Congenital long QT syndrome (LQTS) is a familial cardiac ion channelopathy first described over sixty years ago. It is characterised by prolonged ventricular repolarization (long QT on ECG), ventricular arrhythmias and associated syncope or sudden cardiac death. As the most closely studied cardiac channelopathy, over the decades we have gained a deep appreciation of the complex genetic model of LQTS. Variability in genetic expression and incomplete penetrance leads to a heterogenous phenotype that can be challenging to clinically classify. In recent times, progress has been made in diagnostic method, risk stratification and treatment options. This review has been written as a guide for the general cardiologist to understand the basic pathophysiology, diagnosis, and management priorities for the most encountered LQTS subtypes: LQT1, LQT2 and LQT3. This article is protected by copyright. All rights reserved.
Congenital Long QT Syndrome: A Clinician's Guide