Incidence and risk factors of arrhythmic events in catecholaminergic polymorphic ventricular tachycardia. - Inserm - Institut national de la santé et de la recherche médicale Accéder directement au contenu
Article Dans Une Revue Circulation Année : 2009

Incidence and risk factors of arrhythmic events in catecholaminergic polymorphic ventricular tachycardia.

Résumé

BACKGROUND: The pathophysiological background of catecholaminergic polymorphic ventricular tachycardia is well understood, but the clinical features of this stress-induced arrhythmic disorder, especially the incidence and risk factors of arrhythmic events, have not been fully ascertained. METHODS AND RESULTS: The outcome in 101 catecholaminergic polymorphic ventricular tachycardia patients, including 50 probands, was analyzed. During a mean follow-up of 7.9 years, cardiac events defined as syncope, aborted cardiac arrest, including appropriate discharges from implantable defibrillators, or sudden cardiac death occurred in 27 patients, including 2 mutation carriers with normal exercise tests. The estimated 8-year event rate was 32% in the total population and 27% and 58% in the patients with and without beta-blockers, respectively. Absence of beta-blockers (hazard ratio [HR], 5.48; 95% CI, 1.80 to 16.68) and younger age at diagnosis (HR, 0.54 per decade; 95% CI, 0.33 to 0.89) were independent predictors. Fatal or near-fatal events defined as aborted cardiac arrest or sudden cardiac death occurred in 13 patients, resulting in an estimated 8-year event rate of 13%. Absence of beta-blockers (HR, 5.54; 95% CI, 1.17 to 26.15) and history of aborted cardiac arrest (HR, 13.01; 95% CI, 2.48 to 68.21) were independent predictors. No difference was observed in cardiac and fatal or near-fatal event rates between probands and family members. CONCLUSIONS: Cardiac and fatal or near-fatal events were not rare in both catecholaminergic polymorphic ventricular tachycardia probands and affected family members during the long-term follow-up, even while taking beta-blockers, which was associated with a lower event rate. Further studies evaluating concomitant therapies are necessary to improve outcome in these patients.
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Dates et versions

inserm-00410301 , version 1 (13-07-2011)

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Meiso Hayashi, Isabelle Denjoy, Fabrice Extramiana, Alice Maltret, Nathalie Roux Buisson, et al.. Incidence and risk factors of arrhythmic events in catecholaminergic polymorphic ventricular tachycardia.. Circulation, 2009, 119 (18), pp.2426-34. ⟨10.1161/CIRCULATIONAHA.108.829267⟩. ⟨inserm-00410301⟩
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