TY - JOUR
T1 - Liver cirrhosis and/or hepatocellular carcinoma occurring late after the fontan procedure
T2 - ― A nationwide survey in Japan ―
AU - for the Research Committee of the Japanese Society of Pediatric Cardiology Cardiac Surgery
AU - Kuwabara, Masanari
AU - Niwa, Koichiro
AU - Toyoda, Tomohiko
AU - Shirai, Takeaki
AU - Tateno, Shigeru
AU - Ohuchi, Hideo
AU - Tanaka, Yasuhiko
AU - Ichida, Fukiko
AU - Fujisawa, Tomoo
AU - Akagi, Teiji
AU - Mori, Yoshiki
N1 - Funding Information:
The authors thank the patients and all staff in all the participating institutions (Shizuoka Children’s Hospital, Chiba Cardiovascular Center, Tokyo Women’s Medical University, Okayama University, Kyoto University, Kyoto Prefectural University, Tsukuba University, Toyama University, NTT-East Sapporo Hospital, Amagasaki Hospital, and Okahata Clinic) for their assistance with detailed data collection. The authors thank the staff in the facilities belonging to the JPCCS for help with data collection. M.K. received a grant to study abroad from the Federation of National Public Service Personnel Mutual Aid Association in Japan. Additional members of the Research Committee of Japanese Society of Pediatric Cardiology and Cardiac Surgery are: Takahiro Ishiwata and Takashi Kanai (National Defence Medical College); Hitoshi Kato and Shunsuke Nosaka (National Center for Children Health and Development); Eiichi Kohda and Tsutomu Saji (Toho University); Arata Murakami (Tokyo University); Makoto Nakazawa (Minami Tohoku Hospital); Shigeyuki Echigo (Echigo Clinic); Kisaburo Sakamoto (Shizuoka Children’s Hospital); Takuya Ueda (St. Luke’s International Hospital); and Hideshi Yamamura and Mikiko Shimizu (Tokyo Women’s Medical University).
Publisher Copyright:
© 2018, Japanese Circulation Society. All rights reserved.
PY - 2018
Y1 - 2018
N2 - 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.
AB - 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.
KW - Diagnosis
KW - Hepatocellular carcinoma
KW - Liver cirrhosis
KW - Risk factors
UR - http://www.scopus.com/inward/record.url?scp=85044294131&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85044294131&partnerID=8YFLogxK
U2 - 10.1253/circj.CJ-17-1053
DO - 10.1253/circj.CJ-17-1053
M3 - Article
C2 - 29445059
AN - SCOPUS:85044294131
SN - 1346-9843
VL - 82
SP - 1155
EP - 1160
JO - Circulation Journal
JF - Circulation Journal
IS - 4
ER -