Development of a non-invasive evaluation for liver fibrosis using elastography in patients after Fontan operation

2020 Fiscal Year Final Research Report

• Project/Area Number: 18K15725
• Research Category: Grant-in-Aid for Early-Career Scientists
• Allocation Type: Multi-year Fund
• Review Section: Basic Section 52050:Embryonic medicine and pediatrics-related
• Research Institution: Osaka City University
• Principal Investigator: Cho Yuki 大阪市立大学, 大学院医学研究科, 医員 (80779398)
• Project Period (FY): 2018-04-01 – 2021-03-31
• Keywords: 肝硬度 / 門脈圧 / Fontan術後肝障害 / 肝線維化 / 超音波エラストグラフィ

Outline of Final Research Achievements

We aimed to clarify the correlation between liver stiffness (LS) and liver fibrosis stage in Fontan patients. We undertook liver biopsy, cardiac catheterization, and laboratory tests in 22 of these patients (median age, 14.7 years; range, 9.9-32.1 years) with LS > 11.0 kPa (median, 19.2 kPa; range, 12.2-39.8 kPa); these elevated LS values suggest liver cirrhosis. Histologically, all patients showed mild-to-severe portal and sinusoidal fibrosis but no cirrhosis. Statistically, LS did not predict histological liver fibrosis scores (p = 0.175). Liver stiffness was not correlated with central venous pressure (p = 0.456) or with the hepatic venous pressure gradient (HVPG; p = 0.062), although the p value for HVPG was only slightly above the threshold for significance. Liver stiffness could be influenced by HVPG, and using the conventional cut-off values for LS overestimates and overtreats liver fibrosis in these patients.

Free Research Field

小児期発症慢性肝疾患

Academic Significance and Societal Importance of the Research Achievements

Fontan術後には従来の検査手法（超音波エラストグラフィや血液検査）では正確な評価が難しい特異な肝線維化の進展が高い比率でみられる。少なくとも術後10年以降は肝臓の合併症の有無について専門的な医療機関で定期的な診療を受けていただくことが望ましい。Fontan術後の肝線維化を的確に評価しうるバイオマーカーの開発が今後必要である。