Liver health in adults with Fontan circulation: A multicenter cross-sectional study

Fred M. Wu, Brian Kogon, Michael G. Earing, Jamil A. Aboulhosn, Craig Broberg, Anitha S. John, Amy Harmon, Nisha I. Sainani, Andrew J. Hill, Robert D. Odze, Melanie E. Johncilla, Chinweike Ukomadu, Kimberlee Gauvreau, Anne Marie Valente, Michael J. Landzberg

    Research output: Contribution to journalArticle

    32 Citations (Scopus)

    Abstract

    Objectives Liver disease is an important contributor to morbidity and mortality in patients after Fontan surgery. There has been no large-scale survey of liver health in this population. We sought to explore the prevalence and predictors of liver disease in a multicenter cohort of adults with Fontan physiology. Methods Subjects were recruited from 6 adult congenital heart centers. Demographics; clinical history; and laboratory, imaging, and histopathology data were obtained. Results Of 241 subjects (median age 25.8 years [11.8-59.4], median time since Fontan 20.3 years [5.4-34.5]), more than 94% of those who underwent testing (208 of 221) had at least 1 abnormal liver-related finding. All hepatic imaging (n = 54) and liver histology (n = 68) was abnormal. Subjects with abnormal laboratory values had higher sinusoidal fibrosis stage (2 vs 1, P = .007) and higher portal fibrosis stage (3 vs 1, P = .003) compared with those with all normal values. Low albumin correlated with lower sinusoidal fibrosis stage (1 vs 2; P = .02) and portal fibrosis stage (0 vs 3, P = .002); no other liver studies correlated with fibrosis. Regenerative nodules were seen on 33% of histology specimens. Conclusions Regardless of modality, findings of liver disease are common among adults with Fontan circulation, even those appearing clinically well. Cirrhosis is present in up to one-third of subjects. Correlations between hepatic fibrosis stage and clinical history or findings on noninvasive testing are few. Further research is needed to identify patients at risk for more severe liver disease and to determine the best methods for assessing liver health in this population.

    Original languageEnglish (US)
    Pages (from-to)656-664
    Number of pages9
    JournalJournal of Thoracic and Cardiovascular Surgery
    Volume153
    Issue number3
    DOIs
    StatePublished - Mar 1 2017

    Fingerprint

    Cross-Sectional Studies
    Fibrosis
    Liver
    Health
    Liver Diseases
    Histology
    Health Surveys
    Population
    Albumins
    Reference Values
    Demography
    Morbidity
    Mortality
    Research

    Keywords

    • adult congenital heart disease
    • congenital heart disease
    • congestive hepatopathy
    • Fontan circulation
    • hepatic fibrosis

    ASJC Scopus subject areas

    • Surgery
    • Pulmonary and Respiratory Medicine
    • Cardiology and Cardiovascular Medicine

    Cite this

    Liver health in adults with Fontan circulation : A multicenter cross-sectional study. / Wu, Fred M.; Kogon, Brian; Earing, Michael G.; Aboulhosn, Jamil A.; Broberg, Craig; John, Anitha S.; Harmon, Amy; Sainani, Nisha I.; Hill, Andrew J.; Odze, Robert D.; Johncilla, Melanie E.; Ukomadu, Chinweike; Gauvreau, Kimberlee; Valente, Anne Marie; Landzberg, Michael J.

    In: Journal of Thoracic and Cardiovascular Surgery, Vol. 153, No. 3, 01.03.2017, p. 656-664.

    Research output: Contribution to journalArticle

    Wu, FM, Kogon, B, Earing, MG, Aboulhosn, JA, Broberg, C, John, AS, Harmon, A, Sainani, NI, Hill, AJ, Odze, RD, Johncilla, ME, Ukomadu, C, Gauvreau, K, Valente, AM & Landzberg, MJ 2017, 'Liver health in adults with Fontan circulation: A multicenter cross-sectional study', Journal of Thoracic and Cardiovascular Surgery, vol. 153, no. 3, pp. 656-664. https://doi.org/10.1016/j.jtcvs.2016.10.060
    Wu, Fred M. ; Kogon, Brian ; Earing, Michael G. ; Aboulhosn, Jamil A. ; Broberg, Craig ; John, Anitha S. ; Harmon, Amy ; Sainani, Nisha I. ; Hill, Andrew J. ; Odze, Robert D. ; Johncilla, Melanie E. ; Ukomadu, Chinweike ; Gauvreau, Kimberlee ; Valente, Anne Marie ; Landzberg, Michael J. / Liver health in adults with Fontan circulation : A multicenter cross-sectional study. In: Journal of Thoracic and Cardiovascular Surgery. 2017 ; Vol. 153, No. 3. pp. 656-664.
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    abstract = "Objectives Liver disease is an important contributor to morbidity and mortality in patients after Fontan surgery. There has been no large-scale survey of liver health in this population. We sought to explore the prevalence and predictors of liver disease in a multicenter cohort of adults with Fontan physiology. Methods Subjects were recruited from 6 adult congenital heart centers. Demographics; clinical history; and laboratory, imaging, and histopathology data were obtained. Results Of 241 subjects (median age 25.8 years [11.8-59.4], median time since Fontan 20.3 years [5.4-34.5]), more than 94{\%} of those who underwent testing (208 of 221) had at least 1 abnormal liver-related finding. All hepatic imaging (n = 54) and liver histology (n = 68) was abnormal. Subjects with abnormal laboratory values had higher sinusoidal fibrosis stage (2 vs 1, P = .007) and higher portal fibrosis stage (3 vs 1, P = .003) compared with those with all normal values. Low albumin correlated with lower sinusoidal fibrosis stage (1 vs 2; P = .02) and portal fibrosis stage (0 vs 3, P = .002); no other liver studies correlated with fibrosis. Regenerative nodules were seen on 33{\%} of histology specimens. Conclusions Regardless of modality, findings of liver disease are common among adults with Fontan circulation, even those appearing clinically well. Cirrhosis is present in up to one-third of subjects. Correlations between hepatic fibrosis stage and clinical history or findings on noninvasive testing are few. Further research is needed to identify patients at risk for more severe liver disease and to determine the best methods for assessing liver health in this population.",
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    author = "Wu, {Fred M.} and Brian Kogon and Earing, {Michael G.} and Aboulhosn, {Jamil A.} and Craig Broberg and John, {Anitha S.} and Amy Harmon and Sainani, {Nisha I.} and Hill, {Andrew J.} and Odze, {Robert D.} and Johncilla, {Melanie E.} and Chinweike Ukomadu and Kimberlee Gauvreau and Valente, {Anne Marie} and Landzberg, {Michael J.}",
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    T1 - Liver health in adults with Fontan circulation

    T2 - A multicenter cross-sectional study

    AU - Wu, Fred M.

    AU - Kogon, Brian

    AU - Earing, Michael G.

    AU - Aboulhosn, Jamil A.

    AU - Broberg, Craig

    AU - John, Anitha S.

    AU - Harmon, Amy

    AU - Sainani, Nisha I.

    AU - Hill, Andrew J.

    AU - Odze, Robert D.

    AU - Johncilla, Melanie E.

    AU - Ukomadu, Chinweike

    AU - Gauvreau, Kimberlee

    AU - Valente, Anne Marie

    AU - Landzberg, Michael J.

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    Y1 - 2017/3/1

    N2 - Objectives Liver disease is an important contributor to morbidity and mortality in patients after Fontan surgery. There has been no large-scale survey of liver health in this population. We sought to explore the prevalence and predictors of liver disease in a multicenter cohort of adults with Fontan physiology. Methods Subjects were recruited from 6 adult congenital heart centers. Demographics; clinical history; and laboratory, imaging, and histopathology data were obtained. Results Of 241 subjects (median age 25.8 years [11.8-59.4], median time since Fontan 20.3 years [5.4-34.5]), more than 94% of those who underwent testing (208 of 221) had at least 1 abnormal liver-related finding. All hepatic imaging (n = 54) and liver histology (n = 68) was abnormal. Subjects with abnormal laboratory values had higher sinusoidal fibrosis stage (2 vs 1, P = .007) and higher portal fibrosis stage (3 vs 1, P = .003) compared with those with all normal values. Low albumin correlated with lower sinusoidal fibrosis stage (1 vs 2; P = .02) and portal fibrosis stage (0 vs 3, P = .002); no other liver studies correlated with fibrosis. Regenerative nodules were seen on 33% of histology specimens. Conclusions Regardless of modality, findings of liver disease are common among adults with Fontan circulation, even those appearing clinically well. Cirrhosis is present in up to one-third of subjects. Correlations between hepatic fibrosis stage and clinical history or findings on noninvasive testing are few. Further research is needed to identify patients at risk for more severe liver disease and to determine the best methods for assessing liver health in this population.

    AB - Objectives Liver disease is an important contributor to morbidity and mortality in patients after Fontan surgery. There has been no large-scale survey of liver health in this population. We sought to explore the prevalence and predictors of liver disease in a multicenter cohort of adults with Fontan physiology. Methods Subjects were recruited from 6 adult congenital heart centers. Demographics; clinical history; and laboratory, imaging, and histopathology data were obtained. Results Of 241 subjects (median age 25.8 years [11.8-59.4], median time since Fontan 20.3 years [5.4-34.5]), more than 94% of those who underwent testing (208 of 221) had at least 1 abnormal liver-related finding. All hepatic imaging (n = 54) and liver histology (n = 68) was abnormal. Subjects with abnormal laboratory values had higher sinusoidal fibrosis stage (2 vs 1, P = .007) and higher portal fibrosis stage (3 vs 1, P = .003) compared with those with all normal values. Low albumin correlated with lower sinusoidal fibrosis stage (1 vs 2; P = .02) and portal fibrosis stage (0 vs 3, P = .002); no other liver studies correlated with fibrosis. Regenerative nodules were seen on 33% of histology specimens. Conclusions Regardless of modality, findings of liver disease are common among adults with Fontan circulation, even those appearing clinically well. Cirrhosis is present in up to one-third of subjects. Correlations between hepatic fibrosis stage and clinical history or findings on noninvasive testing are few. Further research is needed to identify patients at risk for more severe liver disease and to determine the best methods for assessing liver health in this population.

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    KW - congenital heart disease

    KW - congestive hepatopathy

    KW - Fontan circulation

    KW - hepatic fibrosis

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