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心脏淀粉样变性诊断与评估中的多模态成像

Multimodality Imaging in the Diagnosis and Assessment of Cardiac Amyloidosis.

作者信息

Velaga Jyothirmayi, Liew Charlene, Choo Poh Angeline Choo, Lee Phong Teck, Lath Narayan, Low Shoen Choon, Bharadwaj Pushan

机构信息

Department of Diagnostic Radiology, Singapore General Hospital, Singapore, Singapore.

Department of Radiology, Changi General Hospital, Singapore, Singapore.

出版信息

World J Nucl Med. 2022 Aug 16;21(3):173-183. doi: 10.1055/s-0042-1751057. eCollection 2022 Sep.

Abstract

Amyloidosis is a rare disorder where abnormal protein aggregates are deposited in tissues forming amyloid fibrils, leading to progressive organ failure. Although any organ can be affected, cardiac involvement is the main cause of morbidity and mortality associated with amyloidosis as diagnosis is often delayed due to the indolent nature of the disease in some forms. An early diagnosis of disease and knowledge of the type/subtype of cardiac amyloidosis (CA) are essential for appropriate management and better outcome. Echocardiography is often the first line of investigation for patients suspected of CA and offers superior hemodynamic assessment. Although cardiovascular magnetic resonance (CMR) imaging is not diagnostic of CA, it provides vital clues to diagnosis and has a role in disease quantification and prognostication. Radiolabeled bone seeking tracers are the mainstay of diagnosis of CA and when combined with screening of monoclonal light chains, bone scintigraphy offers high sensitivity in diagnosing transthyretin type of CA. This review aims to describe the noninvasive imaging assessment and approach to diagnosis of patients with suspected CA. Imaging features of echocardiography, nuclear scintigraphy, and CMR are described with a brief mention on computed tomography.

摘要

淀粉样变性是一种罕见的疾病,异常蛋白质聚集体沉积在组织中形成淀粉样纤维,导致进行性器官衰竭。尽管任何器官都可能受到影响,但心脏受累是与淀粉样变性相关的发病和死亡的主要原因,因为在某些形式的疾病中,由于其隐匿性,诊断往往延迟。疾病的早期诊断以及心脏淀粉样变性(CA)类型/亚型的知识对于适当的管理和更好的结果至关重要。超声心动图通常是疑似CA患者的一线检查方法,可提供出色的血流动力学评估。尽管心血管磁共振(CMR)成像不能诊断CA,但它为诊断提供了重要线索,并在疾病量化和预后评估中发挥作用。放射性标记的亲骨示踪剂是CA诊断的主要手段,当与单克隆轻链筛查相结合时,骨闪烁显像在诊断转甲状腺素蛋白型CA方面具有高灵敏度。本综述旨在描述疑似CA患者的非侵入性成像评估和诊断方法。文中描述了超声心动图、核闪烁显像和CMR的成像特征,并简要提及了计算机断层扫描。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0ea7/9436521/e9d337fd8f5c/10-1055-s-0042-1751057-i12221-1.jpg

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