A 57-year-old woman showed frequent premature ventricular complexes (PVCs) originating from the right ventricular outflow tract (RVOT), and some of the PVCs triggered polymorphic ventricular tachycardia (PVT). Structural heart diseases were ruled out by conventional cardiac examinations. Radiofrequency catheter ablation was successful in eliminating the PVCs and subsequent PVT. However, epinephrine infusion unmasked her prolonged QT interval, and a genetic analysis revealed a KCNH2 mutation (R694H) as the cause of latent type-2 long QT syndrome (LQTS). This case suggests that latent LQTS may work as an arrhythmogenic substrate of PVT triggered by a benign form of RVOT-PVCs in patients with a structurally normal heart.

译文

一名57岁的女性表现出频繁的源自右心室流出道 (RVOT) 的室性早搏 (pvc),其中一些pvc触发了多形性室性心动过速 (PVT)。常规心脏检查排除了结构性心脏病。射频导管消融成功消除了pvc和随后的PVT。然而,肾上腺素输注揭示了她延长的QT间期,基因分析显示KCNH2突变 (R694H) 是潜伏性2型长QT综合征 (LQTS) 的原因。这种情况表明,在结构正常的心脏患者中,潜在的LQTS可能是由良性形式的rvot-pvc触发的PVT的致心律失常底物。

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