The assessment of risk in the asymptomatic patient with long QT syndrome can often be a challenging task, particularly when the available evidence is limited to relatively small retrospective registries, not to mention the need to consider the effect of individual patient factors which are often difficult to quantitate. We describe the relatively uncommon case of a man with a long-standing diag...

Intrauterine and neonatal manifestations of congenital long QT syndrome are associated with a high cardiac risk, particularly when atrioventricular block and excessive QT prolongation (. 600 ms) are present. In a female newborn with these features, treatment with propranolol and mexiletine led to complete reduction of arrhythmia that was maintained 1.5 years later. High throughput genetic analy...

Two patients with a novel variant of long QT syndrome are described. The clinical course was characterised by an in utero onset of ventricular tachycardia and atrioventricular block (at 26 and 30 weeks' gestational age, respectively), and an association with a ventricular septal defect. Studies of both patients' families identified relatives with prolonged QT interval, syncope, or sudden death....

J Cardiol Curr Res 2016, 5(5): 00176 the declining inflow of sodium and calcium. These ion currents utilize specific channels that are under the influence of multiple factors. The long QT syndrome is characterized by QT interval prolongation and a tendency to develop a potentially lethal ventricular tachycardia [1,2]. Several anti-arrhythmic drugs, as well as some non-cardiac medications, block...

L ong QT syndrome causes sudden unexpected death through rapid ventricular tachycardia (‘‘torsades de pointes’’) leading to ventricular fibrillation (see figs 1 and 2). 2 The postmortem examination reveals no cause for the death. These characteristics make long QT syndrome a plausible cause of sudden infant death syndrome (SIDS), and many research efforts have been made to investigate a possibl...

Congenital long QT syndrome type 3 (LQT3) is the third in frequency compared to the 15 forms known currently of congenital long QT syndrome (LQTS). Cardiac events are less frequent in LQT3 when compared with LQT1 and LQT2, but more likely to be lethal; the likelihood of dying during a cardiac event is 20% in families with an LQT3 mutation and 4% with either an LQT1 or an LQT2 mutation. LQT3 is ...

AIMS The long QT syndrome is mainly defined by QT interval prolongation (QTc > 0.44s). However, data obtained in genotyped patients showed that resting QTc measurement alone may be inaccurate for ascertaining the phenotype. The aim of this study was to evaluate the diagnostic performance of QT interval rate-dependence in untreated chromosome 11-linked patients. METHODS The study population co...

Long QT syndrome (LQTS) is an arrhythmogenic cardiovascular disorder resulting from mutations in cardiac ion channels. LQTS is characterized by prolonged ventricular repolarization and frequently manifests itself as QT interval prolongation on the electrocardiogram (ECG). A variety of commonly prescribed anesthetic drugs possess the adverse property of prolonging cardiac repolarization and may ...