Protein-losing enteropathy in Fontan circulation: Pathophysiology, outcome and treatment options of a complex condition.

Protein-losing enteropathy (PLE) represents a rare but severe and potentially life-threatening complication following Fontan operation in patients with a functional single ventricle. PLE is characterized by enteric protein loss, leading to devastating multiorgan involvement with increased morbidity and mortality. In spite of remarkable advances in the care of congenital heart disease in recent years, treatment of PLE is still one of the most challenging tasks due to limited understanding of the underlying mechanisms and lack of high-quality evidence from large scale, controlled studies to test the real efficacy of the several treatment strategies, which have been proposed. For this reason, we herewith aim to discuss the pathogenesis and diagnosis of PLE in Fontan patients as well as provide a comprehensive overview on potential advantages and disadvantages of the currently available therapeutic strategies, in order to propose a stepwise approach for the management of this unique condition.