long-QT syndrome

long-QT syndrome

A genetic disorder in which the interval between the Q and the T waves of the electrocardiogram is usually, but not necessarily, prolonged to 500 msec or more. The condition is due to defects of potassium ion or sodium ion channels in heart muscle and features fast heart rate, heart irregularities, fainting and sometime sudden death. Six different mutations of genes coding for the ion channels have been detected, but in about 50 per cent of cases these mutations do not produce symptoms. Sudden death occurs only in ablout 4 per cent of affected persons.
References in periodicals archive ?
Prevalence of long-QT syndrome gene variants in sudden infant death syndrome.
Compound mutations: a common cause of severe long-QT syndrome.
Chronic Amiodarone Evokes No Torsade de Pointes Arrhythmias Despite QT Lengthening in an Animal Model of Acquired Long-QT Syndrome.
Risk factors for aborted cardiac arrest and sudden cardiac death in children with the congenital long-QT syndrome.
Risk of aborted cardiac arrest or sudden cardiac death during adolescence in the long-QT syndrome.
Effectiveness and limitations of beta-blocker therapy in congenital long-QT syndrome.
Other areas investigated include the origin and role of N-homocysteinylated proteins in cardiovascular disease, the potential role of biomarkers in diagnosing the risk of sudden cardiac death, and pharmacological therapy in children with congenital long-QT syndrome.
Age- and sex-related differences in clinical manifestations in patients with congenital long-QT syndrome.
Bradyarrhythmias, including complete atrioventricular block (CAVB), may predispose to acquired long-QT syndrome (L(ITS) and torsade de pointes (TdP) (1).
Factors that predict a high risk of life-threatening events in adolescents with the hereditary long-QT syndrome are duration of the QT interval, timing and frequency of recent syncope, and male sex, according to a new study by Dr.
A recessive variant of the Romano-Ward long-QT syndrome.