Training/PracticeContemporary Issues in Cardiology PracticeFontan-Associated Liver Disease: Evidence for Early Surveillance of Liver Health in Pediatric Fontan Patients
Section snippets
Pathology
The liver pathology in FALD is thought to be initiated with distension of hepatic sinusoids, causing chronic congestion, inflammation, and sinusoidal fibrosis. This can progress to include centrilobular fibrosis, bridging fibrosis, and portal fibrosis due to portal hypertension. This continues to progress to cirrhosis, at which point liver dysfunction becomes more clinically apparent. The complications of liver failure and risk of hepatocellular carcinoma are significant morbidities at this
Elastography
Liver biopsy is the gold standard for the diagnosis of liver fibrosis and cirrhosis. To minimize the need for this invasive procedure, liver elastography has been validated as a noninvasive technique for the assessment of liver fibrosis and cirrhosis in primary hepatic disease. This technology has been applied in the Fontan population with the intention to use it for diagnosis and surveillance of FALD. Three modalities of elastography have been studied in FALD. Transient elastography (TE) and
Biochemical Tests
Most biochemical tests of liver health in Fontan patients have not been demonstrated to be reliable indicators of pathology. Although often abnormal in Fontan patients, transaminitis and impaired synthetic function have not demonstrated a reliable association with other measures of liver health that include elastography, hemodynamic measurements, or liver fibrosis. Alpha-fetoprotein (AFP) is used as a specific marker for hepatocellular carcinoma (HCC) and would complement ultrasound imaging for
Imaging
The significance of most imaging abnormalities with respect to the severity of FALD remains unclear. Cross-sectional imaging with computed tomography (CT) and magnetic resonance imaging (MRI) often reveals abnormalities in Fontan patients. CT and MRI in Fontan patients often demonstrate abnormalities such as zonal enhancement and reticular changes that are typically associated with liver congestion and fibrosis in other disease processes. Less commonly, hypervascular nodules are found, which
Transplant
The growing knowledge of FALD has led to questions regarding the role of concurrent liver transplantation in those with a failing Fontan who are listed for cardiac transplant. Hepatic failure is typically cited as a reason for combined heart and liver transplant (CHLT) as opposed to an isolated heart transplant (IHT). This concept is being challenged with reports showing positive outcomes following IHT in Fontan patients with FALD. Investigations following IHT in patients with FALD have
Conclusion
FALD is ubiquitous and progressive, but an evidence-based protocol for surveillance has not been established. Laboratory investigations are largely unhelpful in guiding management. Nearly all results of investigations of liver health—including biochemical markers, ultrasound, cross-sectional imaging, and elastography—will be abnormal in Fontan patients. The clinical significance and appropriate action to take upon discovery of mild abnormalities in individual tests is unclear. Patients with
Disclosures
The authors have no conflicts of interest to disclose.
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Cited by (13)
Low Mean Perfusion Pressure Indexed to Body Surface Area is a Powerful Predictor of Poor Outcomes After Heart Transplantation in Patients With High Pre-Transplant Venous Pressure: A Clinical Study With Physiological Insights From Mathematical Modelling of Biventricular Heart Failure
2024, Heart Lung and CirculationEASL-ERN position paper on liver involvement in patients with Fontan-type circulation
2023, Journal of HepatologyPrenatal Diagnosis and Management of Single-Ventricle Heart Disease
2022, Canadian Journal of CardiologyCitation Excerpt :Although the excessive load on the single-ventricle heart is gradually lifted throughout these stages and the saturations gradually improve, the Fontan circulation has its challenges. Systemic venous congestion may occur, manifesting as plastic bronchitis in the lungs, protein-losing enteropathy in the intestines,50 congestive hepatopathy with fibrosis (Fontan-associated liver disease),51,52 or kidney disease53 among others. Cardiac suture lines, among other factors, may lead to arrhythmias, and the single ventricle may decompensate with valve regurgitation and systolic and/or diastolic function, leading to heart failure.
Hepatic and Renal Consequences of Single-Ventricle Physiology Palliated With the Fontan Operation
2022, Canadian Journal of CardiologyCitation Excerpt :Fontan-associated liver disease (FALD) results from the systemic venous connections that are created in the Fontan procedure to separate the venous and arterial circulations in the absence of a pulmonary ventricle. This alters the hemodynamics of venous return, causing chronic liver congestion that can progress to fibrosis and cirrhosis.3,4 The result of having the superior vena cava (SVC) and IVC connect directly to the pulmonary arteries without an intervening pulmonary ventricle elevates the central venous pressure (CVP) that is transmitted to the efferent hepatic circulation.
Portal vein thrombosis in Fontan-associated liver disease
2020, Cardiology in the Young
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