Sigrist T, Bär W, Zink P
Gerichtsmedizinisches Institut am Kantonsspital, St. Gallen, Schweiz.
Z Rechtsmed. 1988;100(2-3):176-89. doi: 10.1007/BF00200758.
Partial absence and fatty replacement of the myocardial compacta of the right ventricular wall was observed in five young male adults, four of whom died suddenly and unexpectedly and one in a car accident. In one of these cases there was only one coronary orifice. Four cases of the so-called Uhl anomaly had histological findings indicating dysmorphic myocardial cells associated with minimal lymphohistiocytic infiltrates within the fatty tissue. In one case, chronic destructive inflammation was a prominent feature. Marked dysmorphic alterations in the myocardial fibers suggested the occurrence of arrhythmic episodes as the main cause of sudden heart failure and death.
在5名年轻男性成年人中观察到右心室壁心肌致密层部分缺失和脂肪替代，其中4人突然意外死亡，1人死于车祸。在这些病例中的1例中，仅存在一个冠状动脉口。4例所谓的乌尔异常病例的组织学结果显示心肌细胞发育异常，脂肪组织内有少量淋巴细胞组织细胞浸润。在1例中，慢性破坏性炎症是一个突出特征。心肌纤维的明显发育异常改变提示心律失常发作是导致猝死和心力衰竭的主要原因。
