Copyright © 1998 by the European Society of Cardiology.
Gastro-intestinal protein loss in late survivors of Fontan surgery and other congenital heart disease
a Grown up Congenital Heart Disease Unit, Department of Biochemistry, National Heart and Lung Institute and Royal Brompton Hospital, London, U.K.
b Department of Biochemistry, National Heart and Lung Institute and Royal Brompton Hospital, London, U.K.
accepted September 5, 1997
Aims
Protein losing enteropathy is a serious complication of Fontan surgery. The aim of this study was to investigate gastro-intestinal protein loss in adults with congenital heart disease, both with and without Fontan surgery, and to correlate findings with systemic venous pressure.
Methods and Results
Forty eight patients were studied. The first group included adult survivors of Fontan surgery. The second and third groups were control patients with congenital heart disease who had not had Fontan surgery and had either normal or chronically elevated systemic venous pressure. Gastro-intestinal protein loss was assessed by measurement of faecal 
-1-antitrypsin. Faecal -1-antitrypsin levels were significantly higher in the Fontan group (0·55±0·15mg.g–1faeces, P=0·002) and the control group with chronically elevated venous pressure (0·60±0·30mg.g–1faeces, P<0·001) compared to the controls with normal venous pressure (0·29±0·12mg.g–1faeces). Of the 15 subjects who were found to have increased gastro-intestinal protein loss, only four had clinical protein-losing enteropathy. The degree of gastro-intestinal protein loss correlated significantly with venous pressure (P=0·01) and with serum aspartate transaminase (P=0·04).
Conclusion
Increased gastro-intestinal protein loss is common in this select group of late survivors of Fontan surgery and in other subjects with congenital heart disease and chronic elevation of systemic venous pressure, and was present in patients who did not have protein-losing enteropathy. Increased faecal -1-antitrypsin is an important finding in these patients as intervention at this stage, before the onset of florid protein-losing enteropathy, might prevent the development of further complications.
Key Words: Fontan • protein losing enteropathy • late results
f1 Correspondence: Dr Sara Thorne, Grown Up Congenital Heart Disease Unit, Royal Brompton Hospital, Sydney Street, London SW3 6NP, U.K.
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