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  

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

作　　者：张丽[1] 李筠[1] 王健怡[1] 肖婷婷[1] 谢利剑[1] 徐萌[1] ZHANG Li;LI Yun;WANG Jianyi;XIAO Tingting;XIE Lijian;XU Meng(Department of Cardiology,Children’s Hospital Affiliated to Shanghai Jiao Tong University,Shanghai 200062,China)

机构地区：[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

出　　处：《临床儿科杂志》2020年第3期209-212,共4页Journal of Clinical Pediatrics

摘　　要：目的 探讨儿茶酚胺敏感性多形性室性心动过速(CPVT)的诊断和治疗.方法 回顾分析1例CPVT患儿的临床资料,并复习相关文献.结果 男性患儿,5岁2个月,间歇性心前区不适8月余,运动时晕厥发作2次.24小时动态心电图以及运动试验,窦性心率>110次/min即出现室性心律失常,随着心率增加室性心律失常频数增加,可见双向及多形性室速.基因检测示RYR2基因变异c.6886 G>A(p.E 2296 K).予大剂量普萘洛尔治疗,症状未缓解且心电检查无明显改善,后加用普罗帕酮获得改善.随访1年,患儿未再有运动胸闷不适,动态心电图及运动试验显示无室性心律失常.结论CPVT以运动或情绪激动诱发双向型和/或多形性室性心动过速为主要临床特征,晕厥、猝死为主要临床表现,普罗帕酮可能可以替代或联合用药治疗CPVT.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.

关 键 词：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 

分 类 号：R725.4[医药卫生—儿科]