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Non-Invasive Biomarkers of Fontan Associated Liver Disease

Surgical palliation (resulting in the Fontan circulation and total cavo-pulmonary connection) is beneficial to palliate symptoms in patients with a range of congenital cardiac diseases and lesions as hypoplastic left heart syndrome and tricuspid atresia.

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Surgical palliation (resulting in the Fontan circulation and total cavo-pulmonary connection) is beneficial to palliate symptoms in patients with a range of congenital cardiac diseases and lesions as hypoplastic left heart syndrome and tricuspid atresia. Passive venous flow directly enters the pulmonary circulation, increasing oxygen saturation in arterial blood. As a result of this increase, there is an abrupt increase in the CVP (Central Venous Pressure), which leads to several other extra-cardiac effects. 


As the population or the patients proceed to adulthood, the FALD (Fontan-associated liver diseases) tends to emerge as the unintentional comorbidity in the patients. Multiple factors contribute to congestive liver disease or hepatopathy as low CO (cardiac output), hypoxia, perioperative insults, and elevated levels of CVP. The limitations of FALD detection are associated with normal laboratory values, less definitive imaging features, and fewer physical exam findings. Following the surgical palliations of the single ventricle congenital heart diseases, FALD is significant morbidity. In this study, different aspects and non-invasive biomarkers have been explored. 
This retrospective study included the post-Fontan pediatric patients who underwent the liver biopsy. A total of 108 patients were included in the study, amongst which the mean age of the study participants was 14-years. The majority of the pediatric patients suffered from tricuspid atresia and hypoplastic left heart syndrome. Other patients were further divided into cirrhosis, bridging fibrosis, and fibrosis alone. 


According to the cardiac evaluation through TTE, 12.3% of patients had a mild, 6.6 (moderate), and more than 6.6% of patients had a severe decrease in the VSF (ventricular systolic function). Furthermore, the study results showed that patients suffering from bridging fibrosis had worse survival from biopsy time to the end of the study. The most common risk factors identified for the FALD were female gender, Hispanic ethnicity, Fontan pressure, and obesity. 


According to the results and observations of the study, advanced fibrosis has a strong association with the overall worse survival rate even in the case of compensated liver function test. The study’s data provides evidence regarding the significance of liver biopsy and function, providing a positive prognosis to the clinical team and the patient. This technique is more beneficial for patients who have a higher risk for decompensating events in the future, such as Fontan failure and other infections. Another result of the study shows underlying fibrotic liver diseases to be another significant risk of disease in adolescent patients who have had decompensating events compared to the adult Fontan population or patients. 


Changes in the APRO/FIB-4 scores and platelet count before the biopsy had a mild-moderate discriminatory power for identifying the patients with complaints of advanced fibrosis. An additional risk factor of steatosis can also cause disease progression in the case of obese patients. There is a need for future research and prospective studies to develop the appropriate strategies for screening FALD in the pediatric or adolescent population. In order to identify predictive FALD progression biomarkers, different lab-based non-invasive markers have been identified and reported in the study. All future studies must focus on the gaps and limitations of the current FALD identification to maximize the prognosis and treatment of this complication. 


Read more:
Emamaullee J, Khan S, Weaver C, Goldbeck C, Yanni G, Kohli R, Genyk Y, Zhou S, Shillingford N, Sullivan PM, Takao C, Detterich J, Kantor PF, Cleveland JD, Herrington C, Ram Kumar S, Starnes V, Badran S, Patel ND. Non-invasive biomarkers of Fontan-associated liver disease. JHEP Rep. 2021 Sep 14;3(6):100362. doi: 10.1016/j.jhepr.2021.100362. PMID: 34693238; PMCID: PMC8517550.
 

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