Heterogenerous Outcomes of Liver Disease after Heart Transplantation for a Failed Fontan Procedure

Purpose Hepatic fibrosis and cirrhosis have been recognized as complications after the Fontan procedure, which may preclude heart-only transplantation (HT). We and others have reported successful short-term survival for HT but to date the fate of the liver after HT is unknown. We hypothesized that HT would lead to reversal of hepatic pathology in the failed Fontan HT recipient. Methods Pre- and post-HT liver imaging by CT scan or MRI were evaluated and classified as 1) no pathology, 2) fibrosis/congestive hepatopathy (F/CH), and 3) cirrhosis by the same evaluator in a similar fashion to our previous analysis. Congestive hepatopathy and early fibrosis were analyzed as one group, as differentiating between the two entities with conventional imaging is challenging. Transaminases, total bilirubin, serum albumin, and INR were measured routinely as part of post-HT surveillance. Results 13 HT recipients for failed Fontan procedure were evaluated: 5 had normal findings (Group A) and 8 [F/CH-6, cirrhosis-2] were abnormal at transplant (Group B). Median age at HT were 8.9 yr (7.5 - 19.8 yr) for Group A and 8.6 yr (4.7-19.2 yr) for Group B. None of the subjects in either group developed abnormalities of serum liver studies or overt liver failure. All patients with F/CH at time of HT had resolution of F/CH post-HT with a median follow-up time of scan of 28 mo (range 9.8-52 mo). Cirrhosis was persistent in one recipient scanned at 23 mo, but had resolved in the other when scanned 46 mo post-HT. 2 subjects in Group A developed liver disease: 1 with cirrhosis at 136 mo post-HT in association with PTLD and 1 with mild F/CH changes at 79 mo post-Tx in association with coronary vasculopathy. Conclusion This pilot study suggests liver disease as detected by conventional imaging in failed Fontan recipients generally resolves post-HT, but new onset liver disease can occur in recipients whose livers were normal at transplant. These findings need to be confirmed in a larger sample of recipients, but suggest all failed Fontan HT recipients should have ongoing surveillance for liver disease after HT regardless of pathology at time of HT.