Multicenter Study of Planar Technetium 99m Pyrophosphate Cardiac Imaging Predicting Survival for Patients With ATTR Cardiac Amyloidosis
Multicenter Study of Planar Technetium 99m Pyrophosphate Cardiac Imaging Predicting Survival for Patients With ATTR Cardiac Amyloidosis
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DOI：
10.1001/jamacardio.2016.2839
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发表时间：
2016-11-01
期刊：
影响因子：
24
通讯作者：
Maurer, Mathew S.
中科院分区：
文献类型：
--
作者：
Castano, Adam;Haq, Muhammad;Maurer, Mathew S.
重要性甲状腺素运载蛋白心脏淀粉样变性（也称为ATTR心脏淀粉样变性）是一个日益认识到的原因心力衰竭与保留射血分数。在单中心研究中，99焦磷酸锝Tc 99 mPYP心脏显像可无创性检测ATTR心脏淀粉样变性，但该技术在多中心研究中的准确性以及Tc 99 mPYP心肌摄取与生存率的关系尚不清楚。目的评价Tc 99 mPYP心脏显像作为ATTR心脏淀粉样变性的诊断工具及其与生存率的关系。和参与者在美国3个心脏淀粉样变性学术专业中心进行的回顾性队列研究，其中229名参与者接受了心脏淀粉样变性评估，并接受了Tc 99 mPYP心脏成像。分析和统计学家的最终确认日期为2016年5月4日。暴露Tc 99 mPYP心脏成像用于检测ATTR心脏淀粉样变性。主要结局和测量使用半定量视觉评分（范围，0 [无摄取]至3 [摄取大于骨]）和定量心脏与对侧（H/CL）比率评估Tc 99 mPYP在心脏中的保留。H/CL比计算为心脏上感兴趣区域的总计数除以对侧胸部上相同大小感兴趣区域的背景计数。结果171例受试者接受99 mPYP显像后死亡时间（121例ATTR心脏淀粉样变性和50例非ATTR心脏淀粉样变性[34例AL淀粉样变性和16例射血分数保留的非淀粉样心力衰竭]; 86%男性;中位[IQR]年龄，73岁[65-79岁]）显示检测ATTR心脏淀粉样变性的灵敏度为91%，特异性为92%，曲线下面积为0.960（95%CI，0.930-0.981）。对ATTR心脏淀粉样变性受试者进行单变量和多变量考克斯比例风险回归分析显示，H/CL比值≥ 1.6预示生存率较差（风险比，3.911 [95%CI，1.155-13.247];单变量分析P = 0.03，多变量分析7.913 [95%CI，1.679-37.296]; P = 0.01）在Kaplan-Meier分析中，5年随访期内，如果H/CL比值大于或等于1.6，而不是小于1.6，则生存率显著更差（对数秩P =.02）。结论和相关性在这项多中心研究中，Tc 99 mPYP心脏显像对ATTR心脏淀粉样变的鉴别诊断具有高度的敏感性和特异性（不考虑基因型）与AL心脏淀粉样变性患者和射血分数正常的非淀粉样心力衰竭患者的差异。在ATTR心脏淀粉样变性患者中，H/CL比值≥ 1.6与生存率较差相关。在临床高度怀疑心脏淀粉样变性的患者中，Tc 99 mPYP可能具有诊断和预后重要性。
IMPORTANCE Transthyretin cardiac amyloidosis (also known as ATTR cardiac amyloidosis) is an increasingly recognized cause of heart failure with preserved ejection fraction. In single-center studies, technetium 99mpyrophosphate (Tc 99m PYP) cardiac imaging noninvasively detects ATTR cardiac amyloidosis, but the accuracy of this technique in a multicenter study and the association of Tc 99mPYPmyocardial uptake with survival are unknown.OBJECTIVE To assess Tc 99mPYP cardiac imaging as a diagnostic tool for ATTR cardiac amyloidosis and its association with survival in a multicenter study.DESIGN, SETTING, AND PARTICIPANTS Retrospective cohort study performed at 3 academic specialty centers for cardiac amyloidosis in the United States in which 229 participants were evaluated for cardiac amyloidosis and also underwent Tc 99mPYP cardiac imaging. The date of analysis and final confirmation from the statistician wasMay 4, 2016.EXPOSURE Tc 99mPYP cardiac imaging for detection of ATTR cardiac amyloidosis.MAIN OUTCOMES AND MEASURES Retention of Tc 99mPYP in the heart was assessed using both a semiquantitative visual score (range, 0 [no uptake] to 3 [uptake greater than bone]) and a quantitative heart to contralateral (H/CL) ratio. The H/CL ratio was calculated as total counts in a region of interest over the heart divided by background counts in an identical size region of interest over the contralateral chest. The outcome measured was time to death after Tc 99mPYP imaging.RESULTS Tc 99mPYP imaging of 171 participants (121 with ATTR cardiac amyloidosis and 50 with non-ATTR cardiac amyloidosis [34 with AL amyloidosis and 16 with nonamyloid heart failure with preserved ejection fraction]; 86% male; median [IQR] age, 73 years [65-79 years]) demonstrated 91% sensitivity and 92% specificity for detecting ATTR cardiac amyloidosis with an area under the curve of 0.960 (95% CI, 0.930-0.981). Univariable and multivariable Cox proportional hazards regression analyses among participants with ATTR cardiac amyloidosis showed that an H/CL ratio of 1.6 or greater predicted worse survival (hazard ratio, 3.911 [95% CI, 1.155-13.247]; P =.03 for univariable analysis and 7.913 [95% CI, 1.679-37.296]; P =.01 for multivariable analysis). In Kaplan-Meier analysis over a 5-year follow-up period, survival was significantly worse if the H/CL ratio was 1.6 or greater rather than less than 1.6 (log-rank P =.02).CONCLUSIONS AND RELEVANCE In this multicenter study, Tc 99m PYP cardiac imaging conferred a high level of sensitivity and specificity for differentiation of patients with ATTR cardiac amyloidosis (irrespective of genotype) from patients with AL cardiac amyloidosis and patients with nonamyloid heart failure with preserved ejection fraction. An H/CL ratio of 1.6 or greater was associated with worse survival among patients with ATTR cardiac amyloidosis. Among patients for whom there is a high clinical suspicion of cardiac amyloidosis, Tc 99mPYP may be of diagnostic and prognostic importance.