Nonaka Y, Mori M, Sugishita Y, Ishii M, Koide T
Jpn Heart J. 1976 Jan;17(1):114-22. doi: 10.1536/ihj.17.114.
Dyspnea, back pain, edema, and cyanosis developed suddenly in a 23-year-old woman during the last trimester of her first pregnancy. Although she had been noticed to have the enlarged heart and exertional shortness of breath to a slight degree, she had been apparently in good condition without any significant heart murmurs. Clinically, recurrent episodes of disseminated intravascular coagulation, including pulmonary thrombosis, were thought to be superimposed to Eisenmenger syndrome associated with toxemia of pregnancy. Anticoagulant and fibrinolytic treatments were tried, but their effectiveness was limited by hemorrhagic diathesis. She died of respiratory and circulatory failure after delivery of a moribund baby. Autopsy revealed Eisenmenger complex (a defect in the membranous portion of the interventricular septum and pulmonary vascular disease) and many fresh hemmorrhages in both lungs with a lot of new and organized thrombi. Fresh thrombi were also seen in the heart, the pancreas and the kidneys. The high peripartal mortality in Eisenmenger syndrome could be attributed to pulmonary thrombosis, which may be related to DIC, as well as to peripartal changes in circulatory function.
一名23岁女性在首次怀孕的最后三个月突然出现呼吸困难、背痛、水肿和发绀。尽管此前发现她心脏轻度扩大且运动时稍有气短，但她此前状况良好，未闻及明显心脏杂音。临床上，包括肺血栓形成在内的反复弥散性血管内凝血发作被认为叠加于与妊娠中毒相关的艾森曼格综合征。尝试了抗凝和纤溶治疗，但因出血素质其效果有限。她在产下一名濒死婴儿后死于呼吸和循环衰竭。尸检发现艾森曼格综合征（室间隔膜部缺损和肺血管疾病）以及双肺多处新鲜出血伴大量新形成和机化的血栓。心脏、胰腺和肾脏也可见新鲜血栓。艾森曼格综合征围产期死亡率高可能归因于肺血栓形成，其可能与弥散性血管内凝血有关，也与围产期循环功能变化有关。
