Singh Gurkirat, Khemani Hemant, Shaikh Shakil, Bade Arun, Singla Rahul, Bansal Narender Omprakash
Department of Cardiology, Grant Medical College and Sir J.J. Group of Hospitals, Mumbai, India.
Cardiol Res. 2019 Apr;10(2):124-127. doi: 10.14740/cr758w. Epub 2019 Apr 11.
Uhl's anomaly is an extremely rare congenital cardiac malformation and is characterized by the partial or complete absence of right ventricular myocardium. The absence of myocardium may be the result of primary non-development of myocytes or a form of selective apoptosis. It is mainly sporadic although some familial occurrences have been reported. Congestive cardiac failure is the most common mode of presentation. Associated congenital cardiac malformations are also reported. We report a case of a 17-year-old male who presented with symptoms and signs of right heart failure, during evaluation found to have large right ventricle free wall thrombus.
乌尔氏异常是一种极其罕见的先天性心脏畸形，其特征是右心室心肌部分或完全缺失。心肌缺失可能是心肌细胞原发性未发育或一种选择性凋亡的形式所致。虽然有一些家族性发病的报道，但主要为散发性。充血性心力衰竭是最常见的表现形式。也有相关先天性心脏畸形的报道。我们报告一例17岁男性患者，其表现出右心衰竭的症状和体征，在评估过程中发现有巨大的右心室游离壁血栓。
