CHAMRAN HEART HOSPITAL, ISFAHAN, IRAN
Department of Cardiology, Chamran Heart Hospital, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran.
Department of Pacemaker and Electrophysiology, Shaheed Rajaie Cardiovascular Medical Center, Iran University of Medical Sciences, Tehran, Iran.
The short QT syndrome (SQTS) is a new member of the genetic arrhythmia family (including long QT syndrome, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia and familial atrial fibrillation) with a high incidence of syncope, sudden death and atrial fibrillation in all age groups. The cause of this syndrome is mutation in genes that encode for the potassium rectifier channels, leading to a gain of function in these channels and heterogeneous aberration of repolarization, such that patients with this syndrome become prone to ventricular tachyarrhythmias.
To date, the implantable cardioverter-defibrillator (ICD) is the only therapeutic option for the prevention of sudden cardiac death. Although many potassium channel (IKr and IKs) blocking drugs have been tested for the treatment of this syndrome, only guanidine (and possibly flecainide) has the potential for effective therapy in patients with SQTS and serves as an adjunct to ICD or as a possible alternative treatment.