Oral Budesonide as a Therapy for Protein-Losing Enteropathy in Patients Having Undergone Fontan Palliation

BACKGROUND: 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. METHODS: We examine our single-institution retrospective experience in eight patients who were treated with this therapy. RESULTS: 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. CONCLUSIONS: Oral budesonide is an additional therapy that has the potential to improve symptoms and delay need for heart transplantation in this patient population.