Usefulness of corticosteroid therapy for protein-losing enteropathy after the Fontan procedure

Chronic pleural and pericardial effusions are a significant cause of morbidity and mortality after the Fontan procedure. Frequently, these patients manifest a protein-losing enteropathy (PLE), defined as severe loss of serum proteins into the gut, with clinical sequelae of edema, anasarca and immunodeficiency.1 Despite standard therapy which includes nutritional alterations, diuretics and serial albumin infusions, mortality remains high.2 We report 2 cases of severe PLE after the Fontan procedure successfully managed with corticosteroid therapy.