Echocardiographic features of right ventricular dilated cardiomyopathy and Uhl's anomaly.

The echocardiographic features of six patients with isolated right ventricular dilated cardiomyopathy and two with Uhl's anomaly are described. The M-mode echocardiogram was similar in both groups showing increased right ventricular dimensions and normal or paradoxical septal motion. Cross-sectional echocardiography confirmed a dilated but poorly contracting right ventricle. However, patients with right ventricular cardiomyopathy had normal ventricular wall thickness, whereas those with Uhl's anomaly showed areas of thin right ventricular myocardium. These areas of the right ventricle also exhibited regional wall motion abnormalities. In conclusion, cross-sectional echocardiography can be used to differentiate between these two forms of right ventricular enlargement.