Uhl's anomaly as a result of progression to ventricular dilation from hypertrophic cardiomyopathy.

A 17-year-old female with clinically diagnosed Uhl's anomaly died of intractable congestive heart failure, liver cirrhosis, and protein losing gastroenteropathy. Cardiac catheterization, echocardiography and nuclear angiography proved to be valuable in making the diagnosis. At autopsy, there was widespread myocardial disarray and extensive fibrosis of both ventricles which led to, in particular, almost total absence of the myocardium of the right ventricle. The present case implicated that certain case of Uhl's anomaly might be related to widespread myocardial disarray and extensive fibrosis, which is known as idiopathic cardiomyopathy.