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接受心脏移植的轻链淀粉样变性患者的长期预后:单中心经验

Long-term outcomes of light chain amyloidosis patients receiving heart transplant: A single-center experience.

作者信息

Patel Khush, Tu Zoe H, Yuen Carrie, Ganguly Siddhartha, Yun Allison N, Gorthi Janardhana R, Trachtenberg Barry H, Guha Ashrith, Kassi Mahwash, Kim Ju, Yousefzai Rayan, Baker Kelty, Rice Lawrence, Kamble Rammurti T, Adrogue Horacio E, Suarez Erik E, Ghobrial Mark, Saharia Ashish, Mobley Constance M, Victor David W, Krisl Jill C, Knight Richard, Osama Gaber A, Bhimaraj Arvind

机构信息

Department of General Surgery, J.C. Walter Jr. Transplant Center, Houston Methodist Hospital, Houston, Texas.

Department of Pharmacy, Houston Methodist Hospital, Houston, Texas.

出版信息

JHLT Open. 2025 Jun 22;9:100328. doi: 10.1016/j.jhlto.2025.100328. eCollection 2025 Aug.

Abstract

BACKGROUND

Cardiac amyloidosis is a less common indication for orthotopic heart transplantation (OHT). Light chain (AL) amyloidosis specifically poses challenges. While previous small studies have established the validity of OHT for AL amyloidosis, we present experience from a large contemporary series at a single center.

METHODS

This was a retrospective single-center study of patients receiving OHT between 2009 and 2023 at our institution. A data warehouse was curated from our electronic medical records and United Network for Organ Sharing databases. Univariable and multivariable analyses of donor and recipient characteristics were performed. Patients who received OHT after 2016 were active in our electronic medical record and hence AL-OHT patients after 2016 had granular details regarding immunosuppression, chemotherapy regimens, amyloidosis, and post-transplant-related outcomes within their first-year post-OHT.

RESULTS

Thirty-one patients received an OHT for cardiac AL while 595 received OHT for non-amyloid indication during the study period. One-, three-, five-, and eight-year survival (87%, 83%, 73%, and 67%, respectively) was not statistically different compared to non-AL OHT patients (92%, 85%, 79%, and 68%, respectively). In a subset of 18 patients with access to electronic medical record data, all patients remained on tacrolimus and prednisone through 1-year post-OHT, but nearly half were off mycophenolate.

CONCLUSION

Select AL patients with advanced cardiac involvement can gain survival advantage with OHT similar to other advanced heart failure patients.

摘要

背景

心脏淀粉样变性是原位心脏移植(OHT)较少见的适应证。轻链(AL)淀粉样变性尤其具有挑战性。虽然先前的小型研究已证实OHT用于AL淀粉样变性的有效性,但我们介绍了来自单一中心的大型当代系列研究的经验。

方法

这是一项对2009年至2023年在我们机构接受OHT的患者进行的回顾性单中心研究。从我们的电子病历和器官共享联合网络数据库中整理出一个数据仓库。对供体和受体特征进行单变量和多变量分析。2016年后接受OHT的患者在我们的电子病历中有记录,因此2016年后的AL-OHT患者在OHT后的第一年有关于免疫抑制、化疗方案、淀粉样变性和移植后相关结局的详细信息。

结果

在研究期间,31例患者因心脏AL接受了OHT,而595例因非淀粉样变性适应证接受了OHT。与非AL OHT患者(分别为92%、85%、79%和68%)相比,1年、3年、5年和8年生存率(分别为87%、83%、73%和67%)无统计学差异。在可获取电子病历数据的18例患者亚组中,所有患者在OHT后1年内均继续使用他克莫司和泼尼松,但近一半患者停用了霉酚酸酯。

结论

部分有晚期心脏受累的AL患者通过OHT可获得与其他晚期心力衰竭患者相似的生存优势。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3721/12275127/bb281fe2d4bf/gr1.jpg

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