Pediatric Cardiology and Cardiac Surgery
Vol.26 No.1 2010 (33-38)

Justin M. Horner and David J. Driscoll

Department of Pediatric and Adolescent Medicine/Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota, USA


Protein losing enteropathy (PLE) is a potentially lethal complication of the modified Fontan operation. PLE can occur in up to 10% of patients after a Fontan operation. It is characterized by fatigue, peripheral edema, ascites, pleural effusions, growth delay, and/or diarrhea. The etiology is not well understood and many mechanisms for the development of PLE have been proposed. These include elevated systemic venous pressure, elevated thoracic duct pressure, elevated levels of brain natriuretic peptide (BNP), small bowel inflammation, elevated mesenteric venous pressure, and low cardiac output. A thorough cardiovascular investigation should be undertaken once PLE develops to identify potentially reversible important hemodynamic abnormalities. Despite more than 25 years of research, no universal or curative treatment has been identified. Overall, management is aimed at decreasing edema and increasing serum protein levels. Other treatment strategies include: nutritional therapy, atrioventricular pacing, diuretics, heparin, steroids, albumin infusion, and cardiac transplantation. Further investigation is needed to enhance our understanding of PLE and to define improved treatment.