In Vivo Quantification of Myocardial Amyloid Deposits in Patients with Suspected Transthyretin-Related Amyloidosis (ATTR).
In Vivo Quantification of Myocardial Amyloid Deposits in Patients with Suspected Transthyretin-Related Amyloidosis (ATTR).
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DOI：
10.3390/jcm9113446
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发表时间：
2020-10-27
影响因子：
3.9
通讯作者：
Hacker M
中科院分区：
文献类型：
--
作者：
Wollenweber T;Rettl R;Kretschmer-Chott E;Rasul S;Kulterer O;Rainer E;Raidl M;Schaffarich MP;Matschitsch S;Stadler M;Traub-Weidinger T;Beiztke D;Loewe C;Duca F;Mascherbauer J;Bonderman D;Hacker M
背景：目前应用核素骨显像诊断甲状腺激素相关淀粉样变性(ATTR)主要基于目测评分和半定量指标。随着新的潜在延长生命的药物的推出，更准确的心肌淀粉样蛋白负荷的量化是改善反应预测和治疗监测的需要。方法：首先利用拟人胸腔体模进行定量化实验。32例患者同时行胸部[99mTc]-3，3-二膦-1，2-丙二羧酸(DPD)平面全身显像和单光子发射计算机体层摄影(SPECT/CT)。测定SPECT/CT标准化心肌摄取值SUVPeak和与骨摄取归一化的SUVPeak(NSUVPeak)。结果：体模测量显示心肌插入部的活动与测量的活动之间有很强的线性关系(r=0.9998，p=0.01)，但测量的活动被系统性地低估了约30%。受试者操作特征(ROC)分析显示，以SUV峰值为3.1作为区分两组患者的临界值，灵敏度和特异度均为100%。结论：应用SPECT/CT新技术对ATTR淀粉样蛋白负荷进行SUV定量是可行的。以峰值值3.1为界，Perugini分级为2级和3级的患者与Perugini分级为0级和1级的患者可以清楚地区分开来。除了ATTR的诊断外，淀粉样蛋白沉积的定量还可能用于心脏ATTR患者的治疗监测和预后。
Background: Current diagnosis of Transthyretin-related Amyloidosis (ATTR) using bone scintigraphy is primarily based on visual scoring and semi-quantitative indices. With the introduction of new potential life-prolonging drugs for ATTR, a more precise quantification of myocardial amyloid burden is desirable for improved response prediction and therapy monitoring. Methods: At first, quantification experiments using an anthropomorphic thorax phantom were performed. Second, 32 patients underwent both planar whole body [99mTc]- 3,3-Diphosphono-1,2-Propanodicarboxylic Acid (DPD)-scintigraphy and quantitative Single-Photon Emission Computed Tomography/Computed Tomography (SPECT/CT) of the thorax. SPECT/CT standardized myocardial uptake values SUVpeak and SUVpeak normalized to bone uptake (nSUVpeak) were determined. Results: Phantom measurements showed a strong linear relationship between the activity in the myocardial insert and the measured activity (r = 0.9998, p = 0.01), but the measured activity was systematically underestimated by approximately 30%. Receiver operating characteristics (ROC) analysis revealed a 100% sensitivity and specificity at a cut-off of 3.1 for SUVpeak for the differentiation of both patient groups. Conclusion: SUV quantification of ATTR amyloid burden is feasible using novel SPECT/CT technology. With a SUVpeak cut-off of 3.1, patients with Perugini grade 2 and 3 could be clearly separated from those with Perugini grade 0 and 1. Besides ATTR diagnostics, quantification of amyloid deposits could potentially be used for therapy monitoring and prognostication in patients with cardiac ATTR.
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影响因子：
120.7
作者：
Berk, John L.;Suhr, Ole B.;Obici, Laura;Sekijima, Yoshiki;Zeldenrust, Steven R.;Yamashita, Taro;Heneghan, Michael A.;Gorevic, Peter D.;Litchy, William J.;Wiesman, Janice F.;Nordh, Erik;Corato, Manuel;Lozza, Alessandro;Cortese, Andrea;Robinson-Papp, Jessica;Colton, Theodore;Rybin, Denis V.;Bisbee, Alice B.;Ando, Yukio;Ikeda, Shu-ichi;Seldin, David C.;Merlini, Giampaolo;Skinner, Martha;Kelly, Jeffery W.;Dyck, Peter J.
通讯作者：
Dyck, Peter J.
影响因子：
5.4
作者：
Banypersad SM;Moon JC;Whelan C;Hawkins PN;Wechalekar AD
通讯作者：
Wechalekar AD
影响因子：
37.8
作者：
Gillmore, Julian D.;Maurer, Mathew S.;Hawkins, Philip N.
通讯作者：
Hawkins, Philip N.
影响因子：
24
作者：
Martinez-Naharro, Ana;Treibel, Thomas A.;Fontana, Marianna
通讯作者：
Fontana, Marianna
影响因子：
6.2
作者：
Hutt, David F.;Fontana, Marianna;Gillmore, Julian D.
通讯作者：
Gillmore, Julian D.