肺动脉高压的分类

肺动脉高压的分类2008年世界卫生组织(WHO)第四届肺动脉高压会议重新修订了肺动脉高压的分类，共分为5大类：动脉性肺动脉高压；左心疾病所致肺动脉高压；肺部疾病和（或）低氧所致肺动脉高压；慢性血栓栓塞性肺动脉高压；未明多因素机制所致肺动脉高压。该分类对于制订患者的治疗方案具有重要的指导意义。一、动脉性肺动脉高压(pulmonary arterial hypertension，PAH )1.特发性(idiopathic)2.遗传性(heritable)骨形成蛋白受体2(bone morphogenetic protein receptor type 2，BMPR2)激活素受体样激酶1(activin receptor-like kinase type 1，ALK1)，内皮因子（伴或不伴遗传性出血性毛细血管扩张症）endoglin (with or without hereditary hemorrhagic telangiectasia)未知遗传因素(unknown)3.药物所致和毒物所致肺动脉高压(drug-and toxin-induced)4.疾病相关性肺动脉高压(associated with)结缔组织疾病(connective tissue diseases)HIV感染(human immunodeficiency virus infection)门静脉高压(portal hypertension)先天性心脏病(congenital heart disease)血吸虫病(schistosomiasis)慢性溶血性贫血(chronic hemolytic anemia)5.新生儿持续性肺动脉高压(persistent pulmonary hypertension of the newborn)肺静脉闭塞病和（或）肺毛细血管瘤样增生症pulmonary veno-occlusive disease (PVOD) and (or) pulmonary capillary hemangiomatosis(PCH) 二、左心疾病所致肺动脉高压（pulmonary hypertension owing to left heart disease）1.收缩性心功能不全（systolic dysfunction）2.舒张性心功能不全（diastolic dysfunction）3.心脏瓣膜病（valvular heart disease）三、肺部疾病和（或）低氧所致肺动脉高压(pulmonary hypertension owing to lung disease and/or hypoxia)1.慢性阻塞性肺疾病(chronic obstructive pulmonary disease)2.间质性肺疾病(interstitial lung disease)3.其他限制性与阻塞性通气障碍并存的肺部疾病（other pulmonary diseases with mixed restrictive and obstructive pattern）4.睡眠呼吸障碍（sleep-disordered breathing）5.肺泡低通气(alveolar hypoventilation disorders)6.长期居住高原环境(chronic exposure to high altitude)7.肺发育异常(developmental abnormalities)四、慢性血栓栓塞性肺动脉高压（chronic thromboembolic pulmonary hypertension，CTEPH）五、未明多因素机制所致肺动脉高压（pulmonary hypertension with unclear multifactorial mechanisms）1.血液系统疾病（hematologic disorders）：骨髓增生异常（myeloproliferative disorder），脾切除（splenectomy）2.系统性疾病（systemic disorders）：结节病（sarcoidosis），肺郎汉斯细胞组织细胞增多(pulmonary Langerhans cell histiocytosis)，淋巴管平滑肌瘤病(lymphangioleiomyomatosis)，神经纤维瘤neurofibromatosis，血管炎(vasculitis)3.代谢性疾病(metabolic disorders：糖原贮积症(glycogen storage disease，戈谢病(Gaucher disease)，甲状腺疾病(thyroid disorders)4.其他（others）：肿瘤阻塞（tumoral obstruction），纤维素性纵隔炎（fibrosing mediastinitis），接受透析治疗的慢性肾功能不全（chronic renal failure on dialysis） 动脉性肺动脉高压、肺部疾病或低氧所致的肺动脉高压、CTEPH及未明多因素机制所致肺动脉高压都属于毛细血管前性肺动脉高压，血流动力学特征mPAP25mmHg，肺毛细血管楔压（pulmonary capillary wedge pressure,PCWP）或左心室舒张末压15mmHg。左心疾病所致肺动脉高压属于毛细血管后性肺动脉高压，血流动力学特征mPAP25mmHg,PCWP或左心室舒张末压5mmHg。肺动脉