摘要
目的评价^99Tc^m焦膦酸盐(PYP)显像在心肌转甲状腺素型淀粉样变(ATTR)中的诊断价值。方法前瞻性纳入自2018年12月至2019年7月因疑诊心肌淀粉样变于北京协和医院行^99Tc^mPYP显像的17例[男9例,女8例,年龄(53.4±13.0)岁]患者,利用视觉评分及半定量方法[心脏/对侧比值(H/CL)],对照心肌或其他部位活组织检查(简称活检)结果及基因检测结果,判断该综合分析方法对心肌ATTR的诊断价值。结果17例患者中有5例视觉评分为2~3分,H/CL≥1.5,诊断为阳性,后续活检或基因检测为阳性,确诊为ATTR;4例患者基因检测为阳性但无心脏症状,视觉评分0~1分,H/CL<1.5,考虑患者年龄较轻,体内淀粉样物质沉积尚未引起脏器病变;另有8例显像阴性患者视觉评分为0~1分,H/CL<1.5,其中2例后续穿刺活检证实为轻链型淀粉样变(AL),3例临床诊断为AL,余3例基本除外ATTR。^99Tc^mPYP显像诊断ATTR心脏受累的准确性为11/11。结论^99Tc^mPYP显像有助于无创地诊断心肌ATTR。
Objective To evaluate the diagnostic value of^99Tc^mpyrophosphate(PYP)in transthyretin cardiac amyloidosis.Methods From December 2018 to July 2019,17 patients(9 males,8 females,age:(53.4±13.0)years)with suspected cardiac amyloidosis underwent^99Tc^mPYP imaging in Peking Union Medical College Hospital were prospectively included.Visual score and semi-quantitative values(heart to contralateral ratio,H/CL)of^99Tc^mPYP uptake were used to diagnose transthyretin amyloidosis(ATTR).Biopsies and genetic measurements were also developed to evaluate the diagnostic value of the imaging.Results Five of the 17 patients were diagnosed as ATTR with a visual score of 2-3,H/CL≥1.5,and confirmed with the biopsy or gene test.Four patients were diagnosed as ATTR with positive genetic results but no cardiac symptoms,and their visual scores were between 0 and 1 with H/CL<1.5.Considering the young age of the patients,amyloid deposition might have not yet caused visceral damage.Visual score of other 8 patients with negative^99Tc^mPYP imaging were also between 0 and 1 with H/CL<1.5,2 of 8 were confirmed with light chain amyloidosis(AL)by biopsy,3 were clinically diagnosed as AL and 3 were ATTR excluded.The accuracy of^99Tc^mPYP imaging for diagnosing ATTR was 11/11.Conclusion^99Tc^mPYP imaging is helpful for non-invasive diagnosis of transthyretin cardiac amyloidosis.
作者
任静芸
任超
杜延荣
何山
田庄
刘鹏
霍力
李方
张抒扬
Ren Jingyun;Ren Chao;Du Yanrong;He Shan;Tian Zhuang;Liu Peng;Huo Li;Li Fang;Zhang Shuyang(Department of Nuclear Medicine,Peking Union Medical College Hospital,Peking Union Medical College,Chinese Academy of Medical Sciences,Beijing Key Laboratory of Molecular Targeted Diagnosis and Therapy in Nuclear Medicine,Beijing 100730,China;Department of Cardiology,Peking Union Medical College Hospital,Peking Union Medical College,Chinese Academy of Medical Sciences,Beijing 100730,China)
出处
《中华核医学与分子影像杂志》
CAS
北大核心
2020年第10期577-582,共6页
Chinese Journal of Nuclear Medicine and Molecular Imaging
基金
国家重点研发计划(2016YFC0901500)
中国医学科学院医学与健康科技创新工程(2018-I2M-3-001)。
