Anderson K R, Lie J T
Am J Cardiol. 1978 Apr;41(4):739-45. doi: 10.1016/0002-9149(78)90826-3.
The typical textbook description of Ebstein's anomaly of the heart usually singles out and emphasizes the downward displacement of septal and posterior leaflets of the tricuspid valve. An anatomic reappraisal of this uncommon anomaly suggests that other structural abnormalities of import should be equally stressed. Among the 15 well preserved autopsy specimens in this series, enlargement of the right atrioventricular (A-V) junction and malalignment of the giant and sometimes muscularized anterior leaflet of the tricuspid valve were consistently found. In addition, massive aneurysmal dilation of the right ventricle was present in almost two thirds (9 of 15) of the hearts. Our observations raise the possibility that abnormal embryonic development of the right A-V junction may be the primary event that leads to malformation of the tricuspid valve apparatus.
典型的教科书对埃布斯坦心脏畸形的描述通常只挑出并强调三尖瓣隔叶和后叶向下移位。对这种罕见畸形进行的解剖学重新评估表明，其他重要的结构异常也应同样予以强调。在本系列15个保存完好的尸检标本中，始终发现右房室交界处扩大以及三尖瓣巨大且有时呈肌性化的前叶排列不齐。此外，几乎三分之二（15个中的9个）的心脏存在右心室巨大动脉瘤样扩张。我们的观察结果提示，右房室交界处异常的胚胎发育可能是导致三尖瓣装置畸形的主要事件。
