Caruso G, Losekoot T G, Becker A E
Br Heart J. 1978 Nov;40(11):1275-9. doi: 10.1136/hrt.40.11.1275.
This report documents 2 patients who presented with cyanosis early in life. In both instances the necropsy showed the simultaneous occurrence of an imcomplete type of persistent atrioventricular canal and an Ebstein's malformation of the "tricuspid" valve component. In the first patient no clinical investigations were done. In the second patient the electrocardiogram was consistent with persistent atrioventricular canal and the angiocardiogram showed the characteristic goose-neck deformity. Moreover, an abnormality of the right cardiac contour was seen, which in retrospect was correlated with the right-sided Ebstein's malformation. Ebstein's anomaly may significantly alter the clinical and haemodynamic profile of atrioventricular canal and should be considered in atypical cases. The presence of Ebstein's anomaly will complicate surgical repair of the atrioventricular canal.
本报告记录了2例在生命早期出现发绀的患者。在这两个病例中，尸检均显示同时存在不完全型持续性房室通道和“三尖瓣”瓣叶的埃布斯坦畸形。第一例患者未进行临床检查。第二例患者的心电图与持续性房室通道相符，心血管造影显示特征性的鹅颈样畸形。此外，还发现右心轮廓异常，回顾起来这与右侧埃布斯坦畸形相关。埃布斯坦畸形可能会显著改变房室通道的临床和血流动力学特征，在非典型病例中应予以考虑。埃布斯坦畸形的存在会使房室通道的手术修复复杂化。
