Abstract

Purpose

Increased cardiac uptake (CU) on early-phase ^99mTc-HMDP scintigraphy has demonstrated diagnostic and prognostic values in amyloid transthyretin (ATTR) cardiac amyloidosis (CA). Extracardiac uptake (ECU) has been poorly studied. We assessed the clinical value of ECU, in combination with CU, on ^99mTc-HMDP scintigraphy using a novel Methodological Amyloidosis Diagnostic Index (MADI).

Methods

We reviewed all patients referred for suspicion of CA, who underwent ^99mTc-HMDP scintigraphy over an 8-year period. ECU, CU, and MADI were determined: MADI0 = neither ECU or CU, MADI1 = ECU alone, MADI2 = CU alone, and MADI3 = ECU + CU.

Results

Of 308 eligible patients, 247 had CA, including 75 ATTRv, 107 ATTRwt, and 65 light-chain (AL), while 61 had another cardiopathy (controls). ECU was observed in 29% of CA and 3% of controls. Most frequent sites of ECU were pleuropulmonary (16% of CA, 3% of controls) followed by the digestive tract and subcutaneous tissues. The liver and spleen ECU was only observed in AL-CA (n = 8). CU was only observed in CA patients (n = 187), of whom 182 had ATTR-CA vs. 5 AL-CA, P < 0.001. MADI0 was only observed in controls (97%) and in AL-CA (60%). MADI1 was mainly observed in AL-CA (positive predictive value, PPV = 91%) while MADI2/3 were more frequent in ATTR-CA (PPV = 97%), P < 0.0001. MADI > 0 vs. MADI0 in AL and MADI3 vs. MADI2 in ATTR were associated with a worse prognosis (P = 0.03 and P = 0.002, respectively).

Conclusions

ECU combined with CU demonstrates high diagnostic and prognostic values in CA patients. MADI seems an easy and reliable score in clinical practice.

中文翻译：

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早期99mTc-HMDP SPECT / CT证实有心外膜软组织摄取，有助于分型心脏淀粉样变性，并具有较高的预后价值。

摘要

目的

早期^99m Tc-HMDP闪烁显像术的心脏摄取（CU）增加已证明淀粉样蛋白甲状腺素（ATTR）心脏淀粉样变性病（CA）的诊断和预后价值。心脏外摄取（ECU）的研究很少。我们使用一种新的方法学淀粉样变性诊断指数（MADI）评估ECU与CU结合在^99m Tc-HMDP闪烁显像上的临床价值。

方法

我们回顾了所有因CA怀疑而转诊的患者，这些患者在8年中接受了^99m Tc-HMDP闪烁显像。确定ECU，CU和MADI：MADI0 =既不使用ECU也不使用CU，MADI1 =单独使用ECU，MADI2 =单独使用CU，以及MADI3 = ECU + CU。

结果

在308名合格患者中，有247名患有CA，其中包括75名ATTRv，107名ATTRwt和65个轻链（AL），而61名患有另一种心脏病（对照）。在29％的CA和3％的对照中观察到ECU。ECU最常见的部位是胸膜肺（CA的16％，对照的3％），其次是消化道和皮下组织。仅在AL-CA中观察到肝和脾ECU（n  = 8）。仅在CA患者中观察到CU（n  = 187），其中182例患有ATTR-CA，而5例AL-CA，P  <0.001。仅在对照组（97％）和AL-CA（60％）中观察到MADI0。MADI1主要在AL-CA中观察到（阳性预测值，PPV = 91％），而MADI2 / 3在ATTR-CA中更常见（PPV = 97％），P <0.0001。AL的MADI> 0与MADI0以及ATTR的MADI3 vs. MADI2与预后较差相关（分别为P  = 0.03和P  = 0.002）。

结论

ECU与CU联用对CA患者具有很高的诊断和预后价值。MADI在临床实践中似乎是一个简单而可靠的分数。