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Role of transient elastography to stage fontan-associated liver disease (Fald) in adults with single ventricle congenital heart disease correction

Title: Role of transient elastography to stage fontan-associated liver disease (Fald) in adults with single ventricle congenital heart disease correction
Authors: Chemello L.; Padalino M.; Zanon C.; Benvegnu' L.; Biffanti R.; Mancuso D.; Cavalletto L.
Contributors: Chemello, L.; Padalino, M.; Zanon, C.; Benvegnu', L.; Biffanti, R.; Mancuso, D.; Cavalletto, L.
Publisher Information: MDPI
Publication Year: 2021
Collection: Padua Research Archive (IRIS - Università degli Studi di Padova)
Subject Terms: CHD; Congenital heart disease; FALD; Fibroscan; Fontan associated liver disease; Fontan palliation; Single ventricle; SV; TE; Transient elastography
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 deter-minates 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®). According to selective criteria for liver disease, we identified 23/43 (53.5%) cases with advanced FALD that showed: older age (p < 0.05), larger hepatic and cava veins diameter (p < 0.05), worsened NYHA class (p < 0.05), abnormal lymphocytes (p < 0.01), platelet count (p < 0.05), and GGT, prothrombin time (INR), albumin and cystatin C levels (p < 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 (p < 0.001). LS, and its combined score with spleen diameter and platelet count (LSPS) successfully helped to detect 100% of cases with portal hyper-tension (p < 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.
Document Type: article in journal/newspaper
File Description: STAMPA
Language: English
Relation: info:eu-repo/semantics/altIdentifier/pmid/34677186; info:eu-repo/semantics/altIdentifier/wos/WOS:000716964700001; volume:8; issue:10; firstpage:117; lastpage:128; numberofpages:12; journal:JOURNAL OF CARDIOVASCULAR DEVELOPMENT AND DISEASE; https://hdl.handle.net/11577/3438860
DOI: 10.3390/jcdd8100117
Availability: https://hdl.handle.net/11577/3438860; https://doi.org/10.3390/jcdd8100117
Rights: info:eu-repo/semantics/openAccess ; license:Creative commons ; license uri:http://creativecommons.org/licenses/by/4.0/
Accession Number: edsbas.42DF5FA8
Database: BASE