Department of Cardiology, Interdisciplinary Center for Health Sciences, Scuola Superiore Sant'Anna, Piazza Martiri della Libertà 33, Pisa 56127, Italy.
Department of Cardiology, Fondazione Toscana Gabriele Monasterio; Interdisciplinary Center for Health Sciences, Scuola Superiore Sant'Anna, Piazza Martiri della Libertà 33, Pisa 56127, Italy.
Heart Fail Clin. 2024 Jul;20(3):307-316. doi: 10.1016/j.hfc.2024.03.003. Epub 2024 Apr 8.
Cardiac amyloidosis (CA) is caused by the myocardial deposition of misfolded proteins, either amyloid transthyretin (ATTR) or immunoglobulin light chains (AL). The paradigm of this condition has transformed, since CA is increasingly recognized as a relatively prevalent cause of heart failure. Cardiac scintigraphy with bone tracers is the unique noninvasive technique able to confirm CA without performing tissue biopsy or advanced imaging tests. A moderate-to-intense myocardial uptake (Perugini grade ≥2) associated with the absence of a monoclonal component is greater than 99% specific for ATTR-CA, while AL-CA confirmation requires tissue biopsy.
心脏淀粉样变性(CA)是由错误折叠的蛋白质,即淀粉样转甲状腺素(ATTR)或免疫球蛋白轻链(AL)在心肌中的沉积引起的。这种情况的范式已经发生了转变,因为 CA 越来越被认为是心力衰竭的一个相对普遍的原因。用骨示踪剂进行心脏闪烁显像术是一种独特的非侵入性技术,无需进行组织活检或高级成像检查即可确认 CA。中等至高强度的心肌摄取(佩鲁吉尼分级≥2),同时无单克隆成分,其特异性大于 99%,提示为 ATTR-CA,而 AL-CA 的确认则需要组织活检。