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The correlation between parameters of two-dimensional echocardiography and signal-averaged ECG (SAECG) in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) is not known well.Thirty-three patients (13 females, 40.3 ± 14.4 years old) were included in this study. Both the right and left ventricular dimensions and systolic function were assessed with two-dimensional echocardiography. The SAECG was performed with high-gain amplification and filtered using bidirectional Butterworth filters between 40 and 250 Hz. We evaluated the correlation between the parameters of the SAECG and two-dimensional echocardiography.The right ventricular (RV) outflow tract was the most frequently (n = 18, 54%) involved segment. Six (18%) patients had only mildly decreased RV systolic function. All the other patients had normal RV systolic function. Although localized left ventricular wall motion abnormalities were observed in 14 (42%) patients, the left ventricular ejection fraction was normal in most (n = 32, 97%). Late potentials were positive in 22 (63%) patients. There was no significant correlation between parameters of the SAECG and two-dimensional echocardiography for the entire patient population.The SAECG parameters exhibited no correlation to any of two-dimensional echocardiography parameters in the patients with ARVC. Fragmented electrical activity may develop with no significant relation to the anatomical changes in the patients with ARVC.