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Increased extracellular volume in the liver of pediatric Fontan patients

BACKGROUND: Patients with single ventricle physiology are at increased risk for developing liver fibrosis. Its extent and prevalence in children with bidirectional cavopulmonary connection (BCPC) and Fontan circulation are unclear. Extracellular volume fraction (ECV), derived from cardiovascular mag...

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Autores principales: de Lange, Charlotte, Reichert, Marjolein J. E., Pagano, Joseph J., Seed, Mike, Yoo, Shi-Joon, Broberg, Craig S., Lam, Christopher Z., Grosse-Wortmann, Lars
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6628496/
https://www.ncbi.nlm.nih.gov/pubmed/31303178
http://dx.doi.org/10.1186/s12968-019-0545-4
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author de Lange, Charlotte
Reichert, Marjolein J. E.
Pagano, Joseph J.
Seed, Mike
Yoo, Shi-Joon
Broberg, Craig S.
Lam, Christopher Z.
Grosse-Wortmann, Lars
author_facet de Lange, Charlotte
Reichert, Marjolein J. E.
Pagano, Joseph J.
Seed, Mike
Yoo, Shi-Joon
Broberg, Craig S.
Lam, Christopher Z.
Grosse-Wortmann, Lars
author_sort de Lange, Charlotte
collection PubMed
description BACKGROUND: Patients with single ventricle physiology are at increased risk for developing liver fibrosis. Its extent and prevalence in children with bidirectional cavopulmonary connection (BCPC) and Fontan circulation are unclear. Extracellular volume fraction (ECV), derived from cardiovascular magnetic resonance (CMR) and T1 relaxometry, reflect fibrotic remodeling and/or congestion in the liver. The aim of this study was to investigate whether pediatric patients with single ventricle physiology experience increased native T1 and ECV as markers of liver fibrosis/congestion. METHODS: Hepatic native T1 times and ECV, using a cardiac short axis modified Look-Locker inversion recovery sequence displaying the liver, were measured retrospectively in children with BCPC- and Fontan circulations and compared to pediatric controls. RESULTS: Hepatic native T1 time were increased in Fontan patients (n = 62, 11.4 ± 4.4 years, T1 762 ± 64 ms) versus BCPC patients (n = 20, 2.8 ± 0.9 years, T1 645 ± 43 ms, p = 0.04). Both cohorts had higher T1 than controls (n = 44, 13.7 ± 2.9 years, T1 604 ± 54 ms, p < 0.001 for both). ECV was 41.4 ± 4.8% in Fontan and 36.4 ± 4.8% in BCPC patients, respectively (p = 0.02). In Fontan patients, T1 values correlated with exposure to cardiopulmonary bypass time (R = 0.3, p = 0.02), systolic and end diastolic volumes (R = 0.3, p = 0.04 for both) and inversely with oxygen saturations and body surface area (R = -0.3, p = 0.04 for both). There were no demonstrable associations of T1 or ECV with central venous pressure or age after Fontan. CONCLUSION: Fontan and BCPC patients have elevated CMR markers suggestive of hepatic fibrosis and/or congestion, even at a young age. The tissue changes do not appear to be related to central venous pressures. TRIAL REGISTRATION: Retrospectively registered data. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (10.1186/s12968-019-0545-4) contains supplementary material, which is available to authorized users.
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spelling pubmed-66284962019-07-23 Increased extracellular volume in the liver of pediatric Fontan patients de Lange, Charlotte Reichert, Marjolein J. E. Pagano, Joseph J. Seed, Mike Yoo, Shi-Joon Broberg, Craig S. Lam, Christopher Z. Grosse-Wortmann, Lars J Cardiovasc Magn Reson Research BACKGROUND: Patients with single ventricle physiology are at increased risk for developing liver fibrosis. Its extent and prevalence in children with bidirectional cavopulmonary connection (BCPC) and Fontan circulation are unclear. Extracellular volume fraction (ECV), derived from cardiovascular magnetic resonance (CMR) and T1 relaxometry, reflect fibrotic remodeling and/or congestion in the liver. The aim of this study was to investigate whether pediatric patients with single ventricle physiology experience increased native T1 and ECV as markers of liver fibrosis/congestion. METHODS: Hepatic native T1 times and ECV, using a cardiac short axis modified Look-Locker inversion recovery sequence displaying the liver, were measured retrospectively in children with BCPC- and Fontan circulations and compared to pediatric controls. RESULTS: Hepatic native T1 time were increased in Fontan patients (n = 62, 11.4 ± 4.4 years, T1 762 ± 64 ms) versus BCPC patients (n = 20, 2.8 ± 0.9 years, T1 645 ± 43 ms, p = 0.04). Both cohorts had higher T1 than controls (n = 44, 13.7 ± 2.9 years, T1 604 ± 54 ms, p < 0.001 for both). ECV was 41.4 ± 4.8% in Fontan and 36.4 ± 4.8% in BCPC patients, respectively (p = 0.02). In Fontan patients, T1 values correlated with exposure to cardiopulmonary bypass time (R = 0.3, p = 0.02), systolic and end diastolic volumes (R = 0.3, p = 0.04 for both) and inversely with oxygen saturations and body surface area (R = -0.3, p = 0.04 for both). There were no demonstrable associations of T1 or ECV with central venous pressure or age after Fontan. CONCLUSION: Fontan and BCPC patients have elevated CMR markers suggestive of hepatic fibrosis and/or congestion, even at a young age. The tissue changes do not appear to be related to central venous pressures. TRIAL REGISTRATION: Retrospectively registered data. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (10.1186/s12968-019-0545-4) contains supplementary material, which is available to authorized users. BioMed Central 2019-07-15 /pmc/articles/PMC6628496/ /pubmed/31303178 http://dx.doi.org/10.1186/s12968-019-0545-4 Text en © The Author(s). 2019 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Research
de Lange, Charlotte
Reichert, Marjolein J. E.
Pagano, Joseph J.
Seed, Mike
Yoo, Shi-Joon
Broberg, Craig S.
Lam, Christopher Z.
Grosse-Wortmann, Lars
Increased extracellular volume in the liver of pediatric Fontan patients
title Increased extracellular volume in the liver of pediatric Fontan patients
title_full Increased extracellular volume in the liver of pediatric Fontan patients
title_fullStr Increased extracellular volume in the liver of pediatric Fontan patients
title_full_unstemmed Increased extracellular volume in the liver of pediatric Fontan patients
title_short Increased extracellular volume in the liver of pediatric Fontan patients
title_sort increased extracellular volume in the liver of pediatric fontan patients
topic Research
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6628496/
https://www.ncbi.nlm.nih.gov/pubmed/31303178
http://dx.doi.org/10.1186/s12968-019-0545-4
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