged QT interval,often leading to irregular recurrent syncope,epilepsy,malignant arrhythmia and even sudden death,but there is no abnormality in cardiac structure.Studies have shown that long QT syndromes are associated with genes mutations in calcium channels,potassium channels,sodium channels,and calmodulin.This paper reviews the research progress on ion channel and long QT syndrome and summarizes the mechanism of long QT syndrome induced by ion channel gene mutation as well as the current clinical treatment status of long QT syndrome,and provides some reference for the prevention and treatment of long QT syndrome.