Di Giovanni Bennett, Gustafson Dakota, Arivalagan Priya, Adamson Mitchell B, Vishram-Nielsen Julie, Delgado Diego
University of Toronto, Toronto, Ontario, Canada.
Department of Medicine, Queen's University School of Medicine, Kingston, Ontario, Canada.
Open Heart. 2025 Aug 21;12(2):e002686. doi: 10.1136/openhrt-2024-002686.
Transthyretin amyloidosis is an under-recognised systemic disease whereby misfolded transthyretin proteins form fibrils capable of depositing in various tissues and organs. Despite improvements to diagnostic modalities, the associations between imaging techniques and clinical laboratory metrics remain unclear.
A single-centre retrospective cohort study was performed including 183 patients aged 18 years or older diagnosed with transthyretin cardiac amyloidosis (ATTR-CA) in a tertiary care setting. Linear regression and multivariate proportional hazard models were used to examine the associations between established imaging modalities (ie, technetium-99m pyrophosphate (Tc-PYP) scintigraphy and echocardiography) and cardiac biomarkers (ie, cardiac troponin I and B-type natriuretic peptide (BNP)). The study included patients who visited the Toronto General Hospital Peter Munk Cardiac Centre between October 2012 and December 2022.
Of the 183 patients included, 143 (78.1%) were male, with a median age (IQR) of 73.0 (66.0-79.0) years. Primary analyses revealed significant associations between positive Tc-PYP grading and echocardiographic parameters, particularly increased left ventricular (LV) mass (β=111.21, p=0.009) and greater interventricular septal thickness at end-diastole (IVSd) (β=0.48, p=0.003) in patients with hereditary transthyretin amyloidosis (ATTRm). Additionally, positive Tc-PYP grades correlated significantly with cardiac biomarkers, including log-transformed BNP (logBNP; β=1.99, p=0.002) in patients with ATTRm and log-transformed Troponin (logTroponin; β=1.68, p=0.007) in patients with wild type ATTR (ATTRwt). Conversely, the heart-to-contralateral lung ratio, a quantitative index derived from Tc-PYP scintigraphy, did not show significant correlations with cardiac biomarkers (logBNP and logTroponin), but demonstrated significant associations with LV mass (β=134.52, p=0.001) and IVSd (β=0.46, p=0.002) in patients with ATTRm.
These data suggest strong associations exist between cardiac biomarkers, structural echocardiographic changes and Tc-PYP scintigraphy, emphasising the importance of a multipronged diagnostic approach stratified by genotype.
转甲状腺素蛋白淀粉样变性是一种未得到充分认识的全身性疾病，在此疾病中，错误折叠的转甲状腺素蛋白形成能够沉积在各种组织和器官中的纤维。尽管诊断方式有所改进，但成像技术与临床实验室指标之间的关联仍不明确。
进行了一项单中心回顾性队列研究，纳入了183名18岁及以上在三级医疗环境中被诊断为转甲状腺素蛋白心脏淀粉样变性（ATTR-CA）的患者。使用线性回归和多变量比例风险模型来研究既定成像方式（即锝-99m焦磷酸盐（Tc-PYP）闪烁扫描和超声心动图）与心脏生物标志物（即心肌肌钙蛋白I和B型利钠肽（BNP））之间的关联。该研究纳入了2012年10月至2022年12月期间就诊于多伦多综合医院彼得·芒克心脏中心的患者。
在纳入的183名患者中，143名（78.1%）为男性，中位年龄（IQR）为73.0（66.0 - 79.0）岁。初步分析显示，在遗传性转甲状腺素蛋白淀粉样变性（ATTRm）患者中，Tc-PYP阳性分级与超声心动图参数之间存在显著关联，特别是左心室（LV）质量增加（β = 111.21，p = 0.009）和舒张末期室间隔厚度增加（IVSd）（β = 0.48，p = 0.003）。此外，在ATTRm患者中，Tc-PYP阳性分级与心脏生物标志物显著相关，包括对数转换后的BNP（logBNP；β = 1.99，p = 0.002），在野生型ATTR（ATTRwt）患者中与对数转换后的肌钙蛋白（logTroponin；β = 1.68，p = 0.007）相关。相反，从Tc-PYP闪烁扫描得出的心脏与对侧肺比值这一量化指标与心脏生物标志物（logBNP和logTroponin）没有显著相关性，但在ATTRm患者中与LV质量（β = 134.52，p = 0.001）和IVSd（β = 0.46，p = 0.002）存在显著关联。
这些数据表明心脏生物标志物、超声心动图结构变化与Tc-PYP闪烁扫描之间存在密切关联，强调了按基因型分层的多管齐下诊断方法的重要性。
