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在衰竭的 Fontan 患者中进行心脏和肝脏联合移植后的死亡率和发病率:一项更新的双中心回顾性研究。

Mortality and morbidity after combined heart and liver transplantation in the failing Fontan: An updated dual center retrospective study.

机构信息

Division of Cardiovascular Medicine, Department of Medicine, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania, USA.

Division of Cardiology, Department of Pediatrics, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.

出版信息

Clin Transplant. 2024 Apr;38(4):e15302. doi: 10.1111/ctr.15302.

DOI:10.1111/ctr.15302
PMID:38567883
Abstract

INTRODUCTION

As the adult Fontan population with Fontan associated liver disease continues to increase, more patients are being referred for transplantation, including combined heart and liver transplantation.

METHODS

We report updated mortality and morbidity outcomes after combined heart and liver transplant in a retrospective cohort series of 40 patients (age 14 to 49 years) with Fontan circulation across two centers from 2006-2022.

RESULTS

The 30-day, 1-year, 5-year and 10-year survival rate was 90%, 80%, 73% and 73% respectively. Sixty percent of patients met a composite comorbidity of needing either post-transplant mechanical circulatory support, renal replacement therapy or tracheostomy. Cardiopulmonary bypass time > 283 min (4.7 h) and meeting the composite comorbidity were associated with mortality by Kaplan Meier analysis.

CONCLUSION

Further study to mitigate early mortality and the above comorbidities as well as the high risk of bleeding and vasoplegia in this patient population is warranted.

摘要

引言

随着患有 Fontan 相关肝疾病的成年 Fontan 人群不断增加,越来越多的患者被转介接受移植,包括心脏和肝脏联合移植。

方法

我们报告了在两个中心从 2006 年至 2022 年接受 Fontan 循环的 40 名(年龄 14 至 49 岁)患者的回顾性队列系列中,心脏和肝脏联合移植后的死亡率和发病率的最新结果。

结果

30 天、1 年、5 年和 10 年的生存率分别为 90%、80%、73%和 73%。60%的患者出现了需要移植后机械循环支持、肾脏替代治疗或气管切开术的复合合并症。根据 Kaplan-Meier 分析,体外循环时间>283 分钟(4.7 小时)和出现复合合并症与死亡率相关。

结论

需要进一步研究以减轻该患者群体的早期死亡率和上述合并症,以及出血和血管扩张的高风险。

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引用本文的文献

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JTCVS Tech. 2024 Dec 23;31:94-96. doi: 10.1016/j.xjtc.2024.12.006. eCollection 2025 Jun.
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Comprehensive Multiomic Analysis Reveals Metabolic Reprogramming Underlying Human Fontan-Associated Liver Disease.综合多组学分析揭示人类Fontan相关肝病背后的代谢重编程。
J Am Heart Assoc. 2025 Mar 18;14(6):e039201. doi: 10.1161/JAHA.124.039201. Epub 2025 Mar 7.