title: Resolution of protein-losing enteropathy and normalization of mesenteric Doppler flow with sildenafil after Fontan.
abstract: A 9.5-year-old girl after Fontan procedure for hypoplastic left heart syndrome had recurrent protein-losing enteropathy (PLE) develop 2 months after partial catheter closure of the Fontan fenestration. Despite satisfactory hemodynamic measurements under general anesthesia, we postulated that she suffered from increased pulmonary vascular reactivity and commenced her on Sildenafil treatment. After 6 weeks of oral Sildenafil treatment, her serum albumin and the fecal alpha-1-antitrypsin levels normalized, and her exercise tolerance had increased. There was also an improvement of the mesenteric arterial flow patterns on Doppler studies. Sildenafil should be considered in the treatment of PLE after the Fontan procedure.
authors.affiliation: Department of Pediatric Cardiology, University Hospital of Wales, Cardiff, United Kingdom. uzun@cf.ac.uk
authors.forename: Orhan
authors.initials: O
authors.lastname: Uzun
authors.forename: Joon Kwang
authors.initials: JK
authors.lastname: Wong
authors.forename: Vinaj
authors.initials: V
authors.lastname: Bhole
authors.forename: Oliver
authors.initials: O
authors.lastname: Stumper
mesh_terms: D002648:Child; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects*; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000601:surgery; D010879:Piperazines / Q000627:therapeutic use*; D011504:Protein-Losing Enteropathies / Q000188:drug therapy* / Q000209:etiology; D011687:Purines; D000068677:Sildenafil Citrate; D013450:Sulfones; D014665:Vasodilator Agents / Q000627:therapeutic use*
chemical_list: D010879:Piperazines; D011687:Purines; D013450:Sulfones; D014665:Vasodilator Agents; D000068677:Sildenafil Citrate