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核医学成像技术能否预测遗传性转甲状腺素蛋白心脏淀粉样变患者的预后并指导医学管理?

Can Nuclear Imaging Techniques Predict Patient Outcome and Guide Medical Management in Hereditary Transthyretin Cardiac Amyloidosis?

机构信息

Cardiology Department, Antoine Béclère Hospital, Assistance Publique Hôpitaux de Paris (AP-HP), UMR-S 1180, University of Paris-Sud, Clamart, France.

French Referral Center for Familial Amyloidotic Polyneuropathy and Other Rare Peripheral Neuropathies (CRMR-NNERF), Bicêtre, France.

出版信息

Curr Cardiol Rep. 2018 Mar 24;20(5):33. doi: 10.1007/s11886-018-0976-9.

Abstract

PURPOSE OF REVIEW

Nuclear imaging recently gained a key role in the diagnosis and prognostic assessment of transthyretin (TTR)-related cardiac amyloidosis. This review aims at summarizing the state-of-the art regarding the implementation of nuclear imaging in the management of hereditary mutated TTR-cardiac amyloidosis (mTTR-CA).

RECENT FINDINGS

Although cardiac uptake of bone tracers is acknowledged as a specific marker of TTR amyloid cardiac burden, recent studies validated the implementation of bone scan in the flow chart for non-invasive diagnosis and follow-up of CA in multicenter trials. Simultaneously, cardiac denervation evidenced by MIBG scintigraphy proved to be a strong and independent prognostic marker of poor outcome in mTTR-CA. By its unique ability to assess both amyloid burden and cardiac denervation, nuclear imaging may prove useful as part of multimodality imaging tools to trigger treatment initiation and monitoring in patients with mTTR-CA.

摘要

目的综述

核成像技术最近在转甲状腺素蛋白(TTR)相关性心脏淀粉样变的诊断和预后评估中发挥了关键作用。本综述旨在总结核成像在遗传性突变型 TTR-心脏淀粉样变(mTTR-CA)管理中的应用现状。

最新发现

虽然骨示踪剂的心脏摄取被认为是 TTR 淀粉样心脏负荷的特异性标志物,但最近的研究在多中心试验中验证了骨扫描在 CA 无创诊断和随访流程中的应用。同时,通过 MIBG 闪烁扫描证实的心脏去神经支配是 mTTR-CA 预后不良的一个强烈且独立的预后标志物。核成像通过其评估淀粉样负荷和心脏去神经支配的独特能力,有望成为多模态成像工具的一部分,用于触发 mTTR-CA 患者的治疗启动和监测。

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