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Catecholaminergic Polymorphic Ventricular Tachycardia with QT Prolongation
Author(s) -
TANAKA YASUAKI,
KAWABATA MIHOKO,
SCHEINMAN MELVIN M.,
HIRAO KENZO
Publication year - 2015
Publication title -
pacing and clinical electrophysiology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.686
H-Index - 101
eISSN - 1540-8159
pISSN - 0147-8389
DOI - 10.1111/pace.12735
Subject(s) - catecholaminergic polymorphic ventricular tachycardia , medicine , flecainide , cardiology , qt interval , long qt syndrome , ventricular tachycardia , prolongation , ryanodine receptor 2 , tachycardia , electrocardiography , anesthesia , ryanodine receptor , calcium , atrial fibrillation
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.