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Abstract：
Transthyretin amyloid cardiomyopathy (ATTR-CM) is an infiltrative disorder characterized by extracellular myocardial deposits of amyloid fibrils, with poor outcome, leading to heart failure and death, with significant treatment expenditure. In the era of a novel therapeutic arsenal of disease-modifying agents that target a myriad of pathophysiological mechanisms, timely and accurate diagnosis of ATTR-CM is crucial. Recent advances in therapeutic strategies shown to be most beneficial in the early stages of the disease have determined a paradigm shift in the screening, diagnostic algorithm, and risk classification of patients with ATTR-CM. The aim of this review is to explore the utility of novel specific non-invasive imaging parameters and biomarkers from screening to diagnosis, prognosis, risk stratification, and monitoring of the response to therapy. We will summarize the knowledge of the most recent advances in diagnostic, prognostic, and treatment tailoring parameters for early recognition, prediction of outcome, and better selection of therapeutic candidates in ATTR-CM. Moreover, we will provide input from different potential pathways involved in the pathophysiology of ATTR-CM, on top of the amyloid deposition, such as inflammation, endothelial dysfunction, reduced nitric oxide bioavailability, oxidative stress, and myocardial fibrosis, and their diagnostic, prognostic, and therapeutic implications.
摘要：
转甲状腺素蛋白淀粉样心肌病（ATTR-CM）是一种浸润性疾病，其特征是淀粉样纤维的细胞外心肌沉积，结果不佳，导致心脏衰竭和死亡,具有显著的治疗支出。在针对无数病理生理机制的新型疾病修饰剂治疗武器库的时代，及时准确的诊断ATTR-CM至关重要。在疾病早期阶段最有益的治疗策略的最新进展决定了筛查的范式转变。诊断算法,和ATTR-CM患者的风险分类。这篇综述的目的是探索新的特异性非侵入性成像参数和生物标志物从筛查到诊断的实用性，预后,风险分层，和监测对治疗的反应。我们将总结诊断的最新进展的知识，预后，和早期识别的治疗定制参数，预测结果，以及更好地选择ATTR-CM中的治疗候选物。此外，我们将提供来自ATTR-CM病理生理学中涉及的不同潜在途径的输入，在淀粉样蛋白沉积之上，比如炎症，内皮功能障碍,一氧化氮生物利用度降低，氧化应激，和心肌纤维化，和他们的诊断，预后，和治疗意义。
