Protein-losing enteropathy after Fontan operation: resolution after baffle fenestration.
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.
Jacobs
M L
ML
Division of Cardiothoracic Surgery, Childrens Hospital of Philadelphia, Pennsylvania 19104, USA.
Rychik
J
J
Byrum
C J
CJ
Norwood
W I
WI
D001201:Ascites / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D004487:Edema / Q000209:etiology; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects*; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000601:surgery; D011504:Protein-Losing Enteropathies / Q000209:etiology* / Q000628:therapy; D012086:Reoperation; D018785:Tricuspid Atresia / Q000601:surgery