Open AccessUHL'S ANOMALY AS A RESULT OF PROGRESSION TO VENTRICULAR DILATION FROM HYPERTROPHIC CARDIOMYOPATHY
Author(s) -
Yutani Chikao,
Imakita Masami,
IshibashiUeda Hatsue,
Nagata Seiki,
Sakakibara Hiroshi,
Nimura Yasuharu
Publication year - 1987
Publication title -
acta patholigica japonica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.73
H-Index - 74
ISSN - 0001-6632
DOI - 10.1111/j.1440-1827.1987.tb02269.x
Subject(s) - medicine , cardiology , ventricle , autopsy , hypertrophic cardiomyopathy , cirrhosis , heart failure , fibrosis , cardiac catheterization , restrictive cardiomyopathy , coronary angiography , myocardial fibrosis , cardiomyopathy , myocardial infarction
A 17‐year‐old female with clinically diagnosed Uhl's anomaly died of intractable congestive heart failure, liver cirrhosis, and protein losing gas‐troenteropathy. 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, inparticular, 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.