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岁的妇女表现出频繁的早产于右室流出道(RVOT)的心室复合物(PVC),并且其中一些PVC触发了多形性室性心动过速(PVT)。通过常规心脏检查排除了结构性心脏病。射频导管消融成功地消除了PVC和随后的PVT。然而,肾上腺素的注入掩盖了她延长的QT间隔,遗传分析显示KCNH2突变(R694H)是潜在的2型长QT综合征(LQTS)的病因。这种情况表明,在心脏结构正常的患者中,潜在的LQTS可能是由良性形式的RVOT-PVC触发的PVT的一种致心律失常底物。

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