Das M, Carroll S F
Surgery. 1985 Feb;97(2):242-6.
This report documents a unique case of Budd-Chiari syndrome associated with antithrombin III deficiency and massive thrombus in the superior vena cava and right atrium. This aberration of the coagulation mechanism is proposed as an etiologic factor in the pathogenesis of hepatic venous obstruction whenever the cause is obscure. Antithrombin III was restored to a level adequate to permit thrombus extraction with cardiopulmonary bypass and relief of portal hypertension with a mesoatrial shunt. The protocol for reversing this hypercoagulable state with fresh frozen plasma and warfarin (Coumadin) is discussed, and technical innovations employed in the management of this complex problem are described.
本报告记录了一例独特的布加综合征病例,该病例与抗凝血酶III缺乏以及上腔静脉和右心房内的大量血栓形成相关。当肝静脉阻塞病因不明时,这种凝血机制异常被认为是其发病机制中的一个病因。通过体外循环使抗凝血酶III恢复到足以允许取出血栓的水平,并通过中房分流术缓解门静脉高压。讨论了使用新鲜冰冻血浆和华法林(香豆素)逆转这种高凝状态的方案,并描述了处理这一复杂问题所采用的技术创新。