Emmanoulides G C, Thanopoulos B, Siassi B, Fishbein M
Am J Cardiol. 1976 Mar 4;37(3):403-9. doi: 10.1016/0002-9149(76)90290-3.
Four infants with the severe form of the syndrome of tetralogy of Fallot and absent pulmonary valve are described. All had severe respiratory distress due to bronchial compression from massively dilated pulmonary arteries. The clinical diagnosis was confirmed by cardiac catheterization and angiocardiography. The infants died at age 3, 5, 30 and 90 days, respectively. Autopsy revealed, in addition to the usual components of the syndrome, absence of the ductus arteriosus or its remnants. The possible relation of "agenesis" of the ductus arteriosus to the pathogenesis of the severe form of this syndrome is discussed and pertinent studies are reviewed.
本文描述了4例患有重症法洛四联症及肺动脉瓣缺如综合征的婴儿。所有患儿均因极度扩张的肺动脉压迫支气管而出现严重的呼吸窘迫。心脏导管检查和心血管造影证实了临床诊断。这些婴儿分别于3天、5天、30天和90天时死亡。尸检发现，除了该综合征常见的组成部分外，动脉导管或其残余部分缺如。本文讨论了动脉导管“发育不全”与该综合征重症形式发病机制之间的可能关系，并对相关研究进行了综述。
