Facchini M, Guldenschuh I, Turina J, Jenni R, Schalcher C, Attenhofer Jost C H
Department of Internal Medicine, University Hospital, Zürich, Switzerland.
J Cardiovasc Surg (Torino). 2000 Aug;41(4):567-70.
At 6 years of age, a girl with tricuspid atresia underwent a Björk modified Fontan procedure with implantation of a Carpentier Edwards bioprosthesis between the right atrium and the right ventricle. Ten years later she developed increasing edema, ascites and pleural effusions. The work-up showed severe stenosis of the bioprosthesis and protein losing enteropathy with a massive decrease of the albumin level to 14 g/l (normal 40-50 g/l). At 17 years of age, the bioprosthesis was replaced with a direct anastomosis between the cavoatrial junction and the right pulmonary artery. Within one month post-operatively, extensive thrombosis of the superior vena cava, anonymous and subclavian veins occurred. Protein-losing enteropathy persisted with an albumin level of 17 g/l. Parallel to the successful treatment of these thrombi with high molecular heparin and urokinase, protein losing enteropathy and hypoalbuminemia resolved completely as long as the antithrombotic treatment with high molecular heparin was continued. Oral anticoagulation was ineffective. Chronic antithrombotic treatment with high molecular heparin may thus be the treatment of choice in these forms of protein-losing enteropathy associated with venous thrombosis.
一名患有三尖瓣闭锁的6岁女孩接受了改良的Björk Fontan手术，在右心房和右心室之间植入了一个Carpentier Edwards生物假体。十年后，她出现了水肿、腹水和胸腔积液加重的情况。检查显示生物假体严重狭窄，伴有蛋白丢失性肠病，白蛋白水平大幅降至14 g/l（正常为40 - 50 g/l）。17岁时，将生物假体更换为腔房交界处与右肺动脉之间的直接吻合术。术后一个月内，上腔静脉、无名静脉和锁骨下静脉发生广泛血栓形成。蛋白丢失性肠病持续存在，白蛋白水平为17 g/l。在使用高分子肝素和尿激酶成功治疗这些血栓的同时，只要继续使用高分子肝素进行抗血栓治疗，蛋白丢失性肠病和低白蛋白血症就完全缓解。口服抗凝治疗无效。因此，对于这些与静脉血栓形成相关的蛋白丢失性肠病形式，长期使用高分子肝素进行抗血栓治疗可能是首选治疗方法。
