SHIMADA ERIKO
Department School of Medicine(Tokyo Women's Medical University Hospital), School of Medicine Position Assistant Professor |
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Article types | Original article |
Language | English |
Peer review | Non peer reviewed |
Title | Risk factors for Fontan-associated hepatocellular carcinoma. |
Journal | Formal name:PloS one Abbreviation:PLoS One ISSN code:19326203/19326203 |
Domestic / Foregin | Foregin |
Volume, Issue, Page | 17(6),pp.e0270230 |
Author and coauthor | KOGISO Tomomi†, SAGAWA Takaomi, TANIAI Makiko, SHIMADA ERIKO, Inai Kei, SHINOHARA Tokuko, TOKUSHIGE Katsutoshi |
Publication date | 2022/01 |
Summary | AIMS:The incidence of hepatocellular carcinoma (HCC) in patients with Fontan-associated liver disease (i.e., FALD-HCC) has increased over time. However, the risk factors for HCC development remain unclear. Here, we compared the levels of non-invasive markers to the survival rate of FALD-HCC patients.METHODS:From 2003 to 2021, 154 patients (66 men, 42.9%) developed liver disease after undergoing Fontan procedures. HCC was diagnosed in 15 (9.7%) (8 men, 53.3%) at a median age of 34 years (range, 21-45 years). We compared FALD-HCC and non-HCC cases; we generated marker level cutoffs using receiver operating characteristic curves. We sought to identify risk factors for HCC and mortality.RESULTS:The incidence of HCC was 4.9% in FALD patients within 20 years after the Fontan procedure. Compared with non-HCC patients, FALD-HCC patients exhibited higher incidences of polysplenia and esophageal varices. At the time of HCC development, the hyaluronic acid (HA) level (p = 0.04) and the fibrosis-4 index (p = 0.02) were significantly higher in FALD-HCC patients than in non-HCC patients; the total bilirubin (T-BIL) level (p = 0.07) and the model for end-stage liver disease score [excluding the international normalized ratio (MELD-XI)] (p = 0.06) tended to be higher in FALD-HCC patients. Within approximately 20 years of the Fontan procedure, 10 patients died (survival rate, 96.9%). Kaplan-Meier curve analysis indicated that patients with T-BIL levels ≥ 2.2 mg/dL, HA levels ≥ 55.5 ng/mL, and MELD-XI scores ≥ 18.7 were at high risk of HCC, a generally poor prognosis, and both polysplenia and esophageal varices. Multivariate Cox regression analyses indicated that the complication of polysplenia [Hazard ratio (HR): 10.915] and a higher MELD-XI score (HR: 1.148, both p < 0.01) were independent risk factors for FALD-HCC.CONCLUSIONS:The complication of polysplenia and a MELD-XI score may predict HCC development and mortality in FALD patients. |
DOI | 10.1371/journal.pone.0270230 |
PMID | 35714161 |