Published online Mar 21, 2025. doi: 10.3748/wjg.v31.i11.103178
Revised: January 12, 2025
Accepted: February 25, 2025
Published online: March 21, 2025
Processing time: 122 Days and 15.6 Hours
Fontan-associated liver disease (FALD) often occurs in patients with single-ventricle physiology following Fontan surgery, and ranges from liver congestion to cirrhosis. The assessment of the severity of FALD using noninvasive methods is challenging. However, transient elastography (TE) may be useful for the non
To evaluate the role of TE in the diagnosis of FALD and its association with clinically relevant events.
This retrospective single-center study (Hospital Universitario La Paz, Madrid), including 91 post-Fontan patients aged > 18 years old. Laboratory and ultrasound findings, and liver stiffness measurements (LSM) by TE (FibroScan®) were assessed. FALD was defined using ultrasound criteria (hepatomegaly, liver surface nodularity, parenchymal heterogeneity, hyperechoic lesions, splenomegaly, collaterals) and advanced FALD was defined according to the European Association for the Study of the Liver-European Reference Network statement (esophageal varices, portosystemic shunts, ascites, splenomegaly). Clinically relevant events included heart or heart-liver transplantation indication, hepatocellular carcinoma, and all-cause mortality.
Patient characteristics were: 60.4% male; Mean age, 33.3 ± 8.2 years; Mean elapsed time since surgery, 24.3 ± 7.7 years; 89% with FALD; 73% with advanced FALD. LSM by TE was associated with FALD [odds ratio (OR) = 1.34; 95% confidence interval (95%CI): 1.10-1.64; P = 0.003] and advanced FALD (OR = 1.10; 95%CI: 1.01-1.19; P = 0.023). Areas under the curve (AUC) were 0.905 and 0.764 for FALD and advanced FALD, respectively. FALD cut-off values comprised: Optimal, 20 kPa (sensitivity: 92.3%; specificity: 80.0%); Rule-out, 15 kPa (sensitivity: 96.9%); Rule-in, 25 kPa (specificity: 100%). A FALD algorithm was proposed based on LSM by TE and elapsed time since surgery (AUC: 0.877; sensitivity, 95.4%; specificity, 80.0%; positive predictive value, 96.9%; negative predictive value, 72.7%). LSM by TE was associated with clinically relevant events (OR = 1.07; 95%CI: 1.01-1.13; P = 0.021) and all-cause mortality (OR = 1.23; 95%CI: 1.02-1.47; P = 0.026).
In adult patients post-Fontan surgery, TE is a useful noninvasive method for FALD diagnosis. The association between LSM by TE and clinically relevant events suggests a role in prognosis.
Core Tip: There was a significant association between liver stiffness measurement (LSM) by transient elastography (TE) and Fontan-associated liver disease (FALD). We obtained areas under the curve (AUC) of 0.905 for FALD and 0.764 for advanced FALD. The optimal cut-off values were 20 kPa for FALD and 25 kPa for advanced FALD. An algorithm for FALD was proposed based on LSM by TE and elapsed time since Fontan surgery with an AUC of 0.877. We also demonstrated an association between LSM by TE and clinically relevant events (heart or heart-liver transplantation indication, hepatocellular carcinoma, and all-cause mortality).