Table 1

Patient Characteristics (N = 80)

Male, %42 (53%)
Age at first symptom, yrs11.0 (6.0–13.0)
Age at diagnosis, yrs11.0 (7.4–14.0)
Follow-up, yrs6.4 (2.2–10.0)
Proband66 (83)
Family history of CPVT19 (24)
History of cardiac arrest29 (36)
Positive genetic testing49 (80)
RyR245 (92)
CASQ22 (4)
KCNJ22 (4)
Antiarrhythmic
 β anti-adrenergic blocker78 (98)
 Flecainide15 (19)
 Calcium channel blocker12 (15)
ICD (n = 49)
 Age at implantation, yrs13.5 (9.3–16.7)
 ICD follow up9.0 (6.0–12.1)
 Dual chamber26 (53)
 Left cardiac sympathetic denervation15 (19)
Deaths3 (4)

Values are n (%) or median (interquartile range).

CPVT = catecholaminergic polymorphic ventricular tachycardia; ICD = implantable cardioverter defibrillator.