黄玮;荆志成;
HUANG Wei;JING Zhi-cheng;The First Afilliated Hospital of Chongqing Medical University;Thrombosis and Vascular Medicine Center,Fuwai Hospital,Chinese Academy of Medical Sciences &Peking Union Medical College;Key Laboratory of Pulmonary Vascular Medicine,Chinese Academy of Medical Sciences State Key Laboratory of Cardiovascular Disease;

• 1:重庆医科大学附属第一医院心内科
• 2:中国医学科学院北京协和医学院阜外医院血栓性疾病诊治中心
• 3:中国医学科学院肺血管医学重点实验室心血管疾病国家重点实验室

摘要(Abstract)：

2018年2月28日至3月1日在法国尼斯举办了第六次世界肺高血压大会。第十一工作组(Task Force 11)对近5年慢性血栓栓塞性肺高血压(chronic thromboembolic pulmonary hypertension,CTEPH)在定义、分类、病理机制、流行病学、诊断和治疗方面的更新进行了总结,并提出了慢性血栓性疾病的新定义及其与CTEPH的区别。CTEPH的诊疗需要多学科综合评估,随着肺动脉球囊成形术的不断改进,其疗效和安全性明显改善,目前已成为CTEPH的重要治疗方式之一,但其与药物治疗的对比研究尚在进行中,联合治疗疗效仍需长期随访。
The 6th World Symposium on Pulmonary Hypertension was held in Nice between February 28 and March 1,2018. The major progress in chronic thromboembolic pulmonary hypertension( CTEPH) in the past 5 years was reviewed by the experts of Task Force 11. The definition,classification,pathologic mechanisms,epidemiology,diagnosis,and treatment were updated based on available evidence. Chronic thromboembolic disease( CTED) was defined and the difference between CTED and CTEPH was summarized. All CTEPH patients should be evaluated by a multidisciplinary team. Balloon pulmonary angioplasty( BPA),with improved efficacy and safety,plays an important role in CTEPH therapy. An ongoing study investigates patients with CTEPH who are not eligible for pulmonary endarterectomy and are randomized into medicine therapy or balloon pulmonary angioplasty( BPA),and compares the effects of the two therapies. The combination therapy for CTEPH needs the evidence of a long-term follow-up.

关键词(KeyWords)： 慢性血栓栓塞性肺高血压;慢性血栓性疾病;肺动脉球囊成形术;药物治疗;联合治疗
chronic thromboembolic pulmonary hypertension;chronic thromboembolic disease;balloon pulmonary angioplasty;medicine therapy;combination therapy

Abstract:

Keywords:

基金项目(Foundation):

作者(Authors): 黄玮;荆志成;
HUANG Wei;JING Zhi-cheng;The First Afilliated Hospital of Chongqing Medical University;Thrombosis and Vascular Medicine Center,Fuwai Hospital,Chinese Academy of Medical Sciences &Peking Union Medical College;Key Laboratory of Pulmonary Vascular Medicine,Chinese Academy of Medical Sciences State Key Laboratory of Cardiovascular Disease;

参考文献(References)：

• [1]Quarck R,Wynants M,Verbeken E,et al.Contribution of inflammation and impaired angiogenesis to the pathobiology ofchronic thromboembolic pulmonary hypertension[J].Eur Respir J,2015,46:431-443.
• [2]Konstantinides SV,Vicaut E,Danays T,et al.Impact of Thrombolytic Therapy on the Long-Term Outcome of Intermediate-Risk Pulmonary Embolism[J].J Am Coll Cardiol,2017,69:1536-1544.
• [3]Ende-Verhaar YM,Cannegieter SC,Vonk Noordegraaf A,et al.Incidence of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism:a contemporary view of the published literature[J].Eur Respir J,2017,49:1601792.
• [4]Gopalan D,Blanchard D,Auger WR.Diagnostic Evaluation of Chronic Thromboembolic Pulmonary Hypertension[J].Ann Am Thorac Soc,2016,13:S222-S239.
• [5]Delcroix M,Lang I,Pepke-Zaba J,et al.Long-Term Outcome of Patients with Chronic Thromboembolic Pulmonary Hypertension:Results from an International Prospective Registry[J].Circulation,2016,133:859-871.
• [6]Wiedenroth CB,Olsson KM,Guth S,et al.Balloon pulmonary angioplasty for inoperable patients with chronic thromboembolic disease[J].Pulm Circ,2018,8:2045893217753122.
• [7]Aoki T,Sugimura K,Tatebe S,et al.Comprehensive evaluation of the effectiveness and safety of balloon pulmonary angioplasty for inoperable chronic thrombo-embolic pulmonary hypertension:long-term effects and procedure-related complications[J].Eur Heart J,2017,38:3152-3159.
• [8]Hosokawa K,Abe K,Oi K,et al.Balloon pulmonary angioplasty-related complications and therapeutic strategy in patients with chronic thromboembolic pulmonary hypertension[J].Int J Cardiol,2015,197:224-226.
• [9]Simonneau G,D'Armini AM,Ghofrani HA,et al.Riociguat for the treatment of chronic thromboembolic pulmonary hypertension:a long-term extension study(CHEST-2)[J].Eur Respir J,2015,45:1293-1302.
• [10]Ghofrani HA,D'Armini AM,Grimminger F,et al.Study Group.Riociguat for the treatment of chronic thromboembolic pulmonary hypertension[J].N Engl J Med,2013,369:319-329.
• [11]Ghofrani HA,Simonneau G,D'Armini AM,et al.MERITstudy investigators.Macitentan for the treatment of inoperable chronic thromboembolic pulmonary hypertension(MERIT-1):results from the multicentre,phase 2,randomised,double-blind,placebo-controlled study[J].Lancet Respir Med,2017,5:785-794.