CAJ | 학술논문

目的对儿茶酚胺敏感性多形性室性心动过速(CPVT)患者的临床特征进行分析.方法连续收集2002年7月至2010年3月经基因诊断确诊为CPVT的6例患者的临床资料,包括病史、体表12导联心电图、24 h动态心电图、二维超声心动图.结果 6例患者(男4例)平均年龄为(13.0±4.2)岁,首发症状均为晕厥.心电图显示右胸导联T波切迹、双峰、双向以及明显的U波,无J点抬高、ST段偏移及QT间期异常,二维超声心动图未及异常.此6例患者心电图、24 h动态心电图、运动试验记录到双向性和(或)多形性室性心动过速的病例数分别为2/6、5/6和3/3.6例患者均给予β受体阻滞剂口服治疗,出院后3个月门诊随访,均未再发晕厥.结论 CPVT是一种遗传性心脏离子通道疾病,静息状态下心电图表现为右胸导联T波改变、明显的U波,发病时表现为典型的双向性室性心动过速、多形性室性心动过速,β受体阻滞剂对其安全有效.
목적 대인다분알민감성다형성실성심동과속(CPVT)환자적림상특정진행분석.방법 련속수집2002년7월지2010년3월경기인진단학진위CPVT적6례환자적림상자료,포괄병사、체표12도련심전도、24 h동태심전도、이유초성심동도.결과 6례환자(남4례)평균년령위(13.0±4.2)세,수발증상균위훈궐.심전도현시우흉도련T파절적、쌍봉、쌍향이급명현적U파,무J점태고、ST단편이급QT간기이상,이유초성심동도미급이상.차6례환자심전도、24 h동태심전도、운동시험기록도쌍향성화(혹)다형성실성심동과속적병례수분별위2/6、5/6화3/3.6례환자균급여β수체조체제구복치료,출원후3개월문진수방,균미재발훈궐.결론 CPVT시일충유전성심장리자통도질병,정식상태하심전도표현위우흉도련T파개변、명현적U파,발병시표현위전형적쌍향성실성심동과속、다형성실성심동과속,β수체조체제대기안전유효.
Objective To describe the clinical features of 6 patients with catecholaminergic polymorphic ventricular tachycardia.Methods Clinical data including signs and symptoms,electrocardiograms,Holter monitoring electrocardiograms and echocardiography was analyzed.Definite diagnosis was made based on the mutations of RYR2 and CASQ2.Results From July 2002 to March 2010,6 consecutive patients referred to our center because of syncope [4 males,mean age (13.0 ± 4.2) years]were diagnosed with CPVT by clinical evaluation and genetic testing.Their electrocardiograms showed T waves with notch or bimodal and tall U waves in right chest leads.There was no J wave,no ST-segment deviation,no prolongation or shortening of QT interval.We captured the so-called "bidirectional and (or)polymorphic ventricular tachycardia(bVT and pVT)" in 2 out of 6 patients by ECG.in 5 out of 6 patients by24-hours Holter monitor,in 3 out of 6 patients by exercise test.All patients received β blockers and no syncope occurred during the 3 months follow-up after discharge from hospital.Conclusions CPVT is an inherited cardiac channelopathy characterized by syncope and (or) sudden death relatived to motion.The ECG shows T wave alteration and tall U wave in right chest leads.The mode of it's onset is bVT and (or)pVT,and can be captured by Holter easily.β blocker is a safe and effective remedy for suppressing it's attack.