Perugini Enrica, Guidalotti Pier Luigi, Salvi Fabrizio, Cooke Robin M T, Pettinato Cinzia, Riva Letizia, Leone Ornella, Farsad Mohsen, Ciliberti Paolo, Bacchi-Reggiani Letizia, Fallani Francesco, Branzi Angelo, Rapezzi Claudio
Institute of Cardiology, University of Bologna and S. Orsola-Malpighi Hospital, Bologna, Italy.
J Am Coll Cardiol. 2005 Sep 20;46(6):1076-84. doi: 10.1016/j.jacc.2005.05.073.
We investigated the diagnostic accuracy of 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid (99mTc-DPD) scintigraphy for differentiation of monoclonal immunoglobulin light-chain (AL) and transthyretin (TTR)-related cardiac amyloidosis.
Differential diagnosis between TTR-related and AL amyloidosis is often complex and time-consuming.
Patients under routine observation with TTR-related/AL systemic amyloidosis and echocardiographic evidence of cardiac involvement were studied with 99mTc-DPD scintigraphy.
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 99mTc-DPD scintigraphy for distinction of TTR-related and AL etiology was 100%. Cardiac 99mTc-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 99mTc-DPD uptake underwent 99mTc-methylene diphosphonate (99mTc-MDP) scintigraphy; all patients showed a 99mTc-MDP myocardial visual score of 0.
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 99mTc-DPD scintigraphy is a useful step in the workup of the differential diagnosis of TTR versus AL etiology in patients with documented cardiac amyloidosis.
我们研究了99m锝-3,3-二膦酸-1,2-丙二羧酸（99mTc-DPD）闪烁扫描术在鉴别单克隆免疫球蛋白轻链（AL）相关和转甲状腺素蛋白（TTR）相关心脏淀粉样变性中的诊断准确性。
TTR相关和AL淀粉样变性之间的鉴别诊断通常复杂且耗时。
对接受常规观察的TTR相关/AL系统性淀粉样变性患者以及有心脏受累超声心动图证据的患者进行99mTc-DPD闪烁扫描术研究。
TTR相关病因（A组；n = 15）和AL病因（B组；n = 10）的心脏受累患者在左心室质量和肾功能方面具有可比性。与B组以及10名未受影响的对照组相比，A组的心脏和心脏/全身示踪剂滞留显著更高（p < 0.05）。在视觉评分中，所有A组患者均有心脏99mTc-DPD摄取，而所有B组患者均无；因此，以基因分型/免疫组织化学作为参考技术，99mTc-DPD闪烁扫描术区分TTR相关和AL病因的准确性为100%。未受影响的对照组中也无心脏99mTc-DPD摄取。以超声心动图作为识别心脏受累的参考标准，闪烁扫描术对A组患者的敏感性和特异性均为100%；在B组中，敏感性为0%，特异性为100%（准确性为50%）。11名有心肌99mTc-DPD摄取的患者接受了99m锝-亚甲基二膦酸盐（99mTc-MDP）闪烁扫描术；所有患者的99mTc-MDP心肌视觉评分为0。
病因是心脏淀粉样变性闪烁扫描术变异性的第三个主要原因——除了受累器官类型（软组织/心脏）和示踪剂类型之外。这是未来研究中一个高度相关的考虑因素。我们得出结论，99mTc-DPD闪烁扫描术是对已记录心脏淀粉样变性患者进行TTR与AL病因鉴别诊断检查的一个有用步骤。
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