Long-term follow-up of patients with short QT syndrome

Carla Giustetto, Rainer Schimpf, Andrea Mazzanti, Chiara Scrocco, Philippe Maury, Olli Anttonen, Vincent Probst, Jean Jacques Blanc, Pascal Sbragia, Paola Dalmasso, Martin Borggrefe, Fiorenzo Gaita

Research output: Contribution to journalArticlepeer-review


Objectives: The aim of this study was to investigate the clinical characteristics and the long-term course of a large cohort of patients with short QT syndrome (SQTS). Background: SQTS is a rare channelopathy characterized by an increased risk of sudden death. Data on the long-term outcome of SQTS patients are not available. Methods: Fifty-three patients from the European Short QT Registry (75% males; median age: 26 years) were followed up for 64 ± 27 months. Results: A familial or personal history of cardiac arrest was present in 89%. Sudden death was the clinical presentation in 32%. The average QTc was 314 ± 23 ms. A mutation in genes related to SQTS was found in 23% of the probands; most of them had a gain of function mutation in HERG (SQTS1). Twenty-four patients received an implantable cardioverter defibrillator, and 12 patients received long-term prophylaxis with hydroquinidine (HQ), which was effective in preventing the induction of ventricular arrhythmias. Patients with a HERG mutation had shorter QTc at baseline and a greater QTc prolongation after treatment with HQ. During follow-up, 2 already symptomatic patients received appropriate implantable cardioverter defibrillator shocks and 1 had syncope. Nonsustained polymorphic ventricular tachycardia was recorded in 3 patients. The event rate was 4.9% per year in the patients without antiarrhythmic therapy. No arrhythmic events occurred in patients receiving HQ. Conclusions: SQTS carries a high risk of sudden death in all age groups. Symptomatic patients have a high risk of recurrent arrhythmic events. HQ is effective in preventing ventricular tachyarrhythmia induction and arrhythmic events during long-term follow-up.

Original languageEnglish
Pages (from-to)587-595
Number of pages9
JournalJournal of the American College of Cardiology
Issue number6
Publication statusPublished - Aug 2 2011


  • arrhythmias
  • channelopathies
  • hydroquinidine
  • implantable cardioverter defibrillator
  • short-QT syndrome
  • sudden death

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine


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