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The 6th World Symposium on Pulmonary Hypertension: Focus on Updates on Definition and Clinical Classification of Pulmonary Hypertension
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摘要: 第六届世界肺高血压会议对肺高血压(pulmonary hypertension, PH)的定义和诊断分类进行了修订和更新。PH的血流动力学定义维持不变, 将毛细血管前性PH定义更新为:肺动脉平均压＞20 mm Hg、肺动脉楔压 < 15 mm Hg且肺血管阻力> 3 WU。诊断分类更新包括:增加急性肺血管扩张试验阳性肺动脉高压(pulmonary arterial hypertension, PAH)亚类, 将甲基苯丙胺(冰毒)和达沙替尼致PAH作用由可能升级为肯定, 将肺静脉闭塞病/肺毛细血管瘤病由特殊的1'更新为PAH的一个亚类, 将脾切除术后和甲状腺疾病从PH诊断分类中移除等。本次PH定义和诊断分类更新将为临床实践提供重要指导意义。Abstract: The definition and clinical classification of pulmonary hypertension (PH) were updated in the 6th World Symposium on Pulmonary Hypertension (WSPH). The hemodynamic definition of PH keeps no change, but pre-capillary PH was redefined as mean pulmonary arterial pressure>20 mm Hg, pulmonary artery wedge pressure < 15 mm Hg and pulmonary vessel resistance>3 WU. The updated clinical classification included:pulmonary arterial hypertension(PAH) with vasoreactivity was added as a subclass of PAH; in drugs and toxins induced PAH, the role of methamphetamines and dasatinib was updated from likely to definite; pulmonary veno-occlusive disease/pulmonary capillary haemangiomatosis was changed from 1' to a subclass of PAH; splenectomy and thyroid diseases were withdrawn from the group 5 of PH, etc. These clinical practice updates will guide clinical practice better.
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表 1 第六届世界肺高血压会议肺高血压临床诊断分类
条目 1 PAH 1.1 特发性 1.2 急性肺血管扩张试验阳性 1.3 遗传性 1.4 药物和毒物相关性 1.5 相关因素 1.5.1 结缔组织病 1.5.2 HIV感染 1.5.3 门静脉高压 1.5.4 先天性心脏病 1.5.5 血吸虫病 1.6 肺静脉闭塞病/肺毛细血管瘤病 1.7 新生儿持续性 2 左心疾病导致的PH 2.1 射血分数保留的心力衰竭 2.2 射血分数降低的心力衰竭 2.3 心脏瓣膜病 2.4 先天性毛细血管后阻塞性病变 3 肺病和/或缺氧导致的PH 3.1 阻塞性肺疾病 3.2 限制性肺疾病 3.3 其他混合性限制/阻塞性肺疾病 3.4 缺氧但不并发肺疾病 3.5 肺发育异常性疾病 4 肺动脉阻塞导致的PH 4.1 慢性血栓栓塞性肺高血压 4.2 其他肺动脉阻塞性病变 5 未知因素导致的PH 5.1 溶血性疾病 5.2 系统性疾病 5.3 其他 5.4 复杂先天性心脏病 PAH:肺动脉高压; PH:肺高血压 
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