Abstract
Protein-losing enteropathy (PLE) is a rare disorder with diverse causes, but the treatments are limited and understudied. It is often associated with significant mortality and morbidity. The survival of hypoplastic left heart syndrome (HLHS) in infants without any intervention is usually 4.5 days, and 30-day mortality is 95%. However, with surgical intervention, survival at 20 years is 80%. HLHS can lead to protein-leading enteropathy and is corrected by the three-step procedures (Norwood, Glenn, and Fontan) during infancy. We report a case of Fontan procedure postoperative HLHS associated with PLE and describe its clinical course and outcome. The main intention of reporting this case is to provide awareness among physicians while dealing with refractory cases of hypoproteinemia and appropriate management based on the literature.