A case report of midodrine to treat protein-losing enteropathy for heart transplant candidacy.

Background: Protein-losing enteropathy (PLE) is seen in up to 15% of patients with Fontan circulation and has important nutritional, immunologic, and haemodynamic consequences especially in the context of advanced heart failure. Midodrine, an alpha-1-receptor agonist, increases lymphatic tone, mitigating leak of lymph into the bowel, and has been shown to have clinical and biochemical benefit in patients with Fontan failure. Case summary: A young woman with Fontan circulation, end-stage heart failure, and PLE developed progressive hypoalbuminaemia, deconditioning, and frailty prohibitive to transplantation candidacy. Three months after commencing oral midodrine therapy, she experienced normalization of her serum albumin and improvements in her oedema, exercise tolerance, and frailty enabling candidacy and eventual successful heart transplantation. Discussion: The development of PLE in patients with Fontan circulations is an indication for cardiac transplantation, but there are often significant barriers due to the many complications. We performed a comprehensive Medline search, and this is the first case demonstrating the use of midodrine to bridge a frail patient with PLE and advanced Fontan heart failure to heart transplantation candidacy and eventual transplant.