Pudis Michal, Bastarrika Alemañ Gorka
Servicio de Medicina Nuclear-PET (IDI), Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat, Barcelona, España.
Servicio de Radiodiagnóstico, Clínica Universidad de Navarra, Pamplona, Navarra, España.
Med Clin (Barc). 2023 Feb 10;160(3):121-128. doi: 10.1016/j.medcli.2022.09.010. Epub 2022 Dec 19.
Cardiac amyloidosis (CA) is an underdiagnosed disease and, if left untreated, rapidly fatal. Emerging therapies for CA increase the urgency of developing non-invasive diagnostic methods for its early detection and for monitoring therapeutic response. Classic imaging features on echocardiography and cardiac magnetic resonance, although typical for cardiac amyloidosis, are not specific enough to distinguish light chain amyloidosis from transthyretin. Myocardial bone-avid radiotracer uptake is highly specific for transthyretin cardiac amyloidosis when plasma cell dyscrasia has been excluded; it is now replacing the need for biopsy in many patients. Detection of early cardiac amyloidosis, quantitation of its burden, and assessment of response to therapy are important next steps for imaging to advance the evaluation and management of cardiac amyloidosis.
心脏淀粉样变性（CA）是一种诊断不足的疾病，若不治疗，会迅速致命。CA的新兴疗法增加了开发非侵入性诊断方法以早期检测和监测治疗反应的紧迫性。超声心动图和心脏磁共振上的典型影像学特征虽然是心脏淀粉样变性的典型表现，但特异性不足以区分轻链淀粉样变性和转甲状腺素蛋白淀粉样变性。当排除浆细胞异常增生时，心肌亲骨放射性示踪剂摄取对转甲状腺素蛋白心脏淀粉样变性具有高度特异性；现在它正在取代许多患者的活检需求。早期心脏淀粉样变性的检测、其负荷的定量以及对治疗反应的评估是影像学推进心脏淀粉样变性评估和管理的重要下一步。
