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Published on: September 13, 2014
Heterogeneous outcomes of liver disease after heart transplantation for a failed Fontan procedure
Aecha M Ybarra1, Geetika Khanna2, Yumirle P Turmelle2
1Department of Pediatrics, Washington University School of Medicine, St. Louis, MO, USA.
Insights
Heart transplant improved Fontan-associated liver disease (FALD) in most patients, with serial imaging showing resolution of congestion and fibrosis. However, outcomes are heterogeneous, underscoring the need for ongoing liver monitoring post-transplant.
Area of Science:
- Cardiology
- Hepatology
- Transplantation Medicine
Background:
- Fontan-associated liver disease (FALD) is a common complication in patients with long-term Fontan physiology.
- The impact of isolated heart transplant (HT) on the progression of FALD remains incompletely understood.
Purpose of the Study:
- To evaluate the effect of isolated heart transplant on Fontan-associated liver disease.
- To assess changes in liver imaging before and after heart transplant in Fontan patients.
Main Methods:
- Retrospective analysis of serial liver imaging in Fontan HT recipients.
- Review of patient demographics, clinical data, and liver biopsy results.
- Assessment of liver imaging at least one year post-heart transplant.
Main Results:
- 15 out of 19 patients showed improvement in liver imaging post-HT.
- 13 patients with pre-HT abnormal liver imaging normalized after transplant.
- Heterogeneous outcomes observed, including one case of cirrhosis progression.
Conclusions:
- Heart transplant can lead to improvement in Fontan-associated liver disease.
- Serial liver imaging is crucial for monitoring FALD post-heart transplant.
- Liver outcomes post-HT in Fontan patients are variable.
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.
