锝[99Tcm]焦磷酸盐(99Tcm-PYP)单光子显像诊断转甲状腺素相关心脏淀粉样变操作指南

任超; 田庄; 何山; 王雪竹; 郝志鑫; 丁洁; 张抒扬; 霍力

doi:10.12376/j.issn.2097-0501.2022.01.012

锝[⁹⁹Tc^m]焦磷酸盐(⁹⁹Tc^m-PYP)单光子显像诊断转甲状腺素相关心脏淀粉样变操作指南

doi: 10.12376/j.issn.2097-0501.2022.01.012

任超^{1, 3},
田庄^{2, 3},
何山^{2, 3},
王雪竹^{1, 3},
郝志鑫^{1, 3},
丁洁^{1, 3},
张抒扬^{2, 3},
霍力^{1, 3, ,}

1.
中国医学科学院北京协和医院核医学科核医学分子靶向诊疗北京市重点实验室, 北京 100730
2.
中国医学科学院北京协和医院心内科, 北京 100730
3.
中国医学科学院北京协和医院疑难重症及罕见病国家重点实验室, 北京 100730

基金项目:

中国医学科学院医学与健康科技创新工程项目 2021-I2M-1-003

详细信息

通信作者:
霍力，E-mail：Huoli@pumch.cn

中图分类号: R144; R541
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出版历程
- 收稿日期: 2021-12-13
- 修回日期: 2022-01-05

Consensus to ^99mTechnetium-Pyrophosphate Scintigraphy in the Diagnosis of Transthyretin-related Cardiac Amyloidosis

REN Chao^{1, 3},
TIAN Zhuang^{2, 3},
HE Shan^{2, 3},
WANG Xuezhu^{1, 3},
HAO Zhixin^{1, 3},
DING Jie^{1, 3},
ZHANG Shuyang^{2, 3},
HUO Li^{1, 3
, ,}

1.
Department of Nuclear Medicine, Beijing Key Laboratory of Molecular Targeted Diagnosis and Therapy in Nuclear Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China
2.
Department of Cardiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China
3.
State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China

Funding:

CAMS Innovation Fund for Medical Sciences 2021-I2M-1-003

More Information

Corresponding author: HUO Li, E-mail: Huoli@pumch.cn

摘要

摘要: 转甲状腺素蛋白相关淀粉样心肌病(transthyretin-related amyloid cardiomyopathy，ATTR-CM)是由于转甲状腺素前体蛋白错误折叠形成的不溶性淀粉样纤维沉积于心肌细胞间隙所致的病变。该病变可导致心肌功能障碍、充血性心力衰竭和死亡。若能对该病变进行早期临床明确诊断，可以尽早对病患使用药物治疗，干预病情进展，从而有效改善患者预后。锝[⁹⁹Tc^m]焦磷酸盐(^99mtechnetium-pyrophosphate, ⁹⁹Tc^m-PYP)单光子发射计算机断层成像术(single-photon emission computed tomography, SPECT)近年来被广泛用于心脏淀粉样变(cardiac amyloidosis, CA)特异性影像学检查，在做到早期无创诊断的同时，可以通过平面显像Perugini视觉评分、心脏与对侧肺(heart to contralateral lung, H/CL)摄取比值半定量分析及SPECT图像分析得到准确的病理学分型。本文介绍了⁹⁹Tc^m-PYP SPECT显像在诊断ATTR-CM中的应用概况、方法及注意事项，为该技术的临床应用提供借鉴。
- 锝[⁹⁹Tc^m]焦磷酸盐 /
- 转甲状腺素相关心脏淀粉样变 /
- 单光子发射计算机断层成像
Abstract: Transthyretin-related amyloid cardiomyopathy (ATTR-CM) is a disease caused by the depo-sition of insoluble amyloid fibers formed by the misfolding of transthyretin precursor protein in the intercellular space of cardiomyocytes. This lesion may lead to myocardial dysfunction, cogestive heart failure, and death.When diagnosed earlier, the patient can be treated with drugs as soon as possible to intervene in the progress of the disease, so as to effectively improve the patient's prognosis.^99mtechnetium-pyrophosphate (⁹⁹Tc^m-PYP)single-photon emission computed tomography (SPECT) has been widely used in the imaging examination of cardiac amyloidosis (CA) in recent years. While achieving early non-invasive diagnosis, accurate pathological classification can be obtained through Perugini visual score analysis, semi-quantitative analysis of heart to contralateral lung (H/CL) ratio, and SPECT image analysis. This article presents the application, methods, and the precautions of ⁹⁹Tc^m-PYPSPECT in the diagnosis of ATTR-CM, aiming to provide clinical reference for the application of this technology.
- ^99mtechnetium-pyrophosphate /
- transthyretin-related amyloidcardiomyopathy /
- single-photon emission computed tomography
注释:

作者贡献：任超负责研究实施、论文撰写；何山、王雪竹、郝志鑫、丁洁负责研究实施；田庄、张抒扬、霍力负责研究指导、论文修改、经费支持。

利益冲突：所有作者均声明不存在利益冲突

HTML全文

图 1 Asp38Asn型突变基因携带者(男，22岁)⁹⁹Tc^m-PYP心肌淀粉样变显像3 h平面显像、全身显像及心肌断层显像

患者无临床症状，超声心动图及实验室检查结果均为阴性; 平面显像前位(A1)及左侧位(A2)示胸骨及肋骨摄取清晰，心脏区域未见放射性摄取，视觉评分2分，H/CL为1.09;全身显像前位(A3)及后位(A4)示全身骨骼摄取清晰可见，未见明确摄取异常增高或减低区，双肾及膀胱可见生理性摄取; 心肌断层显像(A5)示心肌无弥漫性摄取诊断为阴性

下载: 全尺寸图片幻灯片

图 2 Gly73Glu型ATTRv患者(女，37岁)⁹⁹Tc^m-PYP心肌淀粉样变显像3 h平面显像、全身显像及心肌断层显像

患者心脏超声提示双房增大，二、三尖瓣关闭不全，左室限制性舒张功能减低，左室射血分数：66%，室间隔(24 mm)及左室后壁(17 mm)增厚；实验室检查NT-proBNP：2922 pg/mL(0~115)，血尿免疫固定电泳及游离轻链结果均为阴性; 平面显像前位(B1)及左侧位(B2)示心脏区域显著放射性摄取增高(黑色箭头所示)，胸骨及肋骨摄取减低，可见双侧甲状腺及脾脏异常放射性摄取增高，视觉评分3分，H/CL为2.23;全身显像前位(B3)及后位(B4)示全身骨骼摄取不清晰，可见心脏、双侧甲状腺及脾脏异常放射性摄取增高，双肾及膀胱可见生理性摄取; 心肌断层显像(B5)示心肌弥漫性摄取(白色箭头所示)，患者诊断为ATTR-CM阳性，甲状腺及脾脏受累可能

下载: 全尺寸图片幻灯片

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