Short QT Syndrome

Abstract

Short QT syndrome is a genetically transmitted disease caused by mutations in different genes that is associated with atrial arrhythmias but more importantly with a severely increased risk of dying suddenly from ventricular tachyarrhythmias. After its first description in the year 2000, there have been increasing reports on patients with different degrees of QT-interval shortening caused by dramatic shortening of repolarization. In patients at a high risk of sudden death or after aborted sudden death, implantable defibrillator implantation is the therapy of choice. However, quinidine has been proven to be effective in reducing ventricular fibrillation recurrence in patients with frequent shocks and/or in patients who refused defibrillator implantation or were otherwise not suitable for a device. Ongoing research tries to further elucidate the mechanisms and genetic mutations underlying this life-threatening arrhythmia syndrome.