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• W2020637525 abstract "Protein-losing enteropathy (PLE) is a serious complication of the Fontan operation and is associated with pronounced mortality. Medical management of PLE has been only partially successful. A recent report noted dramatic improvement in patients with PLE within 3 weeks of subcutaneous administration of heparin. We report a case of reversal of PLE with resolution of clinical symptoms and normalization of serum albumin, total protein, and fecal α1,-antitrypsin values after several months of heparin treatment. Our findings substantiate those recently reported but suggest that reversal of PLE may necessitate more than a few weeks of heparin therapy. Protein-losing enteropathy (PLE) is a serious complication of the Fontan operation and is associated with pronounced mortality. Medical management of PLE has been only partially successful. A recent report noted dramatic improvement in patients with PLE within 3 weeks of subcutaneous administration of heparin. We report a case of reversal of PLE with resolution of clinical symptoms and normalization of serum albumin, total protein, and fecal α1,-antitrypsin values after several months of heparin treatment. Our findings substantiate those recently reported but suggest that reversal of PLE may necessitate more than a few weeks of heparin therapy. Protein-losing enteropathy (PLE) may occur in up to 13.4% of patients within 10 years after the Fontan operation and is associated with a mortality rate of 56% within 5 years of diagnosis.1Feldt RH Drlscoll DJ Offord KP Cha RH Perrault J Schaff HV et al.Protein-losing enteropathy after the Fontan operation.J Thorac Cardiovasc Surg. 1996; 112: 672-680Abstract Full Text Full Text PDF PubMed Scopus (248) Google Scholar Enteric loss of protein after the Fontan operation is believed to be caused by intestinal lymphangiectasia as a result of increased systemic venous pressure.2Rothman A Snyder J Protem-losing enteropathy following the Fontan operation: resolution with prednisone therapy.Am Heart J. 1991; 121: 618-619Abstract Full Text PDF PubMed Scopus (54) Google Scholar Several investigators have reported higher diastolic atrial pressures in patients who have development of PLE than in those who do not,1Feldt RH Drlscoll DJ Offord KP Cha RH Perrault J Schaff HV et al.Protein-losing enteropathy after the Fontan operation.J Thorac Cardiovasc Surg. 1996; 112: 672-680Abstract Full Text Full Text PDF PubMed Scopus (248) Google Scholar, 3Warnas CA Feldt RH Hagier DJ Protein losing enteropathy after Fontan operation: successful treatment by percutaneous fenestration of the atrial septum.Mayo Clin Proc. 1996; 71: 378-379Abstract Full Text Full Text PDF PubMed Scopus (27) Google Scholar Some evidence shows that mechanical relief of obstruction of right atrial flow may result in resolution of PLE.3Warnas CA Feldt RH Hagier DJ Protein losing enteropathy after Fontan operation: successful treatment by percutaneous fenestration of the atrial septum.Mayo Clin Proc. 1996; 71: 378-379Abstract Full Text Full Text PDF PubMed Scopus (27) Google Scholar Medical management of PLE has included diet modification, albumin infusion, diuretics, and corticosteroids, none of which have been entirely successful. Recently, heparin therapy was reported to diminish symptoms of PLE and essentially reverse enteric protein loss within 3 weeks of administration.5Donnelly JP Rosenthal A Castle VP Holmes RD Reversal of protein-losing enteropathy with heparin therapy in three patients with univentricular hearts and Fontan palliation.JPeöiatr. 1997; 130: 474-478Abstract Full Text Full Text PDF PubMed Scopus (104) Google Scholar We describe the successful use of heparin to treat PLE in a patient with massive ascites, pleural effusions, and scrotal edema that had not responded to conventional therapy for 6 years. Interestingly, our patient did not respond to heparin therapy within 3 weeks as did the previously reported patients. Our findings suggest that heparin therapy may need to be continued longer than 3 weeks before reversal of PLE occurs. A 21-year-old man with tricuspid atresia, a ventricular septal defect, and a common atrium underwent a modified Fontan operation (right atrial-to-right ventricular outflow tract anastomosis) at 8 years of age. In 1990, at 14 years of age, the patient had development of PLE. During the next 6½ years, he was hospitalized 11 times for management of massive ascites, pleural effusions, and scrotal edema due to PLE. He was treated with albumin infusions and diuretics; improvement was transient. In August 1996, the patient was treated with heparin, 5,000 U/day subcutaneously, because dietary changes, albumin infusions, and diuretics had been ineffective. In October 1996, the patient again experienced increased edema, ascites, and dyspnea. He was hospitalized and treated with albumin and furosemide. His weight decreased from 70.25 kg to 58 kg. Results of laboratory studies were as follows: total protein, 4.6 g/dL; albumin, 2.6 g/dL; and fecal a^antitrypsin, 195 mg/dL. Despite apparent failure of heparin therapy, it was continued at 5,000 U/day. In February 1997, correspondence with the patient's physician in Norwich (Great Britain) indicated that the patient's weight had remained stable at 58.5 kg. Physical examination showed only mild peripheral edema, no evidence of ascites, and a decrease in the size of the chronic pleural effusion. During the following months, the patient's weight remained stable, and he was free of edema and ascites. On examination at our institution in July 1997, the patient was completely free of edema and ascites. Chest roentgenography revealed mild cardiomegaly (Fig. 1). An electrocardiogram showed left axis deviation, no evidence of left ventricular hypertrophy, and normal sinus rhythm. An echocardiographic study revealed a patent Fontan anastomosis without obstruction, right atrial dilatation without evidence of thrombi, and good left ventricular function with an ejection fraction of 55%. Laboratory studies revealed the following: total protein, 6.0 g/dL; albumin, 3.5 g/dL; prothrombin time, 9.9 seconds (normal), activated partial thromboplastin time, 24 seconds (normal), and a fecal a,-antitrypsin concentration of 42 mg/dL (normal for our laboratory). Previous medical management of PLE has included diuretics; albumin infusions; high-protein, low-fat, and low-salt diets; and corticosteroids. All these regimens have been associated with limited success and have provided only transient relief of clinical symptoms.1Feldt RH Drlscoll DJ Offord KP Cha RH Perrault J Schaff HV et al.Protein-losing enteropathy after the Fontan operation.J Thorac Cardiovasc Surg. 1996; 112: 672-680Abstract Full Text Full Text PDF PubMed Scopus (248) Google Scholar, 2Rothman A Snyder J Protem-losing enteropathy following the Fontan operation: resolution with prednisone therapy.Am Heart J. 1991; 121: 618-619Abstract Full Text PDF PubMed Scopus (54) Google Scholar, 4Hess J Kruizinga K Bijleveld CM Hardjowijono R Eygelaar A Protein-losingenteropalhy after Fontan operation.J Thorac Cardiovasc Surg. 1984; 38: 606-609Google Scholar, 6Rychik J Piccoli DA Barber G Usefulness of corticosteroid therapy for protein-losing enteropathy after the Fontan procedure.AmJCardiol. 1991; 69: 819-821Abstract Full Text PDF Scopus (75) Google Scholar Recent reports have shown resolution of clinical symptoms and normalization of laboratory values after percutaneous fenestration of the atrial septum.3Warnas CA Feldt RH Hagier DJ Protein losing enteropathy after Fontan operation: successful treatment by percutaneous fenestration of the atrial septum.Mayo Clin Proc. 1996; 71: 378-379Abstract Full Text Full Text PDF PubMed Scopus (27) Google Scholar, 7Martens L Dumoulin M Gewillig M Effects of percutaneous fenes tration of the atrial septum on protein-losing enteropathy after the Fontan operation.Br Heart J. 1994; 72: 591-592Crossref PubMed Scopus (52) Google Scholar Although atrial fenestration seems to be effective, it is associated with cyanosis, reduced exercise tolerance, and the risk of paradoxical emboli.7Martens L Dumoulin M Gewillig M Effects of percutaneous fenes tration of the atrial septum on protein-losing enteropathy after the Fontan operation.Br Heart J. 1994; 72: 591-592Crossref PubMed Scopus (52) Google Scholar Fontan take-down is associated with a 75% mortality rate. Cardiac transplantation has resulted in resolution of PLE in some patients. Our experience with heparin in the treatment of PLE supports previous findings but suggests that the onset of resolution of symptoms may be delayed. Little is known about the exact cause of PLE, and even less is known about the mechanism of heparin in reversing PLE. Heparin is a heterogeneous glycosaminoglycan prepared from porcine gut mucosa and bovine lung. Heparin exerts its anticoagulant effects by binding to antithrombin III (AT III), an outcome resulting in a conformational change that allows AT III to inhibit several coagulation enzymes irreversibly. By binding with heparin, the activity of AT is increased about 4,000 times.8Berry BR Nantel S Heparin therapy: current regimens and principles of monitoring.Postgrad Med. 1996 Jun; 99 (69-76): 64-66PubMed Google Scholar Although a plausible theory is that the anticoagulant effect of heparin is involved in PLE reversal, this seems unlikely in light of the subtherapeutic levels administered as well as the normal prothrombin time and activated partial thromboplastin time in our patient and in patients described in a previous study.5Donnelly JP Rosenthal A Castle VP Holmes RD Reversal of protein-losing enteropathy with heparin therapy in three patients with univentricular hearts and Fontan palliation.JPeöiatr. 1997; 130: 474-478Abstract Full Text Full Text PDF PubMed Scopus (104) Google Scholar Interestingly, thromboembolism has been cited as a common occurrence in patients after the Fontan procedure.9Jahangiri M Ross DB Redington AN Lincoln C Shlnebourne EA Thromboembolism after the Fontan procedure and its modifications.Ann Thorac Surg. 1994; 58: 1409-1413Abstract Full Text PDF PubMed Scopus (123) Google Scholar In addition, recent studies have made an association between thromboembolism in patients who have undergone the Fontan procedure and coagulation factor abnormalities, primarily protein C deficiency.10Cromme-Dijkhuis AH Henkens CM Bijleveld CM Hillege HL Bom VJ van der Meer J Coagulation factor abnormalities as possible thrombotic risk factors after Fontan operations.Lancet. 1990; 336: 1087-1090Abstract PubMed Scopus (123) Google Scholar, 11Jahangiri M Shore D Kakkar V Lincoln C Shinebourne EA Coagulation factor abnormalities after the Fontan procedure and its modifications.J Thorac Cardiovasc Surg. 1997; 113: 989-992Abstract Full Text Full Text PDF PubMed Scopus (106) Google Scholar Possibly, the development of PLE after the Fontan operation is related to coagulation factor abnormalities, and heparin, even at subtherapeutic levels, may have a role in reversing the imbalance between procoagulanl and anticoagulant factors. Investigators have speculated that a more likely mechanism is related to the fact that heparin sulfate is a known component of basement membranes including the intestinal mucosa. As exogenous heparin becomes incorporated into endothelial cells, it may have a stabilizing effect on the capillary endothelium, which reduces protein leakage into the extravascular space and gut.5Donnelly JP Rosenthal A Castle VP Holmes RD Reversal of protein-losing enteropathy with heparin therapy in three patients with univentricular hearts and Fontan palliation.JPeöiatr. 1997; 130: 474-478Abstract Full Text Full Text PDF PubMed Scopus (104) Google Scholar In support of this theory, earlier studies of the role of tissue plasminogen activator (TPA), another component of the coagulation pathway, showed that it has a role in directly affecting vascular permeability.12Kondo M Bamba T Hosokawa K Hosoda S Kawai K Masuda M Tissue plasminogen activator in the pathogenesis of protein-losing gastroenteropathy.Gastroenterology. 1976; 70: 1045-1047PubMed Scopus (30) Google Scholar, 13Ritnoff OD Increased vascular permeability induced by human plas-fnin.JExpMed. 1965; 122: 905-921Crossref Scopus (23) Google Scholar Modulation of the activity of TPA was shown to be effective in reversing PLE in some patients.13Ritnoff OD Increased vascular permeability induced by human plas-fnin.JExpMed. 1965; 122: 905-921Crossref Scopus (23) Google Scholar Heparin may affect vascular permeability in a similar manner, or it may have an effect on modulation of TPA, which in turn affects vascular permeability. Further investigation is needed to elucidate the exact mechanism of heparin in the reversal of PLE. The results of heparin therapy in the reversal of PLE in our patient and in previously reported patients indicate that heparin is a viable and relatively low-risk treatment for PLE. The initial success of heparin therapy in our patient must be tempered by the uncertainty of long-term success. Heparin therapy has been tried in only a few patients, and no long-term results are known. Our experience and that at other medical centers indicate that a dosage of 5,000 U/day administered subcutaneously is adequate for reversal of enteric protein loss. On the basis of the success of heparin therapy in our patient and in previously reported patients, it may be worthwhile to conduct a controlled trial of heparin in patients with PLE to provide further support of its efficacy and to establish long-term success." @default.
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• W2020637525 title "Use of Heparin in the Treatment of Protein-Losing Enteropathy After Fontan Operation for Complex Congenital Heart Disease" @default.
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