Mondor Amyloidosis Network, Créteil, France.
Department of Nuclear Medicine, AP-HP, Henri Mondor Teaching Hospital, 51 Ave. du Mal de Lattre de Tassigny, 94010, Créteil, France.
J Nucl Cardiol. 2018 Dec;25(6):2072-2079. doi: 10.1007/s12350-017-0894-z. Epub 2017 Apr 26.
A decreased longitudinal strain in basal segments with a base-to-apex gradient has been described in patients with cardiac amyloidosis (CA).
Aim was to investigate the left ventricular (LV) regional distribution of early-phase Tc-Hydroxymethylene diphosphonate (Tc-HMDP) uptake in patients with transthyretin-related cardiac amyloidosis (TTR-CA).
All patients underwent a whole-body planar Tc-HMDP scintigraphy acquired at 10-min post-injection (early-phase) followed by a thorax SPECT/CT. The segmental uptake (expressed as % of maximal myocardial HMDP uptake) was investigated on the AHA 17-segment model and 3-segment model (basal, mid-cavity, apical).
Sixty-one TTR-CA patients were included of whom 29 were wild-type (wt-TTR-CA) and 32 had hereditary TTR-CA (m-TTR-CA). Early myocardial Tc-HMDP uptake occurred in all TTR-CA. In all patients, segmental analysis of the LV myocardial distribution of Tc-HMDP uptake showed an increased median uptake (interquartile range) in basal/mid-cavity segments compared to the lowest median uptake of apical segments (respectively, 79% [72%-86%] vs. 72% [64%-81%]; P < 10). This pattern was similar in wt-TTR-CA group (78% [70%-84%] vs. 70% [61%-81%]; P < 10), in m-TTR-CA group (80% [74%-86%] vs. 73 [66%-82%]; P < 10) and remained constant independently of the TTR mutation subtype with P ranging 10 to 0.03.
Early-phase myocardial scintigraphy identified regional distribution of Tc-HMDP uptake characterized by a base-to-apex gradient, corroborating echocardiographic, and cardiac magnetic resonance findings. This apical sparing pattern was similar across TTR-CA and TTR mutation subtypes.
在转甲状腺素蛋白相关心脏淀粉样变性（TTR-CA）患者中，已描述基底段纵向应变随基底至心尖梯度降低。
目的是研究 17 节段和 3 节段模型（基底段、中段、心尖段）的早期 Tc-羟甲基二膦酸盐（Tc-HMDP）摄取在转甲状腺素蛋白相关心脏淀粉样变性（TTR-CA）患者中的左心室（LV）区域性分布。
所有患者均接受全身平面 Tc-HMDP 闪烁显像，注射后 10 分钟（早期）采集，随后进行胸部 SPECT/CT。使用美国心脏协会 17 节段模型和 3 节段模型（基底段、中段、心尖段）研究节段摄取（以最大心肌 HMDP 摄取的百分比表示）。
共纳入 61 例 TTR-CA 患者，其中 29 例为野生型（wt-TTR-CA），32 例为遗传性 TTR-CA（m-TTR-CA）。所有 TTR-CA 患者均出现早期心肌 Tc-HMDP 摄取。所有患者 LV 心肌 Tc-HMDP 摄取的节段分析显示，基底/中段节段的摄取中位数（四分位间距）高于最低中位数（分别为 79%[72%-86%]和 72%[64%-81%]；P<0.001）。wt-TTR-CA 组（78%[70%-84%]和 70%[61%-81%]；P<0.001）、m-TTR-CA 组（80%[74%-86%]和 73%[66%-82%]；P<0.001）的模式相似，且与 TTR 突变亚型无关，P 值在 0.03 到 10 之间。
早期心肌闪烁显像确定了 Tc-HMDP 摄取的区域性分布，其特征为基底至心尖梯度，与超声心动图和心脏磁共振检查结果一致。这种心尖节段保留模式在 TTR-CA 和 TTR 突变亚型中相似。
