临床儿科杂志 ›› 2020, Vol. 38 ›› Issue (3): 209-.doi: 10.3969/j.issn.1000-3606.2020.03.012

• 综合报道 • 上一篇    下一篇

普罗帕酮联合普萘洛尔治疗儿茶酚胺敏感性多形性室性心动过速1 例报告并文献复习

张丽, 李筠, 王健怡, 肖婷婷, 谢利剑, 徐萌   

  1. 上海市儿童医院 上海交通大学附属儿童医院心内科(上海 200062)
  • 发布日期:2020-04-07
  • 通讯作者: 李筠 电子信箱:liyun@shchildren.com.cn

Propafenone combined with propranolol in the treatment of catecholaminergic polymorphic ventricular tachycardia: a case report and literature review

 ZHANG Li, LI Yun, WANG Jianyi, XIAO Tingting, XIE Lijian, XU Meng   

  1. Department of Cardiology, Children’s Hospital Affiliated to Shanghai Jiao Tong University, Shanghai 200062, China
  • Published:2020-04-07

摘要: 儿茶酚胺敏感性多形性室性心动过速; 心脏复律除颤器; β受体阻滞剂; 普罗帕酮

Abstract:  Objective To investigate the diagnosis and treatment of catecholaminergic polymorphic ventricular tachycardia (CPVT). Method The clinical data of CPVT in a child was analyzed and the related literature was reviewed. Results A boy, aged 5 years and 2 months, had intermittent precardiac discomfort for more than 8 months and two syncope attacks triggered by exercises. The 24-hour ECG and treadmill test showed that ventricular arrhythmia occurred when sinus heart rate was > 110 bpm. When the heart rate was increased, the frequency of ventricular arrhythmia was increased, and the bidirectional and polymorphic ventricular tachycardia could be seen. Gene detection showed RYR2 gene mutation c.6886G> A (p.E2296K). After the treatment with high dose of propranolol, the symptoms were not relieved and the ECG examination did not improve significantly, but the symptoms were improved with the addition of polymorphic. After one-year follow-up, the child had no chest discomfort after exercise and no ventricular arrhythmia was found in the 24-hour ECG and treadmill test. Conclusions The main clinical features of CPVT are bidirectional and/or pleomorphic ventricular tachycardia induced by exercise or emotional excitation. Syncope and sudden death are the main clinical manifestations. Propafenone may be a promising alternative or combinated for the treatment of CPVT.

Key words:  catecholaminergic polymorphic ventricular tachycardia; cardioverter defibrillator; β-blocker;  propafenone