Congenital Long QT Syndrome in Relation to a Case Report

Long QT syndrome (LQTS) is a disorder of cardiac ion channels that affect repolarization and is characterized by QT interval prolongation on the ECG and torsade de pointes arrhythmias leading to recurrent syncope or sudden death. It may be congenital or acquired. In congenital LQTS mutations in the genes, encoding ion channels at the myocardial cell membrane, cause this channel malfunction. In acquired LQTS ion channel malfunction is caused by metabolic abnormalities or drugs. In this article, we present a case with congenital LQTS, which was diagnosed and treated as epilepsy for two years. It was shown that syncopal episodes were due to ventricular tachycardia attacks. Because of cardiac arrest development cardioverter defibrillator (ICD) was implanted. During one year of follow up, two ventricular fibrillation attacks were terminated by ICD. Due to this case that resembles epilepsy, the clinical features of congenital LQTS and its treatment were reviewed.