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Ann Thorac Surg 2006;82:e39-e40
© 2006 The Society of Thoracic Surgeons

Case Reports

Resolution of Protein-Losing Enteropathy and Normalization of Mesenteric Doppler Flow With Sildenafil After Fontan

Orhan Uzun, MDa,*, Joon Kwang Wong, MBBSa, Vinaj Bhole, MRCPb, Oliver Stumper, PhD, MDb

a Department of Pediatric Cardiology, University Hospital of Wales, Cardiff, United Kingdom
b Department of Pediatric Cardiology, Birmingham Children’s Hospital, Birmingham, United Kingdom

Accepted for publication August 23, 2006.

* Address correspondence to Dr Uzun, University Hospital of Wales, Department of Paediatric Cardiology, Heath Park, Cardiff, CF 14 4XW, United Kingdom (Email: uzun{at}cf.ac.uk).

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.






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