A patient who has had the Fontan operation for congenital heart disease may develop a protein-losing enteropathy.
Patient selection: history of Fontan operation
Risk factors may include:
(1) right-ventricular morphology with hypoplastic left heart
(2) older age at the time of the Fontan procedure
(3) pleural effusions after the procedure
Clinical features:
(1) chronic diarrhea
(2) abdominal pain
(3) ascites
(4) elevated central venous pressure
(5) pleural effusions
(6) protein malnutrition
(7) peripheral edema
Laboratory findings may include:
(1) hypoalbuminemia
(2) elevated stool alpha-1 antitrypsin
Associated complications of the Fontan procedure may include:
(1) advanced liver disease
(2) plastic bronchitis
(3) gastrointestinal bleeding
(4) heart failure
