Related Experiment Video
Updated: Feb 1, 2026
Detection of a Circulating MicroRNA Custom Panel in Patients with Metastatic Colorectal Cancer
Published on: March 14, 2019
Non-invasive biomarkers of liver disease as prognostic indicators in patients with Fontan circulation
Richard C Collier1, Surabhi Sajith1, Jordan S Sack2
1Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts, USA.
Insights
Biomarkers of liver fibrosis, including FibroSURE, APRI, FIB-4, and MELD-XI, can predict transplant-free survival in adults with Fontan circulation. Higher scores indicate an increased risk of death or transplant.
Area of Science:
- Cardiology
- Hepatology
- Transplantation Medicine
Background:
- The Fontan procedure, while life-saving, is linked to significant long-term cardiac and non-cardiac complications, including early mortality.
- Emerging evidence suggests that liver fibrosis scores may provide prognostic value for patients with Fontan circulation.
Purpose of the Study:
- To evaluate the predictive capability of liver fibrosis biomarkers for transplant-free survival in adults with Fontan circulation.
- To determine if non-invasive fibrosis scores can identify individuals at higher risk of adverse outcomes post-Fontan procedure.
Main Methods:
- A retrospective observational cohort study included 334 adults with Fontan circulation undergoing FibroSURE testing between 2008 and 2022.
- FibroSURE scores, Aminotransferase-to-Platelet Ratio Index (APRI), fibrosis-4 (FIB-4), and Model for End-stage Liver Disease eXcluding INR (MELD-XI) were analyzed against clinical outcomes like death and transplantation.
Main Results:
- Higher FibroSURE scores were significantly associated with an increased hazard of death or transplant, with scores above 0.74 indicating a 3.26-fold increased risk.
- APRI, FIB-4, and MELD-XI scores also demonstrated associations with increased hazard of death or transplant in patients with Fontan circulation.
- Specifically, FibroSURE >0.74, APRI >0.5, FIB-4 >1.45, and MELD-XI >18 were all linked to poorer transplant-free survival.
Conclusions:
- Elevated levels of FibroSURE, APRI, FIB-4, and MELD-XI are significant predictors of adverse outcomes in individuals with Fontan circulation.
- These non-invasive liver fibrosis biomarkers can aid in risk stratification and prognostication for Fontan patients.
- The findings underscore the importance of monitoring liver health in the Fontan population to anticipate and manage potential complications.
Background:
The Fontan procedure is associated with long-term cardiac and non-cardiac complications and early mortality. Recent data suggest that some fibrosis scores may offer prognostic insight for individuals with Fontan circulation. This study aimed to investigate whether biomarkers of liver fibrosis can predict transplant-free survival in Fontan circulation.
Methods:
This was a retrospective observational cohort study of 334 adults with Fontan circulation who had FibroSURE testing between 2008 and 2022 at a large-volume adult congenital heart disease centre. FibroSURE score, Aminotransferase-to-Platelet Ratio Index (APRI), fibrosis-4 (FIB-4) and Model for End-stage Liver Disease eXcluding INR (international normalised ratio) (MELD-XI) were recorded and analysed against clinical outcomes.
Results:
There were 664 FibroSURE results during the study period with 83 (13%) above the nominal cut-off predicting cirrhosis (>0.74). There were 37 deaths (11%) and four transplants (1%) over a median follow-up of 5.6 years (IQR 2.7-9.9). After adjusting for age at FibroSURE and history of hepatitis C, each 0.1 increase in FibroSURE score remained associated with a 1.19-fold increased hazard of death or transplant (95% CI 1.02 to 1.40, p=0.032), and FibroSURE >0.74 remained associated with a 3.26-fold increased hazard of death or transplant (95% CI 1.62 to 6.59, p=0.001). In 314 patients where FibroSURE, APRI, FIB-4 and MELD-XI were all available, FibroSURE >0.74 (HR 5.11, 95% CI 2.66 to 9.83, p<0.001), APRI >0.5 (HR 2.56, 95% CI 1.36 to 4.81, p=0.004), FIB-4 >1.45 (HR 5.07, 95% CI 2.67 to 9.64, p<0.001) and MELD-XI >18 (HR 24.1, 95% CI 5.51 to 106, p<0.001) were all associated with increased hazard of death or transplant.
Conclusions:
Higher FibroSURE, APRI, FIB-4 and MELD-XI are associated with increased hazard of death or transplant in Fontan circulation.
More Related Videos
06:37Electroencephalography Network Indices as Biomarkers of Upper Limb Impairment in Chronic Stroke
Published on: July 14, 2023
05:16Cutoff Value of Phase Angle by Bioelectrical Impedance Analysis at Admission as a Prognostic Factor in Patients with Acute Heart Failure
Published on: June 10, 2025
Related Concept Videos
02:39Indicators
01:26Diseases of the Liver and Gallbladder
Cirrhosis is characterized by the scarring of hepatic lobules in the liver, which are replaced by fibrous tissue, affecting the liver's normal functioning. NAFLD, on the other hand, is caused by an excessive build-up of fat in the liver, not...
01:22Effect of Hepatic Disease on Pharmacokinetics: Pathophysiologic Assessment and Liver Function Test
01:21Coronary Circulation
Coronary circulation begins at the base of the aorta, where two main arteries arise—the left and right coronary arteries. These arteries encircle the heart in the coronary sulcus and supply the...
01:14Fetal Circulation
Two umbilical arteries transport blood from the fetus to the placenta. At the placenta, the blood absorbs oxygen and nutrients while simultaneously eliminating waste products. This oxygen-enriched and nutrient-rich blood then returns to the fetus through one...
01:19Overview of Pulmonary Circulation
The process begins with the right ventricle of the heart pumping deoxygenated blood into the pulmonary trunk. This large vessel extends about 5 centimeters before splitting into the left and right pulmonary arteries. These arteries...