1、p左心疾病相關肺高血壓左心疾病相關肺高血壓l左心收縮功能不全l左心舒張功能不全l瓣膜疾病l先天性/獲得性左心室流入道/流出道梗阻和先天性心肌病pLHD是PH的常見病因之一，占全部PH的首位，約半數被超聲篩查出的PH為LHD相關PH。pPH也是LHD常見而又重要的合并癥之一。pPH是LHD患者預后重要的預測因素之一，合并PH的LHD患者預后不良。 流 行 病 學p心力衰竭l左心收縮功能不全（缺血性心肌病、擴張型心肌病等）l左心舒張功能不全（高血壓病、縮窄性心包炎、限制性心肌病等）p瓣膜病變l二尖瓣狹窄和關閉不全l主動脈瓣狹窄和關閉不全p先天性/獲得性左心室流入道/流出道狹窄 病 因p毛細血管后P

2、Hp以不同程度的肺淤血和肺水腫為主要特點p肺毛細血管楔壓增高(PCWP15 mm Hg) p“被動性PH” “不匹配的PH”p具有一定的可逆性與波動性p具體機制尚完全不清楚病理生理及可能機制不匹配的PH:根據跨肺壓（TPG）判定;提示肺高血壓的壓力不僅來自于肺靜脈，也來自肺動脈本身。ESC / ERS G U ID ELIN ESG ui del i nes f or the di agnosi s and treatm entofpul m onary hypertensi onT he T ask Force f or the D i agnosi s and T reatm ent of

3、Pul m onaryH ypertensi on ofthe European Soci ety ofC ardi ol ogy (ESC ) andthe European Respi ratory Soci ety (ERS),endorsed by theInternati onalSoci ety ofH eart and Lung T ranspl antati on (ISH LT )A uthors/ T ask Force M em bers:N azzareno G al i e (C hairperson) (Ital y)*;M ari us M .H oeper (G

4、 erm any);M arc H um bert (France);A damT orbi cki(Pol and);Jean-Luc V achi ery (France);Joan A l bert B arbera (Spai n);M auri ce Beghetti(Sw i tzerl and);PaulC orri s (U K );Sean G ai ne (Irel and);J.Si m on G i bbs (U K );M i guelA ngelG om ez-Sanchez (Spai n);G ui ll aum e Jondeau (France);W al

5、ter K l epetko (A ustri a)C hri sti an O pi tz (G erm any);A ndrew Peacock (U K );Lew i s Rubi n (U SA );M i chaelZel l w eger(Sw i tzerl and);G eral d Si m onneau (France)ESC C om m i ttee f or Practi ce G ui deli nes (C PG ):A l ec V ahani an (C hairperson) (France);A ngel o A uri cchio(Sw i tzerl

6、 and);Jeroen B ax (T he N etherlands);C l audio C econi(Ital y);V eroni ca D ean (France);G erasi m osFi l i ppatos(G reece);C hri sti an Funck-Brentano (France);Ri chard H obbs (U K );Peter K earney (Irel and);T heresa M cD onagh(U K );K ei th M cG regor (France);Bogdan A .Popescu (Rom ani a);Zel j

7、 ko Rei ner (C roati a);U do Sechtem (G erm any);Per A nton Si rnes (N orw ay);M i chalTendera (Pol and);Panos V ardas (G reece);Petr W i di m sky (C zech Republ i c)D ocum ent Revi ew ers:U do Sechtem(C PG Revi ew C oordi nator) (G erm any);N aw w ar A lA ttar (France);Fel i ci ta A ndreotti(Ital y

8、);M i chaelA scherm ann (C zech Republ i c);Ri ccardo A steggi ano (Ital y);Ray B enza (U SA );Rol fB erger (T he N etherl ands);D am i en Bonnet (France);M ari on D el croi x (Bel gi um );Luke H ow ard (U K );A nastasi a N K i tsi ou (G reece);Irene Lang (A ustri a);A l do M aggioni(Ital y);Jens Er

9、i k N i el sen-K udsk (D enm ark);M yung Park (U SA );Pasqual e Perrone-Fi l ardi(Ital y);Suzanna Pri ce (U K );M ari a Teresa Subi rana D om enech (Spai n);A nton V onk-N oordegraaf(T he N etherl ands);Jose Lui s Zam orano (Spai n)T he di scl osure f orm s ofal lthe authors and revi ew ers are avai

10、 l abl e on the ESC w ebsi te w w w . escardi o. org/ gui del i nesIM PO RT A N T N O T E:Si nce the ori gi nalpubl i cati on ofthese G ui del i nes,the drug si taxentan has been w i thdraw n f rom the m arketdue to l i ver toxi ci ty.Si taxentan w asw i thdraw n i n D ecem ber 2010 (f or f urther i

11、 nf orm ati on pl ease see EurH eartJ2011; 32: 386 387and on the ESC w ebsi te http: / / w w w . escardi o. org/ gui del i nes-surveys/esc-gui del i nes/ Pages/ pul m onary-arteri al -hypertensi on. aspx).T he i nstances w here si taxentan appears i n thi s docum ent have been hi ghl i ghted i n yel

12、 l ow .T abl e ofC ontentsAbbrevi ati ons and acronym s .2494Pream bl e .24951. I ntroducti on .24962. D efini ti ons .24973. Cl i ni calcl assi ficati on ofpul m onary hypertensi on .24984. Pathol ogy ofpul m onary hypertensi on .24995. Pathobi ol ogy ofpul m onary hypertensi on .24996. G eneti cs,

13、epi dem i ol ogy,and ri sk f actors ofpul m onaryhypertensi on .25007. Pul m onary arteri alhypertensi on (group 1) .25017. 1 D i agnosi s .25027. 1. 1 Cl i ni calpresentati on .25027. 1. 2 El ectrocardi ogram .25027. 1. 3 Chestradi ograph .2502*Correspondi ng author. I nsti tute of Cardi ol ogy, Bo

14、l ogna Uni versi t y H ospi t al , Vi a M assarenti , 9, 40138 Bol ogna, I tal y. Tel : 39 051 349 858, Fax: 39 051 344 859,Em ai l :nazzareno. gal i e uni bo. i tThe contentofthese European Soci etyofCardi ol ogy (ESC)G ui del i nes hasbeen publ i shed f orpersonaland educati onaluse onl y.N o com

15、m erci aluse i sauthori zed.N o partoftheESC G ui del i nesm aybe transl ated orreproduced i n any f orm w i thoutw ri tten perm i ssi on f rom the ESC.Perm i ssi on can be obtai ned upon subm i ssi on ofaw ri tten requestto O xf ordU ni versi ty Press,the publ i sher ofthe European HeartJ ournaland

16、 the party authori zed to handl e such perm i ssi ons on behal fofthe ESC.D i scl ai m er.The ESC G ui del i nes representthe vi ew s ofthe ESC and w ere arri ved ataf ter caref ulconsi derati on ofthe avai l abl e evi dence atthe ti m e they w ere w ri tten.H eal thprof essi onal s are encouraged t

17、o take them f ul l y i nto accountw hen exerci si ng thei rcl i ni calj udgem ent.The gui del i nesdo not,how ever,overri de the i ndi vi dualresponsi bi l i ty ofheal thprof essi onal s to m ake appropri ate deci si ons i n the ci rcum stances ofthe i ndi vi dualpati ents,i n consul tati on w i th

18、thatpati ent,and w here appropri ate and necessary the pati entsguardi an or carer.I ti s al so the heal th prof essi onal s responsi bi l i ty to veri f y the rul es and regul ati ons appl i cabl e to drugsand devi ces atthe ti m e ofprescri pti on.&The European Soci ety ofCardi ol ogy 2009.Al lri ghts reserved.For perm i ssi ons pl ease em ai l :j ournal s. perm i ssions oxf ordj ournal s. org.European H eartJ ournal(2009) 30,2493 2537doi : 10. 1093/ eurheartj / ehp297“被動性PH” “不匹配的PH”血流動力學特征為肺毛細血管楔壓增高(15 mm Hg)和肺血管阻力逐漸增強,后期可有跨肺壓升高。不同病因導致PH的發(fā)病率72-79左心衰患者存有不同程度的PH這些患者大多為左心室收縮功能不