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Ann Thorac Surg 1996;61:206-208
© 1996 The Society of Thoracic Surgeons

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Case Report

Protein-Losing Enteropathy After Fontan Operation: Resolution After Baffle Fenestration

Divisions of Cardiothoracic Surgery and Cardiology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania

Accepted for publication July 5, 1995.

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.

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