Protein-losing enteropathy managed with percutaneous enlargement of a restrictive atrial septal defect

Protein-losing enteropathy is one of the most feared complications of the Fontan circulation. The diagnosis of protein-losing enteropathy in this setting should prompt a thorough investigation for the presence of a treatable hemodynamic impairment. In this report, we describe a complete reversal of protein-losing enteropathy following percutaneous enlargement of a restrictive atrial septal defect in a patient with a fenestrated lateral tunnel Fontan and severe mitral stenosis.