Perspective:

Risk stratification for sudden cardiac death or need for implantable cardioverter-defibrillator (ICD) therapy has been difficult in patients with ARVC and no prior sustained ventricular tachycardia. Recently, a risk calculator based on seven clinical factors has been developed. Those clinical factors include age at diagnosis, gender, occurrence of cardiac syncope in the preceding 6 months, number of inverted T waves on 12-lead electrocardiography, maximum premature ventricular contraction count over 24 hours, nonsustained ventricular tachycardia, and right ventricular ejection fraction (https://www.ARVCrisk.com/). The role of programmed ventricular stimulation for arrhythmic risk stratification in primary prevention has been uncertain. The current manuscript, which is based on a pooled population from six international registries, offers evidence of incremental benefit from the programmed ventricular stimulation. Using programmed ventricular stimulation can improve risk prediction, particularly among those with low or intermediate predicted risk based on the clinical risk calculator. If negative, its high negative predictive value (93%) in low and intermediate risk patients may support the decision to forgo ICD use in some patients.