Protein-Losing Enteropathy after Fontan Operation
- 1 September 2007
- journal article
- review article
- Published by Computers, Materials and Continua (Tech Science Press) in Congenital Heart Disease
- Vol. 2 (5), 288-300
- https://doi.org/10.1111/j.1747-0803.2007.00116.x
Abstract
Protein-losing enteropathy (PLE) is a poorly understood and enigmatic disease process affecting patients with single ventricle after Fontan operation. In those afflicted, PLE after Fontan operation results in significant morbidity and mortality. The pathophysiology of the disease is unknown; however, a proposed mechanism incorporates a combination of phenomena including: (1) altered hemodynamics, specifically low cardiac output; (2) increased mesenteric vascular resistance; (3) systemic inflammation; and (4) altered enterocyte basal membrane glycosaminoglycan make-up. A paradigm for the clinical management of PLE after Fontan operation is proposed.Keywords
This publication has 58 references indexed in Scilit:
- Intermediate outcomes after the Fontan procedure in the current eraThe Journal of Thoracic and Cardiovascular Surgery, 2006
- Long-Term Results of the Fontan Operation for Double-Inlet Left VentricleThe American Journal of Cardiology, 2005
- Fecal α1-Antitrypsin Concentrations as a Measure of Enteric Protein Loss After Modified Fontan OperationsJournal of Pediatric Gastroenterology and Nutrition, 2003
- Atrial pacing: An alternative treatment for protein-losing enteropathy after the Fontan operationThe Journal of Thoracic and Cardiovascular Surgery, 2001
- Reduced Heparan Sulfate Accumulation in Enterocytes Contributes to Protein-Losing Enteropathy in a Congenital Disorder of GlycosylationThe American Journal of Pathology, 2000
- Reversal of protein losing enteropathy with prednisone in adults with modified Fontan operations: long term palliation or bridge to cardiac transplantation?Heart, 1999
- Protein-losing enteropathy after the Fontan operation: An international multicenter studyThe Journal of Thoracic and Cardiovascular Surgery, 1998
- Protein-losing enteropathy after the Fontan operationThe Journal of Thoracic and Cardiovascular Surgery, 1996
- Outcome after a "perfect" Fontan operation.Circulation, 1990
- Tricuspid regurgitationAmerican Journal Of Medicine, 1968