Protein Losing Enteropathy after Fontan Surgery – Clinical and Diagnostical Aspects

Abstract

Protein losing enteropathy (PLE) is the massive entericloss of serum protein. PLE may appear in several diseasesassociated with intestinal mucous membranedamages with or without infection. PLE is mostly associatedwith total cavopulmonary connection (TCPC) orFontan-type circulation in patients with a functionallyuniventricular heart. TCPC is performed at an age ofabout 2 years or older and has a high survival rate of>90%. Time of PLE onset after TCPC is variable, the exactetiology is still unclear. Increased central venous pressuredue to the absence of a subpulmonary ventriclemay be a main reason for PLE, affecting 2-15% of the patientswith a survival rate of 40% at 5 years and 20% at 10years. Also immunological reasons for PLE are suspected.Major clinical signs are edemas, ascites, pleural effusion,diarrhea, malnutrition, fatigue, weight loss, and reducedphysical development. The most impaired laboratorysigns are elevated fecal α1-antitrypsin and α1-antitrypsinclearance, hypoproteinemia (hypoalbuminemia,hypo-γ-globulinemia), lymphopenia (selective T helpercell loss) and secondary lymphangiectasia. Therapy considerationsshould have the aim to decrease central venouspressure to improve hemodynamics. Medical treatmentconsists of substitution of e.g. albumin, γ-globulin,glucocorticoid, heparin or calcium, but still >60% of thepatients remain symptomatic.