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Ann Thorac Surg 1996;61:206-208
© 1996 The Society of Thoracic Surgeons

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Case Report

Protein-Losing Enteropathy After Fontan Operation: Resolution After Baffle Fenestration

Divisions of Cardiothoracic Surgery and Cardiology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania

Accepted for publication July 5, 1995.

	Abstract

Top Footnotes Abstract Introduction Case Reports Comment References

 
A 4-year-old child with hypoplastic left heart syndrome and a 6-year-old child with tricuspid atresia had both undergone staged reconstructive operations culminating in a Fontan operation. Peripheral edema, ascites, and hypoalbuminemia refractory to dietary manipulation and steroid therapy developed in both patients. After hemodynamic assessment, each child underwent surgical creation of a 4.8-mm fenestration in the previously placed baffle that separated the systemic venous pathway from the pulmonary venous atrium. Peripheral edema and ascites promptly resolved and serum protein levels normalized within 2 weeks after operation. Systemic arterial saturation is 86% in each child, and both children remain clinically well with no evidence of protein-losing enteropathy on normal diets and without specific medical therapy.

	Introduction

Top Footnotes Abstract Introduction Case Reports Comment References

 
Protein-losing enteropathy has been reported to occur in 5% to 10% of patients after the Fontan operation [1]. Although abnormal loss of protein from the gut is believed to be related to chronic elevation of venous pressure and subsequent impaired lymphatic drainage from the gastrointestinal tract, it is not seen only in those patients who have the most elevated right-sided pressures after a Fontan operation. Overt manifestations of protein-losing enteropathy include ascites, edema, and hypoalbuminemia, but the syndrome can include immunodeficiency, hyponatremia, and hypocalcemia. At the more severe end of the spectrum, protein-losing enteropathy is a significantly morbid condition that is difficult to manage [2]. Dietary manipulation, albumin replacement, and steroid therapy constitute the principal elements of medical management [3]. Failure of medical therapy has in general led to either death or abandonment of the Fontan physiology (takedown or heart transplantation) [4]. We have managed 2 patients with chronic protein-losing enteropathy refractory to medical therapy by surgical creation of a fenestration in the previously placed atrial baffle, and have observed rapid and gratifying responses consisting of resolution of ascites and edema and normalization of serum protein levels.

	Case Reports

Top Footnotes Abstract Introduction Case Reports Comment References

 
Patient 1
Patient 1, born January 16, 1990, presented on day 3 of life with low cardiac output and metabolic acidosis. She was recognized as having hypoplastic left heart syndrome and was transferred to the Children's Hospital of Philadelphia, where a first-stage Norwood procedure was performed. At 7 months of age, a hemi-Fontan procedure was performed. At 13 months of age, pulmonary artery pressure was 9 mm Hg, atrial pressure was 5 mm Hg, and pulmonary to systemic blood flow ratio was 0.7:1. No filling of the left pulmonary artery was noted. At 16 months of age, she underwent a modified Fontan procedure with partial exclusion of hepatic veins, and with reconstruction of the left pulmonary artery. Months after the completion Fontan procedure, progressive cyanosis was evident. Cardiac catheterization 1 year after the Fontan procedure revealed systemic arterial desaturation (79%) due to the presence of intrahepatic venous collaterals resulting in a significant fraction of the systemic venous return bypassing the lungs. Again, the left pulmonary artery was noted to be occluded. Mean pulmonary artery pressure was 13 mm Hg, and ventricular end-diastolic pressure was 9 mm Hg.

In April 1993, at 4 years of age, she underwent repositioning of the lower end of the systemic venous baffle to incorporate all of the hepatic veins into the systemic venous pathway. Patch angioplasty of the left pulmonary artery was performed. Postoperatively, arterial oxygen saturation stabilized at greater than 90%. A right-sided pleural effusion persisted for several weeks and gradually resolved. One year after this surgical revision, she presented with signs and symptoms of protein losing enteropathy including ascites, peripheral edema, and hypoalbuminemia (2.4 g/dL). There was minimal response to diuretics and to dietary protein supplementation. Oral steroids were administered and some improvement was noted, but each attempt at weaning the steroid dose from 2 mg•kg^-1•day^-1 was associated with worsening ascites, peripheral edema, and hypoalbuminemia. Catheterization revealed pressure in the systemic veins and right pulmonary artery of 17 mm Hg and right ventricular end-diastolic pressure of 11 mm Hg. Ventricular systolic function appeared to be satisfactory, and no tricuspid regurgitation was present. Oximetry revealed saturations of 76% in the superior vena cava, 74% in the inferior vena cava, and 93% in the descending aorta. No right-to-left shunts were noted, and as before, all systemic venous return was to the right lung. Serum chemistry values included a total protein level of 4.4 g/dL and albumin level of 2.5 g/dL. An immunoglobulin profile revealed a low immunoglobulin G level of 128 mg/dL (normal, 463 to 1,236 mg/dL), with a normal immunoglobulin M level of 118 mg/dL (normal, 43 to 196 mg/dL) and immunoglobulin A level of 27 mg/dL (normal, 25 to 154 mg/dL).

On October 17, 1994, revision of the Fontan pathway was undertaken. Using hypothermic cardiopulmonary bypass and 7 minutes of hypothermic circulatory arrest, a right-to-left shunt at the atrial level was created by placement of a single fenestration in the Gore-Tex (W.L. Gore & Assoc, Flagstaff, AZ) portion of the systemic venous baffle, using a 4.8-mm punch. The child was extubated on the day of operation. In the postoperative period, atrial pressure was 8 to 10 mm Hg and arterial oxygen saturation ranged from 85% to 90%. No steroids were administered, nor was albumin given intravenously. Ascites and peripheral edema resolved quickly after operation and did not recur. Two weeks after operation serum chemistry values were noteworthy for a total protein level of 7.4 g/dL and serum albumin level of 4.0 g/dL. She has remained well and has returned to preschool. Five months after fenestration of the Fontan pathway, her evaluation was noteworthy for a total protein level of 7.3 g/dL, serum albumin level of 4.6 g/dL, and immunoglobulin G level of 966 mg/dL (normal, 463 to 1,236 mg/dL). Pulse oximetry reveals a systemic saturation of 86%.

Patient 2
Patient 2, born on August 31, 1988, was born with tricuspid atresia type IB. On the third day of life she underwent creation of a systemic-to-pulmonary artery shunt. At age 6 months, she underwent a hemi-Fontan procedure and closure of the right modified Blalock-Taussig shunt. Cardiac catheterization at 2 years of age revealed normal ventricular function, normal pulmonary artery architecture, and systemic arterial saturation of 86%. In July 1990, at 2 years of age, she underwent a completion Fontan procedure. The postoperative course was noteworthy only for the presence of pleural effusions, which resolved over several weeks.

Four months after the Fontan procedure, she was evaluated for peripheral edema and lethargy. Diuretic therapy had little influence on her well-being. Mild cyanosis was attributed to the recurrent presence of pleural effusions. Ascites and peripheral edema increased despite increasing diuretic therapy. Laboratory evaluation revealed hypoalbuminemia and hypoproteinemia.

In December 1991, oral steroid therapy was begun at a dose of 2 mg•kg^-1•day^-1. Serum protein levels continued to fall slowly despite steroid therapy, with levels in June 1992 of 4.1 g/dL for total protein and 2.6 g/dL for albumin. Attempts at decreasing the steroid dose were associated with worsening of the edema and ascites.

Cardiac catheterization on February 15, 1995, revealed pressure in the superior vena cava, inferior vena cava, and systemic venous pathway was 13 mm Hg. Mean pressures in the right and left pulmonary arteries were 13 mm Hg. Pressure in the systemic ventricle was 100/8 mm Hg. Oximetry revealed saturation in the superior vena cava to be 63%; inferior vena cava, 69%; and in the aorta, 93%. Angiography revealed no areas of obstruction within the systemic venous pathway or the pulmonary arteries, and satisfactory shortening of the systemic left ventricle. No atrioventricular valve regurgitation was noted. Despite these seemingly satisfactory hemodynamics, the child remained steroid dependent with physical and biochemical evidence of protein-losing enteropathy.

On February 24, 1995, surgical revision of the Fontan pathway was undertaken. Using hypothermic cardiopulmonary bypass, the Fontan baffle was modified by creation of a single fenestration using a 4.8-mm punch. Neither cardioplegic arrest nor circulatory arrest was required. The postoperative course was entirely unremarkable. No additional steroids were administered, nor was any albumin given. Diuretics were administered, and within days after operation, there was complete resolution of ascites and peripheral edema. Two weeks after operation, serum chemistry values were noteworthy for a total protein level of 6.2 g/dL and albumin level of 3.2 g/dL. Systemic saturation by pulse oximetry was 86%. At most recent follow-up nearly 2 months after operation, serum chemistry values were noteworthy for a total protein level of 7.2 g/dL and albumin level of 4.1 g/dL. Pulse oximetry again revealed a saturation of 86%.

	Comment

Top Footnotes Abstract Introduction Case Reports Comment References

 
Protein-losing enteropathy represents a unique and morbid complication of the Fontan operation. It is believed to be related to chronic increase in pressure in the superior vena cava, and thus to an impedance to drainage of the lymphatic system via the thoracic duct. This is not in and of itself an entirely satisfactory explanation. Protein-losing enteropathy is exceptionally rare after the hemi-Fontan operation or bidirectional Glenn procedure, in which cases the superior vena caval pressure is elevated but the inferior vena cava and hepatic veins drain to the low-pressure pulmonary venous atrium. It has been a matter of conjecture whether the creation of an atrial-level right-to-left shunt by means of fenestration, as advocated by Laks and associates [5], Bridges and colleagues [6], and others for patients with multiple risk factors for Fontan operation, could affect the incidence or persistence of protein-losing enteropathy.

It has been established by perioperative [7] and postoperative [8] hemodynamic studies that ``fenestration'' of the atrial septum or systemic venous baffle at the time of a Fontan procedure is associated with increased cardiac index and potentially increased oxygen delivery at the expense of mild systemic arterial oxygen desaturation. Castañeda [9] reported a diminution in the prevalence of long-lasting pleural and pericardial effusions with the fenestrated cavopulmonary connection as compared with the nonfenestrated cavopulmonary connection or atrial pulmonary connection. It is not known whether the causes of effusive complications and protein-losing enteropathy after Fontan procedures are the same, although these complications certainly have been noted to occur coincidentally or consecutively in some patients.

We have had a gratifying, although small, experience with the eradication of persistent and morbid pleural effusions after Fontan operation by secondary fenestration of the systemic venous baffle, and have therefore applied this strategy to the 2 patients with protein-losing enteropathy described in this case report. A single large fenestration was created to maximize the likelihood of long-term patency, and low-dose aspirin has been administered in hopes of preventing spontaneous closure. It is curious that in the 2 cases described in this report, 1 patient (patient 1) had a marked elevation of systemic venous and pulmonary artery pressures whereas the other patient (patient 2) had hemodynamics that would ordinarily be considered very satisfactory. In each case, the creation of a 4.8-mm fenestration or defect in the atrial baffle resulted in early postoperative resolution of clinical and biochemical signs of protein-losing enteropathy. Reversal of this syndrome has persisted during the respective 7-month and 2-month periods of follow-up in the absence of steroids or specific dietary supplements.

Recently, Mertens and associates [10] described a case wherein protein-losing enteropathy resolved after catheter-directed needle puncture of the native atrial septum in a 23-year-old woman with tricuspid atresia who had undergone a Fontan operation. Most patients currently undergoing modified Fontan procedures have creation of a lateral atrial tunnel using synthetic material such as polytetrafluoroethylene. Our preliminary experience with operative creation of fenestrations in previously constructed polytetrafluoroethylene Fontan baffles has been encouraging. The 2 patients described herein each underwent a technically straightforward operative procedure and had gratifying results. We suggest that such a strategy may have merit for those patients in whom protein-losing enteropathy develops after Fontan operations.

	Footnotes

Top Footnotes Abstract Introduction Case Reports Comment References

 
Address reprint requests to Dr Jacobs, Division of Cardiothoracic Surgery, The Children's Hospital of Philadelphia, 34th St & Civic Center Blvd, Philadelphia, PA 19104.

	References

Top Footnotes Abstract Introduction Case Reports Comment References

 

1. Hill D, Feldt R, Porter C, Puga F, Danielson G, Perrault J. Protein-losing enteropathy after Fontan operation: a preliminary report. Circulation 1989;80(Suppl 2):490.
2. Rothman A, Snyder J. Protein-losing enteropathy following the Fontan operation: resolution with prednisone therapy. Am Heart J 1991;121:618–9.[Medline]
3. Rychik J, Piccoli DA, Barber G. Usefulness of corticosteroid therapy for protein-losing enteropathy after the Fontan procedure. Am J Cardiol 1991;68:819–21.[Medline]
4. Mair DD, Puga FJ, Danielson GK. Late functional status of survivors of the Fontan procedure performed during the 1970s. Circulation 1992;86(Suppl 2):108.
5. Laks H, Haas GS, Pearl JM, et al. The use of an adjustable intraatrial communication in patients undergoing the Fontan and definitive right heart procedures [Abstract]. Circulation 1988;78(Suppl 2):357.
6. Bridges ND, Lock JE, Castañeda AR. Baffle fenestration with subsequent transcatheter closure: modification of the Fontan operation for patients at increased risk. Circulation 1990;82:1681–9.[Abstract/Free Full Text]
7. Mavroudis C, Zales VR, Backer CL, Muster AJ, Latson LA. Fenestrated Fontan with delayed catheter closure: effects of volume loading and baffle fenestration on cardiac index and oxygen delivery. Circulation 1992;86(Suppl 2):85.
8. Hijazi ZM, Fahey JT, Kleinman CS, Kopf GS, Hellenbrand WE. Hemodynamic evaluation before and after closure of fenestrated Fontan. An acute study of changes in oxygen delivery. Circulation 1992;86(Suppl 1):196–202.[Abstract/Free Full Text]
9. Castañeda AR. From Glenn to Fontan. Circulation 1992;86(Suppl 2):80.
10. Mertens L, Dumoulin M, Gewilling M. Effect of percutaneous fenestration of the atrial septum on protein-losing enteropathy after the Fontan operation. Br Heart J 1994;72:591–2.[Free Full Text]

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This Article Abstract Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Marshall L. Jacobs Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Jacobs, M. L. Articles by Norwood, W. I. Search for Related Content PubMed PubMed Citation Articles by Jacobs, M. L. Articles by Norwood, W. I., Jr