Role of Transient Elastography to Stage Fontan-Associated Liver Disease (FALD) in Adults with Single Ventricle Congenital Heart Disease Correction
Fontan-associated liver disease (FALD) is an arising clinical entity that can occur long after a successful Fontan operation for correction of single ventricle (SV) congenital heart disease (CHD). Occurrence of FALD is characterized by liver cirrhosis and other hepatic complications, and determinate...
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| Format: | Article |
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MDPI AG
2021-09-01
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| Series: | Journal of Cardiovascular Development and Disease |
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| Online Access: | https://www.mdpi.com/2308-3425/8/10/117 |
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| author | Liliana Chemello Massimo Padalino Chiara Zanon Luisa Benvegnu’ Roberta Biffanti Daniela Mancuso Luisa Cavalletto |
| author_facet | Liliana Chemello Massimo Padalino Chiara Zanon Luisa Benvegnu’ Roberta Biffanti Daniela Mancuso Luisa Cavalletto |
| author_sort | Liliana Chemello |
| collection | DOAJ |
| description | Fontan-associated liver disease (FALD) is an arising clinical entity that can occur long after a successful Fontan operation for correction of single ventricle (SV) congenital heart disease (CHD). Occurrence of FALD is characterized by liver cirrhosis and other hepatic complications, and determinates an increased morbidity and mortality. Currently, there is no consensus on how to stage FALD. We report here our experience by an observational study in 52 patients with SV-CHD after Fontan operation that were recruited through a period of 36 ± 9.3 months. All cases underwent lab tests and liver and cardiac imaging evaluation, including liver stiffness (LS) measurement by transient elastography (TE) (FibroScan<sup>®</sup>). According to selective criteria for liver disease, we identified 23/43 (53.5%) cases with advanced FALD that showed: older age (<i>p</i> < 0.05), larger hepatic and cava veins diameter (<i>p</i> < 0.05)<i>,</i> worsened NYHA class (<i>p</i> < 0.05), abnormal lymphocytes (<i>p</i> < 0.01), platelet count (<i>p</i> < 0.05), and GGT, prothrombin time (INR), albumin and cystatin C levels (<i>p</i> < 0.05), with respect to cases without advanced FALD. LS values were significantly increased in cases with advanced FALD, at cut-off values higher than 22 kPa (<i>p</i> < 0.001). LS, and its combined score with spleen diameter and platelet count (LSPS) successfully helped to detect 100% of cases with portal hypertension (<i>p</i> < 0.001). In conclusion, LS can be effective to stage FALD and to uncover cases with severe risk of complications, avoiding higher morbidity and mortality related to advanced FALD. |
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| format | Article |
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| institution | Directory Open Access Journal |
| issn | 2308-3425 |
| language | English |
| last_indexed | 2024-03-10T06:28:31Z |
| publishDate | 2021-09-01 |
| publisher | MDPI AG |
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| series | Journal of Cardiovascular Development and Disease |
| spelling | doaj.art-0f001ee27f5b411d8bd9eca6a308d0a72023-11-22T18:40:15ZengMDPI AGJournal of Cardiovascular Development and Disease2308-34252021-09-0181011710.3390/jcdd8100117Role of Transient Elastography to Stage Fontan-Associated Liver Disease (FALD) in Adults with Single Ventricle Congenital Heart Disease CorrectionLiliana Chemello0Massimo Padalino1Chiara Zanon2Luisa Benvegnu’3Roberta Biffanti4Daniela Mancuso5Luisa Cavalletto6Clinica Medica 5, Internal Medicine & Hepatology Unit, Department of Medicine-DIMED, University of Padua Medical School, 35128 Padova, ItalyPediatric and Congenital Cardiac Surgery Unit, Department of Cardiac, Thoracic & Vascular Sciences and Public Health, University of Padua Medical School, 35128 Padova, ItalyClinica Medica 5, Internal Medicine & Hepatology Unit, Department of Medicine-DIMED, University of Padua Medical School, 35128 Padova, ItalyClinica Medica 5, Internal Medicine & Hepatology Unit, Department of Molecular Medicine, University of Padua Medical School, 35128 Padova, ItalyPediatric Cardiology Unit, Department of Woman’s and Child’s Health, University of Padua Medical School, 35128 Padova, ItalyCardiologic Unit, Department of Cardiac, Thoracic & Vascular Sciences and Public Health, University of Padua Medical School, 35128 Padova, ItalyClinica Medica 5, Internal Medicine & Hepatology Unit, Department of Medicine-DIMED, University of Padua Medical School, 35128 Padova, ItalyFontan-associated liver disease (FALD) is an arising clinical entity that can occur long after a successful Fontan operation for correction of single ventricle (SV) congenital heart disease (CHD). Occurrence of FALD is characterized by liver cirrhosis and other hepatic complications, and determinates an increased morbidity and mortality. Currently, there is no consensus on how to stage FALD. We report here our experience by an observational study in 52 patients with SV-CHD after Fontan operation that were recruited through a period of 36 ± 9.3 months. All cases underwent lab tests and liver and cardiac imaging evaluation, including liver stiffness (LS) measurement by transient elastography (TE) (FibroScan<sup>®</sup>). According to selective criteria for liver disease, we identified 23/43 (53.5%) cases with advanced FALD that showed: older age (<i>p</i> < 0.05), larger hepatic and cava veins diameter (<i>p</i> < 0.05)<i>,</i> worsened NYHA class (<i>p</i> < 0.05), abnormal lymphocytes (<i>p</i> < 0.01), platelet count (<i>p</i> < 0.05), and GGT, prothrombin time (INR), albumin and cystatin C levels (<i>p</i> < 0.05), with respect to cases without advanced FALD. LS values were significantly increased in cases with advanced FALD, at cut-off values higher than 22 kPa (<i>p</i> < 0.001). LS, and its combined score with spleen diameter and platelet count (LSPS) successfully helped to detect 100% of cases with portal hypertension (<i>p</i> < 0.001). In conclusion, LS can be effective to stage FALD and to uncover cases with severe risk of complications, avoiding higher morbidity and mortality related to advanced FALD.https://www.mdpi.com/2308-3425/8/10/117transient elastographyTEfibroscanFontan associated liver diseaseFALDsingle ventricle |
| spellingShingle | Liliana Chemello Massimo Padalino Chiara Zanon Luisa Benvegnu’ Roberta Biffanti Daniela Mancuso Luisa Cavalletto Role of Transient Elastography to Stage Fontan-Associated Liver Disease (FALD) in Adults with Single Ventricle Congenital Heart Disease Correction Journal of Cardiovascular Development and Disease transient elastography TE fibroscan Fontan associated liver disease FALD single ventricle |
| title | Role of Transient Elastography to Stage Fontan-Associated Liver Disease (FALD) in Adults with Single Ventricle Congenital Heart Disease Correction |
| title_full | Role of Transient Elastography to Stage Fontan-Associated Liver Disease (FALD) in Adults with Single Ventricle Congenital Heart Disease Correction |
| title_fullStr | Role of Transient Elastography to Stage Fontan-Associated Liver Disease (FALD) in Adults with Single Ventricle Congenital Heart Disease Correction |
| title_full_unstemmed | Role of Transient Elastography to Stage Fontan-Associated Liver Disease (FALD) in Adults with Single Ventricle Congenital Heart Disease Correction |
| title_short | Role of Transient Elastography to Stage Fontan-Associated Liver Disease (FALD) in Adults with Single Ventricle Congenital Heart Disease Correction |
| title_sort | role of transient elastography to stage fontan associated liver disease fald in adults with single ventricle congenital heart disease correction |
| topic | transient elastography TE fibroscan Fontan associated liver disease FALD single ventricle |
| url | https://www.mdpi.com/2308-3425/8/10/117 |
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