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Introduction Long QT syndrome (LQTS) is a congenital disorder characterized by a prolongation of the QT interval on electrocardiograms (ECGs) and a propensity to ventricular tachyarrhythmias, which may lead to syncope, cardiac arrest, or sudden death. T-wave alternans (TWA) refers to the periodic beat-to-beat alternation of T-wave shape, polarity and amplitude on surface ECG during regular heart rhythm. In this report, a case of long QT syndrome with KCNQ1 gene mutation induced TWA in the head-up tilt test (HUTT), which has not been report
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