title: Catecholaminergic Polymorphic Ventricular Tachycardia with QT Prolongation.
abstract: The QT interval in patients with catecholaminergic polymorphic ventricular tachycardia (CPVT) is typically normal. However, CPVT patients are sometimes misdiagnosed as concealed long QT syndrome (LQTS), because patients with LQTS also manifest with syncope or sudden death following periods of exertion or extreme emotion. We report a CPVT patient with a pathogenic RyR2 mutation associated with a marked QT prolongation, which normalized after flecainide therapy.
authors.affiliation: Heart Rhythm Center, Tokyo Medical and Dental University, Tokyo, Japan.
authors.forename: Yasuaki
authors.initials: Y
authors.lastname: Tanaka
authors.affiliation: Heart Rhythm Center, Tokyo Medical and Dental University, Tokyo, Japan.
authors.forename: Mihoko
authors.initials: M
authors.lastname: Kawabata
authors.affiliation: Division of Cardiology, Electrophysiology Section, University of California, San Francisco, California.
authors.forename: Melvin M
authors.initials: MM
authors.lastname: Scheinman
authors.affiliation: Heart Rhythm Center, Tokyo Medical and Dental University, Tokyo, Japan.
authors.forename: Kenzo
authors.initials: K
authors.lastname: Hirao
mesh_terms: D003937:Diagnosis, Differential; D004562:Electrocardiography / Q000379:methods*; D005260:Female; D006801:Humans; D008133:Long QT Syndrome / Q000150:complications* / Q000175:diagnosis*; D017180:Tachycardia, Ventricular / Q000150:complications* / Q000175:diagnosis*; D055815:Young Adult; D000098850:Polymorphic Catecholaminergic Ventricular Tachycardia
keywords: bidirectional ventricular tachycardia; catecholaminergic polymorphic ventricular tachycardia; flecainide; long QT syndrome; sudden cardiac death