Management of protein-losing enteropathy after the Fontan procedure.

Enteric loss of protein with the sequelae of edema, immunodeficiency, and hypercoagulability is being diagnosed with increasing frequency after Fontan procedure. The precise pathophysiological mechanism is unknown; however, protein-losing enteropathy (PLE) after Fontan procedure is likely related to a hemodynamic derangement that is not easily detectable via standard hemodynamic measures presently obtainable in the cardiac catheterization laboratory. Treatment options include (1) symptomatic relief via diuretics, supplemental albumin infusion, and dietary change to high protein/high medium-chain triglyceride intake, (2) hemodynamic improvement via afterload reduction (angiotensin-converting enzyme inhibitors), repair of branch pulmonary artery stenoses, or coil embolization of aortopulmonary collaterals, (3) intestinal cell membrane stabilization via high-dose steroids or heparin infusion, and (4) attempt at alteration of the primary hemodynamic derangement via fenestration of the systemic venous baffle or via heart transplantation. Further understanding of the cause of PLE after Fontan procedure is needed before more effective treatment options can be used. Copyright 1998 by W.B. Saunders Company