European Heart Journal Advance Access originally published online on September 4, 2006
European Heart Journal 2006 27(20):2382-2384; doi:10.1093/eurheartj/ehl223
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© The European Society of Cardiology 2006. All rights reserved. For Permissions, please e-mail: journals.permissions@oxfordjournals.org
Short QT syndrome and arrhythmogenic cardiac diseases in the young: the challenge of implantable cardioverter-defibrillator therapy for children
1 Institute of Cardiology, University of Bologna, Policlinico S. Orsola, Azienda Ospedaliera S. Orsola-Malpighi, via Massarenti 9, Bologna 40138, Italy
2 Department of Pediatric Cardiology, Azienda Ospedaliera S. Orsola-Malpighi, via Massarenti 9, Bologna 40138, Italy
* Corresponding author. Tel: +390 51349858; fax: +390 51344859. E-mail address: cardio1@med.unibo.it
This editorial refers to ‘Short QT syndrome: clinical findings and diagnostic therapeutic implications’
by C. Giustetto et al., on page 2440
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Giustetto et al.1 have reported the largest available study of patients affected by short QT syndrome, an extremely rare genetic ion channel disease that has only recently been identified and described.2,3 The results of this multicentre study reinforce concepts gleaned from previous reports. Short QT syndrome is a genetic arrhythmogenic disease with a high risk of both syncope and sudden death throughout life. The study was based on data from 25 patients belonging to eight affected families, plus a further four sporadic cases. Although syncopes were reported, cardiac arrest was the most common event and often prompted the initial diagnosis of short QT syndrome. Notably, sudden death events occurred in the first year of life, as well as in adolescent age and adulthood. Moreover, other cases of sudden
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Related articles in EHJ:
- Short QT syndrome: clinical findings and diagnostic–therapeutic implications
- Carla Giustetto, Fernando Di Monte, Christian Wolpert, Martin Borggrefe, Rainer Schimpf, Pascal Sbragia, Gianpiero Leone, Philippe Maury, Olli Anttonen, Michel Haissaguerre, and Fiorenzo Gaita
EHJ 2006 27: 2440-2447.[Abstract] [FREE Full Text]
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 E. A. Stephenson and C. I. Berul Electrophysiological Interventions for Inherited Arrhythmia Syndromes Circulation, August 28, 2007; 116(9): 1062 - 1080. [Full Text] [PDF]
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