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(99m technetium⁃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。
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 single⁃photon emission computed tomography(SPECT)has been widely used in the imaging examination of car⁃diac 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 con⁃tralateral 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.
作者
任超
田庄
何山
王雪竹
郝志鑫
丁洁
张抒扬
霍力
REN Chao;TIAN Zhuang;HE Shan;WANG Xuezhu;HAO Zhixin;DING Jie;ZHANG Shuyang;HUO Li(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;Department of Cardiology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Beijing 100730,China;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)
出处
《罕见病研究》
2022年第1期72-77,共6页
Journal of Rare Diseases
基金
中国医学科学院医学与健康科技创新工程项目(2021⁃I2M⁃1⁃003)。
