Liver stiffness versus fibrosis after Fontan-palliation: is transient elastography the solution to early detection of progressive liver disease?

Abstract

Fontan associated liver disease (FALD) is now a well-recognized source of morbidity in the Fontan population. The disease spectrum ranges from hepatic fibrosis to cirrhosis and is a progressive process that has been identified in patients as young as those in their adolescence ( 1 Rathgeber SL, Guttman OR, Lee AF et al. Fontan-Associated Liver Disease: Spectrum of Disease in Children and Adolescents. J Am Heart Assoc. 2020;9:e0 12529. Google Scholar ). It is generally understood that all Fontan patients will eventually develop FALD although the extent and whether it will be clinically apparent remains unclear ( 2 Emamaullee J. Zaidi A.N. Schiano T. et al. Fontan-Associated Liver Disease: Screening, Management, and Transplant Considerations. Circulation. 2020; 142: 591-604 Crossref PubMed Scopus (58) Google Scholar ). FALD is characterized as a congestive hepatopathy that is related to the inherent increase in central venous pressure (CVP) following separation of a pulmonary ventricle from the circulation following Fontan palliation. This process is exacerbated by a corresponding decrease in cardiac output associated with single ventricle physiology, culminating in a setup for chronic and progressive liver injury ( 3 Agnoletti G. Ferraro G. Bordese R. et al. Fontan circulation causes early,severe liver damage: should we offer patients a tailored strategy?. Int J Cardiol. 2016; 209: 60-65 Abstract Full Text Full Text PDF PubMed Scopus (48) Google Scholar ). The recognition of FALD as an inevitability has led to the adoption of routine hepatic surveillance according to published guidelines in the clinical management of single ventricle patients in many centers ( 4 Rychik J. Atz A.M. Celermajer D.S. et al. Evaluation and management of the child and adult with Fontan circulation: a scientific statement from the American Heart Association. Circulation. 2019; : e234-e284 Google Scholar ). Despite the recognition and definition of this problem, the understanding of the true natural history of FALD remains elusive. This is primarily due to the absence of a reliable non-invasive test to accurately identify and monitor the progression of liver pathology. Numerous established tests for liver health have been investigated in FALD without any definitive serum or imaging test being identified as a reliable and reproducible correlate to liver biopsy. The measurement of liver stiffness by transient elastography is one such established modality that has received attention as a potential candidate for the evaluation of FALD. Transient elastography is an ultrasound-based method that is validated for the assessment of fibrosis and cirrhosis in primary hepatic disease. This test can be performed at the bedside without sedation or analgesia and calculates the liver stiffness based on tissue deformation following an induced shear wave. The accessibility of this technology as a test that can be performed in an outpatient setting in a serial manner would be ideal in this context if validated. Butikofer et al ( 5 Butikofer S, Greutmann-Yanitir M, Gubler C, et al. Determinants of advanced liver fibrosis in adult patients after Fontan-palliation: Usefulness of ultrasound transient elastography. Can J Cardiol. (accepted, paper commented on) Google Scholar ) in their manuscript entitled ‘Determinants of advanced liver fibrosis in adult patients after Fontan-palliation: Usefulness of ultrasound transient elastography’ have taken steps to better understand the role of transient elastography in the surveillance of FALD. Their novel presentation of serial transient elastography measurements using Fibroscan® (Echosens, Paris, France) with biopsy data in adult patients has shed new light on our ability to interpret this test in the context of FALD. Determinants of advanced liver fibrosis in adult patients after Fontan-palliation: Usefulness of ultrasound transient elastographyCanadian Journal of CardiologyPreviewFontan-associated liver disease is an increasing concern. Our aim was to assess prevalence and predictors of advanced liver fibrosis with a specific focus on utility of liver stiffness measurement by ultrasound transient elastography. Full-Text PDF Open Access