Short QT Syndrome


Diagnosis

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The hallmark of SQTS is a short QT-interval on the electrocardiogram(ECG).  Basic information about ECGs and Short QT can be found on this website under What is Short QT.

A short QT-interval can be overlooked. It is particularly important to focus attention on the QT-interval in people with family members who have SQTS or have died suddenly. People with atrial fibrillation at a young age or anyone presenting with passing out spells of unknown origin should have an ECG at a time when the heart beats less than 80 beats per minute in order to look for a short QT interval. It is important to emphasize, that an ECG taken during a higher heart rate may not show the short QT-interval.

If the diagnosis is questionable from the standard ECG, the next step is a 24-hour ambulatory ECG recording (Holter monitoring). This is a continuous ECG recording using a small portable recorder (tape or digital technique) with 4-6 electrodes applied to the front of the chest wall. Since the heart rate varies over a 24 hour period, it gives the physician the opportunity to measure the QT-interval at many different heart rates.  The diagnosis can be made with greatest certainty when the heart beats between 60-80 beats per minute.

The next step could be an electrophysiologic study, but the indication for such a study has not been established yet.

The diagnosis can be firmly made in cases where one of the genetic defects is found. How often this will be the case is not known, but many patients have been found to carry the gene abnormality associated with Short QT Syndrome.

 

last updated: 04/02/2009

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