Heart Area, Cardiology Department, Asturias Central University Hospital, Roma Ave., s/n, 33011, Oviedo, Asturias, Spain.
Radiology Department, Asturias Central University Hospital, Oviedo, Asturias, Spain.
J Nucl Cardiol. 2021 Jun;28(3):1117-1125. doi: 10.1007/s12350-020-02107-2. Epub 2020 Apr 24.
We present a case of a 40-year-old Spanish man with cardiac amyloidosis in which a Tc-99m-3,3-diphosphono-1,2-propanodicarboxylic acid (Tc-99m-DPD) scintigraphy was strongly suggestive of cardiac amyloidosis by transthyretin (ATTR) but endomyocardial biopsy (EB) analyzed by immunohistochemistry demonstrated a light chain amyloidosis (AL). Even though the Tc-99m-DPD has proven in different published papers that has high sensibility and specificity for differentiating AL and ATTR cardiac amyloidosis, we present an unusual case of an AL cardiac amyloidosis with a Perugini grade 3 on the scintigraphy. Diagnostic approach of cardiac amyloidosis following consensus documents is discussed to avoid diagnostic mistakes based on imaging techniques.
我们报告了一例 40 岁的西班牙男性患有心脏淀粉样变性，其 Tc-99m-3,3-二膦酸基-1,2-丙烷二羧酸（Tc-99m-DPD）闪烁显像强烈提示转甲状腺素（ATTR）心脏淀粉样变性，但免疫组化分析的心肌活检（EB）显示为轻链淀粉样变性（AL）。尽管 Tc-99m-DPD 已在不同的已发表论文中证明具有高度的敏感性和特异性，可用于区分 AL 和ATTR 心脏淀粉样变性，但我们报告了一例罕见的 AL 心脏淀粉样变性，其闪烁显像为佩鲁吉尼 3 级。讨论了根据共识文件进行心脏淀粉样变性的诊断方法，以避免基于影像学技术的诊断错误。
