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Dante Labs US Report

Short QT Syndrome

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Short QT Syndrome (SQTS) is a rather rare heart disease, characterized by the shortening of the QT interval on the electrocardiogram. The QT interval is a specific stretch of this trace and indicates the time that elapses between one heartbeat and the next, during which the myocardium returns to a rest situation waiting for the next heartbeat. These phenomena of depolarization (beat) and repolarization (rest) are determined by specific sodium and potassium currents, whose passage through the cells is regulated by special ion channels. Due to the reduction of the QT interval, the heart does not have a physiological recovery time and this leads to the typical arrhythmic manifestations of the syndrome.
SQTS can occur at any age, even in childhood, and is transmitted in an autosomal dominant manner.
The mutations that cause its onset occur in the genes that code for the ion channels of the myocardium; for this reason, SQTS is classified as "channelopathy".
This results in a reduction of the entire action potential.

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