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.