Liver diseases usually appears after a Fontan operation (FO), often without obvious clinical features, but it may lead to life-threatening complications.

We aim to assess the usefulness of the liver stiffness (LS), assessed by transient elastography (TE), in the follow-up of Fontan patients.

In our center, patients with a Fontan circulation (FC) were prospectively evaluated since 2012 through an annual work up including physical examination, laboratory tests, transthoracic echocardiography and TE. This work up was also performed in case of clinical complications, which were classified as follows: cardiac complications (including arrhythmias or catheter interventions) and subdiaphragmatic complications (including clinical sign of portal hypertension(PH) or protein losing enteropathy (PLE)).

Forty-eight patients (21.7±8.2 years of age and 9.7±6.5 years post-Fontan) were included, 28 of them (58%) had least two LS measurement. Mean time between first and last LS measurements was 3.27±1.9 years. Mean LS at baseline was 15.3±6.9kPa (4.3–47.2kPa) No correlation was found between LS and age (r=1, P=0.73), or time since FO (r=0.3, P=0.64). LS did not vary regarding the presence of a fenestration (15±6.8 vs. 15.1±6.7kPa, P=0.82). During the follow-up, a clinical complication occurred in 19 patients (39.6%) including 8 cardiac complications and 11 subdiaphragmatic. Among the subdiaphragmatic complications group, 3 had PLE and 8 developed PH. LS was significantly higher in patient with liver complication (17.2±7.7 vs. 13.8±5.9, P<0.01). Patient with an increasing LS value during the follow-up had a higher complications rate than patients with a decreasing or unchanged LS (4/11 (36%) vs. n=5/17 (29%); P<0.04).

LS measurement using TE is a good tool for the non-invasive follow-up of patient with FC. Indeed, a significate elevation of the LS is associated with the occurrence of liver and/or cardiac complications.