Abstract: Objective To investigate the echocardiographic features of cardiomyopathies with predominant right ventricular involvement. Methods Echocardiography was used for diagnosis of cardiomyopathies with predominant right ventricular involvement in 13 cases. Results Obvious right atrial enlargement was observed in 11 patients, and all the 13 patients had tricuspid regurgitation of different degrees with normal or basically normal left ventricular size. One patient had mild left ventricular enlargement. Echocardiography demonstrated in these cases characteristic abnormalities of right ventricular cardiomyopathy, classified into two types based on the echocardiographic features of the right ventricular dilation or apical obliteration. Eight patients had pronounced right ventricular dilatation and hypokinesia with right ventricular wall thinning, evident echo enhancement and irregularity of the moderator band and right ventricular papillary muscles, as well as prominent trabeculations. In the other 5 patients, obliterative changes were found in the apical trabecular region of the right ventricle, presenting deflated and deformed right ventricle with shrinkage or obliteration of the apical trabecular region, increased right ventricular wall thickness producing echo enhancement, but the pericardial thickness remained normal with normal or dilated right ventricular inflow and outflow tracts. Conclusion The echocardiographic features of cardiomyopathies with predominant right ventricular involvement can be classified into two types, one of which is characterized by obviously dilated right ventricle with wall thinning and hypokinesia, and the other by obliterative changes in the apical trabecular region of the right ventricle with deformed right ventricular cavity, stiffened and thickened right ventricular wall with echo enhancement and normal pericardium. The echocardiographic characteristics are useful for the diagnosis of this disease.