138 - Non-invasive liver fibrosis scores at 10 and 20 years after Fontan procedure predict the outcomes of patients with Fontan procedure.

Publication Number: 138.5775

Chaowapong Jarasvaraparn, Riley Hospital for Children, Indianapolis, IN, United States; Anthony Perkins, Indiana University School of Medicine, Indianapolils, IN, United States; Andrew Rodenbarger, Riley Hospital - Indianapolis, IN, Indianapolis, IN, United States; Jessica Thoe, Indiana University School of Medicine, Indianapolis, IN, United States; Ronald M.. Payne, Indiana University School of Medicine, Indianapolis, IN, United States; Larry W.. Markham, Riley Hospital for Children at Indiana University Health, Indianapolis, IN, United States; Jean P. Molleston, Indiana University School of Medicine, Indianapolis, IN, United States

Background: Fontan-associated liver disease (FALD) refers to post-Fontan patients who develop progressive hepatic fibrosis and cirrhosis secondary to hemodynamic consequences of Fontan circulation. FALD impact on survival remains controversial and current diagnostic tools have limitations.

Objective: We aimed to assess the prognostic outcome role of liver fibrosis scores (aminotransferase to platelet ratio [APRI] and fibrosis-4 [FIB-4]) of all Fontan patients at 10 and 20 years after Fontan procedure.

Design/Methods: This was a retrospective, single-center, cohort study of children and adults who had laboratory evaluation at least 10 years after Fontan procedure. Non-invasive liver fibrosis scores were calculated as: APRI=([AST/40]/platelet count [109/L]) *100; FIB-4 = (age [years]*AST)/(platelet count [109/L]*√ALT). Composite outcomes including death, failing Fontan physiology (exercise intolerance, heart failure, ascites, protein losing enteropathy, and plastic bronchitis), and heart transplantation were investigated. Univariate Cox regression models were built to analyze the association between APRI and FIB-4 with all-cause mortality and composite outcomes.

Results: We included 98 composite outcomes-free participants at 10 years and 115 composite outcomes-free participants at 20 years after Fontan procedure. At 10 years after Fontan procedure, 3 patients died, and 8 patients had composite outcomes during subsequent follow-up. APRI was associated with overall mortality (HR 5.19 [1.58–16.99] per unit increase, p=0.007). APRI > 0.7 remained associated with overall mortality (11.65 [1.06-128.6, p = 0.011]) and composite outcomes (4.47 [0.99, 20.02, p = 0.031]). At 20 years after Fontan procedure, 7 patients died, and 16 patients had composite outcomes during subsequent follow-up. APRI > 0.7 (12.36 [2.29-66.7, p = 0.003] and FIB-4 > 1.45 (14 [2.6-74, p = 0.002]) were associated with overall mortality. Lastly, APRI > 0.7 (17.52 [5.07-60.5, p = 0.001]) and FIB-4 > 1.45 (22.62 [6.59-77.6, p = 0.001]) were also associated with composite outcomes.

Conclusion(s): This is the first study investigating longitudinal non-invasive liver fibrosis scores in patients following Fontan procedure to predict outcomes at 10 and 20 years. APRI and FIB-4 scores are associated with long-term all-cause mortality and composite outcomes in patients with Fontan procedure. APRI and FIB-4 scores might serve as non-invasive tools for risk stratification and prognostication of outcomes in patients with FALD.

Figure 1 Survival stratified by an APRI cut-off value of >0.7 at 10 years after Fontan procedure with death (p=0.011) and the composite outcome (0.031).
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