先天性Q T间期延长综合征 (LQTS)是因编码心肌离子通道蛋白的基因突变导致心肌细胞膜离子通道功能障碍而引起的一组临床综合征[1] 。以心电图表现为QT间期延长 ,T波异常为特征 ,易产生室性心律失常和猝死[2 ] 。人群发病率约为 1 30万 ,多见于儿童和青少年 ,女性约为男性的 2倍 Congenital Q T-prolongation Syndrome (LQTS) is a group of clinical syndromes caused by dysfunction of membrane ion channels of myocardial cells caused by gene mutations that encode myocardial ion channel proteins [1]. Electrocardiogram showed prolonged QT interval, T wave abnormalities characterized by easy to produce ventricular arrhythmias and sudden death [2]. The incidence of the population is about 1.3 million, more common in children and adolescents, women about twice as men