Khor Yiu Ming, Cuddy Sarah, Harms Hendrik J, Kijewski Marie F, Park Mi-Ae, Robertson Matthew, Hyun Hyewon, Di Carli Marcelo F, Bianchi Giada, Landau Heather, Yee Andrew, Sanchorawala Vaishali, Ruberg Frederick L, Liao Ronglih, Berk John, Falk Rodney H, Dorbala Sharmila
Division of Nuclear Medicine, Department of Radiology, Brigham and Women's Hospital, Boston, MA, USA.
Cardiac Amyloidosis Program, Division of Cardiology, Department of Medicine, Brigham and Women's Hospital, Boston, MA, USA.
Eur J Nucl Med Mol Imaging. 2020 Jul;47(8):1998-2009. doi: 10.1007/s00259-019-04627-7. Epub 2019 Dec 5.
The clinical diagnosis of pulmonary involvement in individuals with systemic AL amyloidosis remains challenging. [F]florbetapir imaging has previously identified AL amyloid deposits in the heart and extra-cardiac organs. The aim of this study is to determine quantitative [F]florbetapir pulmonary kinetics to identify pulmonary involvement in individuals with systemic AL amyloidosis.
We prospectively enrolled 58 subjects with biopsy-proven AL amyloidosis and 9 control subjects (5 without amyloidosis and 4 with ATTR cardiac amyloidosis). Pulmonary [F]florbetapir uptake was evaluated visually and quantified as distribution volume of specific binding (Vs) derived from compartmental analysis and simpler semiquantitative metrics of maximum standardized uptake values (SUVmax), retention index (RI), and target-to-blood ratio (TBR).
On visual analysis, pulmonary tracer uptake was absent in most AL subjects (40/58, 69%); 12% (7/58) of AL subjects demonstrated intense bilateral homogeneous tracer uptake. In this group, compared to the control group, Vs (median Vs 30-fold higher, 9.79 vs. 0.26, p < 0.001), TBR (median TBR 12.0 vs. 1.71, p < 0.001), and RI (median RI 0.310 vs. 0.033, p < 0.001) were substantially higher. Notably, the AL group without visually apparent pulmonary [F]florbetapir uptake also demonstrated a > 3-fold higher Vs compared to the control group (median 0.99 vs. 0.26, p < 0.001). Vs was independently related to left ventricular SUVmax, a marker of cardiac AL deposition, but not to ejection fraction, a marker of cardiac dysfunction. Also, intense [F]florbetapir lung uptake was not related to [C]acetate lung uptake, suggesting that intense [F]florbetapir lung uptake represents AL amyloidosis rather than heart failure.
[F]florbetapir PET/CT offers the potential to noninvasively identify pulmonary AL amyloidosis, and its clinical relevance warrants further study.
系统性AL淀粉样变性患者肺部受累的临床诊断仍然具有挑战性。[F]氟代硼吡咯成像先前已在心脏和心脏外器官中识别出AL淀粉样蛋白沉积。本研究的目的是确定定量的[F]氟代硼吡咯肺部动力学,以识别系统性AL淀粉样变性患者的肺部受累情况。
我们前瞻性纳入了58例经活检证实为AL淀粉样变性的受试者和9例对照受试者(5例无淀粉样变性,4例患有ATTR心脏淀粉样变性)。通过视觉评估肺部[F]氟代硼吡咯摄取情况,并将其量化为通过房室分析得出的特异性结合分布容积(Vs)以及更简单的半定量指标,即最大标准化摄取值(SUVmax)、滞留指数(RI)和靶血比(TBR)。
在视觉分析中,大多数AL受试者(40/58,69%)肺部无示踪剂摄取;12%(7/58)的AL受试者表现出双侧均匀强烈的示踪剂摄取。在该组中,与对照组相比,Vs(中位数Vs高30倍,9.79对0.26,p<0.001)、TBR(中位数TBR 12.0对1.71,p<0.001)和RI(中位数RI 0.310对0.033,p<0.001)显著更高。值得注意的是,在视觉上未观察到肺部[F]氟代硼吡咯摄取的AL组与对照组相比,Vs也高出3倍以上(中位数0.99对0.26,p<0.001)。Vs与左心室SUVmax独立相关,左心室SUVmax是心脏AL沉积的标志物,但与射血分数无关,射血分数是心脏功能障碍的标志物。此外,强烈的[F]氟代硼吡咯肺部摄取与[C]乙酸盐肺部摄取无关,这表明强烈的[F]氟代硼吡咯肺部摄取代表AL淀粉样变性而非心力衰竭。
[F]氟代硼吡咯PET/CT有潜力无创识别肺部AL淀粉样变性,其临床相关性值得进一步研究。