Zhonghua xin xue guan bing za zhi · 2012
[Clinical features of six patients with catecholaminergic polymorphic ventricular tachycardia].
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Zhao Dong-sheng, Shen Jian-hua, Lu Jing-ping, Yu Sheng-yong, Zhang Gang, Geng Jie, Shan Qi-jun
Аннотация
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.
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.