Gerlis L M, Schmidt-Ott S C, Ho S Y, Anderson R H
Department of Paediatrics, National Heart and Lung Institute, London.
Br Heart J. 1993 Feb;69(2):142-50. doi: 10.1136/hrt.69.2.142.
Since 1905 there have been many reports of cases in which the right ventricle was deficient in myocardium. Several terms have been used to describe this condition. Of these, "Uhl's anomaly" and "arrhythmogenic right ventricular dysplasia" are most often used. Our study investigates the relation between these entities.
Five cases with a primary deficiency of the right ventricular musculature were examined. The findings were compared with those published reports to evaluate the similarities and differences between Uhl's anomaly and arrhythmogenic dysplasia.
The five cases showed two patterns of myocardial deficiency in the right ventricle. On the one hand, the parietal wall was paper thin with complete absence of musculature and apposition of the endocardial and epicardial layers. On the other hand, patchy, localised fibrofatty tissue replacement was found within the parietal musculature. Evidence from our cases, combined with analysis of other publications, showed different modes and timing of clinical presentation of the patients with these two anatomical conditions, congestive heart failure or arrhythmia.
The conditions variously described as Uhl's anomaly and arrhythmogenic dysplasia are separate and distinct morphological entities.
自1905年以来，已有许多关于右心室心肌发育不全病例的报道。人们使用了多个术语来描述这种情况。其中，“乌尔氏异常”和“致心律失常性右心室发育不良”最为常用。我们的研究调查了这些实体之间的关系。
对5例原发性右心室肌肉组织发育不全的病例进行了检查。将检查结果与已发表的报告进行比较，以评估乌尔氏异常和致心律失常性发育不良之间的异同。
这5例病例显示出右心室心肌发育不全的两种模式。一方面，室壁薄如纸张，完全没有肌肉组织，心内膜和心外膜层紧贴在一起。另一方面，在室壁肌肉组织内发现了散在的、局部的纤维脂肪组织替代。我们病例的证据，结合对其他出版物的分析，显示这两种解剖状况的患者出现充血性心力衰竭或心律失常的临床表现方式和时间不同。
被不同描述为乌尔氏异常和致心律失常性发育不良的情况是不同的形态学实体。
