The Clinical Feature Of6Patients With Catecholaminergic Polymerphic Ventricular Tachycardia

Objective:To describe the clinical feature of6patients from Han people with catecholaminergic polymorphic ventricular tachycardia. Methods:Clinical date including signs and symptoms, electrocardiogram, holter monitoring electrocardiogram and echocardiogram were collected. The mutation of RYR2> CASQ2was detected to make a definite diagnosis. In5of the patients, an Intravenous Epinephrine Infusion test was performed. Results:From July2002to March2010,6consecutive patients[4males,mean age (13.0±4.2)years] were diagnosed with CPVT by clinical evaluation and genetic testing. They all referred to our center because of syncope. Their electrocardiogram showed sinus bradycardia, T waves with notch and bimodal, tall U waves, no J wave, no ST-segment deviation, no prolongation or shortening of QT interval. The so-called "bidirectional and (or) polymorphic ventricular tachycardia" was the labeling of their seizures. And we can captured it by Intravenous Epinephrine Infusion test easily. Conclusions:CPVT tends to involve children and adolescents. It’s clinical feature involves: exercise-related syncope、no structural heart disease and long QT interval typical bidirectional and (or) polymorphic ventricular tachycardia. Sinus bradycardia, T waves with notch and bimodal, tall U waves would be the clue for diagnosis. The excitation of adrenergic nerve is the important trigger for its fire, and BB can put out it effectively.