Background

Challenges exist with heterotaxy due to the complexity of heart disease, abnormal venous connections, and infection risks. This study aims to understand heart transplant outcomes for children with heterotaxy.

Methods

All children with congenital heart disease listed for transplant from 1993 to 2018 were included. Those with and without heterotaxy were compared. Waitlist outcomes and survival post-listing and transplant were analyzed. Post-transplant risk factors were identified using multiphase parametric hazard modeling.

Results

There were 4814 children listed, of whom 196 (4%) had heterotaxy. Heterotaxy candidates were older (5.8 ± 5.7 vs 4.2 ± 5.5 years, p < 0.01), listed at a lower urgency status (29.8% vs 18.4%, p < 0.01), more commonly single ventricle physiology (71.3% vs 59.2%, p < 0.01), and less often supported by mechanical ventilation (22% vs 29.1%, p < 0.05) or extracorporeal membrane oxygenation (3.6% vs 7.5%, p < 0.05). There were no differences in waitlist outcomes of transplant, death, or removal. Overall, post-transplant survival was worse for children with heterotaxy: one-year survival 77.2% vs 85.1%, with and without heterotaxy, respectively. Heterotaxy was an independent predictor for early mortality in the earliest era (1993-2004), HR 2.09, CI 1.16-3.75, p = 0.014. When stratified by era, survival improved with time. Heterotaxy patients had a lower freedom from infection and from severe rejection, but no difference in vasculopathy or malignancy.

Conclusions

Mortality risk associated with heterotaxy is mitigated in the recent transplant era. Early referral may improve waitlist outcomes for heterotaxy patients who otherwise have a lower status at listing. Lower freedom from both infection and severe rejection after transplant in heterotaxy highlights the challenges of balancing immune suppression.

中文翻译：

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当前时代先天性心脏病和异位综合征患儿心脏移植存活率的提高：来自儿科心脏移植学会的分析

背景

由于心脏病的复杂性、异常的静脉连接和感染风险，异向性存在挑战。本研究旨在了解异位儿童的心脏移植结果。

方法

纳入了 1993 年至 2018 年列入移植名单的所有先天性心脏病儿童。比较了有和没有异向性的那些。分析了候补名单的结果以及上市和移植后的生存情况。使用多阶段参数风险模型确定移植后风险因素。

结果

列出了 4814 名儿童，其中 196 名（4%）有异位症。异位患者年龄较大（5.8 ± 5.7 vs 4.2 ± 5.5 岁，p < 0.01），列为较低紧急状态（29.8% vs 18.4%，p < 0.01），更常见的是单心室生理学（71.3% vs 59.2%，p < 0.01)，机械通气 (22% vs 29.1%, p < 0.05) 或体外膜肺氧合 (3.6% vs 7.5%, p< 0.05)。移植、死亡或移除的候补名单结果没有差异。总体而言，异位儿童的移植后存活率更差：一年生存率分别为 77.2% 和 85.1%，分别有和没有异位。异位性是最早时期（1993-2004）早期死亡率的独立预测因子，HR 2.09，CI 1.16-3.75，p  = 0.014。当按时代分层时，生存率随着时间的推移而提高。异位性患者的感染和严重排斥反应较低，但血管病变或恶性肿瘤没有差异。

结论

在最近的移植时代，与异向性相关的死亡风险得到了缓解。早期转诊可能会改善异位患者的候补结果，否则这些患者在列表中的地位较低。异种移植后感染和严重排斥的降低突出了平衡免疫抑制的挑战。