Clinical Challenges in Catecholaminergic Polymorphic Ventricular Tachycardia

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 u...

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Bibliographic Details
Published in:Heart, lung & circulation lung & circulation, 2016-08, Vol.25 (8), p.777-783
Main Authors: Imberti, Jacopo F., BS, Underwood, Katherine, BS, Mazzanti, Andrea, MD, Priori, Silvia G., MD, PhD
Format: Article
Language:English
Subjects:
Beta-blocker
Bidirectional ventricular tachycardia
Cardiovascular
CASQ2
Catecholaminergic polymorphic ventricular tachycardia
Exercise-induced cardiac arrest
Exercise-induced syncope
Female
Flecainide
Flecainide - therapeutic use
Humans
Implantable cardioverter defibrillator
Male
Prevalence
RyR2
Sudden cardiac death
Sympathectomy - methods
Tachycardia, Ventricular - diagnosis
Tachycardia, Ventricular - epidemiology
Tachycardia, Ventricular - physiopathology
Tachycardia, Ventricular - therapy
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Summary: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.
ISSN:1443-9506
1444-2892
DOI:10.1016/j.hlc.2016.01.012