As the population of patients with Fontan palliation grows, so does the number of patients with Fontan failure, necessitating heart transplantation. However, due to mainly small-sized studies, outcomes after heart transplantation in these patients remain unclear. The objective of this study was to review the available literature and conduct a meta-analysis to provide well-powered and generalizable estimates of outcomes after heart transplantation in patients with a failing Fontan. PubMed, Embase, and MEDLINE databases were searched for original studies of patients with a failing Fontan who underwent heart transplantation. The outcomes included were 1-year and 5-year survival, acute rejection, renal dysfunction, sepsis, and multi-organ failure. Proportions were pooled using random effects models to derive pooled proportions (PPs) and corresponding 95% confidence intervals (CIs). Meta-regression analysis was done to study the effects of age and gender on key outcomes. Sixteen retrospective single-center cohort studies with 426 Fontan patients undergoing heart transplantation were included in this meta-analysis. Pooled analysis of this study further revealed that 1-year survival after heart transplantation was 79.9% ([75.8%, 83.7%]; I2 = 1.92%), and 5-year survival rate was 72.5% ([62.1%, 81.9%]; I2 = 72.12%). Secondary outcomes after heart transplantation of failed Fontan procedure were acute rejection (PP 20% [7.4%, 36.8%]; I2 = 72.48%), renal dysfunction (PP 31.3% [10.5%, 57.2%]; I2 = 75.42%), multi-organ failure (PP 18.6% [2.8 to 43.9%]; I2= 69.60%), and sepsis (PP 21.1% [9%, 36.8%]; I2 = 61.19%). Cardiac transplantation in patients with a failing Fontan is associated with acceptable interventional success and improved survival rates.

中文翻译：

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丰坦衰竭患者心脏移植的疗效：系统评价和荟萃分析

随着Fontan病患者的数量增加，Fontan衰竭患者的数量也在增加，因此必须进行心脏移植。但是，由于主要是小型研究，这些患者的心脏移植后结局仍不清楚。这项研究的目的是回顾现有文献并进行荟萃分析，以对功能衰竭的Fontan患者进行心脏移植后，提供有力且可概括的结局评估。检索PubMed，Embase和MEDLINE数据库，以获取接受心脏移植的Fontan衰竭患者的原始研究。结果包括1年和5年生存，急性排斥反应，肾功能不全，败血症和多器官衰竭。使用随机效应模型合并比例，以得出合并比例（PPs）和相应的95％置信区间（CIs）。进行元回归分析以研究年龄和性别对关键结局的影响。这项荟萃分析包括16项回顾性单中心队列研究，对426名接受心脏移植手术的Fontan患者进行了研究。这项研究的汇总分析进一步表明，心脏移植后1年生存率为79.9％（[75.8％，83.7％]； I2 = 1.92％），5年生存率为72.5％（[62.1％，81.9％] ； I2 = 72.12％）。心脏移植失败的Fontan手术后的次要结局为急性排斥反应（PP 20％[7.4％，36.8％]; I2 = 72.48％），肾功能不全（PP 31.3％[10.5％，57.2％]; I2 = 75.42％），多器官衰竭（PP 18.6％[2.8至43.9％]； I2 = 69.60％）和败血症（PP 21.1％[9％，36.8％]）；I2 = 61.19％）。Fontan衰竭患者的心脏移植与可接受的介入成功和提高的生存率相关。