The Long and Short of It Insights Into the Short QT Syndrome

Sudden cardiac death in the absence of structural heart disease remains a tragic and often perplexing problem, especially when it affects otherwise healthy young people. Conditions resulting in sudden death, once labeled idiopathic ventricular fibrillation, are now recognized as distinct disorders of ion channel function. A recent addition to this group of sudden death disorders is the short QT syndrome (SQTS). Although it has been recognized for some time that prolongation of cardiac repolarization is a substrate for arrhythmias, the link between accelerated repolarization and arrhythmia has only recently been established (1).