Holmvard Oscar, Póvoa-Corrêa Mariana, Macintyre Innocenzi Adriana, Pacheco Lucio Filgueiras, Parente Daniella Braz, Luiz Ronir Raggio, de Lima Moreira Jessica Pronestino, de Mello Perez Renata, Fernandes Fernanda Padrão, Moll-Bernardes Renata
D'Or Institute for Research and Education (IDOR), Rio de Janeiro, RJ, Brazil.
Maternal and Child Health Department, Federal University of Rio de Janeiro (UFRJ), Macaé, RJ, Brazil.
Pediatr Transplant. 2025 Nov;29(7):e70174. doi: 10.1111/petr.70174.
Fontan-associated liver disease can progress to advanced fibrosis, raising the potential need for combined heart-liver transplantation (CHLT) in selected patients. However, the benefits of CHLT over isolated orthotopic heart transplantation (HT), particularly in terms of mortality, remain uncertain. In this systematic review, we compared mortality outcomes following CHLT versus HT in patients with Fontan circulation, with the aim of supporting clinical decision-making.
This systematic review was conducted according to the 2020 PRISMA guidelines and registered in PROSPERO. PubMed, Scopus, and Embase were searched. Studies examining HT or CHLT in patients with Fontan circulation that provided information about total and/or 1-year mortality were included. Bias risks were assessed using the Newcastle-Ottawa Scale. We used random- and fixed-effect models, depending on heterogeneity, to estimate pooled effects.
Sixteen studies were included in this analysis. CHLT was associated with a lower mortality rate per patient-year compared to HT (0.03 vs. 0.09; p < 0.01). However, after excluding studies in which transplantations were performed before the year 2000, the difference between groups was no longer statistically significant. One-year mortality rates were also not significantly different between CHLT and HT (0.09 vs. 0.14; p = 0.28), with similar results observed after excluding pre-2000 studies.
Overall, this systematic review suggests that CHLT may result in mortality rates comparable to those of isolated HT. These findings support the consideration of CHLT in patients with concomitant liver disease and reinforce the importance of comprehensive liver evaluation in transplant candidates.
与Fontan循环相关的肝病可进展为晚期纤维化，这使得部分患者可能需要进行心脏-肝脏联合移植（CHLT）。然而，CHLT相较于单纯原位心脏移植（HT）的益处，尤其是在死亡率方面，仍不明确。在本系统评价中，我们比较了Fontan循环患者接受CHLT和HT后的死亡率，旨在为临床决策提供支持。
本系统评价按照2020年PRISMA指南进行，并在PROSPERO中注册。检索了PubMed、Scopus和Embase。纳入了对Fontan循环患者进行HT或CHLT且提供了总死亡率和/或1年死亡率信息的研究。使用纽卡斯尔-渥太华量表评估偏倚风险。根据异质性，我们使用随机效应模型和固定效应模型来估计合并效应。
本分析纳入了16项研究。与HT相比，CHLT每患者年的死亡率较低（0.03对0.09；p<0.01）。然而，排除2000年前进行移植的研究后，两组之间的差异不再具有统计学意义。CHLT和HT的1年死亡率也无显著差异（0.09对0.14；p=0.28），排除2000年前的研究后结果相似。
总体而言，本系统评价表明CHLT的死亡率可能与单纯HT相当。这些发现支持在合并肝病的患者中考虑CHLT，并强化了对移植候选者进行全面肝脏评估的重要性。
