Dilated right ventricular cardiomyopathy: Uhl's disease

Uhl's disease is a rare disorder originally described in 1952 in an infant with severe diffuse right ventricular dysfunction with total absence of the myocardium. Uhl considered the disease to be congenital in origin. We report a patient with severe dilated congestive cardiomyopathy limited to the right ventricle but apparently developing in adulthood.