Menon Shaji, Hagler Donald, Cetta Frank, Gloviczki Peter, Driscoll David
Department of Pediatrics, Division of Pediatric Cardiology, Mayo Clinic College of Medicine, Mayo Clinic and Foundation, Rochester, MN 55905, USA.
Cardiol Young. 2008 Jun;18(3):275-81. doi: 10.1017/S1047951108001996. Epub 2008 Mar 7.
Protein-losing enteropathy has been associated with the Fontan operation, restrictive cardiomyopathy, constrictive pericarditis, tricuspid valvar stenosis and insufficiency, and superior caval venous obstruction. The mechanism of development of this complication of cardiac disease likely is multifactorial. We report here our experience with 3 patients with protein-losing enteropathy. Two had superior caval venous occlusion. The third patient had undergone an extracardiac Fontan operation, with stenosis of the extracardiac conduit placed to the right pulmonary artery. In all three cases, the complication was resolved by restoring unobstructed flow of blood in the superior or inferior caval vein. Since the aetiology of protein-losing enteropathy associated with cardiac disease is multifactorial, reduction of pressure in the tributaries of the superior caval vein may, in some cases, be curative. It is more difficult to cure the problem when it is associated with the Fontan operation.
