Protein Losing Enteropathy after Fontan Surgery – Clinical and Diagnostical Aspects

Protein losing enteropathy (PLE) is the massive enteric loss of serum protein. PLE may appear in several diseases associated with intestinal mucous membrane damages with or without infection. PLE is mostly associated with total cavopulmonary connection (TCPC) or Fontan-type circulation in patients with a functionally univentricular heart. TCPC is performed at an age of about 2 years or older and has a high survival rate of >90%. Time of PLE onset after TCPC is variable, the exact etiology is still unclear. Increased central venous pressure due to the absence of a subpulmonary ventricle may be a main reason for PLE, affecting 2-15% of the patients with a survival rate of 40% at 5 years and 20% at 10 years. Also immunological reasons for PLE are suspected. Major clinical signs are edemas, ascites, pleural effusion, diarrhea, malnutrition, fatigue, weight loss, and reduced physical development. The most impaired laboratory signs are elevated fecal α1-antitrypsin and α1-antitrypsin clearance, hypoproteinemia (hypoalbuminemia, hypo-γ-globulinemia), lymphopenia (selective T helper cell loss) and secondary lymphangiectasia. Therapy considerations should have the aim to decrease central venous pressure to improve hemodynamics. Medical treatment consists of substitution of e.g. albumin, γ-globulin, glucocorticoid, heparin or calcium, but still >60% of the patients remain symptomatic.