Suppr超能文献

右心室心肌致密化不全/肥厚型与Ebstein 畸形合并存在。

A combination of right ventricular hypertrabeculation/noncompaction and Ebstein's anomaly.

出版信息

Int J Cardiol. 2010 Aug 20;143(2):e30-3. doi: 10.1016/j.ijcard.2008.12.024. Epub 2009 Jan 10.

Abstract

A 35-year-old man admitted with iterative extremities edema for three months and mild chest complaint associated with activity without chest distress for five days. He underwent an initial 2DE that did not confirm HVM/NVM and 3DE that strengthened the diagnosis of HVM/NVM. The limitations of 2DE may lead to misinterpretation of prominent trabeculations as "false tendons," and may also lead to underestimation of the severity of HVM/NVM. In contrast to 2DE, 3DE provides for pyramid-shaped datasets that encompass the entire right ventricular. Specifically, right ventricular can be sectioned in userselected planes and an unlimited number and angles of such planes can be used. Intracavitary echodensities that are suspicious for trabeculations can be tracked in multiple directions from base to apex. To our knowledge, this is the first report on only right ventricular HVM/NVM associated with Ebstein's malformation. The leaflets of the tricuspid valve develop from endocardial cushion tissue and the myocardium. The tricuspid valve leaflets become freely movable after delamination of the tension apparatus within the myocardium. Although the coexistence of right ventricular HVM/NVM and Ebstein's malformation could be a coincidence, I believe that both defects could be caused by a developmental arrest of the right ventricular myocardium. That is to say, the genetic change that is responsible for Ebstein's anomaly also plays a role in the differentiation of the right myocardium.

摘要

一位 35 岁男性,因反复四肢水肿 3 个月,伴轻度活动后胸闷 5 天入院。患者初始行二维超声心动图(2DE)检查未证实肥厚型室壁心肌疾病(HVM)/非对称性室间隔肥厚(NVM),后行三维超声心动图(3DE)检查,进一步证实 HVM/NVM。2DE 可能导致对明显的心肌小梁的错误解释为“假性腱索”,也可能导致 HVM/NVM 严重程度的低估。与 2DE 相比,3DE 提供了涵盖整个右心室的金字塔形数据集。具体来说,可以在用户选择的平面上对右心室进行分段,并且可以使用无限数量和角度的此类平面。可疑为小梁的心腔内回声密度可以从基底到心尖进行多方向追踪。据我们所知,这是首例仅与 Ebstein 畸形相关的右心室 HVM/NVM 的报告。三尖瓣的瓣叶由心内膜垫组织和心肌发育而来。心肌内张力装置分层后,三尖瓣瓣叶变得可以自由活动。虽然右心室 HVM/NVM 和 Ebstein 畸形的并存可能是巧合,但我认为这两种缺陷可能是由右心室心肌发育停滞引起的。也就是说,导致 Ebstein 异常的基因变化也可能在右心肌分化中起作用。

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型,支持多种主流文档格式。

立即体验