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Clinical experiences of patients with catecholaminergic polymorphic ventricular tachycardia

Published online by Cambridge University Press:  23 December 2008

Alpay Çeliker ,
İlkay Erdoğan ,
Tevfik Karagöz  and
Sema Özer
Alpay Çeliker
Affiliation:
Department of Pediatric Cardiology, Hacettepe University, Ankara, Turkey
İlkay Erdoğan*
Affiliation:
Department of Pediatric Cardiology, Hacettepe University, Ankara, Turkey
Tevfik Karagöz
Affiliation:
Department of Pediatric Cardiology, Hacettepe University, Ankara, Turkey
Sema Özer
Affiliation:
Department of Pediatric Cardiology, Hacettepe University, Ankara, Turkey
*
Correspondence to: Dr İlkay Erdoğan, Hacettepe University, Department of Pediatric Cardiology, 06100 Sihhiye, Ankara, Turkey. Tel: 0312 305 1157; Fax: 0312 309 0220; E-mail: ilkayoerdogan@hotmail.com
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Abstract

Catecholaminergic polymorphic ventricular tachycardia is a rare entity that can occur in children without cardiac disease and with a normal QT interval. It may cause syncope, convulsions, and sudden death during physical activity or emotional distress. We report the clinical features, treatment, and follow-up of 16 children with this diagnosis, emphasizing the potentially fatal nature of the disease.

The mean age of patients at the onset of symptoms and at the time of diagnosis was 7.8 plus or minus 2.5 years, and 10.6 plus or minus 3.5 years, respectively. Syncope was the main complaint in 11, and 7 were treated as erroneously as having epilepsy. Diagnosis was confirmed by exercise and/or infusion of isoproterenol. Once the diagnosis was made, we started propranolol in all patients, and added verapamil if ventricular tachycardia was still inducible on a treadmill exercise test. An intracardiac defibrillator was implanted in 4 patients. Of the 16 patients, 4 died suddenly, giving a rate of mortality of 25%. In 2 of those dying suddenly, there was evidence of poor compliance to the recommended treatment. Another 2 patients had been resusciated because of sudden cardiac arrest.

Catecholaminergic polymorphic ventricular tachycardia must be considered in the differential diagnosis of syncope in children without heart disease but with a normal QT interval. Medical treatment with propranolol and verapamil may decrease the incidence of arrhythmia. Implantation of intracardiac defibrillators should be considered in those resistant to drug therapy. Delay in diagnosis, and inadequate treatment, can result in sudden cardiac death.

Keywords

Syncopeimplantable cardioverter defibrillatorschildhood
Type
Original Article
Information
Cardiology in the Young , Volume 19 , Issue 1 , February 2009 , pp. 45 - 52
Copyright
Copyright © Cambridge University Press 2008

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