长QT间期综合征病儿植入心律转复除颤器1例并文献复习

APPLICATION OF IMPLANTABLE CARDIOVERTER-DEFIBRILLATOR IN CHILDREN WITH LONG-QT SYNDROME:A CASE REPORT AND LITERATURE REVIEW

目的探讨植入型心律转复除颤器(ICD)在长QT间期综合征(LQTS)儿童中的应用。方法对2018年8月我院小儿心脏科收治的1例LQTS病儿的临床资料及诊治过程总结分析,并行相关文献复习。结果病儿入院后心电图显示QTc为580ms,抽搐时心电监护显示尖端扭转性室性心动过速、心室颤动并发生心脏骤停,临床诊断为LQTS;行基因检测提示存在KCNH2基因c.1841C>T(p.A614V)杂合变异,明确诊断为2型LQTS。因病儿曾发生心脏骤停,在应用普萘洛尔基础上,植入ICD作为心脏性猝死二级预防措施。迄今随访6个月,未再发生心脏事件。结论 ICD植入可作为存在心脏骤停病史的LQTS病人有效的二级预防措施。

Objective To explore the application of implantable cardioverter-defibrillator (ICD) in children with long-QT syndrome (LQTS). Methods A summarized analysis was conducted on the clinical data and diagnosis and treatment of a child with LQTS admitted to the Division of Pediatric Cardiology in our hospital in August, 2018, and the related literature was reviewed. Results A clinical diagnosis of LQTS was made based on the facts as follows:QTc was 580 ms on the electrocardiogram on admission;torsades de pointes ventricular tachycardia, ventricular fibrillation, and cardiac arrest were found by electrocardiogram monitoring during a convulsive seizure. Since a heterozygous c.1841C>T (p.A614V) mutation in the KCNH2 gene was identified in the genetic testing, a confirmed diagnosis of type 2 LQTS was made. Given that the child had experienced a cardiac arrest, ICD was implanted as a secondary preventive measure for sudden cardiac death in addition to administration of propranolol. No cardiac events have been observed during the six-month follow-up up to now. Conclusion ICD implantation can be an effective secondary preventive measure for LQTS patients with a history of cardiac arrest.