可分为五大类,具体见下图:
表1肺动脉高压Danapoint分类
Tab.1Danapointclassificationofpulmonaryhypertension
1.动脉性肺动脉高压(Pulmonaryarterialhypertension,PAH)
1.1特发性PAH(IdiopathicPAH,IPAH)
1.2遗传性PAH(HeritablePAH)
1.2.1BMPR2
1.2.2ALK1,Endoglin
1.2.3未知原因(Unknown)
1.3药物和毒素所致(Drugandtoxininduced)
1.4相关(Associatedwith)
1.4.1胶原组织疾病(Collagetissuediseases)
1.4.2HIV感染(HIVinfection)
1.4.3门脉高压性(Portalhypertension)
1.4.4先天性心脏病(Congenitalheartdisease)
1.4.5血吸虫病(Schistosomiasis)
1.4.6慢性溶血性贫血(Chronichaemolyticanameia)
1.5和静脉或毛细血管相关(Associatedwithsignificantvenousorcapillaryinvolvement)
1.5.1肺静脉闭塞疾病(Pulmonaryveno-occlusiondisease)
1.5.2肺毛细血管血管瘤(Pulmonarycapillaryhemangiomatosis)
1.6新生儿持续性肺动脉高压(Persistentpulmonaryhypertensionofthenewborn)
2.左心功能不全继发的PH(静脉性肺动脉高压)(Pulmonaryhypertensionwithleftheartdisease)
2.1左心收缩功能不全(HFrEF,leftventricularsystolicdysfunctionheartfailurewithreducedEF)
2.2左心舒张功能不全(HFpEF,leftventriculardiastolicdysfunctionheartfailurewithpreservedEF)
2.3左心瓣膜性心脏病(left-sidedvalvularheartdisease)
3.肺疾病/低氧血症相关性肺动脉高压(pulmonaryhypertensionassociatedwithlungdiseaseorhypoxemiaorboth)
3.1慢性阻塞性肺疾病(Chronicobtrusivepulmonarydisease)
3.2间质性肺疾病(Interstitiallungdisease)
3.3限制性或阻塞形式的其他肺疾病(Otherpulmonarydiseasewithmixedrestrictiveandobstructivepattern)
3.4呼吸睡眠障碍(Sleepdisorderedbreathing)
3.5肺泡低通气障碍(Alveolarhypoventilationdisorders)
3.6慢性高原反应(Chronicexposuretohighaltitude)
3.7发育异常(Developmentalabnormalities)
4.慢性血栓或(和)栓塞性肺动脉高压(Pulmonaryhypertensionduetochronicthromboticorembolicdiseaseorboth,CTEPH)
5.其他不明原因多因素的肺动脉高压(Pulmonaryhypertensionwithunclearmultifactorialmechanisms)
5.1血液病(Hematologicdisorders)
5.2系统性疾病(Systemicdisorders)
5.3代谢性疾病(Metabolicdisorders)
5.4其他(Others)
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