congenital long QT syndrome

- 2 major syndromes
  Jervell and Lange-Nielsen syndrome (associated with deafness) - autosomal recessive
  Romano Ward syndrome - autosomal dominant
- mutations in cardiac potassium and sodium channel genes
- 3 major subtypes
   LQT1: provoked by exercise (LQT1 most common) - slow delayed rectifier potassium channel mutation
   LQT2: provoked by emotion and acoustic stimuli (eg: door bell or telephone ring)
   LQT3: at rest or when asleep
- presents with syncope and palpitations or sudden death
- management : beta blocker (reduce adrenergic demand and reduce sympathetic activation from left stellate ganglion). Avoid drugs that prolong QT interval