Pediatric Fontan Associated Liver Disease: Non-invasive Evaluation with Serologic Markers and Acoustic Radiation
Sylvia Y Ofei1, Karen Texter2, Cheryl Gariepy3
1University of Kentucky College of Medicine/Kentucky Children's Hospital Department of Pediatrics/Division of Pediatric Gastroenterology, Hepatology and Nutrition 138 Leader Avenue, Rm 206. Lexington, KY 40506. United States of America.
Insights
Pediatric Fontan patients may develop liver disease over time. Decreasing platelet counts and elevated liver enzymes suggest fibrosis, but acoustic radiation force impulse (ARFI) elastography may be limited by hepatic congestion.
Area of Science:
- Pediatric Cardiology
- Hepatology
- Medical Imaging
Background:
- Hepatic fibrosis is a known complication in adult Fontan patients, increasing with time post-surgery.
- Pediatric Fontan patients are not routinely screened for liver disease, necessitating evaluation of noninvasive markers.
- Assessing liver health in this population is crucial due to potential long-term complications.
Purpose of the Study:
- To evaluate the effectiveness of serologic biomarkers and acoustic radiation force impulse (ARFI) elastography for detecting liver disease in pediatric Fontan patients.
- To correlate clinical and imaging findings with time since Fontan operation.
- To identify reliable noninvasive screening methods for hepatic fibrosis in this cohort.
Main Methods:
- Prospective study of pediatric Fontan patients (≥1 year post-operation) including hepatic ultrasound with ARFI elastography and laboratory testing.
- Review of clinical cardiac data (echocardiograms, cardiac catheterizations).
- Statistical analysis using Pearson's correlation, Wilcoxon rank-sum, and Kruskal-Wallis tests.
Main Results:
- Forty patients (median age 11 years, median 6.5 years post-Fontan) were analyzed.
- Platelet count negatively correlated with years since Fontan (p < 0.000); 15% had thrombocytopenia.
- Elevated ALT (p=0.034) and AST (p=0.009) were associated with Extracardiac Conduit Fontan. Heterogeneous echotexture correlated with years since Fontan (p=0.022).
- All ARFI values were elevated but did not correlate with clinical factors, suggesting passive hepatic congestion may limit its utility.
Conclusions:
- Decreasing platelet count may indicate developing liver fibrosis in pediatric Fontan patients.
- ARFI elastography's utility may be limited by passive hepatic congestion in this population.
- Further research is needed to establish reliable noninvasive screening modalities for hepatic fibrosis in Fontan patients.
Abstract:
Hepatic fibrosis is a significant complication in adult Fontan patients suggesting development as a function of time since the surgery. Children with Fontan circulation are not routinely assessed for development of liver disease. We aimed to evaluate the effectiveness of serologic biomarkers and acoustic radiation force impulse (ARFI) elastography to detect liver disease in pediatric Fontan patients. Patients ≥ 1 year after Fontan operation prospectively had hepatic US with acoustic radiation force impulse and laboratory testing. Clinical cardiac data (echocardiograms, cardiac catheterizations) were reviewed. Statistical analysis was performed using Pearson's correlation coefficient, Wilcoxon rank-sum test and Kruskal-Wallis test. Forty patients were enrolled with median age of 11 years and median time since Fontan of 6.5 years. Platelet count negatively correlated with years since Fontan (p < 0.000). Thrombocytopenia was noted in 15% of patients with the lowest platelet count of 78 K/cu mm, in a patient >10 years from the Fontan (DORV) operation. Alanine transaminase (ALT, p = 0.034) and aspartate aminotransferase (AST, p = 0.009) were higher in patients with Extracardiac Conduit Fontan and not in other Fontan operations. Heterogeneous echotexture on liver ultrasound correlated with years since Fontan (p = 0.022), however all acoustic radiation force impulse values were elevated (> 1.34 m/s) and did not correlate with age, years since Fontan, labs or imaging. FibroSure values did not correlate with years since Fontan. This suggests that ARFI may be elevated due to passive hepatic congestion, limiting its value in this patient population. Additional testing is necessary to identify reliable noninvasive screening modalities for hepatic fibrosis in Fontan patients. Our study is the largest pediatric study to evaluate ARFI in patients after the Fontan operation and showed increased shear wave speed for all patients with no correlation with time since palliation. Decreasing platelet count may indicate the development of liver fibrosis.
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