Short QT Syndrome


ICD therapy

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Patients with SQTS belong to a category of patients at high risk of sudden cardiac death who are all candidates for prophylactic ICD implantation as long as features for individual risk stratification are not known and the benefit from medical treatment not proven. A characteristic finding in some patients with SQTS is tall peaked T waves, which may lead to double counting of the ICD and inappropriate shocks. This was encountered in four of the very first SQTS patients who received an ICD, but not been a clinical problem since then because of the ability to program ICDs in such a way that it can be avoided. Other complications to ICD treatment in patients with SQTS was presented in the long-term follow-up study from the European SQTS registry. Among 24 patients who received an ICD 14 (58%) had complications. As already mentioned 4 had T wave over-sensing, but there were no recurrences following reprogramming of the ICD. Four had inappropriate shocks during supraventricular tachycardias, 4 patients needed ICD lead replacement: 3 because of lead fracture and 1 because of infection of the ICD system. One patient had early replacement of the ICD because of a recall. Finally, there was one patient who had severe psychological distress from having an ICD. During the approximately 5 years of follow-up, two patients were successfully defibrillated. One had initially presented with cardiac arrest and the other with syncope.

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