Liver cirrhosis and/or hepatocellular carcinoma occurring late after the fontan procedure: ― A nationwide survey in Japan ―

for the Research Committee of the Japanese Society of Pediatric Cardiology Cardiac Surgery

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9 Citations (Scopus)

Abstract

Background: Fontan-associated liver disease (FALD) is an important late complication involving liver dysfunction, such as liver cirrhosis (LC) and hepatocellular carcinoma (HCC), in patients undergoing the Fontan procedure. However, the prevalence, clinical manifestation, and methods of diagnosis of FALD are still not well established. Methods and Results: This study comprised 2 nationwide surveys in Japan. First, the prevalence of LC and/or HCC in patients undergoing the Fontan procedure was determined. Second, clinical manifestations in patients with LC and/or HCC were analyzed, along with data from blood tests, echocardiography, and right heart catheterization. In the 1st survey, of the 2,700 patients who underwent the Fontan procedure, 31 were diagnosed with LC and/or HCC (1.15%), and 5 died due to liver diseases (mortality: 0.19%). In the 2nd survey, data were collected from 17 patients (12 with LC, 2 with HCC, and 3 with LC+HCC. Of these 17 patients, 5 died (mortality: 29.4%). The mean age at diagnosis of LC and HCC was 23 and 31 years, respectively. Computed tomography followed by ultrasound was most frequently used for diagnosis. Blood tests revealed low platelet counts, increased hemoglobin, aspartate aminotransferase, γ-guanosine triphosphate, and total bilirubin levels, and an elevated international normalized ratio of prothrombin time. Conclusions: LC and/or HCC in patients undergoing the Fontan procedure were not rare late complications and were associated with high mortality rates.

Original languageEnglish
Pages (from-to)1155-1160
Number of pages6
JournalCirculation Journal
Volume82
Issue number4
DOIs
Publication statusPublished - 2018

Keywords

  • Diagnosis
  • Hepatocellular carcinoma
  • Liver cirrhosis
  • Risk factors

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

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