[MIM 609 620, 609 621, 609 622]

Very rare. Autosomal dominant transmission of a mutation in one of the KCNQ1 and KCNH2, KCNJ2 genes coding for cardiac potassium channels. Association of an ECG where QT is short (QT and QTc < 320 msec) with a significant risk of syncope or sudden death on ventricular arrhythmia. Occurs mainly in children and young adults. Frequent early atrial fibrillation (around the age of 40 years).

Treatment: automatic implanted defibrillator

Anesthetic implications : 

presence of an automatic implanted defibrillator.

References : 

• Lupoglazoff J-M, Denjoy I.
  Syndrome du QT court familial.
  Encyclopédie Orphanet février 2007, 6p.

Updated: March 2016