Long QT syndrome (LQTS) is an uncommon, but important, cause of ventricular arrhythmias. The diagnosis is straightforward in symptomatic patients with marked QT prolongation on a resting 12-lead electrocardiogram (ECG). However, in many patients, the ECG findings are dynamic, and to make the diagnosis, clinicians need to be aware of suggestive features. The greatest challenge in managing these patients is risk stratification for a sudden arrhythmic event. Beta-blockers have been shown to reduce the risk for ventricular arrhythmias in all genotype-positive patients, regardless of 12-lead ECG findings. For patients in whom beta-blockers are contraindicated, left cardiac sympathetic denervation may be a useful therapy. Implantable cardioverter–defibrillators have a role to play in preventing sudden cardiac death, although their use should be balanced with associated complications and psychological impact. Population screening for LQTS remains controversial. Screening of first-degree relatives in sudden arrhythmic death syndrome is recommended, but population screening is not currently undertaken in the United Kingdom.
Effectiveness of Implantable Cardioverter-Defibrillators to Reduce Mortality in Patients With Long QT Syndrome
