title: Incidence and risk factors of arrhythmic events in catecholaminergic polymorphic ventricular tachycardia.
abstract: 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.
  
  
  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.
authors.affiliation: Service de Cardiologie, Hôpital Lariboisière, Assistance Publique-Hôpitaux de Paris, Université Paris Diderot, INSERM U942, Paris, France.
authors.forename: Meiso
authors.initials: M
authors.lastname: Hayashi
authors.forename: Isabelle
authors.initials: I
authors.lastname: Denjoy
authors.forename: Fabrice
authors.initials: F
authors.lastname: Extramiana
authors.forename: Alice
authors.initials: A
authors.lastname: Maltret
authors.forename: Nathalie Roux
authors.initials: NR
authors.lastname: Buisson
authors.forename: Jean-Marc
authors.initials: JM
authors.lastname: Lupoglazoff
authors.forename: Didier
authors.initials: D
authors.lastname: Klug
authors.forename: Miyuki
authors.initials: M
authors.lastname: Hayashi
authors.forename: Seiji
authors.initials: S
authors.lastname: Takatsuki
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Villain
authors.forename: Joël
authors.initials: J
authors.lastname: Kamblock
authors.forename: Anne
authors.initials: A
authors.lastname: Messali
authors.forename: Pascale
authors.initials: P
authors.lastname: Guicheney
authors.forename: Joël
authors.initials: J
authors.lastname: Lunardi
authors.forename: Antoine
authors.initials: A
authors.lastname: Leenhardt
mesh_terms: D000293:Adolescent; D000319:Adrenergic beta-Antagonists / Q000627:therapeutic use; D000328:Adult; D002155:Calsequestrin / Q000235:genetics*; D002648:Child; D002675:Child, Preschool; D016757:Death, Sudden, Cardiac / Q000453:epidemiology; D005080:Exercise Test; D005192:Family Health; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D015994:Incidence; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D011110:Polymorphism, Genetic; D012307:Risk Factors; D019837:Ryanodine Receptor Calcium Release Channel / Q000235:genetics*; D013575:Syncope / Q000235:genetics / Q000401:mortality; D017180:Tachycardia, Ventricular / Q000235:genetics* / Q000401:mortality* / Q000517:prevention & control; D055815:Young Adult
chemical_list: D000319:Adrenergic beta-Antagonists; C443109:CASQ2 protein, human; D002155:Calsequestrin; D019837:Ryanodine Receptor Calcium Release Channel