Increased extracellular volume in the liver of pediatric Fontan patients

Charlotte De Lange, Marjolein J.E. Reichert, Joseph J. Pagano, Mike Seed, Shi Joon Yoo, Craig Broberg, Christopher Z. Lam, Lars Grosse-Wortmann

    Research output: Contribution to journalArticle

    Abstract

    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.

    Original languageEnglish (US)
    Article number39
    JournalJournal of Cardiovascular Magnetic Resonance
    Volume21
    Issue number1
    DOIs
    StatePublished - Jul 15 2019

    Fingerprint

    Pediatrics
    Liver
    Central Venous Pressure
    Liver Cirrhosis
    Magnetic Resonance Spectroscopy
    Sequence Inversion
    Body Surface Area
    Cardiopulmonary Bypass
    Fibrosis
    Oxygen

    Keywords

    • Cardiovascular magnetic resonance
    • Fontan circulation
    • Liver cirrhosis
    • Single ventricle
    • T1 mapping

    ASJC Scopus subject areas

    • Radiological and Ultrasound Technology
    • Radiology Nuclear Medicine and imaging
    • Cardiology and Cardiovascular Medicine
    • Family Practice

    Cite this

    De Lange, C., Reichert, M. J. E., Pagano, J. J., Seed, M., Yoo, S. J., Broberg, C., ... Grosse-Wortmann, L. (2019). Increased extracellular volume in the liver of pediatric Fontan patients. Journal of Cardiovascular Magnetic Resonance, 21(1), [39]. https://doi.org/10.1186/s12968-019-0545-4

    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; Lam, Christopher Z.; Grosse-Wortmann, Lars.

    In: Journal of Cardiovascular Magnetic Resonance, Vol. 21, No. 1, 39, 15.07.2019.

    Research output: Contribution to journalArticle

    De Lange, C, Reichert, MJE, Pagano, JJ, Seed, M, Yoo, SJ, Broberg, C, Lam, CZ & Grosse-Wortmann, L 2019, 'Increased extracellular volume in the liver of pediatric Fontan patients', Journal of Cardiovascular Magnetic Resonance, vol. 21, no. 1, 39. https://doi.org/10.1186/s12968-019-0545-4
    De Lange, Charlotte ; Reichert, Marjolein J.E. ; Pagano, Joseph J. ; Seed, Mike ; Yoo, Shi Joon ; Broberg, Craig ; Lam, Christopher Z. ; Grosse-Wortmann, Lars. / Increased extracellular volume in the liver of pediatric Fontan patients. In: Journal of Cardiovascular Magnetic Resonance. 2019 ; Vol. 21, No. 1.
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    abstract = "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.",
    keywords = "Cardiovascular magnetic resonance, Fontan circulation, Liver cirrhosis, Single ventricle, T1 mapping",
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    T1 - Increased extracellular volume in the liver of pediatric Fontan patients

    AU - De Lange, Charlotte

    AU - Reichert, Marjolein J.E.

    AU - Pagano, Joseph J.

    AU - Seed, Mike

    AU - Yoo, Shi Joon

    AU - Broberg, Craig

    AU - Lam, Christopher Z.

    AU - Grosse-Wortmann, Lars

    PY - 2019/7/15

    Y1 - 2019/7/15

    N2 - 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.

    AB - 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.

    KW - Cardiovascular magnetic resonance

    KW - Fontan circulation

    KW - Liver cirrhosis

    KW - Single ventricle

    KW - T1 mapping

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