Shear-wave elastography for monitoring Fontan-associated liver disease: A prospective cohort study

The spectrum of Fontan-associated liver disease (FALD) varies from abnormal liver function tests to fibrosis and even cirrhosis. In this prospective study, we evaluated the role of shear-wave elastography (SWE) in predicting the presence of advanced FALD. Forty-eight patients (30 males, 13.9 [6-21] years) with a Fontan circulation were evaluated at 8.3 (2.1-18.7) years since the Fontan surgery. The median liver stiffness measurement (LSM) value was higher than values in normal children at 15.4 (9.5-38.7) kPa. The LSMs had a weak but significant correlation with age at the time of LSM (r = 0.25, p = 0.01) and duration post-Fontan surgery (r = 0.31, p = 0.02). It had a poor correlation with the concomitant aspartate transaminase-to-platelet ratio index (r = 0.1, p = 0.39). No difference in the elastography values between children with and without ultrasound evidence of advanced liver disease (17.7 [interquartile range, IQR: 4] vs. 16.1 [IQR: 6], p = 0.62] was observed. Further studies are required to determine the precise role of SWE as a noninvasive marker of liver fibrosis in FALD. What is Known The spectrum of Fontan-associated liver disease (FALD) may vary from abnormal liver function tests to fibrosis and even cirrhosis. The role of elastography for the monitoring of FALD is unclear.What is New The median liver stiffness measurement value in children with FALD is much higher than values in normal children. There is a poor correlation between elastography values and ultrasound evidence of advanced liver disease in this population. Further studies are required to determine the precise role of shear-wave elastography as a noninvasive marker of liver fibrosis in FALD.