Short QT Syndrome Patients

Treatment of Short QT Syndrome with ICD

Congenital Short QT Syndrome and Implantable Cardioverter Defibrillator Treatment: Inherent Risk for Inappropriate Shock Delivery Schrimpf R, Wolpert C, Bianchi F, Giustetto C, Gaita F, Bauersfeld U, Borggrefe M.. J Cardiovasc Electrophysiol 2003;14:1273-1277

This paper describes the experience of ICD implantation in 5 patients from the Italian and German families. Due to T-wave oversensing inappropriate shocks were delivered in 3 out of the 5 patients shortly after implantation. Programming lower sensitivities and decay delays prevented further inappropriate discharges.

The oversensing of the T wave lead to the observation, that patients with short QT syndrome often have prominent peaked T waves.

Inappropriate Implantable Cardioverter Defibrillator Therapy in the Short QT Syndrome: Old Problem in a New Disease Passman R. J Cardiovasc Electrophysiol 2003;14:1278-1279 This is an editorial comment to the previous paper.

Short QT syndrome: Successful prevention of sudden cardiac death in an adolescent by implantable cardioverter-defibrillator treatment for primary prophylaxis. Schimpf R, Bauersfeld U, Gaita F, Wolpert C. Heart Rhythm 2005;2:416-417

Case report of a previously asymptomatic 16 y.o. male who received an ICD due to a family history of SQTS and his own QT 248 msec and QTc 252 msec. Six month later the ICD saved his life, when he developed ventricular fibrillation during sleep. He is a member of the first German family described in ref. # 7. The episode occurred during a HR of approximately 60 bpm, initiated by a premature beat with a coupling interval of 180 msec.

This is the first description of a patient with SQTS rescued by an ICD shock.The case shows that patients with SQTS should be considered highly vulnerable to premature ventricular beats below 180 msec, to which normal hearts would be refractory.

Short QT Syndrome and arrhythmogenic cardiac diseases in the young: the challenge of implantable cardioverter-defibrillator therapy for children. Borlani G, Biffi M, Valzania C, Bronzetti G, Martignani C. Eur Heart J 2006;27:2382-2384 (Editorial)

Inappropriate ICD Discharge Due to T-Wave Oversensing in a Patient with Short QT Syndrome Sun Y, Zhang P, Li X, Guo J. PACE 2010;33:113-116

45 yo male with SQTS (QTc: 298 msec) and high peaked T waves received a St. Jude Epic DR V-239 ICD. Ten days later he received 5 inappropriate shocks for T wave oversensing in sinus rhythm. After programming the threshold start value to 100% and decay delay to 0 msec together with a total ventricular refractory period to 432 msec and R wave sensing at 5 mV the patient had no further shocks during 6 months follow-up.

Use of a subcutaneous ICD in a patient with short QT syndrome.

Pierre Mondoly, Christelle Cardin, Anne Rollin, Alexandre Duparc, Philippe Maury, Clin Case Rep. 2016;4(1):35-3

Implantable cardioverter defibrillator replacement guided by T wave safety margin in a short QT syndrome patient Yishi Shen, Weizhong Pan, Chenyang Jiang, Guosheng Fu, Yaxun Sun, Dan Hu Pacing Clin Electrophysiol 2019;42(5):557-559

Long-term follow-up of implantable cardioverter-defibrillators in Short QT syndrome Ibrahim El-Battrawy, Johanna Besler, Uzair Ansari, Volker Liebe, Rainer Schimpf, Erol Tülümen, Boris Rudic, Siegfred Lang, Katja Odening, Lukas Cyganek, Christian Wolpert, Xiaobo Zhou, Martin Borggrefe, Ibrahim Akin Clin Res Cardiol 2019;108(10):1140-1146