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Congenital long QT syndrome (LQTS) is a rare hereditary cardiac disorder characterized by prolongation of the QT interval on electrocardiogram (ECG), predisposing affected individuals to life-threatening arrhythmias. We present a case of a newborn with congenital LQTS and 2:1 atrioventricular (AV) block who presented with bradycardia and QT prolongation. Continuous intravenous lidocaine infusion was initiated, because of hypoglycemia with beta-blockers, resulting in stabilization of AV conduction and prevention of malignant arrhythmias.

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