Long QT syndrome: Diagnosis and management
Long QT syndrome: Diagnosis and management
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DOI：
10.1067/mhj.2002.120295
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发表时间：
2002-01-01
影响因子：
4.8
通讯作者：
Khan, IA
中科院分区：
文献类型：
--
作者：
Khan, IA
背景长QT综合征(LQT)以QT间期延长、尖端扭转性心动过速和心源性猝死为特征。LQT是一种由跨膜钾、钠电流改变引起的心脏复极紊乱。先天性LQT是一种跨膜离子通道蛋白疾病。目前已确定该病的6个遗传位点。这种疾病的零星病例是自发突变的结果。LQT的获得性病因包括药物、电解质失衡、明显的心动过缓、可卡因、有机磷化合物、蛛网膜下腔出血、心肌缺血、无蛋白禁食、自主神经病变和人类免疫缺陷病毒病。临床表现包括头晕、晕厥和猝死。基因筛查主要是作为一种研究工具。LQT的短期治疗旨在防止扭体复发，包括静脉注射镁和钾、临时心脏起搏、停用致癌剂、纠正电解质失衡，以及极少数情况下静脉注射异丙肾上腺素。长期治疗的目的是缩短QT间期，防止扭转和猝死，包括口服P-肾上腺素受体阻滞剂，植入永久起搏器/心律转复除颤器，以及左胸交感神经切除术。钠通道阻滞剂是正在研究中的有前景的药物。记录心电图是为了筛查家庭成员。数据有利于治疗无症状的患者，如果
Background Long QT syndrome (LQT) is characterized by prolongation of the QT interval, causing torsade de pointes and sudden cardiac death. The LQT is a disorder of cardiac repolarization caused by alterations in the transmembrone potassium and sodium currents. Congenital LQT is a disease of transmembrane ion-channel proteins. Six genetic loci of the disease have been identified. Sporadic cases of the disease occur as a result of spontaneous mutations. The acquired causes of LQT include drugs, electrolyte imbalance, marked bradycardia, cocaine, organophosphorus compounds, subarachnoid hemorrhage, myocardial ischemia, protein sparing fasting, autonomic neuropathy, and human immunodeficiency virus disease.Methods Data on the diagnosis and management of LQT were thoroughly reviewed.Results and Conclusions The diagnosis of LQT primarily rests on clinical and electrocardiographic features and family history. The clinical presentations range from dizziness to syncope and sudden death. Genetic screening is available primarily as a research tool. Short-term treatment of LQT is aimed at preventing the recurrences of torsades and includes intravenous magnesium and potassium administration, temporary cardiac pacing, withdrawal of the offending agent, correction of electrolyte imbalance, and, rarely, intravenous isoproterenol administration. The long-term treatment is aimed at reducing the QT-interval duration and preventing the torsades and sudden death and includes use of oral P-adrenergic blockers, implantation of permanent pacemaker/cardioverter-defibrillator, and left thoracic sympathectomy. Sodium channel blockers are promising agents under investigation. Electrocardiograms are recorded for screening of family members. The data favor treating asymptomatic patients, if