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双胞胎中孤立性先天性二尖瓣狭窄的病理解剖特征(作者译)

[Pathoanatomical features of isolated congenital mitral stenosis in twins (author's transl)].

作者信息

Schwarze E W

出版信息

Virchows Arch A Pathol Anat Histol. 1975;366(1):75-85. doi: 10.1007/BF00438680.

Abstract

The pathoanatomical features of isolated congenital mitral stenosis in female twins are presented. At the age of 7 months one of the infants underwent an emergency mitral valvotomy and died the next day due to mitral insufficiency. A total mitral valvectomy and an implantation of a Björk-Shiley prosthesis were performed on the second child at the age of 27 months. Death occurred 6 months later due to malfunction of the prosthesis caused by a circular fibrous endocardial bulge on both the atrial and ventricular side of the prosthesis. The bulging endocardial fibrosis may have been favored by a local thrombosis, which was found circularly around the bed of the prosthesis after it was removed. Since the child suffered from postoperative serum hepatitis, anticoagulants could not be applied on a regular basis in sufficient doses. The relation of the endocardial fibroelastosis to the congenital valve disease and the implantation of a prosthesis is discussed. The site of the postoperative valve in the first child and the state of the second child after implantation of the mitral valve prosthesis are discussed in detail.

摘要

本文介绍了女性双胞胎中孤立性先天性二尖瓣狭窄的病理解剖特征。其中一名婴儿在7个月大时接受了急诊二尖瓣切开术,次日因二尖瓣关闭不全死亡。第二名儿童在27个月大时接受了全二尖瓣切除术并植入了Björk-Shiley人工瓣膜。6个月后,由于人工瓣膜心房和心室侧出现环形纤维性心内膜膨出导致人工瓣膜功能障碍而死亡。取出人工瓣膜后发现其周围有环形血栓,这种膨出的心内膜纤维化可能因局部血栓形成而加重。由于该儿童患有术后血清性肝炎,无法定期足量应用抗凝剂。文中讨论了心内膜弹力纤维增生症与先天性瓣膜疾病及人工瓣膜植入的关系。详细讨论了第一名儿童术后瓣膜的位置以及第二名儿童植入二尖瓣人工瓣膜后的情况。

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