Short QT Syndrome


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Only a small number of patients with SQTS have been identified.  This has limited our overall experience with the disease leaving no clearly established treatment regimen.  The current trend in treatment is provided below.

The rhythm disturbance from the upper chambers of the heart, atrial fibrillation, can probably be treated like in many other situations, where this arrhythmia is seen. We have used a drug called propafenone, with great success, but it is likely that other drugs like sotalol, dofetilide or flecainide might work as well.

One approach to treatment has focused on prolonging the QT interval with the use of a medication called hydroquinidine.  Studies in a small subset of SQTS patients have shown that this medication lengthens the QT interval.  It also decreased the ease of inducing arrhythmias during electrophysiology studies.

The main treatment concern has focused on protecting patients with SQTS from dying suddenly. So far most patients have received an implantable defibrillator (ICD). It is possible; however, that medications like those listed above may be an option for some patients, especially in children where implantation of an ICD may not be possible.  However, to date, ICD implantation is the standard of care in the early experience with SQTS.


last updated: 04/02/2014

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