BACKGROUND: Arrhythmogenic right ventricular (RV) cardiomyopathy (ARVC) is a regional disease of the RV myocardium with variable degrees of left ventricular involvement. Three-dimensional echocardiography and Doppler tissue imaging (DTI) are new echocardiographic modalities for the evaluation of global and regional function, but the diagnostic potential remains to be assessed. METHODS: Twenty patients with previously established ARVC were evaluated by 3-dimensional echocardiography and DTI, and compared with 32 age- and sex-matched control subjects. RESULTS: Using 3-dimensional echocardiography, patients with ARVC had a decreased RV ejection fraction (0.47 +/- 0.08 vs 0.53 +/- 0.05, P < .01), and a decreased peak lateral systolic annular velocity by pulsed wave imaging of both the RV (11.9 +/- 2.6 vs 15.1 +/- 3.7 cm/s, P < .01) and the left ventricle (7.0 +/- 2.6 vs 9.5 +/- 1.9 cm/s, P < .01). DTI showed decreased regional systolic strain, but with wide variation in the measurements. CONCLUSION: Three-dimensional echocardiography identifies decreased RV ejection fraction in ARVC. Assessment of regional contractility by DTI is limited by wide variation. Echocardiographic evaluation of the longitudinal motility appears to be a sensitive marker of preclinical left ventricular involvement.