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[Catecholinergic ventricular tachycardia in children].
- Source :
-
Archives des maladies du coeur et des vaisseaux [Arch Mal Coeur Vaiss] 2005 May; Vol. 98 (5), pp. 506-12. - Publication Year :
- 2005
-
Abstract
- Catecholinergic ventricular tachycardia is an adrenergic induced polymorphic ventricular arrhythmia. It occurs in infancy and is responsible for syncope and sudden death in the absence of any morphological cardiac abnormality. Without treatment the mortality in catecholinergic ventricular tachycardia is very high. We report genetic and clinical data from 25 cases of catecholinergic ventricular tachycardia referred with syncope (n=19) or resuscitated sudden death during exercise (n=6). A family history from the 25 families identified 41 apparent subjects considered as being clinically affected, with an average age of 30 +/- 10 years (11 to 62 years). Analysis of the RyR2 gene showed mutations in 13 of the 25 cases and in 39 of apparent subjects. With betablocker treatment (nadolol: 1.6 +/- 0.15 mg/kg), 96% of patients remained asymptomatic over an average follow-up of between 7.5 +/- 1.5 years, although some of them continued to display polymorphic ventricular extrasystoles on exercise. Nevertheless, 12% of the cases suffered sudden death or further syncope during follow-up. An automatic defibrillator was implanted in 2 patients who had a RyR2 mutation. High dose betablockers are effective in preventing serious rhythm disturbance in children. In adolescence, implanting an automatic defibrillator should be discussed in cases with a history of syncope or resuscitated sudden death. We confirm the importance of genetic studies in these families at high risk of sudden death.
- Subjects :
- Adolescent
Adult
Catecholamines pharmacology
Child
DNA Mutational Analysis
Defibrillators, Implantable
Electrocardiography
Exercise
Female
Humans
Male
Middle Aged
Retrospective Studies
Risk Factors
Ryanodine Receptor Calcium Release Channel genetics
Syncope
Tachycardia, Ventricular drug therapy
Adrenergic beta-Antagonists therapeutic use
Death, Sudden, Cardiac
Tachycardia, Ventricular genetics
Tachycardia, Ventricular pathology
Subjects
Details
- Language :
- French
- ISSN :
- 0003-9683
- Volume :
- 98
- Issue :
- 5
- Database :
- MEDLINE
- Journal :
- Archives des maladies du coeur et des vaisseaux
- Publication Type :
- Academic Journal
- Accession number :
- 15966600