The present study shows that patients who undergo the Fontan procedure are at increased risk of developing liver fibrosis and liver cirrhosis. The risk increases with the age of the patient and the time interval since the Fontan procedure. The noninvasive measurement of liver fibrosis using transient elastography and fibrosis marker scores can be a useful tool to identify patients at risk and for noninvasive surveillance.

In 15 pigs an auxiliary liver was transplanted and cholescintigraphy with 99mTc-diethyl-IDA was performed to study the function of the livers separately. Serial scintigraphy of the recipient and donor liver was performed on days 3, 8 and 18 after transplantation and time-activity curves over 1 h after injection of the radio-tracer were generated for each liver. From these data the time at which the maximal activity was present in the liver (Tmax) and the time at which during the excretion phase half of this activity was still present in the organ (T1/2) were determined. Liver biopsy material from the recipient liver and the donor liver was obtained at (approximately) the day of one of the scintigraphic examinations and at autopsy. In all cases no histological abnormalities were found in the recipient liver. In 4 pigs there were no histological signs of rejection of the donor liver, in which cases the values for Tmax and T1/2 were normal. In 11 pigs histological signs of rejection were noticed and in all of these cases T1/2 was significantly prolonged, whereas no changes in Tmax were observed. Cholescintigraphy revealed an abnormal value for T1/2 a few days before the biopsy was taken in 10 of these 11 cases. Normal functioning or the prediction of rejection of the auxiliary liver transplant in pigs correlated significantly (P less than 0.01) with normal or prolonged T1/2 values. It is concluded that cholescintigraphy may be helpful in establishing the diagnosis of rejection of liver transplants.