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：① 动脉性肺动脉高压；② 左心疾病所致肺动脉高压；③ 肺部疾病和(或)低氧所致肺动脉高压；④ 慢性血栓栓塞性肺动脉高压；⑤ 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)

⑴ 骨形成蛋白受体2(bone morphogenetic protein receptor type 2 ，BMPR2)

⑵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)]

⑶ 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)

⑴ 结缔组织疾病(connective tissue diseases)

⑵ HIV 感染(human immunodeficiency virus infection)

⑶ 门静脉高压(portal hypertension)

⑷ 先天性心脏病(congenital heart disease)

⑸ 血吸虫病(schistosomiasis)

⑹ 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 mecha ni sms)

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(pul mon ary Lan gerha ns cell histiocytosis)，淋巴管平滑肌瘤病(lympha ngioleiomyomatosis)，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、CTEP H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mPAP》25mmHg ,肺毛细血管楔压(pulmonary capillary wedge pressure,PCWP)或左心室舒张末压v 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mPAP》25mmHg,PCWP 或左心室舒张末压〉5mmHg。肺动脉高压的严重程度可根据静息状态下mPAP水平分为“轻”(26~35mmHg )、“中” (36~45mmHg )、“重”(> 45mmHg )三度。