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淀粉样变终末期心力衰竭管理的最新进展:移植及其他。

State-of-the-art review on management of end-stage heart failure in amyloidosis: transplant and beyond.

机构信息

Houston Methodist DeBakey Heart and Vascular Center, Houston Methodist Hospital, Houston, TX, USA.

Department of Internal Medicine, Houston Methodist Hospital, Houston, TX, USA.

出版信息

Heart Fail Rev. 2022 Sep;27(5):1567-1578. doi: 10.1007/s10741-021-10209-3. Epub 2022 Feb 3.

Abstract

Cardiac involvement occurs in light-chain (AL), transthyretin wild-type (wtATTR), and hereditary (hATTR) amyloidosis; other types of amyloidosis account for < 5% of all cardiac amyloidosis (CA). CA can present subclinically on screening, insidiously with symptoms such as exertional dyspnea, or abruptly as cardiogenic shock. Initially, CA patients were thought to be poor candidates for transplant due to short long-term survival; however, there is a marked improvement in heart and multi-organ transplant outcomes over the past 10 years with newer treatments and improvements in support with temporary and durable mechanical circulatory support while awaiting transplant. Patients with AL CA were reported to have worse post-OHT outcomes than patients with ATTR CA, but this gap is quickly closing with improved patient selection, novel chemotherapeutics, and perhaps with selected use of bone marrow transplantation. Waitlist mortality and transplantation rates have markedly improved for CA after the United Network for Organ Sharing (UNOS) policy change in October 2018. In this review, we will evaluate contemporary data from the last 5 years on advances in the field of transplantation and mechanical circulatory support in this patient population.

摘要

心脏受累发生于轻链 (AL)、转甲状腺素蛋白野生型 (wtATTR) 和遗传性 (hATTR) 淀粉样变性;其他类型的淀粉样变性占所有心脏淀粉样变性 (CA) 的 < 5%。CA 可在筛查时亚临床出现,也可隐匿性出现劳力性呼吸困难等症状,或突然出现心源性休克。最初,由于长期生存率短,CA 患者被认为不适合进行移植;然而,在过去 10 年中,随着新的治疗方法的出现以及在等待移植期间临时和永久性机械循环支持的支持方面的改进,心脏和多器官移植的结果有了显著改善。据报道,AL CA 患者的 OHT 后结局比 ATTR CA 患者差,但随着患者选择、新型化疗药物的改进,以及可能选择性使用骨髓移植,这一差距正在迅速缩小。2018 年 10 月,美国器官共享联合网络 (UNOS) 政策改变后,CA 的候补名单死亡率和移植率显著提高。在这篇综述中,我们将评估过去 5 年来该患者群体中移植和机械循环支持领域的最新数据。

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