Catecholaminergic Polymorphic Ventricular Tachycardia in Children

Purpose：Catecholaminergic polymorphic ventricular tachycardia (CPVT) is the disease entity of adrenergic dependent, potentially lethal tachyarrhythmia in a child with no structural heart disease, which manifest itself as a syncope or sudden death.The objective of this study is to present our experiences on this important, although rare, disease.Methods：Retrospective analysis of 5 patients with episodes of syncope related to exercise, who were referred to our hospital from January 1985 to December 1998.Results：All patients were male and the mean age at the time of the first syncopal episode was 5.1±3.2 years (range 1.3 to 10 years).There were no structural cardiac abnormalities in clinical and laboratory evaluations.In all, polymorphic ventricular tachycardia showing the characteristic pattern of CPVT in which, as the heart became stimulated adrenergically, isolated ventricular premature beats appeared, increased with rate, became polymorphic, finally formed burst with bidirectional salvoes and disappeared in resting state was induced during exercise test and/or isoproterenol infusion test.During the mean follow up period of 3.75±3.1 years (range 1 month to 7.3 years), one died suddenly.In this case, low dose of β -blocker was administered because of associated sinus bradycardia resulting in incomplete control of the syncopal episodes.The other 4 cases were alive and asymptomatic by means of adequate modification of β -blocker dosage and method of administration.Conclusions：This study emphasizes that CPVT is an important, although rare, cause of exercise related syncope in children and can be diagnosed by means of exercise test and/or isoproterenol infusion.β -blockers were very effective in all cases, even though increasing amount of β -blocker was frequently necessary to control ventricular arrhythmia in some cases.( ( ( (Korean Circulation J 2000 ; 30( ( ( (2) ) ) ) : 191-197) ) ) ) KEY WORDS：Catecholaminergic polymorphic ventricular tachycardia•Syncope•β -blocker•Children.논문접수일：1999년 7월 19일 심사완료일：1999년 12월 22일 교신저자：노정일, 110-744 서울 종로구 연건동 28번지 서울대학교 의과대학 어린이병원 소아과학교실 전화：(02) 760-3570•전송：(02) 743-3455