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Catecholaminergic Polymorphic Ventricular Tachycardia with QT Prolongation

机译:儿茶酚胺能性多形性室速伴QT延长

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摘要

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.
机译:儿茶酚胺能性多形性室性心动过速(CPVT)患者的QT间隔通常是正常的。然而,CPVT患者有时会被误诊为隐匿性长QT综合征(LQTS),因为LQTS患者还表现出晕厥或劳累或极度激动后突然死亡。我们报告的CPVT患者具有致病性RyR2突变,伴有明显的QT延长,在氟卡尼治疗后已恢复正常。

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