Tuscan Regional Amyloidosis Centre.
Cardiomyopathy Unit.
J Cardiovasc Med (Hagerstown). 2022 Aug 1;23(8):493-504. doi: 10.2459/JCM.0000000000001337.
Cardiac amyloidosis is an infiltrative disease characterized by extracellular deposition of insoluble amyloid fibrils in the heart leading to organ dysfunction. Despite recent diagnostic advances, the diagnosis of cardiac amyloidosis is often delayed or even missed. Furthermore, a long diagnostic delay is associated with adverse outcomes, with the early diagnosed patients showing the longest survival. In this narrative review we aimed to summarize the 'red flags' that may facilitate the correct diagnosis. The red flags may be classified as clinical, biohumoral, electrocardiographic, echocardiographic, and cardiac magnetic resonance features and should promptly raise the suspicion of cardiac amyloidosis in order to start a correct diagnostic pathway and targeted treatment strategies that may improve patients' outcomes.
心脏淀粉样变是一种浸润性疾病,其特征是心脏中不可溶的淀粉样纤维的细胞外沉积,导致器官功能障碍。尽管最近有了诊断进展,但心脏淀粉样变的诊断常常被延迟,甚至被遗漏。此外,较长的诊断延迟与不良预后相关,早期诊断的患者的生存时间最长。在这篇叙述性综述中,我们旨在总结可能有助于正确诊断的“警示信号”。这些警示信号可分为临床、生物化学、心电图、超声心动图和心脏磁共振特征,应及时引起心脏淀粉样变的怀疑,以便开始正确的诊断途径和靶向治疗策略,从而改善患者的预后。