SHIMIZU MIKIKO
   Department   School of Medicine(Tokyo Women's Medical University Hospital), School of Medicine
   Position   Assistant Professor
Article types Original article
Language English
Peer review Peer reviewed
Title Risk factors and serological markers of liver cirrhosis after Fontan procedure.
Journal Formal name：Heart and vessels
Abbreviation：Heart Vessels
ISSN code：09108327/16152573
Domestic / ForeginDomestic
Publisher シュプリンガー･ジャパン株式会社
Volume, Issue, Page 31(9),pp.1514-1521
Author and coauthor SHIMIZU Mikiko†, MIYAMOTO Kenji, NISHIHARA Yunosuke, IZUMI Gaku, SAKAI Shuji, INAI Kei, NISHIKAWA Toshio, NAKANISHI Toshio*
Authorship Lead author
Publication date 2016/09
Summary Liver cirrhosis (LC), which may result in hepatic failure or cancer, has been reported in patients after Fontan procedure. The purpose of this study was to clarify the frequency and histological characteristics of LC, and to evaluate the risk factors and serological markers of LC with Fontan circulation. Retrospective review of contrast-enhanced CT scans (CT) of the liver was carried out in 57 patients after Fontan procedure. Patients were divided into two groups: LC group (n = 31) and no LC group (n = 26). Age at Fontan procedure, duration after Fontan procedure, catheterization data, and history of failing Fontan circulation were compared between groups. Serological data including γ-GTP and hyaluronic acid were compared. Histology of autopsy specimens was assessed when available. Duration after Fontan procedure was significantly longer in LC group than no LC group. History of failing Fontan circulation was more frequent in LC group than in no LC group. There was no correlation between type of procedure (APC/Bjork/lateral tunnel/TCPC) and LC in this series. Serum hyaluronic acid, γ-GTP, and Forns index were significantly higher in LC group. Significant risk factors for LC were duration after Fontan procedure (>20 years). In autopsy specimens, histopathological changes of LC were observed predominantly in the central venous area. LC diagnosed with CT is frequent in patients long after Fontan procedure, especially after 20 years. Hyaluronic acid and γ-GTP could be useful markers to monitor the progression of liver fibrosis in Fontan patients.
DOI 10.1007/s00380-015-0743-4
PMID 26386570