Hong Zhihui, Xue Song, Yu Josef, Calabretta Raffaella, Haberl David, Jiang Zewen, Grünert Stefan, Beitzke Dietrich, Kammerlander Andreas, Hacker Marcus, Li Xiang
Department of Nuclear Medicine, The Second Affiliated Hospital of Soochow University, Suzhou, China.
Division of Nuclear Medicine, Department of Biomedical Imaging and Image-guided Therapy, Vienna General Hospital, Medical University of Vienna, Vienna, 1090, Austria.
Eur J Nucl Med Mol Imaging. 2025 May 5. doi: 10.1007/s00259-025-07308-w.
Amyloidosis is a systemic disorder characterized by amyloid fibril deposition in multiple organs, including the heart, brain, liver, kidneys, and lungs, leading to organ dysfunction and affecting survival. In cardiac amyloidosis (CA), where the heart is primarily affected, the broader impact on other organs, particularly the brain, is not fully understood. This study aimed to assess the extent of amyloid deposition across systemic organs in CA, investigate brain-organ, brain-brain and organ-organ connections, and evaluate their effects on patients' survival.
A retrospective analysis was conducted on 70 patients, categorized into three groups by biopsy: cardiac amyloidosis (CA) (n = 31), non-cardiac amyloidosis (non-CA) (n = 12), and no amyloidosis control (NC) (n = 27). C-PIB PET/CT scans were performed, and standard uptake value mean (SUVmean) values were extracted using automated segmentation techniques. Brain-organ, brain-brain, organ-organ connectivity analyses were conducted using Spearman correlation. Survival outcomes and prognostic factors were evaluated using Kaplan-Meier analysis.
Amyloid deposition in CA patients was prominent in the myocardium, liver, and kidneys, reflected by increased SUV values. Significant brain-organ connectivity was observed, particularly between the posterior cingulate cortex (PCC), parietal lobes, and myocardial amyloid burden. Additionally, CA patients demonstrated markedly stronger inter-organ connectivity, indicating systemic involvement. In contrast, Non-CA and NC patients demonstrated weaker brain-organ and inter-organ interactions. The CA group exhibited a higher mortality rate, highlighting the severe prognosis, particularly in systemic CA (SCA). Furthermore, elevated amyloid deposition in the bone marrow, lungs and spleen within SCA group was strongly associated with increased mortality.
In CA patients, amyloidosis was associated with extensive systemic involvement, as shown by significant brain-organ and inter-organ connectivity patterns. Findings emphasize that cardiac amyloidosis substantially impacts survival, particularly when accompanied by amyloid deposition in the bone marrow, lungs, and spleen, indicating the need for a comprehensive diagnostic and management approach.
Not applicable.
淀粉样变性是一种全身性疾病,其特征是淀粉样纤维沉积于多个器官,包括心脏、大脑、肝脏、肾脏和肺,导致器官功能障碍并影响生存。在心脏淀粉样变性(CA)中,心脏是主要受累器官,而其对其他器官,尤其是大脑的更广泛影响尚未完全明确。本研究旨在评估CA患者全身器官淀粉样沉积的程度,研究脑 - 器官、脑 - 脑和器官 - 器官之间的联系,并评估它们对患者生存的影响。
对70例患者进行回顾性分析,根据活检结果分为三组:心脏淀粉样变性(CA)组(n = 31)、非心脏淀粉样变性(非CA)组(n = 12)和无淀粉样变性对照组(NC)组(n = 27)。进行C - PIB PET/CT扫描,并使用自动分割技术提取标准摄取值均值(SUVmean)。使用Spearman相关性进行脑 - 器官、脑 - 脑、器官 - 器官连通性分析。使用Kaplan - Meier分析评估生存结果和预后因素。
CA患者的淀粉样沉积在心肌、肝脏和肾脏中较为突出,表现为SUV值升高。观察到显著的脑 - 器官连通性,特别是在后扣带回皮质(PCC)、顶叶与心肌淀粉样负荷之间。此外,CA患者表现出明显更强的器官间连通性,表明存在全身受累。相比之下,非CA和NC患者的脑 - 器官和器官间相互作用较弱。CA组的死亡率较高,突出了其严重的预后,特别是在系统性CA(SCA)中。此外,SCA组骨髓、肺和脾中淀粉样沉积的增加与死亡率升高密切相关。
在CA患者中,淀粉样变性与广泛的全身受累相关,表现为显著的脑 - 器官和器官间连通性模式。研究结果强调心脏淀粉样变性对生存有重大影响,特别是当伴有骨髓、肺和脾中的淀粉样沉积时,这表明需要一种全面的诊断和管理方法。
不适用。