Congenital long QT syndrome is an inherited disorder of cardiac repolarization that predisposes to syncope and to sudden death from polymorphic ventricular tachycardia. The disorder should be suspected when the electrocardiogram shows characteristic QT abnormalities, or when there is a family history of long QT syndrome or of an event that raises suspicion of long QT syndrome, such as sudden de...

OBJECTIVES The aims of this study were: 1) to evaluate risk factors influencing the clinical course of mutation-confirmed adult patients with long QT syndrome (LQTS), 2) to study life-threatening cardiac events as a specific end point in adults, and 3) to examine the protective effect of beta-blocker therapy on cardiac events in adult LQTS patients with known cardiac channel mutations. BACKGR...

he advent of genetic testing and the implantable ardioverter-defibrillator (ICD) have so revolutionized the utlook for patients with long QT that the cardiac commuity needs to revise its thinking about long QT syndrome LQTS). This is the underlying message of a report by theridge et al. (1) in this issue of the Journal. Etheridge et al. (1) studied 128 children with LQTS. bout one-half of their...

OBJECTIVES We hypothesized that neonatal long QT syndrome (LQTS) with 2:1 atrioventricular block (AVB) could be related to HERG mutations. BACKGROUND Early onset of LQTS is rare but carries a high risk of life-threatening events such as ventricular arrhythmias and conduction disorders. There are no data on possible gene specificity. METHODS We analyzed the characteristics and outcomes of 23 neo...

BACKGROUND Long QT is a cardiac electrical disorder. One of the symptoms of long QT caused by fatal ventricular arrhythmia is seizure. In some studies it was indicated that up to 35% of seizures induced by long QT may be misdiagnosed as other causes of seizure. METHODS In a case-control study, patients experiencing primary seizure with unknown etiology and referring for clinical diagnosis wer...

The long QT syndrome is electrocardiographically characterized by a prolonged QT interval and by several other, more subtle, ST-T-U wave abnormalities, most of which have not been quantified. To determine the possible usefulness of several new electrocardiographic characteristics in identifying patients with known long QT syndrome, logistic regression models were applied to a data base of seven...