Protein-losing enteropathy after Fontan operation: resolution after baffle fenestration

Ann Thorac Surg. 1996 Jan;61(1):206-8. doi: 10.1016/0003-4975(95)00659-1.

Abstract

A 4-year-old child with hypoplastic left heart syndrome and a 6-year-old child with tricuspid atresia had both undergone staged reconstructive operations culminating in a Fontan operation. Peripheral edema, ascites, and hypoalbuminemia refractory to dietary manipulation and steroid therapy developed in both patients. After hemodynamic assessment, each child underwent surgical creation of a 4.8-mm fenestration in the previously placed baffle that separated the systemic venous pathway from the pulmonary venous atrium. Peripheral edema and ascites promptly resolved and serum protein levels normalized within 2 weeks after operation. Systemic arterial saturation is 86% in each child, and both children remain clinically well with no evidence of protein-losing enteropathy on normal diets and without specific medical therapy.

Publication types

  • Case Reports

MeSH terms

  • Ascites / etiology
  • Child
  • Child, Preschool
  • Edema / etiology
  • Female
  • Fontan Procedure / adverse effects*
  • Humans
  • Hypoplastic Left Heart Syndrome / surgery
  • Protein-Losing Enteropathies / etiology*
  • Protein-Losing Enteropathies / therapy
  • Reoperation
  • Tricuspid Atresia / surgery