Short QT Publications

Click here to find an updated list of Short QT Syndrome publications that includes a description of each publications content.
Last update: September 30, 2007

Reported Cases of Short QT Syndrome
as of December 2005.

Families with Short QT Syndrome: 7

10 Countries: USA, Italy, Spain, The Netherlands, Germany, France, Finland, Brazil, Turkey

Patients with documented short QT interval: 23

32 patients (12 female, 20 male)

Physicians Section

Updated to reflect discovery of new gene for SQTS involving KCNJ2
Last update: August 8, 2005

Patients Section
    Treatment - click here

Updated: August 8, 2005

NEWS – 3.9.2005

The mechanism behind the arrhythmias seen in patients with Short QT Syndrome is not fully understood, but shortening of the effective refractory period in both the atria and ventricles is likely an important factor.

In a recent study by Fabrice Extramiana and Charles Antzelevitch (Amplified Transmural Dispersion of Repolarization as the Basis for Arrhythmogenesis in a Canine Ventricular-Wedge Model  of Short-QT Syndrome. Circulation 2004;110:3661-3666) they demonstrated, that shortening of the QT interval in a canine wedge preparation by Pinacidil (a specific activator of the I_K-ATP channel) was associated with an increased transmural dispersion of repolarization and inducibility of VF by programmed stimulation.

It remains to be demonstrated, however, that similar dispersion of repolarization  exists in patients with Short QT Syndrome, where it is the delayed rectifier currents that are involved and not I_K-ATP.

Last updated: 09/30/2007