• Long QT syndrome is an inherited disorder in which cardiac Na, K, and Ca ion channels are malfunctional.
• On ECG, there is prolonged QTc interval.
• Common types of long QT syndrome are:
Jervell-Lange Nielson syndrome (autosomal recessive inheritance, with resulting deafness)
Romano-Ward syndrome (autosomal dominant inheritance, without resulting deafness)
Andersen-Tawil syndrome (rare, with resulting skeletal abnormalities)
Timothy syndrome (rare, with resulting congenital heart, cognitive, musculoskeletal involvement)
• Long QT syndrome is associated with an increased risk of Torsades de pointes and fatal tachyarrhythmia.
