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限制型心肌病患者的心脏移植结局:器官共享联合网络(UNOS)过去三十年的登记分析

Heart transplant outcomes in restrictive cardiomyopathy: UNOS registry analysis of the last three decades.

作者信息

Miklin Daniel J, DePasquale Eugene C

机构信息

North Shore University Hospital, Northwell Health, Manhasset, New York.

University of Southern California School of Medicine, Los Angeles, California.

出版信息

JHLT Open. 2023 Dec 5;3:100031. doi: 10.1016/j.jhlto.2023.100031. eCollection 2024 Feb.

DOI:10.1016/j.jhlto.2023.100031
PMID:40145113
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11935335/
Abstract

BACKGROUND

Restrictive cardiomyopathy (RCM) comprises diverse etiologies with overall poor prognosis. Emerging therapies have significantly impacted some of these entities. However, these therapies may have limited impact in the end-stages and have only recently become available. We sought to assess outcomes before and after transplant in the RCM population stratified into 3 distinct time periods from the recent era.

METHODS

Retrospective analysis of the United Network of Organ Sharing registry ( = 62,709) for all patients transplanted between 1987 and March 1, 2022, were stratified by RCM status with 1157 patients with RCM. Populations were grouped temporally into (1987-2000), (2000-2013), and (2014-2022) eras. Multiorgan and repeat transplants were excluded from the analysis. Baseline demographics, listing status, hemodynamics, donor information, and life support methods were compared using Kruskal-Wallis and Pearson's tests. Longitudinal survival was assessed via Kaplan-Meier survival analysis. Univariate and multivariate analyses using Cox modeling and competing outcomes analyses were performed.

RESULTS

RCM patients were older, female, with older donors and longer ischemic times ( < 0.001). There were no significant differences in overall survival compared to the non-RCM population, however, with increased transplant rates. Amyloidosis and chemotherapy/radiation portend the worst prognosis but have shorter waitlist times and up-trending survival in the era.

CONCLUSIONS

RCM represents a small but significant population of those requiring heart transplant. RCM transplant outcomes appear to be improving across all subsets with shorter wait times and better survival. Early recognition is important to help mitigate adverse outcomes.

摘要

背景

限制性心肌病(RCM)病因多样,总体预后较差。新兴疗法对其中一些病因产生了重大影响。然而,这些疗法在终末期的影响可能有限,且直到最近才开始应用。我们试图评估近期三个不同时间段分层的RCM人群移植前后的结局。

方法

对器官共享联合网络登记处(n = 62,709)1987年至2022年3月1日期间所有接受移植的患者进行回顾性分析,根据RCM状态分层,其中1157例为RCM患者。人群按时间分为三个时代:(1987 - 2000年)、(2000 - 2013年)和(2014 - 2022年)。多器官移植和再次移植被排除在分析之外。使用Kruskal - Wallis检验和Pearson检验比较基线人口统计学、登记状态、血流动力学、供体信息和生命支持方法。通过Kaplan - Meier生存分析评估纵向生存情况。使用Cox模型进行单因素和多因素分析以及竞争结局分析。

结果

RCM患者年龄较大,女性居多,供体年龄较大且缺血时间较长(P < 0.001)。与非RCM人群相比,总体生存率无显著差异,但移植率有所增加。淀粉样变性和化疗/放疗预示着最差的预后,但在时代III中等待名单时间较短且生存率呈上升趋势。

结论

RCM是需要心脏移植的一小部分但重要的人群。RCM移植结局在所有亚组中似乎都在改善,等待时间更短,生存率更高。早期识别对于减轻不良结局很重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/084c/11935335/6f695d20fef0/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/084c/11935335/94b200f45021/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/084c/11935335/bdf3442f08e0/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/084c/11935335/9820d0fda965/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/084c/11935335/6f695d20fef0/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/084c/11935335/94b200f45021/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/084c/11935335/bdf3442f08e0/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/084c/11935335/9820d0fda965/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/084c/11935335/6f695d20fef0/gr4.jpg

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本文引用的文献

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Restrictive cardiomyopathy: definition and diagnosis.限制型心肌病:定义与诊断。
Eur Heart J. 2022 Dec 1;43(45):4679-4693. doi: 10.1093/eurheartj/ehac543.
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Genetic Insights into Primary Restrictive Cardiomyopathy.原发性限制性心肌病的遗传学见解
J Clin Med. 2022 Apr 8;11(8):2094. doi: 10.3390/jcm11082094.
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Restrictive cardiomyopathy: from genetics and clinical overview to animal modeling.限制型心肌病:从遗传学和临床概述到动物模型。
Rev Cardiovasc Med. 2022 Mar 17;23(3):108. doi: 10.31083/j.rcm2303108.
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Progress and challenges in the treatment of cardiac amyloidosis: a review of the literature.心脏淀粉样变治疗的进展与挑战:文献综述。
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