Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited 
arrhythmia syndrome characterized by bidirectional or polymorphic ventricular 
arrhythmias under conditions of increased sympathetic activity in young patients 
with structurally normal hearts. Patients with CPVT are at high risk of 
developing life-threatening ventricular arrhythmias when untreated. A wide 
variety of arrhythmic event rates on conventional therapy, with β-blockers as 
the cornerstone, has been reported. Here, we systematically review all available 
studies describing the efficacy of β-blocker therapy for prevention of 
arrhythmic events in CPVT. Because of heterogeneity between the studies, a 
random-effects meta-analysis model was used to assess the efficacy of β-blocker 
therapy in preventing any arrhythmic event [syncope, aborted cardiac arrest 
(ACA), and sudden cardiac death (SCD)], near-fatal arrhythmic events (ACA and 
SCD), and fatal arrhythmic events. Eleven studies including 403 patients, of 
whom 354 (88%) had a β-blocker prescribed, were identified. Mean follow-up 
ranged from 20 months to 8 years. Estimated 8-year arrhythmic, near-fatal, and 
fatal event rates were 37.2% [95% confidence interval (CI): 16.6-57.7], 15.3% 
(95% CI: 7.4-23.3), and 6.4% (95% CI: 3.2-9.6), respectively. In addition, we 
review the recent developments in alternate chronic treatment options for CPVT 
patients, including calcium channel blockers, flecainide, left cardiac 
sympathetic denervation, and implantable cardioverter defibrillators. A new 
treatment strategy is proposed, including a stepwise addition of the alternate 
treatment options to β-blockers in patients who do not respond sufficiently to 
this first-line therapy. Finally, future developments in chronic treatment 
options and acute treatment options of ventricular arrhythmias are discussed.