摘要 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.
刘红彬. 遗传性长QT综合征的药物及器械治疗[J]. 实用心电学杂志, 2016, 25(5): 309-312.
LIU Hong-Bin. Drug and device therapies of congenital longQT syndrome. JOURNAL OF PRACTICAL ELECTROCARDIOLOGY, 2016, 25(5): 309-312.
[1] 李翠兰，胡大一.心脏离子通道病引起的晕厥[J].心血管病学进展，2006，27(4):407-412．[2] Schwartz PJ. Pharmacological and nonpharmacological management of the congenital long QT syndrome: the rationale[J]. Pharmacol Ther，2011，131(1):171-177.[3] Priori SG，Schwartz PJ，Napolitano C，et al. Risk stratification in the longQT syndrome[J]. N Engl J Med，2003，348(19):1866-1874.[4] Sauer AJ，Moss AJ，McNitt S，et al. Long QT syndrome in adults[J]. J Am Coll Cardiol，2007，49(3):329-337.[5] Goldenberg I，Moss AJ，Peterson DR，et al. Risk factors for aborted cardiac arrest and sudden cardiac death in children with the congenital longQT syndrome[J]. Circulation，2008，117(17)：2184-2191.[6] Goldenberg I，Zareba W，Moss AJ. Long QT syndrome[J]. Curr Probl Cardiol，2008，33(11):629-694.[7] [JP4]Moss AJ，Shimizu W，Wilde AA，et al. Clinical aspects of type-1 long-QT syndrome by location, coding type, and biophysical function of mutations involving the KCNQ1 gene[J]. Circulation，2007，115(19): 2481-2489.[8] Shimizu W，Moss AJ，Wilde AA，et al. Genotypephenotype aspects of type 2 long QT syndrome[J]. J Am Coll Cardiol，2009，54(22):2052-2062.[9] Migdalovich D，Moss AJ，Lopes CM，et al. Mutation and genderspecific risk in type 2 long QT syndrome: implications for risk stratification for life threatening cardiac events in patients with long QT syndrome[J]. Heart Rhythm，2011，8(10):1537-1543.[10] Schwartz PJ，Spazzolini C，Priori SG，et al. Who are the longQT syndrome patients who receive an implantable cardioverterdefibrillator and what happens to them? data from the european longQT syndrome implantable cardioverterdefibrillator (LQTS ICD) registry[J]. Circulation，2010，122(13):1272-1282.[11] Vincent GM，Schwartz PJ，Denjoy I，et al. High efficacy of betablockers in longQT syndrome type 1: contribution of noncompliance and QTprolonging drugs to the occurrence of betablocker treatment “failures” [J]. Circulation，2009，119(2):215-221.[12] Chockalingam P，Crotti L，Girardengo G，et al. Not all betablockers are equal in the management of long QT syndrome types 1 and 2: higher recurrence of events under metoprolol[J]. J Am Coll Cardiol，2012，60(20):2092-2099.[13] Goldenberg I，Bradley J，Moss A，et al. Betablocker efficacy in highrisk patients with the congenital longQT syndrome types 1 and 2: implications for patient management[J]. J Cardiovasc Electrophysiol，2010，21(8):893-901.[14] Schwartz PJ，Priori SG，Cerrone M，et al. Left cardiac sympathetic denervation in the management of highrisk patients affected by the longQT syndrome[J]. Circulation，2004，109(15):1826-1833.[15] Epstein AE，DiMarco JP，Ellenbogen KA，et al.2012 ACCF/AHA/HRS focused update incorporated into the ACCF/AHA/HRS 2008 guidelines for devicebased therapy of cardiac rhythm abnormalities: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines and the Heart Rhythm Society[J]. J Am Coll Cardiol，2013，61(3):e6-e75.[16] 李国栋，郑燕列.倍他乐克联合起搏器植入对长QT综合征并发尖端扭转性室速的疗效观察[J]. 现代医学，2016，4(2) : 245-247.[17] 陈旭华，浦介麟，王靖，等.永久起搏器和埋藏式心脏复律除颤器（ICD）在遗传性长QT综合征患者中的应用[J].中国分子心脏病学杂志，2014，14(3):945-948.[18] Jons C，Moss AJ，Goldenberg I，et al. Risk of fatal arrhythmic events in long QT syndrome patients after syncope[J]. J Am Coll Cardiol，2010，55(8):783-788.[19] Schwartz PJ，Spazzolini C，Crotti L，et al. The Jervell and LangeNielsen syndrome: natural history, molecular basis, and clinical outcome[J]. Circulation，2006，113(6):783-790.[20] Dorostkar PC，Eldar M，Belhassen B，et al. Long term followup of patients with long-QT syndrome treated with betablockers and continuous pacing[J]. Circulation，1999，100(24):2431-2436.[21] Burke MC，Gold MR，Knight BP，et al. Safety and efficacy of the totally subcutaneous implantable defibrillator: 2year results from a pooled analysis of the IDE study and EFFORTLESS registry[J]. J Am Coll Cardiol，2015，65(16):1605-1615.
2016, Vol. 25 
