![]() 7 – 10 Based on this distribution, the “normal QTc” interval may be defined as values that fall within 2 standard deviations (SD) from the mean. Several large scale population studies have shown that the corrected QT interval (QTc, using the Bazett formula) of healthy individuals conforms to a gaussian normal distribution, that is, a bell-shaped curve. The definition of a pathophysiologic long QT interval evolved over a period of several decades and it may take some time to define what constitutes a pathophysiologic short QT interval. The purpose of this review is to summarize the available data concerning SQTS from bench to bedside. ![]() Since its initial introduction in 2000, significant progress has been made in defining the clinical, genetic, 6 and ionic basis of the disease as well as approaches to therapy. 5 They described 6 patients of SQTS in 2 unrelated European families with strong family history of sudden death in association with short QT intervals on the ECG. 1 The familial nature and arrhythmic potential of the disease was further highlighted by Gaita et al. 4 SQTS was first described as a new clinical entity by Gussak et al in 2000. Cases of SQTS have been reported with presentation as early as in the first year of life, suggesting that it could be one of the etiologies underlying sudden infant death syndrome. 1 – 3 It is a relatively recent addition to the list of inherited channelopa-thies responsible for sudden cardiac death (SCD) in individuals with structurally normal hearts. ![]() Short QT syndrome (SQTS) is an inheritable primary electric disease of the heart characterized by abnormally short QT intervals on the ECG and an increased propensity to develop atrial and ventricular tachyarrhythmias. ![]()
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