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.