Original Surgical Procedure for the Treatment of Protein-Losing Enteropathy in Fontan Patients: Report of Two Midterm Successes.

August 23, 2016 625 CORRPONDENCE The surgical connection of the vena cava to the pulmonary arteries creates a circulation in series and defines the Fontan operation for univentricular hearts. The central venous pressure is elevated (≈15 mm Hg) and drives the blood into the pulmonary vessels. This elevated venous pressure overwhelms the postsinusoidal regulatory mechanism normally responsible for maintaining portal pressure at ≈10 mm Hg. The elevated venous pressure is transmitted to the sinusoids, portal vein, and gut capillaries. Protein-losing enteropathy (PLE) is a complication of this hemodynamic state, where proteins leach into the gut from submucosal lymphangiectasis. PLE occurs in ≈5% of patients undergoing the Fontan procedure and is often fatal within 5 years of onset. We performed a novel surgical procedure to restore a gradient similar to the physiological one between the portal vein and the inferior vena cava (IVC) in 2 Fontan patients with PLE. Hospital Human Ethics Committee’s approval and informed consent were obtained. Under cardiopulmonary bypass, the 3 major hepatic veins (right, middle, and left) were separated and connected to the common atrium. An extracardiac conduit without fenestration, placed in the hepatic portion of the IVC, completed the Fontan circuit. The other hepatic veins, including the vein draining the caudate lobe and multiple venules that normally drain into the IVC, were occluded by a custom-made covered stent1 placed in the retrohepatic IVC deployed percutaneously immediately after surgery (Figure). The venous drainage of the territories occluded with the covered stent rerouted itself toward the 3 major hepatic veins, with no resulting hepatic dysfunction. The portal flow (now routed directly to the common atrium) thus became a right-to-left shunt equivalent to ≈20% of systemic venous flow. Our first patient received a fenestrated extracardiac Fontan at age 4. PLE was diagnosed 18 months postoperatively. The Fontan pressure was 15 mm Hg with a patent fenestration. Endoscopy and small-bowel biopsy revealed duodenal lymphangiectasis. Despite treatment with high-dose budesonide, diuretics, and albumin transfusions, the albumin level was constantly <1.4 g/dL, and α1-antitrypsin clearance (a measure of net gastrointestinal protein loss) was 450 mL/d before surgery. Postoperatively, his liver transaminases did not increase. Warfarin was instituted (target international normalized ratio, 2–3). There was no lessening of PLE initially; parenteral nutrition and fractionated heparin were added. Seven weeks postoperatively, signs of PLE subsided. The α1-antitrypsin clearance was normal (<16 mL/24 h) and serum albumin was 4.0 g/dL. The patient resumed normal activities and diet with a resting oxygen saturation >82%. Catheterization showed no communication between the Fontan circuit and the liver. The Fontan pressure was 12 mm Hg and hepatic venous pressure was 8 mm Hg. Endoscopy and small-bowel biopsy showed resolution of the lymphangiectasis. Christian P. Brizard, MD, MS Geoffrey K. Lane, MBBS, FRACP, FCSANZ George Alex, MBBS, FRACP, PhD, MMed, MRCP Michael M.H. Cheung, MB ChB, FRACP, MD