Congestive hepatopathy usually appears and develops slowly after a Fontan operation, often without obvious clinical features, but it may lead to life-threatening complications.

We aim to assess the potential usefulness of the liver stiffness (LS) in the longitudinal follow up of Fontan patients.

Patients were prospectively evaluated using clinical examination, laboratory tests, echocardiography and LS using transient elastography (TF). This work up was made annually or in case of clinical complication.

Forty-six patients (22.1±8,1 years of age and 9.7±6.5 years post-Fontan) were enrolled. Mean time between first and last work up was 3.27±1.9 years. During this period clinical complication occurred in 12/46 (26%) including 6 arrhythmias, 2 cirrhosis, 1 steato-hepatitis, 3 exudative enteropathies and 2 venous-collateral. Mean LS at baseline was 14±7.4kPa. LS was significantly higher in patient with complications compared to those who were free from any complication (17.2±7.7 vs. 13.8±5.9kPa, P=0.019). No significant change in stiffness was also observed with age (P=0.73), time since Fontan (P=0.64), presence fenestration (15±6.8 vs. 15.1±6.7kPa, P=0.82) or ventricular morphology (15±13.1 vs. 16±14.1 vs. 10.9±11.6kPa, P=0.09).

Fibroscan appears to be a good tool for the non-invasive follow-up of FP. Indeed, a significate elevation of the LS is associated with the occurrence of complications.