Long QT Syndrome overview

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [2]

Overview

The long QT syndrome (LQTS) is a heart condition associated with prolongation of repolarisation (recovery) following depolarisation (excitation) of the cardiac ventricles. It is associated with syncope (fainting) and sudden death due toventricular arrhythmias. Arrhythmias in individuals with LQTS are often associated with exercise or excitement. LQTS is associated with the rare, ventricular arrhythmia torsade de pointes, which can deteriorate into ventricular fibrillation and ultimately death.

Individuals with LQTS have a prolongation of the QT interval on the ECG. The Q wave on the ECG corresponds to ventricular depolarization while the T wave corresponds to ventricular repolarization. The QT interval is measured from the Q point to the end of the T wave. While many individuals with LQTS have persistent prolongation of the QT interval, some individuals do not always show the QT prolongation; in these individuals, the QT interval may prolong with the administration of certain medications.

Classification

There are multiple genetic mutations that account for LQTs, but LQT1, LQT2, and LQT3 account for 75% of cases of LQT.

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