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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

任超, 田庄, 何山, 王雪竹, 郝志鑫, 丁洁, 张抒扬, 霍力. 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[J]. 罕见病研究, 2022, 1(1): 72-77. doi: 10.12376/j.issn.2097-0501.2022.01.012
引用本文: 任超, 田庄, 何山, 王雪竹, 郝志鑫, 丁洁, 张抒扬, 霍力. 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[J]. 罕见病研究, 2022, 1(1): 72-77. doi: 10.12376/j.issn.2097-0501.2022.01.012
REN Chao, TIAN Zhuang, HE Shan, WANG Xuezhu, HAO Zhixin, DING Jie, ZHANG Shuyang, HUO Li. Consensus to 99mTechnetium-Pyrophosphate Scintigraphy in the Diagnosis of Transthyretin-related Cardiac Amyloidosis[J]. Journal of Rare Diseases, 2022, 1(1): 72-77. doi: 10.12376/j.issn.2097-0501.2022.01.012
Citation: REN Chao, TIAN Zhuang, HE Shan, WANG Xuezhu, HAO Zhixin, DING Jie, ZHANG Shuyang, HUO Li. Consensus to 99mTechnetium-Pyrophosphate Scintigraphy in the Diagnosis of Transthyretin-related Cardiac Amyloidosis[J]. Journal of Rare Diseases, 2022, 1(1): 72-77. doi: 10.12376/j.issn.2097-0501.2022.01.012

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

doi: 10.12376/j.issn.2097-0501.2022.01.012
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

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

Funding: 

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

More Information
  • 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[99Tcm]焦磷酸盐(99mtechnetium-pyrophosphate, 99Tcm-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99Tcm-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。

     

  • 图  1  Asp38Asn型突变基因携带者(男,22岁)99Tcm-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

    图  2  Gly73Glu型ATTRv患者(女,37岁)99Tcm-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

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出版历程
  • 收稿日期:  2021-12-13
  • 修回日期:  2022-01-05

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