Abstract 17227: Late Hepatic Complications Following Fontan Conversion Surgery
Introduction: Fontan conversion surgery has extended survival for patients with univentricular heart, yet raised concerns regarding durability of hepatic function.
Methods: We reviewed liver outcomes among transplant-free survivors of Fontan conversion with arrhythmia surgery at our institution. Hepatic screening included a combination of annual liver function testing with clinically indicated imaging and/or biopsy.
Results: Between 1994 - 2014, 145 patients underwent Fontan conversion surgery at a median age of 23 (3-47) years, following initial Fontan surgery at median age of 6 (1 -35) years. Initial Fontan surgery types were atriopulmonary (79%), atrioventricular (13%), and lateral tunnel (8%). Early mortality was 2% following Fontan conversion surgery, with 10-year transplant free-survival of 84%. During median follow-up of 9 (1-20) years among 130 transplant-free survivors, significant liver disease developed in 6 patients (4.6%): hepatocellular carcinoma in 5 patients (primary 4, metastatic from appendix 1), and fulminant liver failure in one patient. Median age at diagnosis of hepatocellular carcinoma/liver failure was 35 (28-47) years, a median of 27 (22-33) years following initial Fontan surgery. Carcinoma was detected on annual hepatic screening with ultrasound in 3, and by elevated alpha-fetoprotein level prompting imaging in 2 patients. None of the patients had clinical variceal bleeding. Preoperative status included heterotaxy syndrome in 2, NYHA Classification III-IV in 5, and long standing ascites in 2 patients. Death occurred in 3/6 patients at a mean of 1.4 years following diagnosis of hepatic disease.
Conclusion: Late hepatocellular carcinoma/liver failure was found in 4.6% of non-transplanted Fontan conversion patients, indicating the need for serial monitoring of liver status. Early detection of carcinoma with routine screening using American Association for the Study of Liver Diseases guidelines may allow for improved treatment strategies and prolonged survival.
Author Disclosures: B.J. Deal: None. J. Johnson: None. G. Webster: None. S. Tsao: None. B.S. Marino: None. J.M. Costello: None. A. De Freitas: None. D.R. Ganger: None.
- © 2015 by American Heart Association, Inc.