BACKGROUND:The extent of myocardial bone tracer uptake with technetium pyrophosphate, hydroxymethylene diphosphonate, and 3,3-diphosphono-1,2-propanodicarboxylate in transthyretin amyloid cardiomyopathy (ATTR-CM) might reflect cardiac amyloid burden and be associated with outcome.METHODS:Consecutive patients with ATTR-CM who underwent diagnostic bone tracer scintigraphy with acquisition of whole-body planar and cardiac single-photon emission computed tomography (SPECT) images from the National Amyloidosis Centre and 4 Italian centers were included. Cardiac uptake was defined according to the Perugini classification: 0=absent cardiac uptake; 1=mild uptake less than bone; 2=moderate uptake equal to bone; and 3=high uptake greater than bone. Extent of right ventricular (RV) uptake was defined as focal (basal segment of the RV free wall only) or diffuse (extending beyond basal segment) on the basis of SPECT imaging. The primary outcome was all-cause mortality.RESULTS:Among 1422 patients with ATTR-CM, RV uptake accompanying left ventricular uptake was identified by SPECT imaging in 100% of cases at diagnosis. Median follow-up in the whole cohort was 34 months (interquartile range, 21 to 50 months), and 494 patients died. By Kaplan-Meier analysis, diffuse RV uptake on SPECT imaging (n=936) was associated with higher all-cause mortality compared with focal (n=486) RV uptake (77.9% versus 22.1%; P<0.001), whereas Perugini grade was not associated with survival (P=0.27 in grade 2 versus grade 3). On multivariable analysis, after adjustment for age at diagnosis (hazard ratio [HR], 1.03 [95% CI, 1.02–1.04]; P<0.001), presence of the p.(V142I) TTR variant (HR, 1.42 [95% CI, 1.20–1.81]; P=0.004), National Amyloidosis Centre stage (each category, P<0.001), stroke volume index (HR, 0.99 [95% CI, 0.97–0.99]; P=0.043), E/e’ (HR, 1.02 [95% CI, 1.007–1.03]; P=0.004), right atrial area index (HR, 1.05 [95% CI, 1.02–1.08]; P=0.001), and left ventricular global longitudinal strain (HR, 1.06 [95% CI, 1.03–1.09]; P<0.001), diffuse RV uptake on SPECT imaging (HR, 1.60 [95% CI, 1.26–2.04]; P<0.001) remained an independent predictor of all-cause mortality. The prognostic value of diffuse RV uptake was maintained across each National Amyloidosis Centre stage and in both wild-type and hereditary ATTR-CM (P<0.001 and P=0.02, respectively).CONCLUSIONS:Diffuse RV uptake of bone tracer on SPECT imaging is associated with poor outcomes in patients with ATTR-CM and is an independent prognostic marker at diagnosis.

中文翻译：

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右心室摄取对骨闪烁扫描在运甲状腺素蛋白淀粉样变心肌病中的临床和预后意义

背景：转甲状腺素蛋白淀粉样心肌病 (ATTR-CM) 中焦磷酸锝、羟亚甲基二磷酸盐和 3,3-二膦酰基-1,2-丙二甲酸的心肌骨示踪剂摄取程度可能反映心脏淀粉样蛋白负荷并与结果相关。方法：纳入连续接受诊断性骨示踪闪烁扫描的 ATTR-CM 患者，并从国家淀粉样变性中心和 4 个意大利中心获取全身平面和心脏单光子发射计算机断层扫描 (SPECT) 图像。心脏摄取根据Perugini分类来定义：0=不存在心脏摄取； 1=低于骨的轻度吸收； 2=中等吸收，等于骨； 3=高于骨的高摄取。根据 SPECT 成像，右心室 (RV) 摄取范围被定义为局灶性（仅 RV 游离壁的基底段）或弥漫性（延伸超出基底段）。主要结局是全因死亡率。 结果：在 1422 名 ATTR-CM 患者中，100% 的病例在诊断时通过 SPECT 成像识别出 RV 摄取伴随左心室摄取。整个队列的中位随访时间为 34 个月（四分位数范围，21 至 50 个月），494 名患者死亡。通过 Kaplan-Meier 分析，与局灶性 (n=486) RV 摄取相比，SPECT 成像上弥漫性 RV 摄取 (n=936) 与更高的全因死亡率相关（77.9% 对比 22.1%；P <0.001），而 Perugini 分级与生存率无关（2 级与 3 级的P = 0.27）。在多变量分析中，调整诊断时的年龄后（风险比 [HR]，1.03 [95% CI，1.02–1.04]；P <0.001），p.(V142I) TTR变异的存在（HR，1.42 [95%] CI，1.20–1.81]；P = 0.004），国家淀粉样变性中心分期（每个类别，P <0.001），每搏输出量指数（HR，0.99 [95% CI，0.97–0.99]；P = 0.043），E/e ’（HR，1.02 [95% CI，1.007–1.03]；P = 0.004），右心房面积指数（HR，1.05 [95% CI，1.02–1.08]；P = 0.001），以及左心室整体纵向应变（ HR，1.06 [95% CI，1.03–1.09]；P <0.001），SPECT 成像上的弥漫性 RV 摄取（HR，1.60 [95% CI，1.26–2.04]；P <0.001）仍然是全因的独立预测因子死亡。在每个国家淀粉样变性中心分期以及野生型和遗传性 ATTR-CM 中，弥漫性 RV 摄取的预后价值均保持不变（分别为P <0.001 和P = 0.02）。结论：SPECT 成像上骨示踪剂的弥漫性 RV 摄取是与 ATTR-CM 患者的不良预后相关，并且是诊断时的独立预后标志物。