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Chronic Thromboembolic Pulmonary Hypertension: Novel Opinion of The 6th World Symposium on Pulmonary Hypertension
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摘要: 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。
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关键词:
- 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
Abstract: 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.利益冲突 无 -
表 1 除CTEPH外的其他肺动脉阻塞性疾病分类
欧洲心脏协会/欧洲呼吸协会(2015) 第六次世界肺高血压大会(2018) 4.2.1 血管肉瘤 4.2.1 肉瘤(高度或中度)或者血管肉瘤 4.2.2 其他血管内肿瘤 4.2.2 其他恶性肿瘤 肾癌 子宫肿瘤 生殖细胞肿瘤 其他肿瘤 4.2.3 动脉炎 4.2.3 非恶性肿瘤 子宫肌瘤 4.2.4 先天性肺动脉狭窄 4.2.4 非结缔组织病的动脉炎 4.2.5 寄生虫(包虫病) 4.2.5 先天性肺动脉狭窄 4.2.6 寄生虫 包虫病 CTEPH：慢性血栓栓塞性肺高血压 
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表 2 CTEPH与CTED诊断比较
特点 CTEPH CTED ·症状 运动时呼吸困难 运动时呼吸困难 ·右心导管 mPAP≥25 mm Hg, PAWP≤15 mm Hg mPAP＜25 mm Hg(通常为21~24 mm Hg), PAWP≤15 mm Hg ·运动右心导管 - PQ斜率＞3 mm Hg/(L·min) ·通气/血流灌注扫描 通气/血流灌注不匹配 通气/血流灌注不匹配 ·血管造影 CTEPH的典型发现 CTEPH的典型发现 ·心肺功能运动试验 - 排除通气限制、缺少运动 ·经胸超声心动图 - 排除左心室心肌及瓣膜疾病 ·抗凝作用 至少3个月 至少3个月 mPAP：平均肺动脉压；PAWP：肺动脉楔压；CTED：慢性血栓栓塞性疾病；CTEPH：同表 1
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表 3 肺动脉血栓内膜剥脱术手术获益与风险分析
特点 低风险，可预测远期预后 高风险，不可预测远期预后 既往史 既往有深静脉血栓形成或肺栓塞 既往无深静脉血栓形成或肺栓塞 检查 无右心衰竭体征 有右心衰竭体征 伴随疾病 无 合并明确的肺部或左心疾病 心功能 心功能Ⅱ~Ⅲ级 心功能Ⅳ级 影像学 影像学表现与病变表现一致 影像学表现与病变表现不一致 疾病类型 双侧下叶病变 病变不位于下叶 血液动力学 PVR＜12.5 WU, 与影像学上闭塞的部位和数量成比例，肺动脉压高 PVR＞15 WU, 与影像学上闭塞的部位和数量不成比例, 肺动脉舒张压高 PVR：肺血管阻力
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