Heterogeneous outcomes of liver disease after heart transplantation for a failed Fontan procedure

Abstract

Background Fontan‐associated liver disease (FALD) uniformly affects patients with long‐term Fontan physiology. The effect of isolated heart transplant (HT) on the course of FALD post‐HT is not well understood. Methods We evaluated serial liver imaging pre‐ and post‐HT to assess liver changes over time in a single‐center retrospective analysis of Fontan HT recipients who had pre‐ and ≥1‐year post‐HT liver imaging. Available patient demographic and clinical data were reviewed, including available liver biopsy results. Results Serial liver imaging was available in 19 patients with a median age at HT of 12 years (range 3–23), the median age from Fontan to HT of 5.7 years (range 0.8–16), and the median time from imaging to follow up of 27 months (range 12–136 months). Pre‐HT liver imaging was classified as follows: normal (n=1), congested (n=9), fibrotic (n=7), and cirrhotic (n=2). The majority of transplanted patients (15/19) had improvement in their post‐HT liver imaging, including 13 patients with initially abnormal imaging pre‐HT having normal liver imaging at follow‐up. One patient had persistent cirrhosis at 26‐month follow‐up, one patient had unchanged fibrosis at 18‐month follow‐up, and one patient progressed from fibrosis pre‐HT to cirrhosis post‐HT at 136 months. No patients had overt isolated liver failure during pre‐ or post‐HT follow‐up. Liver biopsy did not consistently correlate with imaging findings. Conclusions Post‐HT liver imaging evaluation in Fontan patients reveals heterogeneous liver outcomes. These results not only provide evidence for the improvement of FALD post‐HT but also show the need for serial liver imaging follow‐up post‐HT.

A Novel Strategy for the Mechanical Subpulmonary Support in Failing Fontan Patients

“… 4 17 Indeed, FALD is also one of the major causes of heterogenous liver outcomes after 1 year post-HTx. 21 Reliable prognostic markers for HTx candidacy are currently lacking. 1 20 FALD may be associated with different manifestations, such as hepatic congestion, fibrosis, cirrhosis, focal nodular hyperplasia, hypervascular lesions, hepatocellular carcinoma, portal hypertension, varices, and ascites.…”

Review of pediatric combined heart‐liver transplantation: A roadmap to success

“…25 Retrospective review of a small cohort of 12 Fontan patients at St. Louis Children's Hospital with liver imaging past 1 year following HTA encompassed all possible outcomes, including worsening, stabilization, and improvement in fibrosis or congestion. 26 Taken together, this suggests that a subset of patients have the potential for substantial benefit in undergoing CHLT versus HTA. An American Society for Transplantation White Paper proposed an algorithm for HTA versus CHLT in the Fontan population.…”

“…Persistent liver fibrosis on serial imaging of patients with FALD following HTA is variable and dependent on imaging modality, with advanced fibrosis present in 50% of pre‐transplant and 54% post‐transplant patients using any modality 25 . Retrospective review of a small cohort of 12 Fontan patients at St. Louis Children's Hospital with liver imaging past 1 year following HTA encompassed all possible outcomes, including worsening, stabilization, and improvement in fibrosis or congestion 26 . Taken together, this suggests that a subset of patients have the potential for substantial benefit in undergoing CHLT versus HTA.…”

Fontan‐associated liver disease

“…[ 151 ] A retrospective study of 41 patients with Fontan physiology with liver imaging evaluated for IHT compared liver imaging studies obtained pretransplant with those done 1 year or longer after transplant. [ 152 ] The median age at the time of IHT was 12 years (range 3–23). Of these 41 patients, 12 patients (29.2%) had imaging evidence of cirrhosis before IHT.…”