Gerlis L M, Ho S Y, Sweeney A E
Department of Paediatrics, National Heart & Lung Hospital, London, England, U.K.
Am J Cardiovasc Pathol. 1993;4(4):294-301.
The original description of Ebstein's anomaly mentioned a mild deformity of the mitral valve. Reviews of large series of cases with Ebstein's malformation have, however, tended to omit reference to accompanying mitral valve malformations. We have compared the mitral valves in 19 specimens with Ebstein's malformation with those in 30 age-matched controls in order to determine the prevalence and nature of these anomalies. In the Ebstein's group the mitral valve was abnormal in 14 hearts with orificial anomalies in four, leaflet anomalies in 11, cord anomalies in 11 and papillary muscle anomalies in 13. In the control group, five cases showed mild shortening of the tendinous cords. Half of the changes found in the Ebstein's group were of moderate or significant degree suggesting a primary involvement of the mitral valve. In time, dynamic alterations within the left ventricle might become superimposed and contribute to the development of clinically significant lesions.
埃布斯坦畸形（Ebstein's anomaly）最初的描述提到二尖瓣有轻度畸形。然而，对大量埃布斯坦畸形病例系列的回顾往往忽略了伴随的二尖瓣畸形。我们将19例埃布斯坦畸形标本的二尖瓣与30例年龄匹配的对照者的二尖瓣进行了比较，以确定这些异常的发生率和性质。在埃布斯坦畸形组中，14例心脏的二尖瓣异常，其中4例有瓣口异常，11例有瓣叶异常，11例有腱索异常，13例有乳头肌异常。在对照组中，5例显示腱索轻度缩短。在埃布斯坦畸形组中发现的改变有一半为中度或重度，提示二尖瓣原发性受累。随着时间的推移，左心室内的动态改变可能会叠加，促使具有临床意义的病变发展。
