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.
Introduction:
Recent studies have suggested a relationship between mortality and center volume in pediatric heart transplantation; however, these studies utilize databases that lack granularity to assess patient risk profiles.
Hypothesis:
Utilizing the Pediatric Heart Transplant Society (PHTS) database, we hypothesized that there would be a difference in outcomes among centers based on yearly transplant volume, and that the patient’s clinical profiles would differ by center volume.
Methods:
We performed a retrospective analysis of the PHTS dataset from all pediatric (≤ 18 years of age) cases from 2009-2018. Centers were stratified into 5 groups based on annual volume: ≤ 4, 4-6, 6-9, 9-14, and > 14 transplants. We evaluated specific variables, including those identified as significant risk factors in previous PHTS studies including mechanical ventilation, PRA > 10%, high BUN, gender, and Fontan completion, within each group. We used Kaplan Meier for survival, contingency tables with Pearson Chi-square test for association, and analysis-of-variance to compare means. P< 0.05 was considered statistically significant.
Results:
There were 6800 cases among 55 centers. Table 1 demonstrates that there was no significant difference in one-year post transplant survival among the groups, and the patient variables showing statistically significant differences followed no specific pattern among the center volume groups. It also demonstrates that there is no clear association between cumulative risk factors within individual patients and center volume.
Conclusions:
One-year post transplant survival is similar within the PHTS for all centers regardless of case volume. These group differences were different for different variables and did not segregate consistently between lower volume and higher volume groups suggesting there exists a complicated relationship among risk factors and center volume.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.