Systematic Review of the Etiology of Protein Loss Enteropathy

Background: Patients born with a single ventricle physiology typically undergo three stages of palliative procedure. One of these, the Fontan procedure was developed 1971 by Dr. Francois Marie Fontan to connect the systemic venous return to the pulmonary arteries. Protein Losing Enteropathy (PLE) is the enteric loss of proteins, that present as a secondary comorbidity witnessed in patients post Fontan procedure. Objective: This systematic review aims to assess the risk factors, etiology, and treatment of PLE following the Fontan procedure. Methods: I searched PubMed, Embase, PsycINFO, CINAHL, Web of Science, and Cochrane Library. Articles were selected based on mesh words: PLE, Fontan, Congenital Heart Defects (CHD), and Total Cavo-pulmonary Connection (TCPC). Results: The risk factors associated with the development of PLE include: 1.) Chronic state of central venous hypertension, 2.) Single Ventricle (SV) Performance, 3.) Prolonged Cardiopulmonary Bypass Time and 4.) The type of SV anatomy. For the etiology of PLE there were three prevailing hypotheses which include autoimmune/inflammatory response, cellular structure breakdown, and elevated arterial pressures causing lymphatic dysfunction. Lastly, the treatment of PLE includes dietary modifications, Heparin, Budesonide, and IV of immunoglobulin. Conclusion: The systematic review provides and assesses the clinical spectrum of CHD, specifically Single Ventricle defects, the Fontan Circulation, and PLE. The risk factors, etiology, and treatment of PLE provide a clinical framework for clinicians treating PLE patients post Fontan procedure.