Noninvasive etiologic diagnosis of cardiac amyloidosis using 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid scintigraphy
Noninvasive etiologic diagnosis of cardiac amyloidosis using 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid scintigraphy
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DOI：
10.1016/j.jacc.2005.05.073
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发表时间：
2005-09-20
影响因子：
24
通讯作者：
Rapezzi, C
中科院分区：
文献类型：
--
作者：
Perugini, E;Guidalotti, PL;Rapezzi, C
目的 我们研究了 (99m)-Tc-3,3-二膦酰基-1,2-丙二甲酸 ((99m)-Tc-DPD) 闪烁扫描术区分单克隆免疫球蛋白轻链 (AL) 和运甲状腺素蛋白 (TTR) 相关心脏淀粉样变性的诊断准确性。 背景 TTR 相关性和 AL 淀粉样变性之间的鉴别诊断通常很复杂，并且方法 采用 Tc-99m-DPD 闪烁显像技术对常规观察的 TTR 相关/AL 系统性淀粉样变性患者和超声心动图心脏受累证据进行研究。 结果 TTR 相关心脏受累患者（A 组；n = 15）和 AL（B 组；n 10）病因学的患者左心室质量和肾功能具有可比性。与 B 组和 10 个未受影响的对照组相比，A 组的心脏和心脏/全身示踪剂保留显着更高 (p < 0.05)。在视觉评分时，所有 A 组患者均存在心脏 99mTc-DPD 摄取，而所有 B 组患者均不存在心脏 99mTc-DPD 摄取；因此，使用基因分型/免疫组织化学作为参考技术，Tc-99m-DPD 闪烁扫描区分 TTR 相关和 AL 病因的准确性为 100%。在未受影响的对照中，心脏 Tc-99m-DPD 摄取也不存在。以超声心动图作为识别心脏受累的参考标准，A组患者显像的敏感性和特异性均为100%； B 组的敏感性为 0%，特异性为 100%（准确性为 50%）。 11 名心肌 (99m)-Tc-DPD 摄取的患者接受了 (99m)-Tc-亚甲基二膦酸盐 ((99m)-Tc-MDP) 闪烁扫描；所有患者的 (99m)-Tc-MDP 心肌视觉评分均为 0。 结论 除受累器官类型（软组织/心脏）和示踪剂类型外，病因学是心脏淀粉样变性闪烁照相变异性的第三个主要原因。这是未来研究的一个高度相关的考虑因素。我们得出的结论是，(99m)-Tc-DPD 闪烁扫描是对有记录的心脏淀粉样变性患者进行 TTR 与 AL 病因学鉴别诊断的有用步骤。
OBJECTIVES We investigated the diagnostic accuracy of (99m)-Tc-3,3-diphosphono-1,2-propanodicarboxylic acid ((99m)-Tc-DPD) scintigraphy for differentiation of monoclonal immunoglobulin light-chain (AL) and transthyretin (TTR)-related cardiac amyloidosis.BACKGROUND Differential diagnosis between TTR-related and AL amyloidosis is often complex and time-consuming.METHODS Patients under routine observation with TTR-related/AL systemic amyloidosis and echocardiographic evidence of cardiac involvement were studied with Tc-99m-DPD scintigraphy.RESULTS Patients with cardiac involvement of TTR-related (group A; n = 15) and AL (group B; n 10) etiology were comparable for left ventricular mass and renal function. Heart and heart/whole-body tracer retention were significantly higher (p < 0.05) in group A as compared with group B and with 10 unaffected controls. At visual scoring, cardiac 99mTc-DPD uptake was present in all group A patients and absent in all group B patients; thus, using genotyping/immunohistochemistry as the reference technique, the accuracy of Tc-99m-DPD scintigraphy for distinction of TTR-related and AL etiology was 100%. Cardiac Tc-99m-DPD uptake was also absent among unaffected controls. Using echocardiography as the reference standard for recognition of cardiac involvement, sensitivity and specificity of scintigraphy were both 100% for group A patients; in group B, sensitivity was 0% and specificity was 100% (accuracy, 50%). Eleven patients with myocardial (99m)-Tc-DPD uptake underwent (99m)-Tc-methylene diphosphonate ((99m)-Tc-MDP) scintigraphy; all patients showed a (99m)-Tc-MDP myocardial visual score of 0.CONCLUSIONS Etiology is a third major cause-in addition to type of organ-involved (soft-tissue/heart) and tracer type-of scintigraphic variability in cardiac amyloidosis. This is a highly relevant consideration for future studies. We conclude that (99m)-Tc-DPD scintigraphy is a useful step in the workup of the differential diagnosis of TTR versus AL etiology in patients with documented cardiac amyloidosis.