Long QT syndrome (LQTS) is an inherited condition associated with delayed repolarization of the ventricles. It is important to recognise as it may lead to ventricular tachycardia/torsade de pointes and can therefore cause collapse/sudden death. The most common variants of LQTS (LQT1 & LQT2) are caused by defects in the alpha subunit of the slow delayed rectifier potassium channel. A normal corrected QT interval is less than 430 ms in males and 450 ms in females.

Causes of a prolonged QT interval:

Congenital Drugs* Other
• Jervell-Lange-Nielsen syndrome (includes deafness and is due to an abnormal potassium channel)
• Romano-Ward syndrome (no deafness) amiodarone, sotalol, class 1a antiarrhythmic drugs
tricyclic antidepressants,
SSRIs (especially citalopram)
methadone
chloroquine
terfenadine^^
erythromycin
haloperidol
Anti-emetics: ondanestron, metoclopramide, domperidone Malnutrition & electrolyte imbalance: hypocalcaemia, hypokalaemia, hypomagnesaemia
acute myocardial infarction
myocarditis
hypothermia
CNS: subarachnoid haemorrhage, ischaemic stroke

Features

Management

^ the usual mechanism by which drugs prolong the QT interval is blockage of potassium channels. See the link for more details

^^ a non-sedating antihistamine and classic cause of prolonged QT in a patient, especially if also taking P450 enzyme inhibitor, e.g. Patient with a cold takes terfenadine and erythromycin at the same time

^^^ note sotalol may exacerbate long QT syndrome