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It is not known how many people are
born with a short QT-interval in their ECG and it is therefore not known
how many people are at risk of developing SQTS. A short QT interval has
never previously been considered to have great clinical significance and
mostly encountered in patients with diseases, where the calcium level in
the blood is high (hypercalcemia).
So far SQTS has been diagnosed in seven families. There are two families in the
US, two in Germany and one in each of the three countries, Spain, The
Netherlands and Italy. One of the families in the US has just recently
been diagnosed with the disease and all the details about this family
are not known yet. In the six families where information is available,
15 people have an ECG with a short QT-interval and two of these have
died suddenly. In addition seven people from these families, who had no
ECG prior to death, have died suddenly and therefore presumed SQTS
patients. Out of this total of 22 patients classified as having SQTS, 10
are women and their ages ranging from 3 months to 84 years. The ages at
the time of sudden cardiac death varied from 3 months to 62 years with
an average of 37 years.
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