Heart Centre, Cardiology, Department of Public Health and Clinical Medicine, Umeå University, 901 85, Umeå, Sweden.
Heart Centre, Department of Surgical and Perioperative Sciences, Clinical Physiology, Umeå University, Umeå, Sweden.
J Nucl Cardiol. 2018 Feb;25(1):240-248. doi: 10.1007/s12350-016-0638-5. Epub 2016 Sep 19.
DPD scintigraphy has been advocated for imaging cardiac amyloid in ATTR amyloidosis. PET utilizing C-Pittsburgh compound B (PIB) is the gold standard for imaging brain amyloid in Alzheimer's disease. PIB was recently shown to identify cardiac amyloidosis in both AL and ATTR amyloidosis. In the ATTR population, two types of amyloid fibrils exist, one containing fragmented and full-length TTR (type A) and the other only full-length TTR (type B). The aim of this study was to further evaluate PIB-PET in patients with hereditary ATTR amyloidosis.
Ten patients with biopsy-proven V30M ATTR amyloidosis and discrete or no signs of cardiac involvement were included. Patients were grouped according to TTR-fragmentation. All underwent DPD scintigraphy, echocardiography, and PIB-PET. A left ventricular PIB-retention index (PIB-RI) was established and compared to five normal volunteers.
PIB-RI was increased in all patients (P < 0.001), but was significantly higher in type B than in type A (0.129 ± 0.041 vs 0.040 ± 0.006 min, P = 0.009). Cardiac DPD uptake was elevated in group A and absent in group B.
PIB-PET, in contrast to DPD scintigraphy, has the potential to specifically identify cardiac amyloid depositions irrespective of amyloid fibril composition. The heart appears to be a target organ for amyloid deposition in ATTR amyloidosis.
放射性核素标记的二苯基丁酸（DPD）闪烁扫描术已被用于心肌淀粉样变的影像学诊断。正电子发射断层扫描术（PET）使用 C-Pittsburgh 化合物 B（PIB）是阿尔茨海默病脑淀粉样变的金标准。最近的研究表明，PIB 可用于识别 AL 和 ATTR 淀粉样变中的心脏淀粉样变。在 ATTR 患者中，存在两种类型的淀粉样纤维，一种含有片段化和全长 TTR（A型），另一种仅含有全长 TTR（B 型）。本研究旨在进一步评估遗传性 ATTR 淀粉样变患者的 PIB-PET。
本研究纳入了 10 例经活检证实的 V30M ATTR 淀粉样变患者，且无或仅有不明显的心脏受累迹象。根据 TTR 片段化情况对患者进行分组。所有患者均接受了 DPD 闪烁扫描、超声心动图和 PIB-PET 检查。建立左心室 PIB 保留指数（PIB-RI），并与 5 名正常志愿者进行比较。
所有患者的 PIB-RI 均升高（P<0.001），但 B 型患者的 PIB-RI 明显高于 A 型（0.129±0.041 比 0.040±0.006 min，P=0.009）。A 型患者的心脏 DPD 摄取增加，而 B 型患者则无。
与 DPD 闪烁扫描相比，PIB-PET 具有特异性识别心脏淀粉样沉积的潜力，而与淀粉样纤维组成无关。心脏似乎是 ATTR 淀粉样变中淀粉样沉积的靶器官。
