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

The Value of Implantable Cardioverter-defibrillator(ICD) and Permanent Cardiac Pacemaker in Patients with Congenital Long QT Syndrome(LQTS)

目的总结合并窦性心动过缓的遗传性长QT综合征(以下简称遗传性LQTS)患者植入永久起搏器和埋藏式心脏复律除颤器(以下简称ICD)的治疗效果,对比分析这两种治疗在预防患者猝死中的差异。方法对我院从2003年6月到2013年6月出院诊断为遗传性LQTS合并窦性心动过缓、植入了永久起搏器或ICD的全部21例患者,结合门诊、电话和程控随访了解患者的生存状况、手术并发症以及晕厥、室性恶性心律失常的发作情况。结果起搏器组男性2例,女性9例,年龄39.3±14.3岁,随访时间50.6±26.3个月,1例患者猝死,2例患者再发晕厥前兆,其中1例最终更换为ICD。ICD组男性2例,女性8例,年龄34.5±11.9岁,随访时间61.4±43.5个月,3例患者接受了ICD的适当治疗,另2例患者接受了ICD的不适当治疗,1例患者术后出现囊袋感染,1例患者更换为永久起搏器。治疗有效率在起搏器组及ICD组分别为72.7%(8/11)和100.0%(10/10),未达到统计学差异(p=0.21)。不良事件发生率在起搏器组及ICD组分别为27.3%(3/11)和30.0%(3/10),也未达到统计学差异(p=0.63)。结论对于不能植入ICD的合并窦性心动过缓的遗传性LQTS患者,植入永久起搏器可能是一个较好的替代方法,但对于QTc≥539ms的患者,只有植入ICD才能预防猝死。植入ICD后长期无心脏事件发生的患者,根据患者意愿,可考虑更换为永久起搏器。

Objective To investigate the differences between the efficacy of implantable cardioverter-defibrillator（ICD） and permanent pacemaker in patients with congenital long QT syndrome（LQTS） combined with sinus bradycardia. Methods An analysis was conducted in 21 patients with congenital LQTS combined with sinus bradycardia who underwent ICD or permanent cardiac pacemaker implantations between June 2003 and June 2013 in Fuwai Hospital. The incidence of postoperative sudden death, syncope, reccurence of malignant ventricular arrhythmia and operation-related side-effect were evaluated during follow-up. Results Patients were devided into 2 groups. Group A consisted of 11 patients in whom permanent pacemaker were implanted [2 males,（39.3±14.3）ys],and Group B consisted of 10 patients in whom ICD were implanted[2 males,（34.5±11.9）ys].The properties of 2 groups were similar.1 patient sudden died, 2 patients experienced faint and 1 patient＇s permanent pacemaker was upgraded to ICD during a mean follow-up of 50.6±26.3 months in group A.3 patients experienced appropriate shocks, 2 patients experienced inappropriate shocks,1 patient had subcutaneous pouch infection and 1 patient＇s ICD was replaced by permanent pacemaker during a mean follow-up of 61.4±43.5 months in group B.There was no statistically significant difference between group A and B either in the total effective rate（72.7% vs 100.0%, p=0.21） or the adverse events（27.3% vs 30.0%, p=0.63）. Conclusion Permanent cardiac pacemaker could be an alteRNAtive for ICD in patients with congenital long QT syndrome combined with sinus bradycardia who can＇t afford ICD in order to prevent sudden death, except those with a QTc≥539ms.ICD could be replaced by permanent cardiac pacemaker cautiously in selected patients who haven＇t any cardiac events during a long period of time after ICD implantation.