Oral budesonide as a therapy for protein-losing enteropathy in patients having undergone Fontan palliation

Protein-losing enteropathy is associated with Fontan palliation for single-ventricle physiology and has been difficult to treat. Limited data suggest the successful use of oral budesonide (Entocort, AstraZeneca) as a palliative measure.

We examine our single-institution retrospective experience in eight patients who were treated with this therapy.

Median pretherapy albumin level was 1.7 g/dL (range 1.0-2.7 g/dL), 3 months after therapy was 3.1 g/dL (range 2-4.8 g/dL), and by the end of the first year was 3.4 g/dL (range 2.1-5.3 g/dL). All patients had at least a transient improvement, and at latest follow-up (median 29 months, range 3-51 months) five patients remain on therapy. Five of eight patients had required pretherapy albumin transfusions; one patient required albumin infusions after therapy. Four patients had side effects associated with the medication.

Oral budesonide is an additional therapy that has the potential to improve symptoms and delay need for heart transplantation in this patient population.