遗传性长QT综合征（longQT syndrome，LQTS）以心电图上QT间期延长及其相关的尖端扭转型室性心动过速（torsade de pointes,TdP）为特征，易导致心脏性猝死。它的危险因素有QTc长度、基因型、晕厥史等。LQTS的治疗手段包括β受体阻滞剂治疗、外科心脏左侧交感神经节切除术（LCSD）、心脏起搏治疗以及植入式心律转复除颤器（ICD）治疗。β受体阻滞剂治疗是LQTS治疗的基石；心脏起搏治疗适用于伴有心动过缓的LQTS，可明显降低心脏事件的复发率，但不降低死亡率。对高危的LQTS应植入ICD，皮下ICD并发症较少，值得推广。LCSD技术实用性较差，更适用于ICD植入后频繁电击治疗的患者.
Abstract
Congenital longQT syndrome(LQTS) is characterized by the prolongation of QT interval in ECG and relevant torsade de pointes(TdP), which easily leads to sudden cardiac death. Its risk factors include the length of QTc interval, genotype, medical history of syncope, etc. The treatment of congenital LQTS involves β blockers, surgical left cardiac sympathetic denervation(LCSD), cardiac pacing and implantable cardioverter defibrillators(ICD). β blockers are the firstline treatment for all the LQTS patients. Cardiac pacing therapy is fit for LQTS patients complicating bradycardia, which can significantly reduce the recurrence rate of cardiac events, but not the mortality. Highrisk LQTS patients should be implanted with ICD. Less subcutaneous complications of ICD are found and thus the therapy is worthy of being promoted. LCSD is poorly practical and is more suitable for those ICDimplanted patients frequently undergoing electroshock.
LIU Hong-Bin. , {{custom_author.name_en}}et al.
Drug and device therapies of congenital longQT syndrome[J]. JOURNAL OF PRACTICAL ELECTROCARDIOLOGY, 2016, 25(5): 309-312 https://doi.org/10.13308/j.issn.2095-9354.2016.05.002
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