Department of Cardiovascular Medicine, Graduate School of Medical Sciences, Kumamoto University.
Department of Diagnostic Radiology, Graduate School of Medical Sciences, Kumamoto University.
Circ J. 2022 Jun 24;86(7):1113-1120. doi: 10.1253/circj.CJ-22-0118. Epub 2022 May 21.
The accurate sensitivity of amyloid deposition in extracardiac tissue (subcutaneous tissue and gastrointestinal tract) has not been evaluated in transthyretin amyloidosis cardiomyopathy (ATTR-CM) patients. This study aimed to evaluate the sensitivity of amyloid deposition in obtained endomyocardial and extracardiac biopsies.
This study retrospectively evaluated 175 consecutive ATTR-CM patients (wild-type [ATTRwt]: 134, hereditary [ATTRv]: 41) who had positive findings on Tc-labeled pyrophosphate (Tc-PYP) scintigraphy and underwent tissue biopsy of at least one organ (subcutaneous tissue, gastrointestinal tract, and endomyocardium). Amyloid deposition was observed in the subcutaneous tissue of 57/150 patients (38%), gastrointestinal tract of 80/131 patients (61%), and endomyocardium of 108/109 patients (99%). Compared to patients with ATTRv, ATTRwt had significantly lower sensitivity in subcutaneous tissue (73% vs. 25%, P<0.01) and tended to be lower in the gastrointestinal tract (74% vs. 57%, P=0.08) biopsies. Among 124 patients who underwent both subcutaneous tissue and gastrointestinal tract biopsies, amyloid was detected in at least 1 specimen in 91 (73%) patients. The sensitivity of the combination of extracardiac biopsies was 66% and 94% in ATTRwt-CM and ATTRv-CM, respectively. Multivariate analysis reveals that ATTRv was the only significant predictor of amyloid deposition in the subcutaneous tissue.
Subcutaneous tissue and gastrointestinal tract biopsy sensitivity are inadequate, especially in patients with ATTRwt; however, the combination of these extracardiac biopsies contributes to increased sensitivity in patients with positive Tc-PYP scintigraphy findings.
在转甲状腺素蛋白淀粉样变心肌病（ATTR-CM）患者中，尚未评估细胞外组织（皮下组织和胃肠道）中淀粉样沉积的准确敏感性。本研究旨在评估心肌和细胞外活检中淀粉样沉积的敏感性。
本研究回顾性评估了 175 例连续的 ATTR-CM 患者（野生型[ATTRwt]：134 例，遗传性[ATTRv]：41 例），这些患者 Tc 标记焦磷酸盐（Tc-PYP）闪烁显像阳性，并进行了至少一种器官（皮下组织、胃肠道和心肌）的组织活检。在 150 例患者中有 57 例（38%）在皮下组织中观察到淀粉样沉积，在 131 例患者中有 80 例（61%）在胃肠道中观察到淀粉样沉积，在 109 例患者中有 108 例（99%）在心肌中观察到淀粉样沉积。与 ATTRv 患者相比，ATTRwt 患者在皮下组织中的敏感性明显较低（73%比 25%，P<0.01），在胃肠道中的敏感性也较低（74%比 57%，P=0.08）。在 124 例同时进行皮下组织和胃肠道活检的患者中，91 例（73%）患者至少有 1 个标本检测到淀粉样蛋白。ATTRwt-CM 和 ATTRv-CM 患者中外周活检的敏感性分别为 66%和 94%。多变量分析表明，ATTRv 是皮下组织中淀粉样沉积的唯一显著预测因子。
皮下组织和胃肠道活检的敏感性不足，尤其是在 ATTRwt 患者中；然而，这些外周活检的组合可提高 Tc-PYP 闪烁显像阳性患者的敏感性。
