DOI: http://dx.doi.org/10.18203/2320-6012.ijrms20194623

An unusual cause of edema in a child with congenital heart disease post Fontan procedure

Minal Wade, Shweta Shettiwar, Ankita Shah

Abstract


Protein Losing Enteropathy Post Fontan procedure. Protein Losing Enteropathy (PLE) is an uncommon cause of edema in children with congenital heart disease. Protein-Losing Enteropathy may be defined as excessive loss of proteins across the intestinal mucosa and is due to either a primary gastrointestinal abnormality or secondary to cardiac disease. Protein-losing enteropathy (PLE) is a rare complication of the Fontan palliation for functional single-ventricle. Although PLE occurs in about 3.5% of patients post-Fontan, it confers marked morbidity and high mortality within 5 years of diagnosis. The pathogenesis of Fontan-related PLE is not completely understood, and it is unclear why it develops in some patients post-Fontan and not others. We describe a child with Double Inlet Right Ventricle who had undergone Fontan procedure, and presented to us with generalised oedema. The child had hypoproteinaemia, the common causes for which were ruled out and was diagnosed as Protein Losing Enteropathy (PLE) related to his surgical intervention. Though, not frequently encountered it should be kept in mind as one of the causes of anasarca.


Keywords


Alpha -1- antitrypsin, Congenital heart disease, Edema, Fontan procedure, Heparin, Lymphatic pressure, Protein Losing Enteropathy, Steroids

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References


Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax. 1971 May 1;26(3):240-8.

Davidson J, Waldmann TA, Goodman DS, GORDON Jr R. Protein-losing 48 gastroenteropathy in congestive heart-failure. Lancet. 1961;1:899-902.

Wilkinson P, Pinto B, Senior JR. Reversible protein-losing enteropathy with intestinal lymphangiectasia secondary to chronic constrictive pericarditis. N Eng J Med. 1965 Nov 25;273(22):1178-81.

Feldt RH, Driscoll DJ, Offord KP, Cha RH, Perrault J, Schaff HV, et al. Protein-losing enteropathy after the Fontan operation. J Thoracic Cardiovascular Surgery. 1996 Sep 1;112(3):672-80.

Giannico S, Hammad F, Amodeo A, Michielon G, Drago F, Turchetta A, et al. Clinical outcome of 193 extracardiac Fontan patients: the first 15 years. J Am Coll Cardiol. 2006 May 16;47(10):2065-73.

Hess J, Kruizinga K, Bijleveld CM, Hardjowijono R, Eygelaar A. Protein-losing enteropathy after Fontan operation. J Thorac Cardiovasc Surg. 1984 Oct;88(4):606-9.

Rychik J, Piccoli DA, Barber G. Usefulness of corticosteroid therapy for protein-losing enteropathy after the Fontan procedure. Am J Cardiol. 1991 Sep 15;68(8):819-21.

Ringel RE, Peddy SB. Effect of high-dose spironolactone on protein-losing enteropathy in patients with Fontan palliation of complex congenital heart disease. Am J Cardiol 2003;91(8):1031-2.

Castaneda AR. From Glenn to Fontan. A continuing evolution. Circulation. 1992 Nov;86(5 Suppl):1180-4.

Bejiqi R, Retkoceri R, Zeka N, Bejiqi H, Vuqiterna A, Maloku A. Treatment of Children with Protein–Losing Enteropathy After Fontan and Other Complex Congenital Heart Disease Procedures in Condition with Limited Human and Technical Resources. Materia socio-medica. 2014 Feb;26(1):39-42.

Khambadkone S. The Fontan pathway: What's down the road?. Annals of pediatric cardiology. 2008 Jul;1(2):83-92.

Agnoletti G, Borghi A, Vignati G, Crupi GC. Fontan conversion to total cavopulmonary connection and arrhythmia ablation: clinical and functional results. Heart. 2003 Feb 1;89(2):193-8.