Abstract
This report describes the efficacy of tolvaptan for the treatment of protein-losing enteropathy (PLE) that developed in two patients who underwent the Fontan operation. The first patient was an 8-year-old boy with asplenia, dextrocardia, common atrioventricular canal (CAVC), univentricular heart (UVH), pulmonary atresia and total anomalous pulmonary venous connection. Although intravenous heparin, oral corticosteroids and intravenous albumin were administered, serum albumin and total protein levels were only approximately 2 g/dL and 3 g/dL, respectively. Subsequent administration of tolvaptan resulted in normalization of serum albumin and total protein levels and a decrease in PLE-related diarrhea. The second patient was a 19-year-old man with polysplenia, UVH, CAVC, double-outlet right ventricle, pulmonary atresia and interrupted inferior vena cava (azygos connection). Tolvaptan administration in this patient resulted in an increase in serum albumin and total protein levels and a decrease in ascites. These observations suggest that tolvaptan may be an effective treatment option for PLE in the patients who have undergone the Fontan operation.
