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European Heart Journal Advance Access published online on August 22, 2006
European Heart Journal, doi:10.1093/eurheartj/ehl185
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European Heart Journal © The European Society of Cardiology 2006; all rights reserved
Received February 7, 2006
Revised July 4, 2006
Accepted July 20, 2006

Clinical research

Short QT syndrome: clinical findings and diagnostic-therapeutic implications

Carla Giustetto 1 *, Fernando Di Monte 1, Christian Wolpert 2, Martin Borggrefe 2, Rainer Schimpf 2, Pascal Sbragia 3, Gianpiero Leone 4, Philippe Maury 5, Olli Anttonen 6, Michel Haissaguerre 7, and Fiorenzo Gaita 1

1 Department of Cardiology, Cardinal Massaia Hospital, Asti, Italy
2 Department of Medicine-Cardiology, University Hospital Mannheim, Germany
3 Division of Cardiology, Hopital Nord, Marseille, France
4 Division of Cardiology, Hospital, Aosta, Italy
5 Federation of Cardiology, University Hospital Rangueil, Toulouse, France
6 Division of Cardiology, Lahti Central Hospital, Lahti, Finland
7 Hopital Cardiologique du Haut-Leveque, Bordeaux-Pessac, France

* To whom correspondence should be addressed.
Carla Giustetto, E-mail: cgiustetto{at}mac.com


  Abstract

Aims Clinical presentation, occurrence of sudden infant death, and results of the available therapies in the largest group of patients with short QT syndrome (SQTS), studied so far, are reported.

Methods and results Clinical history, physical examination, electrocardiogram (ECG), exercise stress testing, electrophysiological study, morphological evaluation, genetic analysis and therapy results in 29 patients with SQTS and personal and/or familial history of cardiac arrest are reported. The median age at diagnosis was 30 years (range 4-80). In all subjects, structural heart disease was excluded. Eighteen patients were symptomatic (62%): 10 had cardiac arrest (34%) and in 8 (28%) this was the first clinical presentation. Cardiac arrest had occurred in the first months of life in two patients. Seven patients had syncope (24%); 9 (31%) had palpitations with atrial fibrillation documented even in young subjects. At ECG, patients exhibited a QT interval ≤320 ms and QTc ≤340 ms. Fourteen patients received an implantable cardioverter-defibrillator (ICD) and 10 hydroquinidine prophylaxis. At a median follow-up of 23 months (range 9-49), one patient received an appropriate shock from the ICD; no patient on hydroquinidine had sudden death or syncope.

Conclusion SQTS carries a high risk of sudden death and may be a cause of death in early infancy. ICD is the first choice therapy; hydroquinidine may be proposed in children and in the patients who refuse the implant.

Keywords: Short QT syndrome; Sudden death; Ion channelopathies; SIDS.
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