Congestive hepatopathy after Fontan operation and related factors assessed by transient elastography.

OBJECTIVE: Congestive hepatopathy after a Fontan operation can have a major effect on long-term morbidity. We evaluated congestive hepatopathy in patients with Fontan circulation using transient elastography to determine which risk factors for hepatopathy are related to liver stiffness (LS).

METHODS: We evaluated 46 patients with Fontan circulation and 26 with right side heart failure (RHF) and hepatic congestion using laboratory tests, the aspartate aminotransferase-to-platelet ratio index, ultrasonography, and transient elastography.

RESULTS: The LS value was significantly greater in the Fontan group (21.1±8.0 kPa) than in the RHF group (10.0±9.0 kPa). The total bilirubin and albumin serum levels, white blood cell count, and aspartate aminotransferase to platelet ratio index correlated significantly with LS in the Fontan group. Of the risk factors, age at evaluation (r=0.42, P=.004), age at Fontan completion (r=0.51, P<.001), inferior vena cava diameter (r=0.35, P=.02), and spleen size (r=0.53, P=.002) correlated significantly with LS. Nineteen patients in the Fontan group (41.3%) had abnormal ultrasound findings, and the frequency of abnormal findings increased with increasing LS (P=.002). In the subgroup with the greatest LS value (≥30 kPa), 88.9% had abnormal ultrasound findings and 44.4% liver cirrhosis. Multivariate analysis revealed that age at Fontan procedure completion and total bilirubin were independent risk factors for hepatopathy.

CONCLUSIONS: The present study revealed that congestive hepatopathy developed in a significant fraction of patients with long-term Fontan circulation and that transient elastography could be an easy and useful method to assess congestive hepatopathy in these patients.