[Short QT syndrome]

Abstract

Short QT syndrome was first described in 2000. It is a sporadic or familial ion channel disease that is associated with abbreviation of the QT interval permanently or transiently. The time of first manifestation of symptoms such as atrial fibrillation or syncope or even sudden death is between the 2nd and 4th decade. Sudden death has also been described for newborns and adolescents. Therapy depends on the severity of the symptoms. The therapy of choice for secondary prevention of sudden death is the implantable cardioverter-defibrillator (ICD). Quinidine has been shown to be effective in preventing arrhythmias in a number of patients. It is mostly used as an adjunct to the ICD but has also been used with considerable success in children and individuals who refused ICD implantation.