Department of Pediatrics, Washington University School of Medicine, St. Louis, MO, USA.
Department of Radiology, Washington University School of Medicine, St. Louis, MO, USA.
Pediatr Transplant. 2021 Dec;25(8):e14094. doi: 10.1111/petr.14094. Epub 2021 Jul 22.
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.
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.
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.
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.
Fontan 相关肝疾病（FALD）普遍影响长期接受 Fontan 生理的患者。孤立心脏移植（HT）对 HT 后 FALD 病程的影响尚不清楚。
我们评估了 HT 前后的系列肝脏影像学检查，以在单中心回顾性分析中评估 Fontan HT 受者的肝脏变化，这些受者具有 HT 前后的肝脏影像学检查。回顾了可获得的患者人口统计学和临床数据，包括可用的肝活检结果。
19 名患者的系列肝脏影像学检查可用，HT 时的中位年龄为 12 岁（范围 3-23），从 Fontan 到 HT 的中位年龄为 5.7 岁（范围 0.8-16），从影像学检查到随访的中位时间为 27 个月（范围 12-136 个月）。HT 前的肝脏影像学检查分类如下：正常（n=1）、充血（n=9）、纤维化（n=7）和肝硬化（n=2）。大多数移植患者（15/19）的 HT 后肝脏影像学检查得到改善，包括 13 名 HT 前影像学异常的患者在随访时肝脏影像学检查正常。1 名患者在 26 个月随访时仍有肝硬化，1 名患者在 18 个月随访时纤维化无变化，1 名患者在 136 个月时从 HT 前纤维化进展为肝硬化。在 HT 前后随访期间，没有患者出现明显孤立性肝衰竭。肝活检与影像学检查结果不一致。
Fontan 患者的 HT 后肝脏影像学评估显示出不同的肝脏结局。这些结果不仅为 HT 后 FALD 的改善提供了证据，也表明需要对 HT 后进行连续的肝脏影像学随访。
