Division of Cardiovascular Medicine, Department of Internal Medicine, Japan.
Division of Cardiovascular Surgery, Department of Surgery, Kobe University Graduate School of Medicine, Japan.
Intern Med. 2021 Apr 15;60(8):1225-1229. doi: 10.2169/internalmedicine.6035-20. Epub 2020 Nov 23.
We present an extremely rare case of mitral Ebstein's anomaly that resulted in severe mitral regurgitation (MR). A 41-year-old woman with a history of tuberous sclerosis underwent surgery. Preoperatively, it was assumed that MR had occurred due to leaflet tethering related to left ventricular posterior wall motion asynergy due to a scarred rhabdomyoma. However, surgical inspection revealed a dysplastic posterior leaflet adhering to the ventricular wall, which was completely covered by the endocardium. Both congenital mitral Ebstein's anomaly and acquired wall motion abnormality due to a scarred rhabdomyoma may have contributed to the development of severe MR in this case.
我们呈现了一例极其罕见的二尖瓣 Ebstein 畸形导致重度二尖瓣反流（MR）的病例。一名 41 岁的女性，有结节性硬化症病史，接受了手术治疗。术前，考虑到 MR 是由于左心室后壁运动不同步导致的瓣叶牵拉所致，这是由于瘢痕性横纹肌瘤引起的。然而，手术检查发现一个发育不良的后瓣叶附着在心室内壁上，完全被心内膜覆盖。在这种情况下，先天性二尖瓣 Ebstein 畸形和由于瘢痕性横纹肌瘤引起的心室壁运动异常都可能导致重度 MR 的发生。
