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Nhịp tim nhanh thất đa hình Catecholaminergic Lâm sàng trình bày catecholaminergic nhịp tim nhanh thất đa hình (CPVT) là một bệnh được mô tả bởi Coumel et al. vào năm 1978 [54], và đặc trưng bởi rối loạn nhịp tâm thất | Inherited arrhythmogenic diseases 141 reduction in the wavelength of the reentrant circuit which reduces hie path length required for maintenance of reentry 53 . Catecholaminergic polymorphic ventricular tachycardia Clinical presentation The catecholaminergic polymorphic ventricular tachycardia CPVT is a disease described by Coumel et al. in 1978 54 and characterized by exercize-induced polymorphic ventricular arrhythmias syncope occurring during physical activity or acute emotion a normal resting electrocardiogram and the absence of structural cardiac abnormalities. Supraventricular tachyarrythmias are also part of the manifestations of CPVT. Family history of one or multiple sudden cardiac deaths ÍS evident in 30 of cases 55 . Symptoms usually develop during childhood or adolescence although cases in which the first symptoms appeared during adulthood have been reported. The resting ECG is unsemarkabSe with the excephon of ìíuus CradccatCia anp promsnent U waves joot d m smme paSients 54 . Therpfdre hie diegnosie is nwt always straightforward. Given the fact that in approximately 15 of patients cardiac arrest is the first manifestation of the disease 56 in some patients it may be initially considered as idiopathic ventricular fibrillation IFV 55-57 . To establish the diagnosis of CPVT it is critical to observe exercise or emotion induced polymorphic VT. These arrhythmias are reproducibly induced by exercise stress test but not by PES. The most typical arrhythmias of CPVT is the so-called bidirectional VT in which the VT presents with an alternating 183 QRS axis on a beat-to-beat basis. Genetic bases CPVT1 - autosomal dominant The first oocus lor CWT was identifieb by Swmi etal. who mapped the disease to chromosome 1q42-43 58 . In 2331 Priori et al. demonstrated that the disease is caused by a mutation in the RyR2 gene encoding for the cardiac Ryanodine receptor 55 . RyR2 is a large p en that Setramerizec acroth ehe membrane of the sarcoplasmic reticulum SR and .
