Prognostic utility of the Perugini grading of 99mTc-DPD scintigraphy in transthyretin (ATTR) amyloidosis and its relationship with skeletal muscle and soft tissue amyloid
Prognostic utility of the Perugini grading of 99mTc-DPD scintigraphy in transthyretin (ATTR) amyloidosis and its relationship with skeletal muscle and soft tissue amyloid
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DOI：
10.1093/ehjci/jew325
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发表时间：
2017-12-01
影响因子：
6.2
通讯作者：
Gillmore, Julian D.
中科院分区：
文献类型：
--
作者：
Hutt, David F.;Fontana, Marianna;Gillmore, Julian D.
[目的]近年来，经骨显像证实，（99 mTc）标记的3，3-二膦酰基-1，2-丙二羧酸（99 m-DPD）在心肌高摄取（Perceptii 2级或3级）中对心脏甲状腺素运载蛋白（ATTR）淀粉样变性具有较高的诊断敏感性和特异性。我们试图确定患者分层Permalini等级的Tc-99 m-DPD血管造影术是否具有预后意义，在ATTR amyloidosis.Methods和Results患者生存时间的Tc-99 m-DPD血管造影术确定在602例ATTR淀粉样变性，包括377野生型ATTR（ATTRwt）和225突变型ATTR（ATTRm）淀粉样变性。患者根据Tc-99 m-DPD扫描的Perdini分级（0-3）进行分层。确定基线时其他患者和疾病相关因素的预后意义。在整个队列中，根据共识标准，Percusi 0级Tc-99 m-DPD扫描（n = 28）的结果总是与心脏淀粉样蛋白的缺乏相关，并且与Percusi 1级（n = 28），2级（n = 436）或3级（n = 110）Tc-99 m-DPD扫描相比，患者生存率显著更高（P < 0.005）。在ATTRwt（n = 369）、V122 I相关ATTRm（n = 92）或T60 A相关ATTRm（n = 59）淀粉样变性中，99 mTc-DPD扫描1级、2级或3级患者的生存率无差异。通过平衡对比心脏磁共振成像确定的心脏淀粉样蛋白负荷，在Pereini 2级和Pereini 3级Tc-99 m-DPD扫描的患者之间相似，但后一组的骨骼肌/软组织与股骨的比率显著较高结论Tc-99 m-DPD心肌显像对心肌ATTR淀粉样蛋白的检出具有较高的敏感性，但按诊断时阳性的Percushi分级分层没有预后意义。
Aims High-grade (Perugini grade 2 or 3) cardiac uptake on bone scintigraphy with (99m)Technetium labelled 3,3-diphosphono-1,2-propanodicarboxylic acid (Tc-99m-DPD) has lately been confirmed to have high diagnostic sensitivity and specificity for cardiac transthyretin (ATTR) amyloidosis. We sought to determine whether patient stratification by Perugini grade on Tc-99m-DPD scintigraphy has prognostic significance in ATTR amyloidosis.Methods and results Patient survival from time of Tc-99m-DPD scintigraphy was determined in 602 patients with ATTR amyloidosis, including 377 with wild-type ATTR (ATTRwt) and 225 with mutant ATTR (ATTRm) amyloidosis. Patients were stratified according to Perugini grade (0-3) on Tc-99m-DPD scan. The prognostic significance of additional patient and disease-related factors at baseline were determined. In the whole cohort, the finding of a Perugini grade 0 Tc-99m-DPD scan (n = 28) was invariably associated with absence of cardiac amyloid according to consensus criteria as well as significantly better patient survival compared to a Perugini grade 1 (n = 28), 2 (n = 436) or 3 (n = 110) Tc-99m-DPD scan (P < 0.005). There were no differences in survival between patients with a grade 1, grade 2 or grade 3 99mTc-DPD scan in ATTRwt (n = 369), V122I-associated ATTRm (n = 92) or T60A-associated ATTRm (n = 59) amyloidosis. Cardiac amyloid burden, determined by equilibrium contrast cardiac magnetic resonance imaging, was similar between patients with Perugini grade 2 and Perugini grade 3 Tc-99m-DPD scans but skeletal muscle/soft tissue to femur ratio was substantially higher in the latter group (P < 0.001).Conclusion Tc-99m-DPD scintigraphy is exquisitely sensitive for identification of cardiac ATTR amyloid, but stratification by Perugini grade of positivity at diagnosis has no prognostic significance.