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The Clinical Spectrum of Fontan-Associated Liver Disease
Study Questions:
What is the spectrum of liver disease seen in Fontan patients, and what is the usefulness of noninvasive methods to assess biopsy-confirmed liver fibrosis?
Methods:
A prospective study was performed at a single center. All patients underwent liver biopsy scored with Fontan-specific fibrosis scores and collagen proportionate area. Noninvasive screening included laboratory testing, ultrasonography, elastography, and contrast-enhanced magnetic resonance imaging (MRI) or computed tomography (CT) scan.
Results:
Thirty-eight of 49 patients (27 ± 6.6 years, 73.7% male) underwent the complete screening protocol. Liver biopsy demonstrated fibrosis in all patients, with severe fibrosis (stages 3-4) present in 68%. Enhanced liver fibrosis (ELF®) and liver stiffness were elevated, although MELD-XI scores were low in all patients. Fibrosis severity did not correlate with ELF® and liver stiffness. Additionally, fibrosis severity did not correlate with fibrosis parameters on MRI/CT. Esophageal varices were present in 50% of patients, while hyperenhancing nodules were present in 25% of patients.
Conclusions:
The authors concluded that the range of Fontan-associated liver disease (FALD) includes hepatic congestion and severe fibrosis, signs of portal hypertension, and hyperenhancing nodules as common manifestations. Routine imaging, elastography, and serum biomarkers are unable to accurately assess severity of liver fibrosis in Fontan patients.
Perspective:
As the clinical importance of FALD has been increasingly recognized, there has been great interest in establishing noninvasive screening protocols to assess the severity of liver disease. This well-conducted study correlated results of a variety of widely performed noninvasive liver studies with liver biopsy. The patient population was relatively young, with a mean age of 27 years, and had predominately systemic left ventricle anatomy. All patients had fibrosis on liver biopsy and 50% had significant clinical sequelae including varices. This study showed significant limitations in noninvasive measures of liver fibrosis as compared with the gold standard of liver biopsy, and suggests a need for and emphasis on the development of new screening tests. Always in the background of discussions of the diagnosis of FALD is the need to develop effective management strategies to address the diagnosis.
Clinical Topics: Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Invasive Cardiovascular Angiography and Intervention, Noninvasive Imaging, Cardiac Surgery and CHD and Pediatrics, Congenital Heart Disease, CHD and Pediatrics and Imaging, CHD and Pediatrics and Interventions, Interventions and Imaging, Interventions and Structural Heart Disease, Computed Tomography, Echocardiography/Ultrasound, Magnetic Resonance Imaging, Nuclear Imaging
Keywords: Biomarkers, Diagnostic Imaging, Elasticity Imaging Techniques, Fibrosis, Fontan Procedure, Heart Defects, Congenital, Liver Cirrhosis, Liver Diseases, Magnetic Resonance Imaging, Tomography, Tomography, X-Ray Computed, Ultrasonography
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