Long QT Syndrome

Long QT syndrome represents a heterogeneous family of cardiac electrophysiological disorders characterised by QT prolongation and T-wave abnormalities.

Risk assessment should consider age, gender, prior syncopal history, QT-interval duration, and genetic factors. Beta-blockers are the mainstay of therapy for long QT syndrome, but left cardiac sympathetic denervation and ICD implantation should be considered in patients who remain symptomatic despite beta-blocker therapy. Studies are evaluating genotype-specific therapies that may reduce the risk for life-threatening cardiac events in high-risk patients.