Long QT syndrome

Abstract

The so-called congenital long QT syndrome (LQTS) is an inherited arrhythmia syndrome predisposing to life-threatening ventricular arrhythmias and sudden death. It is caused by prolongation of the repolarization phase of the cardiac action potential, which may manifest as lengthening of the heart rate-corrected QT interval (QTc) on the surface electrocardiogram (ECG). This chapter reviews the clinical presentation of LQTS, its diagnosis, and principles of management in the context of recent clinical advances and molecular genetics, with a focus on the most common forms of LQTS - LQT1, LQT2, and LQT3.