Resolution of protein-losing enteropathy with standard high molecular heparin and urokinase after Fontan repair in a patient with tricuspid atresia

At 6 years of age, a girl with tricuspid atresia underwent a Bjork modified Fontan procedure with implantation of a Carpentier Edwards bioprosthesis b etween the right atrium and the right ventricle. Ten years later she develo ped increasing edema, ascites and pleural effusions, The work-up showed sev ere stenosis of the bioprosthesis and protein losing enteropathy with a mas sive decrease of the albumin level to 14 g/l (normal: 40-50 g/l). At 17 yea rs of age, the bioprosthesis was replaced with a direct anastomosis between the cavoatrial junction and the right pulmonary artery. Within one month p ostoperatively, extensive thrombosis of the superior vena cava, anonymous a nd subclavian veins occurred, Protein-losing enteropathy persisted with an albumin level of 17 g/l. Parallel to the successful treatment of these thro mbi with high molecular heparin and urokinase, protein losing enteropathy a nd hypoalbuminemia resolved completely as long as the antithrombotic treatm ent with high molecular heparin was continued Oral anticoagulation was inef fective. Chronic antithrombotic treatment with high molecular heparin may t hus be the treatment of choice in these forms of protein-losing enteropathy associated with venous thrombosis.