Abstract
Protein-losing enteropathy (PLE) is one of the major complications after Fontan operation and usually results in a poor outcome. The mechanism of PLE is not fully understood, and the specific treatment remains to be studied. Recently, some reports showed that the phosphodiesterase inhibitor V, sildenafil, might improve PLE through the vasodilation of not only the pulmonary artery but also the mesenteric artery. Here, we report three patients with PLE after the Fontan operation whose symptoms were improved by administration of sildenafil. [Case 1] A twenty-one-year old male with single ventricle developed edema due to PLE 6 years after surgery. Sildenafil was administered at a dose of 30 mg per day, and edema was resolved after titrating it up to 40 mg per day. [Case 2] A seventeen-year-old male with single ventricle developed cyanosis and edema due to pulmonary arterial-venous fistula and PLE 2 years after surgery. Sildenafil was administered at a dose of 1 mg/kg per day, and his symptom was resolved after titrating it up to 4 mg/kg per day. [Case 3] A twelve-year-old girl with double outlet right ventricle developed edema and ascites due to PLE a year after surgery. The symptoms were refractory to oral prednisolone (1 mg/kg per day). After sildenafil was administered at a dose of 0.5 mg/kg per day and titrated up to 8 mg/kg per day, ascites finally resolved. No major complications were observed in each case. Sildenafil may be a safe and effective therapeutic option for PLE after the Fontan operation with dose dependent efficacy.
