title: Clinical Challenges in Catecholaminergic Polymorphic Ventricular Tachycardia.
abstract: 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.
authors.affiliation: Molecular Cardiology, IRCCS Salvatore Maugeri Foundation, Pavia, Italy.
authors.forename: Jacopo F
authors.initials: JF
authors.lastname: Imberti
authors.affiliation: Molecular Cardiology, IRCCS Salvatore Maugeri Foundation, Pavia, Italy.
authors.forename: Katherine
authors.initials: K
authors.lastname: Underwood
authors.affiliation: Molecular Cardiology, IRCCS Salvatore Maugeri Foundation, Pavia, Italy.
authors.forename: Andrea
authors.initials: A
authors.lastname: Mazzanti
authors.affiliation: Molecular Cardiology, IRCCS Salvatore Maugeri Foundation, Pavia, Italy; Department of Molecular Medicine, University of Pavia, Pavia, Italy. Electronic address: silvia.priori@fsm.it.
authors.forename: Silvia G
authors.initials: SG
authors.lastname: Priori
mesh_terms: D005260:Female; D005424:Flecainide / Q000627:therapeutic use; D006801:Humans; D008297:Male; D015995:Prevalence; D013562:Sympathectomy / Q000379:methods; D017180:Tachycardia, Ventricular / Q000175:diagnosis / Q000453:epidemiology / Q000503:physiopathology / Q000628:therapy; D000098850:Polymorphic Catecholaminergic Ventricular Tachycardia
chemical_list: D005424:Flecainide
keywords: Beta-blocker; Bidirectional ventricular tachycardia; CASQ2; Catecholaminergic polymorphic ventricular tachycardia; Exercise-induced cardiac arrest; Exercise-induced syncope; Flecainide; Implantable cardioverter defibrillator; RyR2; Sudden cardiac death