(99m)Tc-pyrophosphate scintigraphy for differentiating light-chain cardiac amyloidosis from the transthyretin-related familial and senile cardiac amyloidoses.
(99m)Tc-pyrophosphate scintigraphy for differentiating light-chain cardiac amyloidosis from the transthyretin-related familial and senile cardiac amyloidoses.
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DOI：
10.1161/circimaging.112.000132
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发表时间：
2013-03-01
期刊：
影响因子：
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通讯作者：
Maurer MS
中科院分区：
文献类型：
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作者：
Bokhari S;Castaño A;Pozniakoff T;Deslisle S;Latif F;Maurer MS
考虑到对预后、治疗和遗传咨询的影响，区分免疫球蛋白轻链 (AL) 与转甲状腺素蛋白相关的心脏淀粉样变性 (ATTR) 势在必行。我们验证了 99mTc-焦磷酸盐闪烁扫描 (99mTc-PYP) 在 AL 与 TTR 相关心脏淀粉样变性中的区分能力。 45 名受试者（12 名 AL、16 名 ATTR 野生型和 17 名 ATTR 突变体）接受了 99mTc-PYP 平面和单光子正发射计算机断层扫描 (SPECT) 心脏成像。扫描由经验丰富的核心脏病专家进行，对受试者的队列分配不知情。心脏潴留通过半定量视觉评分（范围0，无摄取到3，弥漫性摄取）和通过在心脏上绘制感兴趣区域（ROI）并校正对侧计数并计算心脏与对侧比率（H/CL）的定量分析来评估。患有 ATTR 心脏淀粉样蛋白的受试者的半定量心脏视觉评分显着高于 AL 队列（2.9±0.06 vs. 0.8±0.27，p<0.0001），并且定量评分也较高（1.80±0.04 vs.1.21±0.04，p<0.0001）。使用与强烈弥漫性心肌示踪剂保留一致的 aH/CL 比率 ≥ 1.5 来识别 ATTR 心脏淀粉样变性，其灵敏度为 97%，特异性为 100%，曲线下面积为 0.992，p<0.0001。 99mTc-PYP 心脏成像可区分 AL 和 ATTR 心脏淀粉样变性，并且可能是一种简单、广泛使用的方法，用于识别 ATTR 心脏淀粉样变性受试者，应以更大的前瞻性方式进行研究。
Differentiating immunoglobulin light-chain (AL) from transthyretin-related cardiac amyloidoses (ATTR) is imperative given implications for prognosis, therapy, and genetic counseling. We validated the discriminatory ability of 99mTc-pyrophosphate scintigraphy (99mTc-PYP) in AL vs. TTR-related cardiac amyloidoses. 45 subjects (12 AL, 16 ATTR wild-type, and 17 ATTR mutants) underwent 99mTc-PYP planar and single-photon positive emission computed tomography (SPECT) cardiac imaging. Scans were performed by experienced nuclear cardiologists blinded to the subjects’ cohort assignment. Cardiac retention was assessed with both a semi-quantitative visual score (range 0, no uptake to 3, diffuse uptake) and by quantitative analysis by drawing a region of interest (ROI) over the heart corrected for contralateral counts and calculating a heart-to-contralateral ratio (H/CL). Subjects with ATTR cardiac amyloid had a significantly higher semi-quantitative cardiac visual score than the AL cohort (2.9±0.06 vs. 0.8±0.27, p<0.0001) as well as a higher quantitative score (1.80±0.04 vs.1.21±0.04, p<0.0001). Using aH/CL ratio ≥ 1.5 consistent with intensely diffuse myocardial tracer retention had a 97% sensitivity and 100% specificity with area under the curve 0.992, p<0.0001 for identifying ATTR cardiac amyloidosis. 99mTc-PYP cardiac imaging distinguishes AL from ATTR cardiac amyloidosis and may be a simple, widely available method for identifying subjects with ATTR cardiac amyloidosis which should be studied in a larger prospective manner.