A286 MULTIFACTORIAL ETIOLOGY OF PROTEIN LOSING GASTROENTEROPATHY FOLLOWING FONTAN’S PROCEDURE

Protein-Losing Enteropathy (PLE) post Fontan operation is thought to be due to multiple aetiologies including venous congestion, abnormal mesenteric vascular resistance, primary and\or secondary intestinal lymphangiectasia, & inflammation. An incidence of 3.7% has been reported in multicentre series of >3000 Fontan patients. We report a case where take down of the Fontan did not improve PLE. This highlights the importance of non-vascular mechanisms or irreversible vascular changes in these patients. A detailed review of the case and the literature was undertaken. A 20-year-old Caucasian female was referred to the adult GI clinic with a history of PLE since 2004. She has a complex history of congenital heart disease including dextrocardia, transposition of the great arteries, pulmonary atresia and VSD. In 2000, she underwent a non-fenestrated Fontan procedure. In 2004, she developed progressive PLE presenting with diarrhea, ascites & edema. The albumin was 11g/L, IgG <1.09 g/L, IgA < 0.4 g/L, IgM <0.25 g/L along with iron deficiency anemia, hypocalcaemia, hypomagnesemia and secondary hyperparathyroidism. Other complications include osteoporosis with multiple vertebral fractures, recurrent respiratory infections, and problematic soft tissue infections requiring multiple admissions for IV antibiotics and IVIG. In 2007, she underwent takedown of her Fontan in an attempt to control her PLE. Diarrhea and hypoalbuminemia persisted and she was given 5 years to live. She eventually had a partial response to medical treatment which consisted of budesonide, enoxaparin, and a diet of high protein and high medium chain triglyceride intake. There was partial improvement of symptoms and the hypoalbuminemia 18-20g/L. The budesonide was weaned down from 9mg daily to 3mg every other day. Any attempts at further weaning or stopping of the enoxaparin have resulted in worsening of her symptoms and biochemistry. A CT scan in 2008 showed mild dilatation of small bowel, extensive collateralisation, high attenuation in the distal small bowel extending to the rectum, and relative mucosal enhancement within segments of small bowel. A Doppler US showed patent hepatic and portal veins. This case highlights the importance of the multifactorial nature of PLE post Fontan procedure. Reversing the hemodynamics of the heart is not enough to reverse the PLE in some cases. Permanent secondary intestinal lymphangiectasia, inflammation with enterocyte dysfunction as well as tissue matrix remodelling may be other possible aetiologies that contribute to the pathogenesis of this condition. We speculate that the gut undergoes irreversible vascular and lymphatics changes due to the congenital abnormalities in hemodynamics.We also wonder if the high intraluminal protein and Ig levels have a detrimental effect on the microbiome hence leading to secondary autoimmune disease. None

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