To document the PVC burden and determine whether periodic Holter monitoring was continuously linked with persistent ventricular arrhythmias in persons with ARVC; In the follow-up research, patients with a confirmed ARVC diagnosis and baseline Holter monitoring data at disease identification were enrolled from 6 ARVC registries in North America and Europe. From June 1 to September 15, 2021, data were collected. The relation between prespecified variables retrieved at each Holter monitoring follow-up (i.e., overall PVC burden; the presence of sudden PVC spikes, defined as an absolute increase in PVC burden more than or equal to 5,000 per 24 hours or a relative more than or equals 75% increase, with an absolute increase of more than or equal to 1,000 PVCs; the presence of nonsustained ventricular tachycardia [NSVT]; and use of β-blockers and class III antiarrhythmic drugs) and sustained ventricular arrhythmias were occurring within 12 months after that Holter examination was assessed using a mixed logistical model. In 169 ARVC patients with a mean age of 36.3 (95 men [56.2%]) and an average follow-up of 54 months, 723 Holter tests were done during a median follow-up of 54 months (IQR, 42-63), detecting 75 PVC spikes and 67 sustained ventricular arrhythmias. The PVC burden reduced particularly from the first to the second Holter examination (mean, 2,906 [95% CI, 1581-4231] PVCs per 24 hours; P<.001). A model including 24-hour PVC burden (odds ratio [OR] 1.50 [95% CI, 1.10-2.03]; P=.01), PVC spikes (OR, 6.20 [95 CI, 2.74-13.99]; P<.001), and NSVT (OR, 2.29 [95% CI, 1.10-4.51]; P=.03) in following months. The findings suggested that in patients with ARVC, changes in parameters derived from each Holter examination performed during follow-up were associated with the risk of sustained ventricular arrhythmias within 12 months of disease diagnosis.