Non-invasive biomarkers of Fontan-associated liver disease

Background & Aims: Fontan-associated liver disease (FALD) has emerged as an important morbidity following surgical palliation of single ventricle congenital heart disease. In this study, non-invasive biomarkers that may be associated with severity of FALD were explored. Methods: A retrospect...

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Autores principales:	Juliet Emamaullee, Sara Khan, Carly Weaver, Cameron Goldbeck, George Yanni, Rohit Kohli, Yuri Genyk, Shengmei Zhou, Nick Shillingford, Patrick M. Sullivan, Cheryl Takao, Jon Detterich, Paul F. Kantor, John D. Cleveland, Cynthia Herrington, S. Ram Kumar, Vaughn Starnes, Sarah Badran, Neil D. Patel
Formato:	article
Lenguaje:	EN
Publicado:	Elsevier 2021
Materias:	Congenital heart disease Univentricular heart disease Congestive hepatopathy Diseases of the digestive system. Gastroenterology RC799-869
Acceso en línea:	https://doaj.org/article/0ffbc80adb954ee9ba63a6625523ab24
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spelling	oai:doaj.org-article:0ffbc80adb954ee9ba63a6625523ab242021-11-20T05:11:46ZNon-invasive biomarkers of Fontan-associated liver disease2589-555910.1016/j.jhepr.2021.100362https://doaj.org/article/0ffbc80adb954ee9ba63a6625523ab242021-12-01T00:00:00Zhttp://www.sciencedirect.com/science/article/pii/S2589555921001385https://doaj.org/toc/2589-5559Background & Aims: Fontan-associated liver disease (FALD) has emerged as an important morbidity following surgical palliation of single ventricle congenital heart disease. In this study, non-invasive biomarkers that may be associated with severity of FALD were explored. Methods: A retrospective cohort of paediatric patients post-Fontan who underwent liver biopsy at a high volume at a paediatric congenital heart disease centre was reviewed. Results: Among 106 patients, 66% were male and 69% were Hispanic. The mean age was 14.4 ± 3.5 years, and biopsy was performed 10.8 ± 3.6 years post-Fontan. The mean BMI was 20.8 ± 5 kg/m2, with 27.4% meeting obesity criteria. Bridging fibrosis was observed in 35% of patients, and 10.4% of all patients had superimposed steatosis. Bridging fibrosis was associated with lower platelet counts (168.3 ± 58.4 vs. 203.9 ± 65.8 K/μl for congestive hepatic fibrosis score [CHFS] 0–2b, p = 0.009), higher bilirubin (1.7 ± 2.2 vs. 0.9 ± 0.7 mg/dl, p = 0.0090), higher aspartate aminotransferase-to-platelet ratio index [APRI] and fibrosis-4 [FIB-4] scores (APRI: 0.5 ± 0.3 vs. 0.4 ± 0.1, p <0.01 [AUC: 0.69] and FIB-4: 0.6 ± 0.4 vs. 0.4 ± 0.2, p <0.01 [AUC: 0.69]), and worse overall survival (median 2 years follow-up post-biopsy, p = 0.027). Regression modelling of temporal changes in platelet counts before and after biopsy correlated with fibrosis severity (p = 0.005). Conclusions: In this large, relatively homogeneous adolescent population in terms of age, ethnicity, and Fontan duration, bridging fibrosis was observed in 35% of patients within the first decade post-Fontan. Bridging fibrosis was associated with worse survival. Changes in platelet counts, even years before biopsy, and APRI/FIB-4 scores had modest discriminatory power in identifying patients with advanced fibrosis. Steatosis may represent an additional risk factor for disease progression in obese patients. Further prospective studies are necessary to develop strategies to screen for FALD in the adolescent population. Lay summary: In this study, the prevalence of Fontan-associated liver disease (FALD) in the young adult population and clinical variables that may be predictive of fibrosis severity or adverse outcomes were explored. Several lab-based, non-invasive markers of bridging fibrosis in FALD were identified, suggesting that these values may be followed as a prognostic biomarker for FALD progression in the adolescent population.Juliet EmamaulleeSara KhanCarly WeaverCameron GoldbeckGeorge YanniRohit KohliYuri GenykShengmei ZhouNick ShillingfordPatrick M. SullivanCheryl TakaoJon DetterichPaul F. KantorJohn D. ClevelandCynthia HerringtonS. Ram KumarVaughn StarnesSarah BadranNeil D. PatelElsevierarticleCongenital heart diseaseUniventricular heart diseaseCongestive hepatopathyDiseases of the digestive system. GastroenterologyRC799-869ENJHEP Reports, Vol 3, Iss 6, Pp 100362- (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Congenital heart disease Univentricular heart disease Congestive hepatopathy Diseases of the digestive system. Gastroenterology RC799-869
spellingShingle	Congenital heart disease Univentricular heart disease Congestive hepatopathy Diseases of the digestive system. Gastroenterology RC799-869 Juliet Emamaullee Sara Khan Carly Weaver Cameron Goldbeck George Yanni Rohit Kohli Yuri Genyk Shengmei Zhou Nick Shillingford Patrick M. Sullivan Cheryl Takao Jon Detterich Paul F. Kantor John D. Cleveland Cynthia Herrington S. Ram Kumar Vaughn Starnes Sarah Badran Neil D. Patel Non-invasive biomarkers of Fontan-associated liver disease
description	Background & Aims: Fontan-associated liver disease (FALD) has emerged as an important morbidity following surgical palliation of single ventricle congenital heart disease. In this study, non-invasive biomarkers that may be associated with severity of FALD were explored. Methods: A retrospective cohort of paediatric patients post-Fontan who underwent liver biopsy at a high volume at a paediatric congenital heart disease centre was reviewed. Results: Among 106 patients, 66% were male and 69% were Hispanic. The mean age was 14.4 ± 3.5 years, and biopsy was performed 10.8 ± 3.6 years post-Fontan. The mean BMI was 20.8 ± 5 kg/m2, with 27.4% meeting obesity criteria. Bridging fibrosis was observed in 35% of patients, and 10.4% of all patients had superimposed steatosis. Bridging fibrosis was associated with lower platelet counts (168.3 ± 58.4 vs. 203.9 ± 65.8 K/μl for congestive hepatic fibrosis score [CHFS] 0–2b, p = 0.009), higher bilirubin (1.7 ± 2.2 vs. 0.9 ± 0.7 mg/dl, p = 0.0090), higher aspartate aminotransferase-to-platelet ratio index [APRI] and fibrosis-4 [FIB-4] scores (APRI: 0.5 ± 0.3 vs. 0.4 ± 0.1, p <0.01 [AUC: 0.69] and FIB-4: 0.6 ± 0.4 vs. 0.4 ± 0.2, p <0.01 [AUC: 0.69]), and worse overall survival (median 2 years follow-up post-biopsy, p = 0.027). Regression modelling of temporal changes in platelet counts before and after biopsy correlated with fibrosis severity (p = 0.005). Conclusions: In this large, relatively homogeneous adolescent population in terms of age, ethnicity, and Fontan duration, bridging fibrosis was observed in 35% of patients within the first decade post-Fontan. Bridging fibrosis was associated with worse survival. Changes in platelet counts, even years before biopsy, and APRI/FIB-4 scores had modest discriminatory power in identifying patients with advanced fibrosis. Steatosis may represent an additional risk factor for disease progression in obese patients. Further prospective studies are necessary to develop strategies to screen for FALD in the adolescent population. Lay summary: In this study, the prevalence of Fontan-associated liver disease (FALD) in the young adult population and clinical variables that may be predictive of fibrosis severity or adverse outcomes were explored. Several lab-based, non-invasive markers of bridging fibrosis in FALD were identified, suggesting that these values may be followed as a prognostic biomarker for FALD progression in the adolescent population.
format	article
author	Juliet Emamaullee Sara Khan Carly Weaver Cameron Goldbeck George Yanni Rohit Kohli Yuri Genyk Shengmei Zhou Nick Shillingford Patrick M. Sullivan Cheryl Takao Jon Detterich Paul F. Kantor John D. Cleveland Cynthia Herrington S. Ram Kumar Vaughn Starnes Sarah Badran Neil D. Patel
author_facet	Juliet Emamaullee Sara Khan Carly Weaver Cameron Goldbeck George Yanni Rohit Kohli Yuri Genyk Shengmei Zhou Nick Shillingford Patrick M. Sullivan Cheryl Takao Jon Detterich Paul F. Kantor John D. Cleveland Cynthia Herrington S. Ram Kumar Vaughn Starnes Sarah Badran Neil D. Patel
author_sort	Juliet Emamaullee
title	Non-invasive biomarkers of Fontan-associated liver disease
title_short	Non-invasive biomarkers of Fontan-associated liver disease
title_full	Non-invasive biomarkers of Fontan-associated liver disease
title_fullStr	Non-invasive biomarkers of Fontan-associated liver disease
title_full_unstemmed	Non-invasive biomarkers of Fontan-associated liver disease
title_sort	non-invasive biomarkers of fontan-associated liver disease
publisher	Elsevier
publishDate	2021
url	https://doaj.org/article/0ffbc80adb954ee9ba63a6625523ab24
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Non-invasive biomarkers of Fontan-associated liver disease

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