After the first publication in 2000 about the potential danger in having a short QT interval in the ECG several large studies including thousands of people were performed in order to find out how common a short QT was. The problem was that it was not totally agreed upon when to call a QT interval short, but using 340-350 millisecond as the lower limit for a normal QT interval at a heart rate of 60 beats per minute, such a short QT interval was seen in less than one in a thousand and none of the people with such a short QT interval were having Short QT Syndrome. It was therefore obvious that Short QT Syndrome was an extremely rare hereditary condition seen in only a few families.
By 2014 altogether a little over a hundred patients have been published in the literature with more than half of them from European countries followed by North-America, Japan and China. Most of the patients have been men and the age has been from newborn to elderly with the majority young adults. Most patients have been found when a family had an ECG done because of sudden cardiac death of a younger apparently healthy family member.