BB01.0
动脉性肺动脉高压
Pulmonary arterial hypertension
- 上级条目
- BB01 肺动脉高压
- 层级
- 4 级
定义
肺动脉高压是一种临床症状，其特点是在没有其他原因的情况下出现毛细血管前肺动脉高压，如由于肺部疾病、慢性血栓栓塞性肺动脉高压或其他罕见疾病。它包括不同形式，具有相似的临床表现和几乎相同的肺部微循环的病理变化。
Pulmonary arterial hypertension is a clinical condition characterised by the presence of pre-capillary pulmonary hypertension in the absence of other causes of pre-capillary pulmonary hypertension, such as due to lung diseases, chronic thromboembolic pulmonary hypertension, or other rare diseases. It includes different forms that share a similar clinical picture and virtually identical pathological changes of the lung microcirculation.
包含词 该编码包含的临床情况，通常不作为独立编码
- 原发性肺高压
- 特发性肺动脉高压
英文词条（2）
- primary pulmonary hypertension
- Idiopathic pulmonary arterial hypertension
索引词 可用于检索该条目的同义词与别名
- 动脉性肺动脉高压
- Arrillaga Ayerza综合征
- 原发性肺高压
- PAH[肺动脉高压]
- 动脉性PH
- 特发性肺动脉高压
- 家族性肺动脉高压
- 遗传性肺动脉高压
- BMPR2基因突变引起的家族性肺动脉高压
- ALK1或Endoglin基因突变引起的家族性肺动脉高压
- 原因不明的家族性肺动脉高压
- 药物或毒素所致肺动脉高压
- 与其他情况有关的肺动脉高压
- APAH[相关性肺动脉高压]
- APAH[相关性肺动脉高压]
- 结缔组织病相关性肺动脉高压
- HIV感染相关性肺动脉高压
- 门静脉高压相关性肺动脉高压
- 门脉高压相关性肺动脉高压
- 先天性心脏病相关性肺动脉高压
- 先天性心脏病合并肺动脉高压
- 先天性心脏病并肺动脉高压
- 艾森曼格综合征
- Eisenmenger综合征
- 艾森门格综合征
- 先天性体肺分流相关性肺动脉高压
- 血吸虫病相关性肺动脉高压
- 其他病因相关性肺动脉高压
- 肺静脉闭塞病
- 肺毛细血管瘤病
英文词条（23）
- Pulmonary arterial hypertension
- Arrillaga Ayerza syndrome
- PAH - [pulmonary arterial hypertension]
- primary pulmonary hypertension
- Idiopathic pulmonary arterial hypertension
- Familial pulmonary arterial hypertension
- heritable pulmonary arterial hypertension
- Familial pulmonary arterial hypertension due to BMPR2 mutations
- Familial pulmonary arterial hypertension due to ALK1 or endoglin mutations
- Familial pulmonary arterial hypertension of unknown origin
- Pulmonary arterial hypertension induced by drugs or toxins
- Pulmonary arterial hypertension associated with other conditions
- APAH - [Associated pulmonary arterial hypertension]
- Pulmonary arterial hypertension associated with connective tissue diseases
- Pulmonary arterial hypertension associated with HIV infection
- Pulmonary arterial hypertension associated with portal hypertension
- Pulmonary arterial hypertension associated with congenital heart disease
- Eisenmenger syndrome
- Pulmonary arterial hypertension associated with congenital systemic-to-pulmonary shunt
- Pulmonary arterial hypertension associated with schistosomiasis
- Pulmonary arterial hypertension associated with other aetiologies
- Pulmonary veno-occlusive disease
- Pulmonary capillary haemangiomatosis
后组配轴 Postcoordination
后组配是在主干编码之后追加的可选扩展轴（如侧别、具体解剖部位、严重程度），用于把「部位 + 侧别 + 病程」等细节一次表达完整。只有标注「必填」的轴在组合编码时必须给出，其余可留空。
- 相关疾患associatedWith可选
- 严重程度hasSeverity可选