Yutani C, Imakita M, Ishibashi-Ueda H, Nagata S, Sakakibara H, Nimura Y
Division of Pathology, National Cardiovascular Center, Osaka, Japan.
Acta Pathol Jpn. 1987 Sep;37(9):1477-88. doi: 10.1111/j.1440-1827.1987.tb02269.x.
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.
一名临床诊断为乌尔氏异常的17岁女性死于顽固性充血性心力衰竭、肝硬化和蛋白丢失性胃肠病。心脏导管检查、超声心动图和核血管造影在诊断中被证明是有价值的。尸检时，发现广泛的心肌排列紊乱和双心室广泛纤维化，特别是导致右心室心肌几乎完全缺失。本病例提示，某些乌尔氏异常病例可能与广泛的心肌排列紊乱和广泛纤维化有关，后者被称为特发性心肌病。
