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Background Arrhythmogenic right ventricular (RV) cardiomyopathy is a progressive disease characterized by the replacement of the normal myocardium with fibrofatty tissue. This study aimed to determine the value of echocardiographic RV deformation parameters in predicting electrical progression as assessed by serial changes in RV lead sensing and threshold in patients with arrhythmogenic RV cardiomyopathy. Methods The present study recruited 40 patients with a definitive diagnosis of arrhythmogenic RV cardiomyopathy at a mean (SD) age of 38.6 (14.2) years between 2018 and 2020. All patients had received an implantable cardioverter-defibrillator for the primary or secondary prevention of sudden cardiac death. The patients underwent 2-dimensional (2D) and 3-dimensional (3D) transthoracic echocardiographic examinations and RV 2D and 3D strain analyses, comprising free-wall longitudinal strain, global longitudinal strain, and strain rate. They were then followed up for electrical progression. Results During a mean (SD) follow-up period of 20 (6) months, the RV lead amplitude decreased from 7.95 (IQR, 4.53-10.25) mV to 5.25 (IQR, 2.88-8.55) mV (P < .001), and the lead threshold increased from 0.75 (IQR, 0.50-0.79) V to 0.75 (IQR, 0.75-1.00) V (P < .001). Right ventricular 2D free-wall (ρ = 0.56, P = .01), RV 2D global (ρ = 0.58, P = .007), and RV 3D free-wall (ρ = 0.65; P = .003) longitudinal strain correlated with electrical progression. Conclusion Right ventricular 2D and 3D deformation parameters were found to be significant predictors of electrical progression during follow-up of patients with arrhythmogenic RV cardiomyopathy. These findings suggest that echocardiography has a pivotal role in predicting patients at high risk for electrical progression.