Hot Point Problems in Clinical Diagnosis, Treatment and Management of Pulmonary Hypertension: Comparison and Interpretation of 2022 ESC/ERS Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension and Chinese Guidelines for the Diagnosis and Treatment of Pulmonary Artery Hypertension (2021 Edition)

doi:10.12114/j.issn.1007-9572.2022.0692

Chinese General Practice ›› 2023, Vol. 26 ›› Issue (03): 255-261.DOI: 10.12114/j.issn.1007-9572.2022.0692

Special Issue: 指南/共识最新文章合集

• Guide·Consensus·Pathway • Next Articles

Hot Point Problems in Clinical Diagnosis, Treatment and Management of Pulmonary Hypertension: Comparison and Interpretation of 2022 ESC/ERS Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension and Chinese Guidelines for the Diagnosis and Treatment of Pulmonary Artery Hypertension (2021 Edition)

1.Department of Respiratory and Critical Care Medicine, Beijing Anzhen Hospital, Capital Medical University, Beijing 100029, China
2.Department of Pulmonary and Critical Care Medicine, Center of Respiratory Medicine, ChinaJapan Friendship Hospital, Beijing 100029, China

Received:2022-08-29 Revised:2022-09-22 Published:2023-01-20 Online:2022-09-30
Contact: ZHAI Zhenguo
About author:
WAN J, ZHAI Z G. Hot point problems in clinical diagnosis, treatment and management of pulmonary hypertension: comparison and interpretation of 2022 ESC/ERS Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension and Chinese Guidelines for the Diagnosis and Treatment of Pulmonary Artery Hypertension (2021 Edition) [J] . Chinese General Practice, 2023, 26 (3) : 255-261, 267.

肺动脉高压临床诊治和管理中需要关注的热点问题——基于《2022 ESC/ERS肺动脉高压诊治指南》与《中国肺动脉高压诊断与治疗指南（2021版）》的比较与解读

1．100029　北京市，首都医科大学附属北京安贞医院呼吸与危重症医学科
2．100029　北京市，中日友好医院呼吸中心呼吸与危重症医学科

通讯作者: 翟振国

作者简介:

万钧，医学博士，主任医师，副教授，硕士生导师，首都医科大学附属北京安贞医院呼吸与危重症医学科副主任、感染科执行副主任，长期从事呼吸与危重症医学专业，尤其擅长肺动脉高压、肺栓塞等疾病诊治。学术任职：中国医师协会呼吸医师分会第一届肺栓塞与肺血管病工作委员会委员；国家卫生健康委人才交流服务中心人才评价专家；《中华结核和呼吸杂志》第九届编辑委员会委员；北京整合医学会血栓与止血分会第一届委员；北京医学会呼吸病学分会第八届委员会肺血管病学组委员；北京医学会鉴定专家；北京市卫生健康委员会第三批健康科普专家。学术成果：中国《肺血栓栓塞症诊治与预防指南（2018版）》专家组与核心秘书组成员；《中国血栓性疾病防治指南》核心秘书组成员；《新型冠状病毒肺炎相关静脉血栓栓塞症防治建议（试行）》执笔专家；《中国肺动脉高压诊断与治疗指南（2021版）》秘书组组长、执笔专家组及统稿专家组成员；《右心漂浮导管检查操作流程专家共识》执笔专家组组长。

【本期执行主编】　翟振国，医学博士，主任医师，教授，博士生导师，博士后流动站导师，中日友好医院呼吸中心副主任、呼吸与危重症医学科副主任、呼吸实验研究部副主任、国家呼吸医学中心管理委员会研究办公室主任、肺栓塞与肺血管病团队学术带头人。学术任职：中华医学会呼吸病学分会肺栓塞与肺血管病学组副组长；中国医师协会呼吸医师分会肺栓塞与肺血管病工作委员会主任委员；国际肺血管病研究院（PVRI）委员；美国胸科医师学会（ACCP）资深委员；国际VTE研究联盟（INVENT组织）中国区域负责人。学术成果：主持国家重点研发计划精准医学专项，建设全国肺栓塞与肺血管病临床研究网络，开展肺栓塞、肺动脉高压临床表型、自然病程与组学研究，承担并推广全国肺栓塞和深静脉血栓形成防治能力建设项目。主持制订《肺血栓栓塞症诊治与预防指南》等多部指南。获得国家科技进步二等奖1项、中华医学科技二等奖1项。

万钧，翟振国.肺动脉高压临床诊治和管理中需要关注的热点问题——基于《2022 ESC/ERS肺动脉高压诊治指南》与《中国肺动脉高压诊断与治疗指南（2021版）》的比较与解读[J].中国全科医学，2023，26（3）：255-261，267.[www.chinagp.net] 作者贡献：万钧进行论文的构思与撰写；翟振国负责文章的质量控制及审校。

Abstract

Abstract:

The clinical and translational research of pulmonary hypertension (PH) has been continuously deepened, which has promoted the updating of PH related guidelines. On August 26, 2022, the European Society of Cardiology (ESC) and the Respiratory Society (ERS) jointly released the 2022 ESC/ERS Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension (hereinafter referred to as the "2022 ESC/ERS Guidelines") . In January 2021, Chinese Thoracic Society and Chinese Association of Chest Physicians jointly released the Chinese Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension (2021 Edition) (hereinafter referred to as the "Chinese Guidelines") , in which a lot of recommendations were put forward in line with China's national conditions combining with the research at home and abroad at that time. This paper will focus on the interpretation of the update points of the ESC/ERS Guidelines in combination with the relevant contents of the Chinese Guidelines.

Key words: Pulmonary arterial hypertension, Diagnosis, Therapy, Guidebook, Interpret, Disease management

摘要：

肺动脉高压（PH）的临床与转化医学研究不断深入，促使PH相关指南不断更新。2022-08-26欧洲心脏学会（ESC）与呼吸学会（ERS）联合发布了《2022 ESC/ERS肺动脉高压诊断与治疗指南》（简称2022 ESC/ERS指南），指南内容较前进行了大量更新。2021年1月中华医学会呼吸病学分会与中国医师协会呼吸医师分会联合发布了《中国肺动脉高压诊断与治疗指南（2021版）》（简称中国指南），结合当时的国内外研究提出了很多符合我国国情的推荐意见。本文将结合中国指南相关内容，就ESC/ERS指南的更新要点进行重点解读。

关键词: 肺动脉高压, 诊断, 治疗, 指南, 解读, 疾病管理

WAN Jun,ZHAI Zhenguo. Hot Point Problems in Clinical Diagnosis, Treatment and Management of Pulmonary Hypertension: Comparison and Interpretation of 2022 ESC/ERS Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension and Chinese Guidelines for the Diagnosis and Treatment of Pulmonary Artery Hypertension (2021 Edition)[J]. Chinese General Practice, 2023, 26(03): 255-261. DOI: 10.12114/j.issn.1007-9572.2022.0692.
万钧,翟振国. 肺动脉高压临床诊治和管理中需要关注的热点问题——基于《2022 ESC/ERS肺动脉高压诊治指南》与《中国肺动脉高压诊断与治疗指南（2021版）》的比较与解读[J]. 中国全科医学, 2023, 26(03): 255-261. DOI: 10.12114/j.issn.1007-9572.2022.0692.

Figures/Tables 9

References 19

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定义	血液动力学特征
PH	mPAP>20 mm Hg
毛细血管前PH	mPAP>20 mm Hg
	PAWP≤15 mm Hg
	PVR>2 WU
孤立性毛细血管后PH	mPAP>20 mm Hg
	PAWP>15 mm Hg
	PVR≤2 WU
毛细血管后和毛细血管前混合性PH	mPAP>20 mm Hg
	PAWP>15 mm Hg
	PVR>2 WU
ePH	静息和运动之间的mPAP/CO斜率>3 mm Hg·L^-1·min^-1

定义	血液动力学特征
PH	mPAP>20 mm Hg
毛细血管前PH	mPAP>20 mm Hg
	PAWP≤15 mm Hg
	PVR>2 WU
孤立性毛细血管后PH	mPAP>20 mm Hg
	PAWP>15 mm Hg
	PVR≤2 WU
毛细血管后和毛细血管前混合性PH	mPAP>20 mm Hg
	PAWP>15 mm Hg
	PVR>2 WU
ePH	静息和运动之间的mPAP/CO斜率>3 mm Hg·L^-1·min^-1

分类	亚类
1.动脉性肺动脉高压（PAH）	1.1特发性（IPAH） 1.1.1急性血管反应试验无反应者 1.1.2急性血管反应试验阳性者 1.2遗传性（HPAH） 1.3药物与毒物相关（DPAH） 1.4疾病相关 1.4.1结缔组织疾病 1.4.2 HIV感染 1.4.3门静脉高压 1.4.4先天性心脏病 1.4.5血吸虫病 1.5具有静脉/毛细血管（PVOD/PCH）受累特征的PAH 1.6新生儿持续性肺动脉高压（PPHN）
2.左心疾病相关性肺动脉高压	2.1心力衰竭 2.1.1射血分数保留的心力衰竭 2.1.2射血分数降低或轻度降低的心力衰竭 2.2瓣膜性心脏病 2.3导致毛细血管后肺动脉高压的先天性/获得性心血管病
3.肺部疾病和/或缺氧相关性肺动脉高压	3.1阻塞性肺疾病或肺气肿 3.2限制性肺疾病 3.3限制性/阻塞性并存的肺疾病 3.4低通气综合征 3.5非肺部疾病导致的低氧血症（如高海拔） 3.6肺发育障碍性疾病
4.肺动脉阻塞相关肺动脉高压	4.1慢性血栓栓塞性肺动脉高压（CTEPH） 4.2其他肺动脉阻塞性疾病
5.未明和/或多因素相关肺动脉高压	5.1血液系统疾病 5.2系统性疾病 5.3代谢性疾病 5.4伴或不伴血液透析的慢性肾衰竭 5.5肺肿瘤血栓性微血管病 5.6纤维性纵隔炎

分类	亚类
1.动脉性肺动脉高压（PAH）	1.1特发性（IPAH） 1.1.1急性血管反应试验无反应者 1.1.2急性血管反应试验阳性者 1.2遗传性（HPAH） 1.3药物与毒物相关（DPAH） 1.4疾病相关 1.4.1结缔组织疾病 1.4.2 HIV感染 1.4.3门静脉高压 1.4.4先天性心脏病 1.4.5血吸虫病 1.5具有静脉/毛细血管（PVOD/PCH）受累特征的PAH 1.6新生儿持续性肺动脉高压（PPHN）
2.左心疾病相关性肺动脉高压	2.1心力衰竭 2.1.1射血分数保留的心力衰竭 2.1.2射血分数降低或轻度降低的心力衰竭 2.2瓣膜性心脏病 2.3导致毛细血管后肺动脉高压的先天性/获得性心血管病
3.肺部疾病和/或缺氧相关性肺动脉高压	3.1阻塞性肺疾病或肺气肿 3.2限制性肺疾病 3.3限制性/阻塞性并存的肺疾病 3.4低通气综合征 3.5非肺部疾病导致的低氧血症（如高海拔） 3.6肺发育障碍性疾病
4.肺动脉阻塞相关肺动脉高压	4.1慢性血栓栓塞性肺动脉高压（CTEPH） 4.2其他肺动脉阻塞性疾病
5.未明和/或多因素相关肺动脉高压	5.1血液系统疾病 5.2系统性疾病 5.3代谢性疾病 5.4伴或不伴血液透析的慢性肾衰竭 5.5肺肿瘤血栓性微血管病 5.6纤维性纵隔炎

预后因素	低危	中危	高危
A：WHO功能分级	Ⅰ~Ⅱ	Ⅲ	Ⅳ
B：6MWD	>440 m	165~440 m	<165 m
C：血浆NT-proBNP/BNP水平或RAP	BNP<50 ng/L NT-proBNP<300 ng/L或RAP<8 mm Hg	BNP 50~300 ng/L NT-proBNP 300~1 400 ng/L或RAP 8~14 mm Hg	BNP>300 ng/L NT-proBNP>1 400 ng/L或RAP>14 mm Hg
D：CI或SvO₂	CI≥2.5 L·min^-1·（m²）^-1或SvO₂>65%	CI 2.0~2.4 L·min^-1·（m²）^-1或SvO₂ 60%~65%	CI<2.0 L·min^-1·（m²）^-1或SvO₂<60%

肺动脉高压临床诊治和管理中需要关注的热点问题——基于《2022 ESC/ERS肺动脉高压诊治指南》与《中国肺动脉高压诊断与治疗指南（2021版）》的比较与解读

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预后评估（1年死亡率）	低危<5%	中危5%~20%	高危>20%
右心衰竭的临床表现	无	无	有
症状进展	无	慢	快
晕厥	无	偶尔晕厥	反复晕厥
WHO功能分级	Ⅰ、Ⅱ	Ⅲ	Ⅳ
6MWD	>440 m	165~440 m	<165 m
心肺运动试验	最大氧耗量>15 ml·min^-1·kg^-1（>65%预计值）二氧化碳通气当量斜率<36	最大氧耗量11~15 ml·min^-1·kg^-1（35%~65%预计值）二氧化碳通气当量斜率36~44	最大氧耗量<11 ml·min^-1·kg^-1（<35%预计值）二氧化碳通气当量斜率>44
血浆BNP或血浆NT-proBNP	BNP<50 ng/L NT-proBNP<300 ng/L	BNP 50~800 ng/L NT-proBNP 300~1 100 ng/L	BNP>800 ng/L NT-proBNP>1 100 ng/L
超声心动图	右心房面积<18 cm² TAPSE/sPAP >0.32 mm/mm Hg 无心包积液	右心房面积18~26 cm² TAPSE/sPAP 0.19~0.32 mm/mm Hg 少量心包积液	右心房面积>26 cm² TAPSE/sPAP<0.19 mm/mm Hg 中等或大量心包积液
cMRI	RVEF>54% SVI>40 ml/m² RVESVI<42 ml/m²	RVEF 37%~54% SVI 26~40 ml/m² RVESVI 42~54 ml/m²	RVEF<37% SVI<26 ml/m² RVESVI>54 ml/m²
血流动力学	RAP<8 mm Hg CI≥2.5 L·min^-1·（m²）^-1 SVI>38 ml/m² SvO₂>65%	RAP 8~14 mm Hg CI 2.0~2.4 L·min^-1·（m²）^-1 SVI 31~38 ml/m² SvO₂ 60%~65%	RAP>14 mm Hg CI<2.0 L·min^-1·（m²）^-1 SVI<31 ml/m² SvO₂<60%

预后因素	低危	中低危	中高危	高危
赋分值	1分	2分	3分	4分
WHO功能分级	Ⅰ、Ⅱ	—	Ⅲ	Ⅳ
6MWD	>440 m	320~440 m	165~319 m	<165 m
血浆BNP或血浆NT-proBNP	<50 ng/L <300 ng/L	50~199 ng/L 300~649 ng/L	200~800 ng/L 650~1 100 ng/L	>800 ng/L >1 100 ng/L

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