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心脏淀粉样变性中的心脏移植

Heart transplantation in cardiac amyloidosis.

作者信息

Sousa Matthew, Monohan Gregory, Rajagopalan Navin, Grigorian Alla, Guglin Maya

机构信息

University of Kentucky, Lexington, KY, USA.

出版信息

Heart Fail Rev. 2017 May;22(3):317-327. doi: 10.1007/s10741-017-9601-z.

Abstract

"Cardiac amyloidosis" is the term commonly used to reflect the deposition of abnormal protein amyloid in the heart. This process can result from several different forms, most commonly from light-chain (AL) amyloidosis and transthyretin (ATTR) amyloidosis, which in turn can represent wild-type (ATTRwt) or genetic form. Regardless of the origin, cardiac involvement is usually associated with poor prognosis, especially in AL amyloidosis. Although several treatment options, including chemotherapy, exist for different forms of the disease, cardiac transplantation is increasingly considered. However, high mortality on the transplantation list, typical for patients with amyloidosis, and suboptimal post-transplant outcomes are major issues. We are reviewing the literature and summarizing pros and cons of listing patients with amyloidosis for cardiac or combine organ transplant, appropriate work-up, and intermediate and long-term outcomes. Both AL and ATTR amyloidosis are included in this review.

摘要

“心脏淀粉样变性”是一个常用术语,用于反映异常蛋白质淀粉样物质在心脏中的沉积。这个过程可能由几种不同形式引起,最常见的是轻链(AL)淀粉样变性和转甲状腺素蛋白(ATTR)淀粉样变性,而后者又可表现为野生型(ATTRwt)或遗传形式。无论其起源如何,心脏受累通常与预后不良相关,尤其是在AL淀粉样变性中。尽管针对不同形式的该疾病存在多种治疗选择,包括化疗,但心脏移植越来越受到关注。然而,淀粉样变性患者在移植等待名单上的高死亡率以及移植后不理想的结果是主要问题。我们正在回顾文献,并总结将淀粉样变性患者列入心脏或联合器官移植名单的利弊、适当的检查以及中长期结果。本综述涵盖了AL和ATTR淀粉样变性。

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