Güler Niyazi, Demirbag Recep, Eryonucu Beyhan, Gül Abdulaziz
Department of Cardiology, Yuzuncu Yil University, Yuksek Ihtisas Hospital, 65200, Van, Turkey.
Int J Cardiol. 2003 Feb;87(2-3):283-5. doi: 10.1016/s0167-5273(02)00306-6.
Uhl's anomaly, or parchment right ventricle is a myocardial disorder of unknown cause that mainly involves the right ventricle. Uhl's anomaly may represent a cause of right heart dilatation, failure, and premature sudden death due to ventricular arrhythmias. Although most of the cases of Uhl's anomaly end fatally in infancy or childhood, a limited number of cases have been reported in advanced ages. Also, in pregnant women, this situation increases the risk to both mother and baby and requires special management. This is the first report of six successful consecutive gestations and vaginal deliveries without special managements in a patient with Uhl's anomaly.
乌尔氏异常，即羊皮纸样右心室，是一种病因不明的心肌疾病，主要累及右心室。乌尔氏异常可能是右心扩张、衰竭以及因室性心律失常导致过早猝死的一个原因。尽管大多数乌尔氏异常病例在婴儿期或儿童期以死亡告终，但也有少数高龄病例的报道。此外，对于孕妇，这种情况会增加母婴双方的风险，需要特殊处理。本文首次报道了一例患有乌尔氏异常的患者连续成功妊娠并顺产六次，且未进行特殊处理的病例。
