A 20-year-old patient, who had double outlet right ventricle, mitral atresia, pulmonary atresia, and bilateral superior vena cava and underwent successful lateral tunnel total cavo-pulmonary connection at 6 years old, presented with frequent watery diarrhea, general malaise, and tetany. He was known to have intractable protein-losing enteropathy (PLE) from 7 years of age that was resistant to various treatments. To keep hemodynamics stable, he required intravenous albumin infusion every day. Fontan fenestration partially improved his condition and allowed to stop albumin infusion, however still he showed muddy stool and cachexia with low serum albumin <20 g/L and immunoglobulin <3 g/L. Because of serious risk of infection, we placed him on regular subcutaneous immunoglobulin supplementation with rescue intravenous immunoglobulin that improved his PLE within a month and allowed him to be discharged. This case illustrates that immunoglobulin supplementation can be one of the choices of treatment for intractable PLE. <Learning objective: Combined subcutaneous and intravenous immunoglobulin infusion therapy can be one of the choices of treatment for intractable protein-losing enteropathy that does not respond to multiple medications.>.
Keywords: Fontan type operation; Immunoglobulin therapy; Protein-losing enteropathy; Subcutaneous immunoglobulin supplementation.