标准与规范
ENGLISH ABSTRACT
心脏淀粉样变超声心动图规范化检查中国专家共识(2023版)
中国医师协会超声医师分会心脏超声专业委员会
作者及单位信息
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DOI: 10.3760/cma.j.cn131148-20230716-00010
Chinese expert consensus on standardized diagnosis of cardiac amyloidosis using echocardiography (2023 edition)
Echocardiography Committee of Chinese Ultrasound Doctor Association
Shu Xianhong
Authors Info & Affiliations
Echocardiography Committee of Chinese Ultrasound Doctor Association
Shu Xianhong
Department of Echocardiography, Zhongshan Hospital, Fudan University, Shanghai Institute of Cardiovascular Diseases, Shanghai Institute of Medical Imaging, Shanghai 200032, China
·
DOI: 10.3760/cma.j.cn131148-20230716-00010
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摘要

心脏淀粉样变(cardiac amyloidosis,CA)是一种浸润性限制型心肌病,由体内可溶性蛋白发生错误折叠形成不可溶的淀粉样蛋白纤维,沉积于心肌间质,导致心脏形态和功能异常。90%以上CA的致病蛋白前体为免疫球蛋白轻链或转甲状腺素蛋白,前者多由骨髓浆细胞合成,后者由肝细胞合成 [1,2]。CA致死率高,中位生存期少于5年,患者死亡的原因多为心源性。近年来有效药物的问世,使转甲状腺素蛋白心脏淀粉样变(transthyretin amyloid cardiac amyloidosis,ATTR-CA)患者的预后得到了显著改善,也意味着对CA的早期识别与诊断分型至关重要。然而,CA多系统性和异质性的临床表现以及起病隐匿的特点,导致临床漏诊率较高,治疗不及时 [3]。CA诊断的金标准是侵入性心内膜心肌活检,但目前多模态影像技术在CA诊断中的应用正处于迅速推广阶段,尤其是超声心动图,由于其简便、安全、无辐射、可以在床旁进行等独特的优点,已经成为CA影像学检查中不可或缺的工具之一。2020年起国内外多个心血管和影像学会基于CA的研究进展,发布了新版临床诊断指南 [4,5,6,7]。我国许多学者也已开展了CA的超声心动图诊断研究 [8,9,10,11,12,13,14],但目前国内缺乏统一的CA规范化超声检查方法。在此背景下,中国医师协会超声医师分会心脏超声专业委员会组织专家,在查阅国内外相关文献和指南、总结专家经验、结合临床应用现状和新技术进展的基础上,撰写了本共识,针对CA的超声心动图表现、主要参考指标、鉴别诊断等给出了具体建议,旨在进一步推进CA的超声规范化检查。

引用本文

中国医师协会超声医师分会心脏超声专业委员会. 心脏淀粉样变超声心动图规范化检查中国专家共识(2023版)[J]. 中华超声影像学杂志,2023,32(12):1021-1029.

DOI:10.3760/cma.j.cn131148-20230716-00010

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心脏淀粉样变(cardiac amyloidosis,CA)是一种浸润性限制型心肌病,由体内可溶性蛋白发生错误折叠形成不可溶的淀粉样蛋白纤维,沉积于心肌间质,导致心脏形态和功能异常。90%以上CA的致病蛋白前体为免疫球蛋白轻链或转甲状腺素蛋白,前者多由骨髓浆细胞合成,后者由肝细胞合成 [ 1 , 2 ]。CA致死率高,中位生存期少于5年,患者死亡的原因多为心源性。近年来有效药物的问世,使转甲状腺素蛋白心脏淀粉样变(transthyretin amyloid cardiac amyloidosis,ATTR-CA)患者的预后得到了显著改善,也意味着对CA的早期识别与诊断分型至关重要。然而,CA多系统性和异质性的临床表现以及起病隐匿的特点,导致临床漏诊率较高,治疗不及时 [ 3 ]。CA诊断的金标准是侵入性心内膜心肌活检,但目前多模态影像技术在CA诊断中的应用正处于迅速推广阶段,尤其是超声心动图,由于其简便、安全、无辐射、可以在床旁进行等独特的优点,已经成为CA影像学检查中不可或缺的工具之一。2020年起国内外多个心血管和影像学会基于CA的研究进展,发布了新版临床诊断指南 [ 4 , 5 , 6 , 7 ]。我国许多学者也已开展了CA的超声心动图诊断研究 [ 8 , 9 , 10 , 11 , 12 , 13 , 14 ],但目前国内缺乏统一的CA规范化超声检查方法。在此背景下,中国医师协会超声医师分会心脏超声专业委员会组织专家,在查阅国内外相关文献和指南、总结专家经验、结合临床应用现状和新技术进展的基础上,撰写了本共识,针对CA的超声心动图表现、主要参考指标、鉴别诊断等给出了具体建议,旨在进一步推进CA的超声规范化检查。
其中,本共识对检查方法推荐类别(class of recommendation,COR)的表述沿用国际通用的方式:
Ⅰ类指征(COR Ⅰ):指已证实和(或)一致公认有益、有用和有效的操作或治疗。
Ⅱ类指征(COR Ⅱ):指有用和(或)有效的证据尚有矛盾或存在不同观点的操作或治疗。
Ⅱa类指征(COR Ⅱa):有关证据/观点倾向于有用和(或)有效,应用这些操作或治疗是合理的。
Ⅱb类指征(COR Ⅱb):有关证据/观点尚不能被充分证明有用和(或)有效,可考虑应用。
Ⅲ类指征(COR Ⅲ):指已证实和(或)一致公认无用和(或)无效,并对一些病例可能有害的操作或治疗,不推荐使用。
对证据来源水平(level of evidence,LOE)表述如下:
证据水平A(LOE A):资料来源于多项随机临床试验或荟萃分析。
证据水平B(LOE B):资料来源于单项随机临床试验或多项非随机对照研究。
证据水平C(LOE C):仅为专家共识意见和(或)小型临床试验、回顾性研究或注册登记研究。
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备注信息
A
舒先红,复旦大学附属中山医院心脏超声诊断科 上海市心血管病研究所 上海市影像医学研究所,上海 200032, Email: nc.defhsab.latipsoh-szgnohnaix.uhs
B
所有作者均声明不存在利益冲突
C
国家自然科学基金 (82071933,82071991)
上海市医学创新研究专项项目 (20Y11912000)
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