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