Recurrent Protein-losing Enteropathy and Tricuspid Valve Insufficiency in a Transplanted Heart: A Causal Relationship?

Division of Pediatric Cardiology Department of Cardiovascular Surgery, Children's Hospital of Michigan, 3901 Beaubien Blvd., Detroit, MI 48201, USA.
Congenital Heart Disease (Impact Factor: 1.08). 07/2011; 7(3):E10-3. DOI: 10.1111/j.1747-0803.2011.00557.x
Source: PubMed


This case report describes a toddler who developed a protein-losing enteropathy (PLE) 4 years after orthotopic heart transplantation (OHT). He was born with a hypoplastic left heart syndrome for which he underwent a successful Norwood procedure, a Hemi-Fontan palliation, and a Fontan palliation at 18 months of age. Fifteen months following the Fontan operation, he developed a PLE and Fontan failure requiring OHT. Four years after OHT, he developed a severe tricuspid regurgitation and a PLE. His PLE improved after tricuspid valve replacement. It is now 2 years since his tricuspid valve replacement and he remains clinically free of ascites and peripheral edema with a normal serum albumin level. His prosthetic tricuspid valve is functioning normally.

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