Imberti Jacopo F, Underwood Katherine, Mazzanti Andrea, Priori Silvia G
Molecular Cardiology, IRCCS Salvatore Maugeri Foundation, Pavia, Italy.
Molecular Cardiology, IRCCS Salvatore Maugeri Foundation, Pavia, Italy; Department of Molecular Medicine, University of Pavia, Pavia, Italy.
Heart Lung Circ. 2016 Aug;25(8):777-83. doi: 10.1016/j.hlc.2016.01.012. Epub 2016 Feb 16.
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inheritable cardiac disorder associated with exercise- and stress-induced sudden death in young individuals. Although important steps forward have been made in the comprehension and treatment of this disease, several aspects remain unclear. Firstly, from an epidemiological standpoint the actual prevalence of CPVT is still unknown and possibly underestimated. In addition, the diagnostic process remains very challenging and can be supported by genetic analysis in only about half of the cases. Finally, up to one third of CPVT patients continue to present complex arrhythmias despite beta blocker treatment; the role of newer therapeutic options, such as flecainide and left cardiac sympathetic denervation, needs to be further elucidated. All these points constitute challenges for the cardiologist in the management of CPVT patients and fuel research into new diagnostic, prognostic and therapeutic approaches.
儿茶酚胺能多形性室性心动过速（CPVT）是一种遗传性心脏疾病，与年轻人运动和应激诱发的猝死有关。尽管在对该疾病的理解和治疗方面已经取得了重要进展，但仍有几个方面尚不清楚。首先，从流行病学角度来看，CPVT的实际患病率仍然未知，可能被低估了。此外，诊断过程仍然极具挑战性，仅约一半的病例可通过基因分析得到支持。最后，尽管使用了β受体阻滞剂治疗，仍有多达三分之一的CPVT患者继续出现复杂心律失常；氟卡尼和左心交感神经去神经等新型治疗选择的作用需要进一步阐明。所有这些问题对心脏病专家管理CPVT患者构成了挑战，并推动了对新诊断、预后和治疗方法的研究。
