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接受心脏移植的心脏淀粉样变性患者的结局。

Outcomes in Patients With Cardiac Amyloidosis Undergoing Heart Transplantation.

机构信息

Division of Cardiology, University of Colorado Denver, Denver, Colorado.

Stanford Amyloid Center, Stanford University School of Medicine, Stanford, California.

出版信息

JACC Heart Fail. 2020 Jun;8(6):461-468. doi: 10.1016/j.jchf.2019.12.013. Epub 2020 May 6.

Abstract

OBJECTIVES

The purpose of this study is to report outcomes after heart transplantation in patients with cardiac amyloidosis based on a large single-center experience.

BACKGROUND

Cardiac amyloidosis causes significant morbidity and mortality, often leading to restrictive cardiomyopathy, progressive heart failure, and death. Historically, heart transplantation outcomes have been worse in patients with cardiac amyloidosis compared with other heart failure populations, in part due to the systemic nature of the disease. However, several case series have suggested that transplantation outcomes may be better in the contemporary era, likely in part due to the availability of more effective light chain suppressive therapies for light chain amyloidosis.

METHODS

This study examined all patients seen between 2004 and 2017, either at the Stanford University Medical Center or the Kaiser Permanente Santa Clara Medical Center, who were diagnosed with cardiac amyloidosis and ultimately underwent heart transplantation. This study examined pre-transplantation characteristics and post-transplantation outcomes in this group compared with the overall transplantation population at our center.

RESULTS

During the study period, 31 patients (13 with light chain amyloidosis and 18 with transthyretin [ATTR] amyloidosis) underwent heart transplantation. Patients with ATTR amyloidosis were older, were more likely to be male, had worse baseline renal function, and had longer waitlist times compared with both patients with light chain amyloidosis and the overall transplantation population. Post-transplantation, there were no differences in post-operative bleeding, renal failure, infection, rejection, or malignancy. There was no significant difference in mortality between patients who underwent heart transplantation for amyloid cardiomyopathy and patients who underwent heart transplantation for all other indications.

CONCLUSIONS

In carefully selected patients with cardiac amyloidosis, heart transplantation can be an effective therapeutic option with outcomes similar to those transplanted for other causes of heart failure.

摘要

目的

本研究旨在报告基于大型单中心经验的心脏淀粉样变性患者心脏移植后的结果。

背景

心脏淀粉样变性可导致严重的发病率和死亡率,常导致限制型心肌病、进行性心力衰竭和死亡。历史上,与其他心力衰竭人群相比,心脏淀粉样变性患者的心脏移植结果更差,部分原因是该疾病的系统性。然而,几项病例系列研究表明,在当代,移植结果可能更好,部分原因可能是针对轻链淀粉样变性的更有效的轻链抑制疗法的可用性。

方法

本研究检查了 2004 年至 2017 年间在斯坦福大学医学中心或 Kaiser Permanente Santa Clara 医疗中心就诊的所有被诊断为心脏淀粉样变性并最终接受心脏移植的患者。本研究比较了该组患者的移植前特征和移植后结果与我们中心的整体移植人群。

结果

在研究期间,31 例患者(13 例患有轻链淀粉样变性,18 例患有转甲状腺素 [ATTR] 淀粉样变性)接受了心脏移植。与轻链淀粉样变性患者和整体移植人群相比,ATTR 淀粉样变性患者年龄较大,更可能为男性,基线肾功能更差,等待时间更长。移植后,在术后出血、肾功能衰竭、感染、排斥反应或恶性肿瘤方面没有差异。因淀粉样心肌病接受心脏移植的患者与因其他原因接受心脏移植的患者之间,死亡率无显著差异。

结论

在精心挑选的心脏淀粉样变性患者中,心脏移植可以是一种有效的治疗选择,其结果与因其他原因导致心力衰竭而接受移植的患者相似。

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