title: Resolution of protein-losing enteropathy with standard high molecular heparin and urokinase after Fontan repair in a patient with tricuspid atresia.
abstract: 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.
authors.affiliation: Department of Internal Medicine, University Hospital, Zürich, Switzerland.
authors.forename: M
authors.initials: M
authors.lastname: Facchini
authors.forename: I
authors.initials: I
authors.lastname: Guldenschuh
authors.forename: J
authors.initials: J
authors.lastname: Turina
authors.forename: R
authors.initials: R
authors.lastname: Jenni
authors.forename: C
authors.initials: C
authors.lastname: Schalcher
authors.forename: C H
authors.initials: CH
authors.lastname: Attenhofer Jost
mesh_terms: D002648:Child; D005260:Female; D005343:Fibrinolytic Agents / Q000627:therapeutic use*; D018729:Fontan Procedure; D006493:Heparin / Q000627:therapeutic use*; D006801:Humans; D008970:Molecular Weight; D010960:Plasminogen Activators / Q000627:therapeutic use*; D011183:Postoperative Complications / Q000188:drug therapy*; D011504:Protein-Losing Enteropathies / Q000188:drug therapy* / Q000209:etiology; D018785:Tricuspid Atresia / Q000601:surgery; D014568:Urokinase-Type Plasminogen Activator / Q000627:therapeutic use*; D020246:Venous Thrombosis / Q000150:complications / Q000188:drug therapy*
chemical_list: D005343:Fibrinolytic Agents; D006493:Heparin; D010960:Plasminogen Activators; D014568:Urokinase-Type Plasminogen Activator