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心脏淀粉样变患者心脏移植的中期结果。

Intermediate-term outcomes of heart transplantation for cardiac amyloidosis in the current era.

机构信息

Smidt Cedars-Sinai Heart Institute, Los Angeles, CA, USA.

Department of Hematology/Oncology, Cedars-Sinai Medical Center, Los Angeles, CA, USA.

出版信息

Clin Transplant. 2021 Jun;35(6):e14308. doi: 10.1111/ctr.14308. Epub 2021 Apr 19.

Abstract

BACKGROUND

Cardiac amyloidosis (CA) has been historically noted with poor outcomes after heart transplant (HTx). However, strict patient selection, appropriate multi-organ transplant, and aggressive post-transplant therapy can result in favorable outcomes. We present the experience in the largest single-center cohort of CA patients post-HTx in the recent era.

METHODS

Between January 2010 and December 2018, 51 CA patients underwent HTx-13 light-chain amyloidosis (AL) and 38 transthyretin amyloidosis (ATTR), 49 were included. Endpoints included 3-year survival, freedom from cardiac allograft vasculopathy (CAV), and freedom from non-fatal major adverse cardiac events (NF-MACE).

RESULTS

Overall 3-year survival was 81.6% (69.2% for AL and 86% for ATTR) and was comparable to survival for patients transplanted for non-amyloid restrictive cardiomyopathy (RCM) in the same period (89%, p = .46). Three-year freedom from CAV (84% vs. 89%, p = .98), NF-MACE (82% vs. 83%, p = .96), and any-treated rejection (95% vs. 89%, p = .54) were also comparable in both groups. No recurrence in amyloid was noted in endomyocardial biopsies. Six patients (46%) with AL amyloidosis underwent autologous stem cell transplant 1-year post-HTx, and two patients (8%) with variant ATTR-CA underwent combined heart-liver transplant due to cardiac cirrhosis.

CONCLUSION

In the current era, both AL and ATTR cardiac amyloidosis patients have acceptable outcomes after heart transplantation.

摘要

背景

心脏淀粉样变性(CA)在心脏移植(HTx)后预后一直较差。然而,严格的患者选择、适当的多器官移植和积极的移植后治疗可以带来良好的结果。我们报告了最近一个时代最大的单一中心 CA 患者 HTx 后经验。

方法

2010 年 1 月至 2018 年 12 月,51 例 CA 患者接受了 HTx-13 轻链淀粉样变性(AL)和 38 例转甲状腺素淀粉样变性(ATTR),其中 49 例被纳入。终点包括 3 年生存率、无心脏移植物血管病(CAV)和无非致命性主要不良心脏事件(NF-MACE)。

结果

总体 3 年生存率为 81.6%(AL 为 69.2%,ATTR 为 86%),与同期接受非淀粉样限制性心肌病(RCM)移植的患者生存率相当(89%,p=0.46)。3 年无 CAV(84%比 89%,p=0.98)、NF-MACE(82%比 83%,p=0.96)和任何治疗排斥反应(95%比 89%,p=0.54)在两组之间也相似。在心肌活检中未发现淀粉样物质复发。6 例(46%)AL 淀粉样变性患者在 HTx 后 1 年接受了自体干细胞移植,2 例(8%)变异 ATTR-CA 患者因心脏肝硬化接受了心脏-肝联合移植。

结论

在当前时代,AL 和 ATTR 心脏淀粉样变性患者在心脏移植后有可接受的结果。

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