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The prognostic role of liver volumetry in Fontan patients
Khalid Al Najashi1, Nejoud Al Najashi2, Taghreed Ahmed1
1Pediatric Cardiology Department, Prince Sultan Cardiac Center, Riyadh, Saudi Arabia.
Insights
Elevated liver volume in Fontan patients indicates significant congestion and predicts adverse outcomes, including mortality and transplantation. This finding highlights liver volumetry as a key biomarker for Fontan-associated liver disease management.
Area of Science:
- Cardiology
- Hepatology
- Medical Imaging
Background:
- High venous pressures and hepatic congestion are primary drivers of Fontan-associated liver disease.
- The prognostic value of hepatomegaly as a congestion marker in Fontan patients requires further definition.
Purpose of the Study:
- To investigate the prognostic significance of liver volume as a marker of hepatic congestion in Fontan patients.
- To determine if liver volumetry can predict adverse outcomes following the Fontan operation.
Main Methods:
- Retrospective analysis of liver ultrasound scans from 199 Fontan patients.
- Liver volumes were determined and divided into tertiles for analysis.
- Outcomes included mortality, heart/liver transplantation, arrhythmia, and protein-losing enteropathy.
Main Results:
- Patients with the largest liver volumes (highest tertile) had a lower rate of functioning fenestration.
- The highest liver volume tertile was associated with increased mortality and transplantation rates (p=0.016).
- Liver volumes predicted combined adverse outcomes with 61% accuracy (p=0.009).
Conclusions:
- Liver volumetry shows potential as a significant biomarker for predicting adverse outcomes in Fontan patients.
- Increased liver volume may indicate a higher risk of complications and poorer prognosis post-Fontan operation.
Background And Hypotheses:
High venous pressures and associated hepatic congestion are important drivers for Fontan-associated liver disease. The prognostic significance of hepatomegaly as a marker of congestion however is not well defined and is further explored in this research study.
Methods:
Fontan patients who have had liver ultrasound scans were identified from the Prince Sultan Cardiac Centre Fontan Database and had their anatomic, surgical, clinical histories abstracted from the electronic medical records following institutional ethics approval. Liver volumes were determined retrospectively from reviewing individual US images, and these, divided into tertiles, were analysed in the context of the predefined endpoints of (i) Primary - death or heart or liver transplantation, or (ii) Secondary - combined endpoint of death, transplantation, arrhythmia, or protein-losing enteropathy.
Results:
Mean indexed liver volumes for the entire cohort (n = 199) were 1065.1 ± 312.1 ml/m2, range 387 to 2071 ml/m2. Patients with the largest liver volumes (highest tertile) were less likely to have a functioning fenestration compared to those in the lowest tertile 44% versus 56% p = 0.016 and experienced the highest burden of mortality and heart or heart-liver transplantation, p = 0.016, and were more likely to reach the composite endpoint of death, protein-losing enteropathy, arrhythmia, or transplantation, p = 0.010. Liver volumes had an overall predictive accuracy for the combined outcome of 61% (CI 53%, 67%, p = 0.009).
Conclusions:
Liver volumetry may serve as a potentially important congestion biomarker for adverse outcomes after the Fontan operation.
