肺动脉高压病因 诊断及治疗进展word格式论文.docxVIP

英汉缩略语名词对照英文缩写英文全称中文全称PAHPulmonary arterialhypertensionIPAHIdiopathic PAH肺动脉高压 特发性肺动脉高 压FPAHFamilialPAH家族性肺动脉高 压APAHAssociatedPAH相关因素所致肺CTEPHPulmonary hypertension due to chronic thrombotic and/or embolic disease动脉高压慢性血栓和/或栓 塞性肺动脉高压PPHNPersistent pulmonary hypertension of newborn新生儿持续肺动脉高压PVODPulmonaryveno--occlusive disease肺静脉闭塞症 PCHPulmonary capillary hemangiomatosis肺毛细血管瘤 CMRcardiovascular mangetic resonance心血管磁共振成像HHTHereditarhemorrhagic telangiectasia遗传性出血性毛细血管扩张症PPARPeroxisome proli-ferator-activated receptor过氧化物酶增殖物激活受体1肺动脉高压病因、诊断及治疗进展摘要肺动脉高压(pulmonary arterial hypertension，PAH)是以肺动脉系统循环阻 力进行性增加为特征的一组疾病，可出现右心负荷增大、右心功能不全、肺血流 减少，进而引起一系列临床表现[17]。其病理变化包括肺血管收缩与重构、肺血管 平滑肌和内皮细胞的异常增殖、血栓形成等，最终结局导致右心功能衰竭而死亡。 目前将肺动脉高压大致分为：特发性肺动脉高压（idiopathic PAH,IPAH）、家族 性肺动脉高压（familial PAH，FPAH）、相关因素所致肺动脉高压（associated PAH ,APAH ）、肺动脉高压伴左心疾病（pulmonary hypertension with left heart disease ） 、 肺 疾 病 和 低 氧 血 症 相 关 的 肺 动 脉 高 压 （pulmonary hypertension associated with lung disease and hypox-emia）、以及慢性血栓和/或栓塞性 肺动脉高压（pulmonary hypertension due to chronic thrombotic and/or embolic disea，CTEPH）、新生儿持续肺动脉高压（persistent pulmonary hypertension of newborn，PPHN）。往往出现症状就诊时已经到达中重度肺动脉高压，治疗棘 手。因此提高认识、早期筛查、早期治疗尤为重要。关键词:肺动脉高压，肺动脉高压分类，肺动脉高压治疗，靶向药物2PULMONARY HYPERTENSION ETIOLOGY，DIAGNOSIS AND TREATMENT PROGRESSABSTRACTPulmonary arterial hypertension, PAH,is a group of diseasescharacterized by progressive increase in right heart load , right cardiacinsufficiency, decrease pulmonary blood flow , resulting in a series ofclinical manifestations. The pathological changes include pulmonaryvasoconstriction, reconstruction, abnormal pulmonary vascular smoothmuscle， endothelial cell proliferation and thrombosis。 These changesultimately result in right heart failure and death. Pulmonary hypertension isdivided into: idiopathic pulmonary arterial hypertension (IPAH), familialPAH(FPAH), factors causingpulmonary hypertension (associated PAH,APAH), pulmonary hypertension associated with left heart disease,pulmonary hypertensi