Protein-losing enteropathy seven years after total right ventricular exclusion procedure for arrhythmogenic right ventricular dysplasia.

A 27-year-old man who was diagnosed with arrhythmogenic right ventricular dysplasia (ARVD) underwent the total right ventricular (RV) exclusion procedure: the RV free wall was resected and an extracardiac total cavopulmonary connection (TCPC) was created using a 24-mm-diameter polytetrafluoroethylene (PTFE) graft. After an uneventful period of 7 years, he began to develop protein-losing enteropathy (PLE), which was resistant to medical therapy, moderate mitral regurgitation (MR) and right femoral arteriovenous fistula due to heart catheterization. Therefore, fenestration of TCPC, mitral annuloplasty and ligation of arteriovenous fistula were performed. After the operation, symptoms and levels of total protein and albumin were immediately improved and normalized.