Masanari Kuwabara1,2,3, Koichiro Niwa2, Tomohiko Toyoda4, Takeaki Shirai5, Shigeru Tateno4, Hideo Ohuchi6, Yasuhiko Tanaka7, Fukiko Ichida8, Tomoo Fujisawa9, Teiji Akagi10, Yoshiki Mori11. 1. Department of Cardiology, Toranomon Hospital. 2. Department of Cardiology, Cardiovascular Center, St. Luke's International Hospital. 3. Division of Renal Diseases and Hypertension, School of Medicine, University of Colorado Denver. 4. Chiba Cardiovascular Center. 5. Kakogawa City Hospital Organization. 6. National Cerebral and Cardiovascular Center. 7. Shizuoka Children's Hospital. 8. Department of Pediatrics, Faculty of Medicine, University of Toyama. 9. Saiseikai Yokohama City Tobu Hospital. 10. Cardiac Intensive Care Unit, Okayama University Graduate School of Medicine. 11. Seirei Hamamatsu Hospital.
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.
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.
Authors: Roberta Angelico; Veronica Lisignoli; Lidia Monti; Rosanna Pariante; Chiara Grimaldi; Maria Cristina Saffioti; Maria Giulia Gagliardi; Marco Spada Journal: Int J Surg Case Rep Date: 2019-05-23