Abstract Managing protein-losing enteropathy after Fontan surgery is challenging. We report a case of protein-losing enteropathy associated with Fontan circulation in a 14-year-old boy. He was treated medically for 2 years, without improvement until Fontan takedown and pulsatile bidirectional cavopulmonary shunting were performed. Fontan procedure , Protein-losing enteropathy , Surgical procedure INTRODUCTION Post-Fontan surgery survival has improved over the decades; however, long-term complications of Fontan physiology such as protein-losing enteropathy (PLE) have been noted . Corticosteroid, sildenafil and unfractionated heparin have demonstrated some efficacy for PLE after Fontan surgery . However, there is no consistently effective treatment for PLE, except for heart transplantation . Early mortality in heart transplantation is higher in patients with Fontan circulation than in those with non-congenital heart disease . Moreover, the chance of receiving a donor heart is relatively low for children. Thus, new management strategies for refractory PLE after Fontan surgery are needed. CASE REPORT An 11-year-old boy was admitted because of bilateral leg and scrotal swelling. He underwent a bidirectional cavopulmonary shunt at the age of 1 year and extracardiac Fontan surgery using a 20-mm conduit without fenestration at the age of 2 years because of a functional single ventricle with left isomerism, double-outlet right ventricle, complete atrioventricular septal defect and pulmonary stenosis. At admission, the serum albumin was 2.4 g/dl. His random stool alpha-1-antitrypsin was 338 (normal range 0–54) mg/dl. Echocardiography showed moderate atrioventricular valve regurgitation and good ventricular function. His mean inferior vena cava (IVC) pressure was 15 mmHg via cardiac catheterization. From these findings, he was diagnosed with PLE. Following diagnosis, combinations of diuretics, sildenafil, corticosteroid and unfractionated heparin were administered for 2 years but were ineffective or briefly effective (Fig. 1). During the 2-year treatment, he was admitted 9 times for albumin replacement. Figure 1: View largeDownload slide The graph shows the level of serum albumin (linear line) and random stool alpha-1-antitrypsin (dotted line) since the diagnosis of PLE. Medical treatments and oral prednisolone dose (from 1.25 to 30 mg/day) are also shown. The black arrows represent 2 methyl prednisolone pulses (15 mg/kg/dose for 3 days), and the vertical dotted line represents the procedure date. α1-AT: alpha-1-antitrypsin; PD: prednisolone; PLE: protein-losing enteropathy; SC: subcutaneous. Figure 1: View largeDownload slide The graph shows the level of serum albumin (linear line) and random stool alpha-1-antitrypsin (dotted line) since the diagnosis of PLE. Medical treatments and oral prednisolone dose (from 1.25 to 30 mg/day) are also shown. The black arrows represent 2 methyl prednisolone pulses (15 mg/kg/dose for 3 days), and the vertical dotted line represents the procedure date. α1-AT: alpha-1-antitrypsin; PD: prednisolone; PLE: protein-losing enteropathy; SC: subcutaneous. We considered that resolving the venous congestion of the liver and bowel could ameliorate the PLE. We performed follow-up catheterization to evaluate central venous pressure, and the mean IVC pressure was only 10 mmHg. We assumed that this IVC pressure did not reflect hepatic venous congestion because of the persistent hypoalbuminaemia. Finally, we performed a Fontan takedown to improve the hepatic venous congestion. To maintain sufficient pulmonary blood flow and provide pulsatility to the pulmonary circulation, we created a ventricle-to-pulmonary artery conduit with a 12-mm polytetrafluoroethylene valved conduit, approximately half the size of the prosthetic valve used in adult pulmonary valve replacement. Additionally, we replaced the atrioventricular valve with a mechanical valve to correct atrioventricular valve regurgitation (Fig. 2). Figure 2: View largeDownload slide A schematic drawing of Fontan takedown, pulsatile bidirectional cavopulmonary shunt and atrioventricular valve replacement. AVVR: atrioventricular valve replacement; FP: Fontan pathway; IVC: inferior vena cava; PA: pulmonary artery; SVC: superior vena cava. Figure 2: View largeDownload slide A schematic drawing of Fontan takedown, pulsatile bidirectional cavopulmonary shunt and atrioventricular valve replacement. AVVR: atrioventricular valve replacement; FP: Fontan pathway; IVC: inferior vena cava; PA: pulmonary artery; SVC: superior vena cava. Immediately after surgery, the serum albumin was low. However, the serum albumin increased, and the PLE symptoms gradually improved. Nine months post-surgery, the serum albumin level stabilized and exceeded 3.5 g/dl, without albumin replacement. Two years post-surgery, serum albumin was 4.4 g/dl, and random stool alpha-1-antitrypsin was 26.6 mg/dl (Fig. 1). The patient’s oxygen saturation decreased to 75–80% (preprocedural 93–97%). The patient is currently receiving low-dose corticosteroid (5 mg/day), sildenafil (17 mg tid), furosemide (10 mg qd) and spironolactone (12.5 mg qd). DISCUSSION The mechanism of PLE after Fontan surgery is unknown but high central venous pressure and hepatic venous congestion  may comprise the initial pathophysiology. Conventional treatments to improve high central venous pressure and hepatic venous congestion are only partially effective in resolving PLE. After transcatheter Fontan tract fenestration, the recurrence of PLE was frequent . After Fontan takedown, pulmonary blood flow is decreased to half. Furthermore, there is no previous report on performing a Fontan takedown just for managing PLE. Two new surgical strategies have recently been introduced. The first, thoracic duct decompression, basically involves redirecting the innominate vein to the systemic atrium to improve lymphatic drainage of the bowel . The other strategy, hepatic vein decompression, involves hepatic vein redirection to the systemic atrium and retrohepatic IVC stenting to occlude hepatic venules to IVC . These procedures allow a minimal decrease in pulmonary blood flow. Our new surgical strategy decompresses the entire IVC similar to Fontan takedown. However, unlike Fontan takedown, it additionally creates a ventricle-to-pulmonary artery conduit to maintain sufficient pulmonary blood flow and to provide pulsatility to the pulmonary circulation. We performed catheterization 2 years postoperatively. The postoperative IVC pressure was 17 mmHg, which exceeded the preoperative value; serum albumin level normalization may be responsible for this phenomenon. Another possible explanation for PLE after Fontan surgery might be the loss of pulmonary artery pulsatility and not just elevated venous pressure. More cases are required to verify this finding. Fontan takedown and pulsatile bidirectional cavopulmonary shunt may be used to control refractory PLE after Fontan surgery; however, more studies and long-term follow-up are necessary to validate the outcomes. Conflict of interest: none declared. REFERENCES 1 Ohuchi H. Adult patients with Fontan circulation: what we know and how to manage adults with Fontan circulation? J Cardiol 2016; 68: 181– 9. Google Scholar CrossRef Search ADS PubMed 2 Bernstein D, Naftel D, Chin C, Addonizio LJ, Gamberg P, Blume ED et al. Outcome of listing for cardiac transplantation for failed Fontan: a multi-institutional study. Circulation 2006; 114: 273– 80. Google Scholar CrossRef Search ADS PubMed 3 Vyas H, Driscoll DJ, Cabalka AK, Cetta F, Hagler DJ. Results of transcatheter Fontan fenestration to treat protein losing enteropathy. Catheter Cardiovasc Intervent 2007; 69: 584– 9. Google Scholar CrossRef Search ADS 4 Antonio M, Gordo A, Pereira C, Pinto F, Fragata I, Fragata J. Thoracic duct decompression for protein-losing enteropathy in failing Fontan circulation. Ann Thorac Surg 2016; 101: 2370– 3. Google Scholar CrossRef Search ADS PubMed 5 Brizard CP, Lane GK, Alex G, Cheung MM. Original surgical procedure for the treatment of protein-losing enteropathy in Fontan patients: report of two midterm successes. Circulation 2016; 134: 625– 7. Google Scholar CrossRef Search ADS PubMed © The Author(s) 2018. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved. This article is published and distributed under the terms of the Oxford University Press, Standard Journals Publication Model (https://academic.oup.com/journals/pages/about_us/legal/notices)
Interactive CardioVascular and Thoracic Surgery – Oxford University Press
Published: Apr 16, 2018
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