Porcari Aldostefano, Rossi Maddalena, Dore Franca, Imazio Massimo, Fontana Marianna, Merlo Marco, Sinagra Gianfranco
Centro per la Diagnosi ed il Trattamento delle Cardiomiopatie, Dipartimento Cardiovascolare, Azienda Sanitaria Universitaria Giuliano-Isontina (ASUGI) e Università degli Studi di Trieste, Trieste.
Dipartimento di Medicina Nucleare, Azienda Sanitaria Universitaria Giuliano-Isontina (ASUGI) e Università degli Studi di Trieste, Trieste.
G Ital Cardiol (Rome). 2022 Jun;23(6):424-432. doi: 10.1714/3810.37938.
Although traditionally considered a rare and incurable condition, the perspective on cardiac amyloidosis (CA) has changed deeply over the last 10 years following major advances in diagnosis and treatment options. First, the development of non-invasive diagnostic methods, predominantly cardiac scintigraphy with bone tracers, has dramatically changed the epidemiology of the disease, revealing the real prevalence of the transthyretin-related form (ATTR), and has consistently reduced the need for histological amyloid confirmation by endomyocardial biopsy. Second, the availability of new specific therapies, such as tafamidis, has made CA early recognition a clinical need with major impact on patients' management and natural history. Since the demonstration of the essential role of bone tracer scintigraphy to non-invasively differentiate ATTR from immunoglobulin light-chain amyloidosis (AL), this imaging technique has become part of the vocabulary and clinical practice of physicians of many specialties, including cardiologists managing patients with cardiomyopathies.In this paper, we address the main questions regarding cardiac scintigraphy with bone tracers and CA, providing cardiologists with the knowledge to guide clinical indications, evaluation and clinical interpretation of images.
尽管心脏淀粉样变性（CA）传统上被认为是一种罕见且无法治愈的疾病，但在过去10年中，随着诊断和治疗方法的重大进展，对其的看法发生了深刻变化。首先，非侵入性诊断方法的发展，主要是使用骨显像剂的心脏闪烁扫描，极大地改变了该疾病的流行病学，揭示了转甲状腺素蛋白相关型（ATTR）的实际患病率，并持续减少了通过心内膜心肌活检进行组织学淀粉样变确认的需求。其次，新的特异性疗法的出现，如塔非酰胺，使得CA的早期识别成为一项临床需求，对患者的管理和自然病程产生重大影响。自从证明骨显像剂闪烁扫描在非侵入性鉴别ATTR与免疫球蛋白轻链淀粉样变性（AL）中的关键作用以来，这种成像技术已成为许多专业医生（包括管理心肌病患者的心脏病专家）的常用词汇和临床实践的一部分。在本文中，我们探讨了有关使用骨显像剂的心脏闪烁扫描和CA的主要问题，为心脏病专家提供指导临床适应症、评估和图像临床解读的知识。
