Clinical features of six patients with catecholaminergic polymorphic ventricular tachycardia
10.3760/cma.j.issn.0253-3758.2012.10.009
- VernacularTitle:儿茶酚胺敏感性多形性室性心动过速临床特征分析
- Author:
Dong-Sheng ZHAO
1
;
Jian-Hua SHEN
;
Jing-Ping LU
;
Sheng-Yong YU
;
Gang ZHANG
;
Jie GENG
;
Qi-Jun SHAN
Author Information
1. 210029,南京医科大学第一附属医院心血管内科
- Keywords:
Tachycardia,ventricular;
Catecholamines;
Aadolescent
- From:
Chinese Journal of Cardiology
2012;40(10):844-848
- CountryChina
- Language:Chinese
-
Abstract:
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.
