What is Short QT Syndrome

How Common Is Short QT Syndrome

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 in these studies 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 2018 altogether a little over a couple of hundred patients had been published in the literature with more than half of them from European countries followed by Japan and China. Most of the patients were men at the age from newborn to elderly with the majority being 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.


Title Men Women MAZ14 Total # Pts
SCD/Ab SCD 17 10 19 46
Incidental finding 6 2 17 25
Sudden death in the family 9 3 2 14
Syncope 1 1 9 11
Syncope in a family with a hx of SCD 3 1 0 4
Palpitations/VT 1 0 0


AF/Severe bradycardia at young age 8 8  0
TOTAL (Including 47 from 38Maz14) 45(64%) 25(36%) 47 117
Maz14: Publication by Mazzanti A et.al..Novel insights into Natural History of Short QT syndrome. Am J. Coll.Cardiol 2014;63(13): 1300-1308