文档介绍：肺动脉高压
（ Pulmonary hypertension， PH）
诊断标准：
For an adult at sea level, pulmonary hypertension is said to exist when the mean pulmonary arterial pressure is greater than 25 mm Hg at rest or greater than 30 mm Hg during exercise. However, this level, which would represent a modest increase for adults at sea level, is normal for adults at high altitude. ；肺毛细血管楔压（PAWP）＜15mmHg。
严重程度分级：
轻（26-35mmHg）；中（36-45mmHg）；重（＞45mmHg）。
概述
PH的分类（2009修订）
1、Pulmonary arterial hypertension
2、Pulmonary hypertension owing to left heart disease
3、Pulmonary hypertension owing to lung diseases and/or hypoxia
4、Chronic thromboembolic pulmonary hypertension
5、Pulmonary hypertension with unclear multifactorial mechanisms
分类
机制
Pulmonary vasoconstriction
Normal
Normal
Pulmonary vascular remodeling
PAH
PAH
特发性肺动脉高压
（idiopathic pulmonary hypertension,IPH)
是一种不明原因的肺动脉高压。
病理上表现为动脉中层肥厚、向心或偏心性内膜增生及丛状损害和坏死性动脉炎等构成。
特发性肺动脉高压
特发性肺动脉高压病因迄今不明，可能：
1、遗传因素Genetics
Mutations in two receptors of the transforming growth factor-β family, bone morphogenetic protein receptor type II (BMPR-II) and activin receptor–like kinase type 1 (ALK-1), have been identified in patients with familial pulmonary arterial hypertension 。
2、免疫因素
抗核抗体水平明显升高，但缺乏结缔组织病的特异性抗体。
3、肺血管内皮功能障碍
Imbalances in the production or metabolism of vasoactive mediators of pulmonary vascular tone include increased thromboxane and endothelin and decreased prostacyclin and nitric oxide.
病因和发病机制
4、血管壁平滑肌细胞钾离子通道缺陷
IPH患者存在电压依赖性钾离子通道（Kv）功能缺陷，K离子外流减少，Ca离子进入细胞，使血管处于收缩状态。
病因和发病机制
With mild pulmonary hypertension, the earliest complaints are often fatigue and vague chest discomfort.
1、呼吸困难dyspnea on exertion ：心脏排血↓，V/Q比例失调。
2、胸痛atypical chest pain ：右心负荷↑，耗氧↑，冠脉供血↓。
3、头晕或晕厥syncope ：脑组织供血↓。
4、咯血hemoptysis ：通常较少。
临床表现
临床表现
体征：
肺动脉高压和右心功能不全有关。
Each underlying or associated condition affects the clinical findings. For example, COPD is usually associated with hyperinflation of the lungs. With interstitial lung disease, tachypnea inva