Short QT Syndrome: A Familial Cause of Sudden Death

By F. Gaita, C. Giustetto, F. Bianchi, C. Wolpert, R. Schimpf, R. Riccardi, S. Grossi, E. Richiardi, M. Borggrefe. Circulation, 2003

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ACC/AHA/ESC 2006 guidelines for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: A report of the American College of Cardiology/American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines (2006, background): “In 2003, Gaita et al.(811) reported 2 families with SQTS and showed that all affected patients had a QTc less than 300 ms with a QT interval less than 280 ms.”
2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the preventionof sudden cardiac death (2015, neutral): “40%.(119,447) Given the small size of the populations reported so far, the high lethality may partially reflect a reporting bias related to the underdetection of SQTS in asymptomatic patients.”
Recommendations for interpretation of 12-lead electrocardiogram in the athlete. (2009, background): “Short-QT syndrome is an inheritable ion-channel disease that is characterized by an abnormally short cardiac repolarization predisposing to life-threatening ventricular arrhythmias.(101,102) QT interval in symptomatic index patients has been reported to be consistently 320 ms (QTc 340 ms).”