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1、英文縮寫PAHIPAHFPAHAPAHCTEPHPPHNPVODPCHCMRHHTPPAR中文全稱肺動脈高壓特發(fā)性肺動脈高壓家族性肺動脈高壓相關(guān)因素所致肺動脈高壓慢性血栓和/或栓塞性肺動脈高壓新生兒持續(xù)肺動脈高壓肺靜脈閉塞癥肺毛細血管瘤心血管磁共振成像遺傳性出血性毛細血管擴張癥過氧化物酶增殖英漢縮略語名詞對照英文全稱PulmonaryarterialhypertensionIdiopathicPAHFamilialPAHAssociatedPAHPulmonaryhypertensionduetochronicthromboticand/orembolicdiseasePer
2、sistentpulmonaryhypertensionofnewbornPulmonaryveno—occlusivediseasePulmonarycapillaiyhemangiomatosiscardiovascularmangeticresonaneeHereditarhemorrhagictelangiectasiaPeroxisomeproli-ferator-activatedreceptor物激活受體肺動脈高壓病因、診斷及治療進展摘要肺動脈高壓(pulmonaryarterialhypertension,PAH)是以肺動脈系統(tǒng)循環(huán)阻力進行性增加為特征的一組疾
3、病，可出現(xiàn)右心負荷增大、右心功能不全、肺血流減少，進而引起一系列臨床表現(xiàn)陽。其病理變化包括肺血管收縮與重構(gòu)、肺血管平滑肌和內(nèi)皮細胞的異常增殖、血栓形成等，最終結(jié)局導(dǎo)致右心功能衰竭而死亡。目前將肺動脈高壓大致分為:特發(fā)性肺動脈高壓(idiopathicPAH,TPAH)、家族性肺動脈高壓(fam訂ialPAU,FPAII)、相關(guān)因素所致肺動脈高壓(associatedPAI1,APAI1)、肺動脈高壓伴左心疾病(pulmonaryhypertensionwithleftheartdisease)、肺疾病和低氧血癥相關(guān)的肺動脈高壓(pulmonaryhypertensionass
4、ociatedwithlungdiseaseandhypox-emia)、以及慢性血栓和/或栓塞性月市動脈高壓(pulmonaryhypertensionduetochronicthromboticand/orembolicdisea,CTEPH)、新生兒持續(xù)肺動脈高壓(persistentpulmonaryhypertensionofnewborn,PPHN)。往往出現(xiàn)癥狀就診時已經(jīng)到達中重度肺動脈高壓，治療棘手。因此提高認識、早期篩查、早期治療尤為重要。關(guān)鍵詞:肺動脈高壓，肺動脈高壓分類，肺動脈高壓治療，靶向藥物PULMONARYHYPERTENSIONETIOLOGYD
5、IAGNOSISANDTREATMENTPROGRESSABSTRACTPulmonaryarterialhypertension,PAH,isagroupofdiseasescharacterizedbyprogressiveincreaseinrightheartload,rightcardiacinsufficiency,decreasepulmonarybloodflow,resultinginaseriesofclinicalmanifestations.Thepathologicalchangesincludepulmonaryvasoconstriction,r
6、econstruction,abnormalpulmonaryvascularsmoothmuscle,endothelialcellproliferationandthrombosisoThesechangesultimatelyresultinrightheartfailureanddeath?Pulmonaryhypertensionisdividedinto:idiopathicpulmonaryarterialhypertension(IPAH),familialPAH(FPAH),factorscausingpulmonaryhypertension(associ
7、atedPAH,APAH),pulmonaryhypertensionassociatedwithleftheartdisease,pulmonaryhypertensionwithleftheartdiseases),lungdiseaseandhypoxemiapulmonaryhypertension,pulmonaryhypertensionassociatedwithlungdiseasesandhypoxemia),andchronicthrombosisand/orembolismpulm
