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Zhonghua Xin Xue Guan Bing Za Zhi. 2012 Oct;40(10):844-8.

[Clinical features of six patients with catecholaminergic polymorphic ventricular tachycardia].

[Article in Chinese]

Author information

  • 1Department of Cardiology, the First Affiliated Hospital of Nanjing Medical University, Nanjing, China.

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.

PMID:
23302672
[PubMed - indexed for MEDLINE]
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