title: [Clinical features of six patients with catecholaminergic polymorphic ventricular tachycardia].
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 by 24-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 its onset is bVT and(or) pVT, and can be captured by Holter easily. β blocker is a safe and effective remedy for suppressing its attack.
authors.affiliation: Department of Cardiology, the First Affiliated Hospital of Nanjing Medical University, Nanjing, China.
authors.forename: Dong-sheng
authors.initials: DS
authors.lastname: Zhao
authors.forename: Jian-hua
authors.initials: JH
authors.lastname: Shen
authors.forename: Jing-ping
authors.initials: JP
authors.lastname: Lu
authors.forename: Sheng-yong
authors.initials: SY
authors.lastname: Yu
authors.forename: Gang
authors.initials: G
authors.lastname: Zhang
authors.forename: Jie
authors.initials: J
authors.lastname: Geng
authors.forename: Qi-jun
authors.initials: QJ
authors.lastname: Shan
mesh_terms: D000293:Adolescent; D002395:Catecholamines / Q000009:adverse effects; D002648:Child; D004562:Electrocardiography; D005260:Female; D006801:Humans; D008297:Male; D017180:Tachycardia, Ventricular / Q000175:diagnosis / Q000503:physiopathology; D055815:Young Adult; D000098850:Polymorphic Catecholaminergic Ventricular Tachycardia
chemical_list: D002395:Catecholamines