Graham T P, Smith C W
Cathet Cardiovasc Diagn. 1977;3(4):397-407. doi: 10.1002/ccd.1810030410.
A previously unreported cardiovascular malformation is presented consisting of aneurysmal dilatation of the right ventricular outflow tract associated with a cardiomyopathy, a possible variant of Uhl's anomaly. The patient presented at 8 days of age with mild cyanosis and congestive failure. Cardiac catheterization revealed severe cardiomegaly associated with very poor pump function of both right and left ventricles. The present case is unique in showing 1) severe aneurysmal dilatation of the right ventricular outflow tract. 2) only partially absent right ventricular musculature documented before death by the pressure tracing and cineangiocardiograms, and 3) the first quantitation of both right and left ventricular size and function in this syndrome. A review of previous cases of Uhl's anomaly indicates that this case may represent a variant of that condition and that intrauterine myocarditis or myocardial infarct should be considered in regard to etiology.
本文报道了一种此前未报告的心血管畸形，其特征为右心室流出道动脉瘤样扩张并伴有心肌病，可能是乌尔氏畸形的一种变体。该患者8日龄时出现轻度紫绀和充血性心力衰竭。心导管检查显示心脏严重扩大，左右心室泵功能极差。本病例的独特之处在于：1）右心室流出道严重动脉瘤样扩张；2）生前通过压力描记和心血管造影记录仅部分右心室肌组织缺失；3）该综合征中首次对左右心室大小和功能进行定量分析。回顾先前的乌尔氏畸形病例表明，本病例可能代表该疾病的一种变体，病因方面应考虑宫内心肌炎或心肌梗死。
