Razvi Yousuf, Porcari Aldostefano, Hutt David F, Lazari Jonathan, Ioannou Adam, Patel Rishi K, Rauf Muhammad Umaid, Rezk Tamer, Hague Oliver, Filisetti Stefano, Lachmann Helen J, Wechalekar Ashutosh D, Petrie Aviva, Whelan Carol J, Venneri Lucia, Martinez-Naharro Ana, Gilbertson Janet A, Rowczenio Dorota, Moody William E, Steeds Richard, Pinney Jennifer H, Hawkins Philip N, Fontana Marianna, Gillmore Julian D
National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Hospital, London, United Kingdom.
National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Hospital, London, United Kingdom; Center for Diagnosis and Treatment of Cardiomyopathies, Cardiovascular Department, Azienda Sanitaria Universitaria Giuliano-Isontina, University of Trieste, Italy.
JACC Cardiovasc Imaging. 2025 Aug;18(8):899-908. doi: 10.1016/j.jcmg.2025.03.014. Epub 2025 Jul 9.
Technetium-99m-labeled 3,3-diphosphono-1,2-propanodicarboxylic acid (Tc-DPD) scintigraphy is a critical part of the validated nonbiopsy diagnostic algorithm for transthyretin amyloid cardiomyopathy (ATTR-CM). With the advent of novel disease-modifying therapies for ATTR-CM, there is intense interest in establishing the utility of DPD scans as an indicator of treatment response.
The authors conducted a retrospective multimodality imaging study to determine the utility of Tc-DPD to track treatment response in ATTR-CM.
ATTR-CM patients from the United Kingdom National Amyloidosis Centre who were receiving amyloid-specific disease-modifying therapy (DMT) and underwent pre- and post-treatment Tc-DPD/single-photon emission computed tomography-computed tomography scans were included. Myocardial percentage injected dose (PID), a proposed scintigraphic indicator of cardiac amyloid burden, was measured at baseline and follow-up. Change from baseline in this variable was compared with that of other validated biomarkers of disease severity.
A total of 66 patients with ATTR-CM who received DMT underwent serial Tc-DPD scans. At follow-up, with a median time between Tc-DPD scans of 27.5 months (Q1-Q3: 21.9-33.1 months), there was a mean reduction from pre-DMT myocardial PID on Tc-DPD scintigraphy of 1.5 ± 1.5%. No statistically significant correlation between the change in PID at follow-up and change in echocardiographic, biochemical, or cardiac magnetic resonance parameters was identified. Discordance in which there was an improvement on follow-up Tc-DPD scintigraphy despite disease progression was observed in 28/66 patients (42.4%).
Our study indicates a poor correlation between reduction in Tc-DPD uptake at follow-up and numerous established biomarkers of ATTR-CM treatment response. Changes in DPD uptake in ATTR-CM patients receiving DMT should be interpreted with caution.
锝-99m标记的3,3-二膦酰基-1,2-丙二羧酸（Tc-DPD）闪烁扫描术是经验证的转甲状腺素蛋白淀粉样心肌病（ATTR-CM）非活检诊断算法的关键部分。随着针对ATTR-CM的新型疾病修饰疗法的出现，人们对确定DPD扫描作为治疗反应指标的效用产生了浓厚兴趣。
作者进行了一项回顾性多模态成像研究，以确定Tc-DPD在追踪ATTR-CM治疗反应中的效用。
纳入来自英国国家淀粉样变性病中心的接受淀粉样特异性疾病修饰疗法（DMT）且在治疗前和治疗后进行了Tc-DPD/单光子发射计算机断层扫描-计算机断层扫描的ATTR-CM患者。在基线和随访时测量心肌注射剂量百分比（PID），这是一种提议的心脏淀粉样蛋白负荷的闪烁扫描指标。将该变量相对于基线的变化与其他经过验证的疾病严重程度生物标志物的变化进行比较。
共有66例接受DMT的ATTR-CM患者接受了系列Tc-DPD扫描。在随访时，Tc-DPD扫描之间的中位时间为27.5个月（第一四分位数-第三四分位数：21.9-33.1个月），Tc-DPD闪烁扫描显示治疗前心肌PID平均降低了1.5±1.5%。随访时PID的变化与超声心动图、生化或心脏磁共振参数的变化之间未发现统计学上的显著相关性。在66例患者中有28例（42.4%）观察到不一致的情况，即尽管疾病进展，但随访时Tc-DPD闪烁扫描显示有改善。
我们的研究表明，随访时Tc-DPD摄取减少与众多已确立的ATTR-CM治疗反应生物标志物之间的相关性较差。对于接受DMT的ATTR-CM患者，DPD摄取的变化应谨慎解释。
