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儿茶酚胺敏感性多形性室性心动过速(catecholaminergic polymorphic ventricular tachycardia,CPVT)是一种较少见的严重的原发性心脏电紊乱,多发生于无器质性心脏病、QT间期正常的儿童或青少年,以运动或情绪激动诱发双向性室性心动过速(bVT)或多形性室性心动过速(pVT),可自行恢复抑或恶化为室颤导致晕厥和猝死为特征。在CPVT受累个体中,心脏性猝死发生率较高。CPVT真正的发病率尚不清楚,初步估计为1/10000。1975年Reid首次报道CPVT病例,1978年Coumel对该病进行了较为系统的描述。1999年Swan等对两个患有CPVT的芬兰家系进行基因连锁分析,
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare and severe primary cardiac electrical disorder that occurs mostly in those with non-structural heart disease and normal QT interval Children or adolescents with motor or emotional activation induced bidirectional ventricular tachycardia (bVT) or pleomorphic ventricular tachycardia (pVT) may be self-recovery or worsening of ventricular fibrillation leading to syncope and sudden death characterized. In CPVT affected individuals, the incidence of sudden cardiac death is higher. The true incidence of CPVT is not yet known, initially estimated at 1/10000. Reid first reported in 1975, CPVT cases, Coumel 1978, a more systematic description of the disease. In 1999, Swan et al. Performed genetic linkage analysis on two Finnish pedigrees with CPVT,