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The QT interval on the ECG reflects the duration of action potential (AP) in ventricle cardiomyocytes, in turn representing the sum of ventricular depolarisation and repolarisation.1 AP is caused by transmembrane flow of ions, including inward depolarising currents mainly through sodium and calcium channels, that is, the sodium current (INa) and the L(long-lasting)-type calcium current (ICaL), and outward repolarising currents mainly through potassium channels, particularly the transient outward current (Ito), the rapid and the slow component of the delayed rectifier potassium current (rapid component, IKr, and slow component, IKs) and the inward rectifier potassium current (IK1) (figure 1).1
The long QT syndrome (LQTS) is a multifactorial disorder characterised by a prolonged heart rate-corrected QT interval (QTc), which predisposes to life-threatening ventricular arrhythmias, particularly torsades de pointes (TdP), that can degenerate into ventricular fibrillation and cause sudden cardiac death.2 ,3 Although …