锝 99m 焦磷酸盐诊断转甲状腺素心脏淀粉样变性：一项不断发展的试验。
Diagnosing Transthyretin Cardiac Amyloidosis by Technetium Tc 99m Pyrophosphate: A Test in Evolution.
机构信息
Seymour, Paul, and Gloria Milstein Division of Cardiology, Department of Medicine, Columbia University Irving Medical Center, New York, New York, USA.
Seymour, Paul, and Gloria Milstein Division of Cardiology, Department of Medicine, Columbia University Irving Medical Center, New York, New York, USA; Department of Radiology, Columbia University Irving Medical Center, New York, New York, USA.
出版信息
JACC Cardiovasc Imaging. 2021 Jun;14(6):1221-1231. doi: 10.1016/j.jcmg.2020.08.027. Epub 2020 Nov 18.
OBJECTIVES
This study aimed to characterize trends in technetium Tc 99m pyrophosphate (Tc-PYP) scanning for amyloid transthyretin cardiac amyloidosis (ATTR-CA) diagnosis, to determine whether patients underwent appropriate assessment with monoclonal protein and genetic testing, to evaluate use of single-photon emission computed tomography (SPECT) in addition to planar imaging, and to identify predictive factors for ATTR-CA.
BACKGROUND
Tc-PYP scintigraphy has been repurposed for noninvasive diagnosis of ATTR-CA. Increasing use of Tc-PYP can facilitate identification of ATTR-CA, but appropriate use is critical for accurate diagnosis in an era of high-cost targeted therapeutics.
METHODS
Patients undergoing Tc-PYP scanning 1 h after injection at a quaternary care center from 2010 to 2019 were analyzed; clinical information was abstracted; and SPECT results were analyzed.
RESULTS
Over the decade, endomyocardial biopsy rates remained stable with scanning rates peaking at 132 in 2019 (p < 0.001). Among 753 patients (516 men, mean age 77 years), 307 (41%) had a visual score of 0, 177 (23%) of 1, and 269 (36%) of 2 or 3. Of 751 patients with analyzable heart to contralateral chest ratios, 249 (33%) had a ratio ≥1.5. Monoclonal protein testing status was assessed in 550 patients, of these, 174 (32%) did not undergo both serum immunofixation and serum free light chain analysis tests, and 331 (60%) did not undergo all 3 tests-serum immunofixation, serum free light chain analysis, and urine protein electrophoresis. Of 196 patients with confirmed ATTR-CA, 143 (73%) had genetic testing for transthyretin mutations. In 103 patients undergoing cardiac biopsy, grades 2 and 3 99mTc-PYP had sensitivity of 94% and specificity of 89% for ATTR-CA with 100% specificity for grade 3 scans. With respect to SPECT as a reference standard, planar imaging had false positive results in 16 of 25 (64%) grade 2 scans.
CONCLUSIONS
Use of noninvasive testing with Tc-PYP scanning for evaluation of ATTR-CA is increasing, and the inclusion of monoclonal protein testing and SPECT imaging is crucial to rule out amyloid light chain amyloidosis and distinguish myocardial retention from blood pooling.
目的
本研究旨在描述锝 Tc 99m 焦磷酸盐（Tc-PYP）扫描用于诊断淀粉样转甲状腺素蛋白心脏淀粉样变（ATTR-CA）的趋势，确定患者是否接受了单克隆蛋白和基因检测的适当评估，评估单光子发射计算机断层扫描（SPECT）在平面成像之外的应用，并确定ATTR-CA 的预测因素。
背景
Tc-PYP 闪烁扫描已被重新用于非侵入性诊断ATTR-CA。随着 Tc-PYP 的使用增加，可以更方便地识别 ATTR-CA，但在高成本靶向治疗时代，适当使用对于准确诊断至关重要。
方法
对 2010 年至 2019 年在一家四级保健中心进行注射后 1 小时 Tc-PYP 扫描的患者进行分析；提取临床信息；并分析 SPECT 结果。
结果
在过去的十年中，心内膜活检率保持稳定，扫描率在 2019 年达到 132 峰值（p<0.001）。在 753 名患者（516 名男性，平均年龄 77 岁）中，307 名（41%）的视觉评分得分为 0，177 名（23%）得分为 1，269 名（36%）得分为 2 或 3。在 751 名可分析心脏与对侧胸部比值的患者中，249 名（33%）比值≥1.5。在 550 名接受分析的患者中评估了单克隆蛋白检测状态，其中 174 名（32%）未同时进行血清免疫固定和血清游离轻链分析检测，331 名（60%）未进行所有 3 项检测-血清免疫固定，血清游离轻链分析和尿液蛋白电泳。在 196 名确诊为 ATTR-CA 的患者中，143 名（73%）接受了转甲状腺素蛋白基因突变的基因检测。在 103 名接受心脏活检的患者中，99mTc-PYP 分级 2 和 3 的敏感性分别为 94%和 89%，对 ATTR-CA 的特异性为 100%，对分级 3 扫描的特异性为 100%。以 SPECT 作为参考标准，平面成像在 25 个分级 2 扫描中有 16 个（64%）出现假阳性结果。
结论
使用 Tc-PYP 扫描进行非侵入性ATTR-CA 评估的检测方法正在增加，包括单克隆蛋白检测和 SPECT 成像对于排除淀粉样轻链淀粉样变性和区分心肌保留与血液积聚至关重要。
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