European Heart Journal Advance Access originally published online on August 22, 2006
European Heart Journal 2006 27(20):2440-2447; doi:10.1093/eurheartj/ehl185
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Short QT syndrome: clinical findings and diagnostic–therapeutic implications
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
Received 7 February 2006; revised 4 July 2006; accepted 20 July 2006; online publish-ahead-of-print 22 August 2006.
* Corresponding author. Tel: +39 0141 487121; fax: +39 0141 487134. E-mail address: cgiustetto{at}mac.com
See page 2382 for the editorial comment on this article (doi:10.1093/eurheartj/ehl223)
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.
Key Words: Short QT syndrome • Sudden death • Ion channelopathies • SIDS
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