Jacobs M L, Rychik J, Byrum C J, Norwood W I
Division of Cardiothoracic Surgery, Childrens Hospital of Philadelphia, Pennsylvania 19104, USA.
Ann Thorac Surg. 1996 Jan;61(1):206-8. doi: 10.1016/0003-4975(95)00659-1.
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.
