GASTROINTESTINAL PROTEIN LOSS IN LATE SURVIVORS OF FONTAN SURGERY ANDOTHER CONGENITAL HEART-DISEASE

Aims Protein losing enteropathy is a serious complication of Fontan su rgery. The aim of this study was to investigate gastro-intestinal prot ein loss in adults with congenital heart disease, both with and withou t Fontan surgery, and to correlate findings with systemic venous press ure. 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 no t had Fontan surgery and had either normal or chronically elevated sys temic venous pressure. Gastro-intestinal protein loss was assessed by measurement of faecal alpha-1-antitrypsin. Faecal alpha-1-antitrypsin levels were significantly higher in the Fontan group (0.55+/-0.15 mg.g (-1) faeces, P=0.002) and the control group with chronically elevated venous pressure (0.60+/-0.30 mg.g(-1) faeces, P=0.001) compared to the controls with normal venous pressure (0.29+/-0.12 mg.g(-1) faeces). O f the 15 subjects who were found to have increased gastro-intestinal p rotein loss, only four had clinical protein-losing enteropathy. The de gree of gastro-intestinal protein loss correlated significantly with v enous pressure (P=0.01) and with serum aspartate transaminase (P=0.04) . Conclusion Increased gastro-intestinal protein loss is common in thi s select group of late survivors of Fontan surgery and in other subjec ts with congenital heart disease and chronic elevation of systemic ven ous pressure, and was present in patients who did not have protein-los ing enteropathy. Increased faecal alpha-1-antitrypsin is an important finding in these patients as intervention at this stage, before the on set of florid protein-losing enteropathy, might prevent the developmen t of further complications.