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《肺动脉高压的分类.doc》由会员上传分享,免费在线阅读,更多相关内容在行业资料-天天文库。
1、肺动脉高压的分类2008年世界卫生组织(WHO)第四届肺动脉高压会议重新修订了肺动脉高压的分类,共分为5大类:①动脉性肺动脉高压;②左心疾病所致肺动脉高压;③肺部疾病和(或)低氧所致肺动脉高压;④慢性血栓栓塞性肺动脉高压;⑤未明多因素机制所致肺动脉高压。该分类对于制订患者的治疗方案具有重要的指导意义。一、动脉性肺动脉高压(pulmonaryarterialhypertension,PAH)1.特发性(idiopathic)2.遗传性(heritable)⑴骨形成蛋白受体2(bonemorphogeneticproteinrece
2、ptortype2,BMPR2)⑵激活素受体样激酶1(activinreceptor-likekinasetype1,ALK1),内皮因子(伴或不伴遗传性出血性毛细血管扩张症)[endoglin(withorwithouthereditaryhemorrhagictelangiectasia)]⑶未知遗传因素(unknown)3.药物所致和毒物所致肺动脉高压(drug-andtoxin-induced)4.疾病相关性肺动脉高压(associatedwith)⑴结缔组织疾病(connectivetissuediseases)⑵HI
3、V感染(humanimmunodeficiencyvirusinfection)⑶门静脉高压(portalhypertension)⑷先天性心脏病(congenitalheartdisease)⑸血吸虫病(schistosomiasis)⑹慢性溶血性贫血(chronichemolyticanemia)5.新生儿持续性肺动脉高压(persistentpulmonaryhypertensionofthenewborn)肺静脉闭塞病和(或)肺毛细血管瘤样增生症[pulmonaryveno-occlusivedisease(PVOD)a
4、nd(or)pulmonarycapillaryhemangiomatosis(PCH)]二、左心疾病所致肺动脉高压(pulmonaryhypertensionowingtoleftheartdisease)1.收缩性心功能不全(systolicdysfunction)2.舒张性心功能不全(diastolicdysfunction)3.心脏瓣膜病(valvularheartdisease)三、肺部疾病和(或)低氧所致肺动脉高压(pulmonaryhypertensionowingtolungdiseaseand/orhypoxi
5、a)1.慢性阻塞性肺疾病(chronicobstructivepulmonarydisease)2.间质性肺疾病(interstitiallungdisease)3.其他限制性与阻塞性通气障碍并存的肺部疾病(otherpulmonarydiseaseswithmixedrestrictiveandobstructivepattern)4.睡眠呼吸障碍(sleep-disorderedbreathing)5.肺泡低通气(alveolarhypoventilationdisorders)6.长期居住高原环境(chronicexpos
6、uretohighaltitude)7.肺发育异常(developmentalabnormalities)四、慢性血栓栓塞性肺动脉高压(chronicthromboembolicpulmonaryhypertension,CTEPH)五、未明多因素机制所致肺动脉高压(pulmonaryhypertensionwithunclearmultifactorialmechanisms)1.血液系统疾病(hematologicdisorders):骨髓增生异常(myeloproliferativedisorder),脾切除(splene
7、ctomy)2.系统性疾病(systemicdisorders):结节病(sarcoidosis),肺郎汉斯细胞组织细胞增多(pulmonaryLangerhanscellhistiocytosis),淋巴管平滑肌瘤病(lymphangioleiomyomatosis),神经纤维瘤neurofibromatosis,血管炎(vasculitis)3.代谢性疾病(metabolicdisorders:糖原贮积症(glycogenstoragedisease,戈谢病(Gaucherdisease),甲状腺疾病(thyroiddiso
8、rders)4.其他(others):肿瘤阻塞(tumoralobstruction),纤维素性纵隔炎(fibrosingmediastinitis),接受透析治疗的慢性肾功能不全(chronicrenalfailureondialysis)动脉性肺动脉高压、肺
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