Protein-losing enteropathy after the Fontan operation: successful treatment by percutaneous fenestration of the atrial septum.

Protein-losing enteropathy (PLE) after the Fontan operation is a life-threatening complication that may be refractory to medical therapy. Herein we describe a percutaneous atrial fenestration that was performed in a 42-year-old man with a double-inlet left ventricle who had undergone a Fontan operation 9 years earlier. Severe PLE developed, and despite frequent infusions of protein, his albumin level was 1.8 g/dL. The diagnosis of PLE was confirmed by an alpha(1)-antitrypsin clearance of 425 mL in 24 hours (normal 27 or less). Percutaneous atrial fenestration resulted in dramatic clinical improvement and resolution of the PLE. At 5-month follow-up, the patient's albumin level was 4.2 g/dL, his alpha(1)-antitrypsin clearance was normal, and he was free of ascites and edema.