베타차단제에 반응하는 카테콜라민 다형성 심실빈맥

Abstract

Catecholaminergic polymorphic ventricular tachycardia (CPVT), a familial electrical disorder in a structurally normal heart, is one of the conditions that are related to sudden cardiac death, especially in young patients. In most cases, it is triggered by emotional stress or exercise and can be prevented by a ß-blocker. We present the case of a 12-year-old boy with CPVT, responsive to a ß-blocker. The patient experienced recurrent syncope and dizziness. The initial Holter monitoring showed polymorphic VT. Non-sustained bidirectional VT, which is a representative characteristic of CPVT, was induced by exercise test and isoproterenol infusion. The patient was given 60 mg propranolol per day and since then, recurrent syncope disappeared.