Recent Advancement of Treatment in Catecholaminergic Polymorphic Ventricular Tachycardia

概要

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Background: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is malignant arrhythmia characterized physical induced bidirectional or polymorphic ventricular tachycardia (VT). Methods and Results: From the Japanese registry of CPVT, 69 patients (M:F=25:44, age=11.7±7.8 years) were enrolled in this study. These patients were divided into 35 patients who were diagnosed during 1900s and 34 patients after 2000s. The incidence of sustained VT, nonsustained VT, and VF (10:22:3 vs 7:24:0, ns), the type of VT, and the rate of VT (194±29 vs 196±58, ns) were not different in these 2 groups. However, there was no sudden death in 2000s group, but 8 patients were died in 1900s group (p=0.003). Although statistically insignificant, there was no sudden death in 7 patients who was diagnosed over 20 year-old, however 8 of 62 patients were died who was diagnosed before 20 year-old. Conclusion: The prognosis of CPVT was markedly improved in the last 2 decade. This may be the result of propagation of the necessity of the restriction of exercise in the patients with CPVT, and also from the recent advancement of the use of antiarrhythmic medication, such as flecainide. The patients who were first diagnosed in adulthood may have better prognosis than the patients who were diagnosed in childhood.