Clinical Challenges in Catecholaminergic Polymorphic Ventricular Tachycardia

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• 作者：Jacopo F. Imberti ; BS^a ; Katherine Underwood ; BS^a ; Andrea Mazzanti ; MD^a ; Silvia G. Priori ; MD ; PhD^a ; ^b ; ^{silvia.priori@fsm.it" class="auth_mail" title="E-mail the corresponding author}
• 关键词：bpm ; beats per minute ; CASQ2 ; Calsequestrin 2 ; CPVT ; Catecholaminergic Polymorphic Ventricular Tachycardia ; ECG ; Electrocardiogram ; HR ; Heart Rate ; ICD ; Implantable Cardioverter Defibrillator ; LBBB ; Left Bundle Branch Block ; LCSD ; Left Cardiac Sympathetic Denervation ; RyR2 ; Ryanodine Receptor 2 ; SCD ; Sudden Cardiac Death ; SR ; Sarcoplasmic Reticulum ; VPB ; Ventricular Premature Beat ; VT ; Ventricular Tachycardia
• 刊名：Heart, Lung and Circulation
• 出版年：2016
• 出版时间：August 2016
• 年：2016
• 卷：25
• 期：8
• 页码：777-783
• 全文大小：585 K

文摘

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.