2023单纯门静脉再通术治疗非肝硬化性慢性肝外门静脉阻塞引起的重度门静脉高压.docx

2023单纯门静脉再通术治疗非肝硬化性慢性肝外门静脉阻塞引起的重度门静脉高压非肝硬化性慢性肝外门静脉阻塞(CNC-EHPVo)是一种罕见的肝脏疾病，有发生肝前性门静脉高压及其相关并发症的风险，包括胃肠道(GI)出血、门脉胆管病、腹痛、腹水、血栓蔓延或复发(Intagliatazetal.Gastroenterology.2019,156,1582-1599.e1;NoronhaFerreirazetal.Hepatology.2016z63z1640-1650;Hernandez-Geazetal.JournalofHepatology.2019z71z175-199;Khannazetal.JournalofHepatology.2014z60z421-441;Rodriguesfetal.AlimentaryPharmacologyandTheraPeUtics.2019,49,20-30)o同时，约40%的CNC-EHPVO患者伴有肌少症(Lattanzizetal.LiverInternational.2019z39z1937-1942)o肝硬化患者中，经颈静脉肝内门体分流术(TIPS)后门静脉高压的改善与肌少症的好转相关(Artruzetal.AlimentaryPharmacologyandTherapeutics.2020z52,1516-1526;Tsienzetal.EuropeanJournalofGastroenterologyandHepatology.2013z25,85-93;Gioiazetal.LiverInternational.2021,41,2965-2973)o在急性门静脉血栓患者中，门静脉再通术(PVR)会导致不良事件，这可能是因为在这种情况下常需联合局部或全身溶栓(VallazLiverInternational.2020z40(Suppl.1)z142-148;Hall,etal.WorldJournalofSurgery.2011l35,2510-2520;Thornburg,etal.JournalofVascularandInterventionalRadiology.2017,28z1714-1721.e2)o然而，鉴于CNC-EHPVO的血栓性质，不宜行溶栓治疗。有几项研究报道了肝硬化或非肝硬化患者行TIPS联合肝外门静脉阻塞再通术的良好结局，并证实了该方法的可行性(Kallini,etal.Hepatology.2016,63z1387-1390;Marotzetal.DiagnosticandInterventionalImaging.2019z100z147-156;Senzolo,etal.AlimentaryPharmacologyandTherapeutics.2006z23,767-775;Sarin,etal.Gastroenterology.2016z151,574-577.e3;Knightzetal.Hepatology.2021f74t2735-2744)o然而，对于无明显肝纤维化的窦性门静脉高压患者，辅助TIPS治疗可能是没必要的(Habib,etal.JournalofVascularandInterventionalRadiology.2015,26,499-506)oJHEPReports于2022年8月正式发表了一篇题为单纯门静脉再通术治疗非肝硬化性慢性肝外门静脉阻塞引起的重度门静脉高压的文章，该研究旨在评估CNC-EHPVO患者在未行TIPS的情况下进行PVR的长期预后，并确定PVR失败和支架闭塞的预测因素。ArtrU等纳入了2000年至2019年期间的31例接受了PVR而未行TIPS的CNC-EHPVo患者(平均年龄：50岁；中位随访时间：52个月)。主要操作步骤包括：经皮经肝穿刺进入门静脉，将导管插入肝内门静脉分支，使用0.035英寸J形刚性亲水性导丝再通阻塞血管，在阻塞段放置球囊和自扩张银钛诺支架将其扩张至6mm。术前、术后在肠系膜/脾静脉处测量门静脉压力，并行门静脉造影。当经肝途径不可行时，尝试经脾途径。结果显示，27例患者成功放置支架，6例发生不良事件，未发生死亡。在手术成功的患者中，5年首次通畅率为73%,其与1年后肌肉质量改善(P=O.007)和脾脏体积减小(P=O.01)相关。此外，21例(78%)PVR技术成功的患者在5年内未发生门静脉高压并发症。总的来说，对于既往发生过门静脉高压并发症或处于高风险的CNC-EHPVo三,仅行PVR是安全可行的。重要研究结果分析及其临床意义1.患者的主要基线特征Tablr1.MainClurMtcrfetkso(the11IMtkmgWbounderwentEjIWlkItkMlPrOCcdiltVforCNC-BIPVOwKhPHT.No.9t×CNC-EHPVOCMCIndkJtkmo(rrcjMiwtk>nDeUybetween(MagiwKandrccjuliMton(mooch)Mcordbig(0Sirtnet;,/Mjr(rtetM"typesExtmN>10mrwaterk/spknkveinsSuccmLPrimMy(Mten<y4PHTco<nplkJCk>nJfUrSuccrttlblrecauiiMt>nMSyeirs145.MAfterUvefSUfeRyRroirrcnCGlb/edingO>NoneYesYesNo246.MAflrrpMret«urg«yRHrjctoryjsctMdncnhjemrr!ugicFHT1nMesemencYesYesNo3b5,MChronicPanCratItbBeforeSUrgF2Meemen:andSPknKY«NoNo44B.MAfterPanmJtiCairg«yRecunencClbedingOnSplenicYesY«NoSb4S.FAfterPanmJtkMgcyRecurrencClbleeding1mMeemrrrandp)enYesY«Wst6。0MlOthramboticdisorder:FllAndFVcompositeKetaOzymmRecurrentClblredingJndchronicJbdOfnlIUIPjtn1283/2NoneYcsY«No7*».MChronicPAnCre*!kBeforeSUrIery25中MedrnCandlcnYcsYCNo8v50.MSuspectedumdmti6cdpro(KromboticdisorderBcfbrrUlrXefy873RMremrrrandSPknKY«YcsNo9lQ.FSuspectedUnidentiMpr<MKrombotdisorderRecurrentClbedinf19WMescntrncandSPknKYesYCNo10*».MNecroticMcrcafQsRecurrentGlbcdint2WMescmerrandsp)cnYesYesNo11»5B.MChronicfMt×rcMtBR>talChCUnfQathy83RMesenierrandSp)CnXYesNO¥n12*26.MRodiromboUcdsorde:aniphosptx>bpdsyndromeExirnsionofthrombosisunderm(×OdfuUnigjW493/2XtesentercandSPIenKYesYesNo13*6D.MChronicPtlnCreMNbRecurrentGlbedinc353MttemercJndsplenNaOWlnXtoMtfrjhepMicexIrrvaanNMWA14Q.FAfterpanmjtagFScwreClbleedingO1/1MeemerYesY«No1574MChronicponcreMitsIbrtalchdjnDpjthO3/2MeieniercYCYeNo1628.MAftercolonicsurgeryChromeAbdomnilpain73MesemencJndsplenicNaowing10HUrjhepjocexIrnaanNMWA!731.MOthramboncdisorder:antiphcptM)bpdsyrdrcmeChrome>bdmnjiMn23/3MnenterKYesNo18S3.MChrankpjncrejtitsChrantCJbdOmmapain3MnenlerYesNoWs1978.MAfterCDlonicsurgeryRecurrentGlbeding33/2MncnlrrcY«YeiNo2070.FThrombotkdisorder:ParaneopUsticTrousscausyndrome(ENToncercurativecmtrd4yearsbdoceGIblrcdn)SewreClbleeding481/1Hnemrri:YesYesNo213.MThrombotkdisorder:antIptaphobptdsyndromeChromcabdomnalpn151/1McscmerrYesNoWs2271.MAllerUVefSitffnyChrome4bdommpainandbcites151/2NoneNaowintontrjh11xexIensanN/AWA2321.FAfterPAncreaticUHgerySevereefMkMcopccPHT733/2MeientencJndIP)CfuCYesYesNo2457.MChronicPJnaeMItiISevereQbleeding1inMttenleri:JndSPknKYesYesNo2573.MChrcnicpancrejtHBBeforesurvey33/1MesemecandSPknKYesY«NO26?42.FSuspededUrudenliftedProthromboticdisorderSevereendoscopicPHT116311MttemencY«YesNo(conontMtfonntxragf)1.lble1(c<mnu<d)No.e.KXCNC-EHPVOCMCIndkMkmofIvcanalKitionDelaybetween<U<nosttandrecjfulution(month)CUssMcjtk>nJCCordinxcoSarinWMnMv<rt“WtypeExlcn¼0<1Iomesenteric/pnicwiatSuccess5-yearPrinMrypotency,PHTCoinpIkMionsJflersuc<rttklrrc4uIiwttoaMSyeirS2758.FIhromboticdisordermyrlopratifierjtivesynd<xncJK2*RecurrentabdomuulpainandchronicdnrrhN1933/2MeseniencandsplenicY«Y«No2831.F*11rombotcdisordermyek)prdtferMvesyndromeJK2-Chromeabdcmmalpain183/2MeiemercandspkmcNaowntoIntrAhepatiCextemicnN/AN/AO.MChrenicPjnCr3it.RrairrenlClblerdng91/1MrcntrrYesYCNoW23.Ma11womboticdtiorder:PMOtysmaInoaurndhamoglobmurucloneandMTHFRhomozygotuRc<urrntGlblrcdins1013/2McVCiMerKYesYCNo3C44.MThromboticdisorder:antithrombinddkiencyR>rtJChcMngiopalhyBO3/2MnenirrrYesNoYnChSSi6abonaccmdi:toSJrilIetat"is«follows:type1.onlytrank:KyPC2.onlybranches):type3.tnnkandbranch(clCIjssifkatKmMCDrdinStoMJrotetaLnisaslollowt:ype1.OCdittgIcTmCdtotheorg>natthrnumPOItJlveinJnaortothenghorWtpenalbrjnce:type2.typrIPiUSmtrnuontoth*or<>nofSfnIenmbtanches:type3.type2pbsCXtrnN8todistalbundleCKt<EWVQachr11Mcxwih<xc<xtahepbcpruJremobstrocton:F.Ieimle:Ci.$IIStrOirtStalM.uir:HfA.not4pf44biePHIPoIUlMpcrlrmion*PnmIryPItrneyWWdefvcdasKtvabsenceofcmetr0rntoazlUSOnonH)OWgcrossSeClXMUlUnJsInbP<imbin<ludinoutPmnDiB4uc11bsPaKm皿presented2earlynonscetrIVCKTmanofPHTrcUiedClbtec<krwithCXCrlirntDopplrrnd%noftheAoWconcocdatwiththeCT.sugsrs0nSHlPdenCyoftheMrnL研究纳入了31例仅行PVR的CNC-EHPVO患者；其中，男性23例、女性8例，平均年龄为50岁，年龄范围为2378岁。24例（77%）患者有内镜下门静脉高压表现，包括11例（35%）孤立性食管静脉曲张、1例（3%孤立性胃静脉曲张、7例（23%）食管胃静脉曲张和13例（42%）门脉高压性胃病。大多数患者CNC-EHPVO局限在肝内门静脉。根据Sarin等的门静脉血栓分类，14例（45%）患者为1型（血栓仅累及门静脉主干）。根据Marot等的门静脉血栓分类，16例（52%）患者为1型（血栓仅累及门静脉主干伴或不伴门静脉左支或右支）。29例（94%）患者门静脉主干完全闭塞。28例（90%）患者血栓蔓延至肠系膜上静脉，伴或不伴脾静脉血栓。术中门静脉造影证实所有患者均有门静脉海绵样变。2、再通过程UMe1UnIvartMeaiMlyUtoffadonauocMCrd*thtechnicalsucrrao(POrUiveinrccatuiiMtiaainPJtiCncIwithCNC<HPVOunderwentrrc4ulfcwtionprocedurebetween1Jinuwy20and31Dcccmbrr2019LTcctaiicJlMKrranJkMtion(n27)F<iueofEandhJtiCn(n4)Sjrinal,n(%)I4(52)1(25)0D72O(O)O(O)3B(48)3(75)MJrOtett,"n(X)16()O(O)0.0052»(37)2(50)3IW2(50)LtxcnsKMiwtnthenumu>stvamvmt.n(X)AtacncrofCXtenMOn2(7)(25)02SpknvetoJtonr(0)O(O)MnenIenCwmAkmeB(48)O(O)BothSPleIcJndmesentveins12(44)3(75)IenKIhoftheextensionwithm(heminYefftUatreGan3(1)4(1-5)05Upstreamextemk)ninUceulbc<vhes.n(X)AtcncedUtcultrancheOCdUUon»(37)(25)QIICr2lxcr*brarrhrtoccluded»(37)O(O)>2btrralbranchesocdudrd7(26)3(75)CompleteOCdUSion.n(X)26(96)3(75)aIndiatsondreoruhsMion.n(%)QNc<ng11(44)1(25)0D2Abdomirulpam4g3(75)Other11(«)ScrumJibumin.g/L36(26-38)36(3S-4t)Serumbhrub.IanOIA10(8-17)8(5-18)SeumAlP.Ml106(62-186)73(42-110)SumGGIMl56(21-219)55(29-116)S<runASIIllJ*wnAITIlfl29(22-38)23(2G-32)S<riMnCTrMlnine.moll69(9-8O)75(-92)FVachrofnbinrace,%8S(70-!)%(BS-100)HMmogIotan,g/LI12(96!23)124(95-162)IOHWBC»*l7(4-9)7(4-11)FtitcletecuntGJL225(10-338)325(I5S-J53)FailureofrrciHMcuck*nwmdeAnrdbythratarnccofP(Mtprocedure(tentOKincMiCn(bluredUreCInMVTiCInOCimvdiMcthranbottoftheacncdrtpitrmauldAjuiionXDatJJfeCpreSinmedu*n(IQIt)OcnumberjndPCTeenUge.CocnpjrtsombetweenPMIentSwithIcChnKdSUeEesSJnd(JUUreofrecJiwiiMiionWCTePeffOrmedUdntfeMjn-V¼tiA<vUuMfaquMitMwVMubkgCr(hrChi-fquMCtefofac<*xkilwUNrvPdybaMdrno<rubr<JMfn"r<LUk48PrOPoWdbySJttnetalvumfc>lkws:type1.Oclytnak:type2.onlybcjrh(es):type1tn*andbrxc(oCUs6cj>oeproposedbyMarotaat"SjSfotkws:cpeLOCdUnOnbmtcdtoeOdfin<rfthenumPOftJivemandtotf11t<OCWtpulbrjndnc«献2.type1PhBetmmio(hea*inofSepnentjlbrjncs:type).<>pe2plusnxeffikxtodiaalbrjnchr,AU*.VlMUnepoptMUirALT.aUnineMnaundcv*;CNMHFV0.thtMcntMwiakmraXPMko<ulwinOtanjaion：Cl.gMo>mttuLCCIs>nwnrluumyl(11mierjae;3CwhitebloodcdL27例(87%)患者技术成功；再通前，肠系膜上静脉/脾静脉与门静脉主干/分支之间的中位压力梯度为10mmHg(614mmHg),再通后为0mmHg(02mmHg)(P<0.0001)。6例(20%)患者发生了与手术相关的不良事件。在单因素分析中，与PVR手术失败相关的因素包括：根据Marot等的门静脉血栓分类评估为血栓蔓延至肝内门静脉(P=O.005);腹痛作为再通指征(P=O.02)o3、五年首次和二次通畅率E <95、Cl 539Z)3<S4S4¾7(WCt¾M>¾20htMn to H mtjM ¼r JTCtOCtf XWVM600900 INoo 1.5001,800Time (days)OL 0300Mnbon Io g. G Abt pcotol mM16009001.200Time (days)1,5001,8002222Hg.tRvc-yearprimaryandsecondarypatencyratesestimatedusingtheKaplan-Meiermethod.（八）5-yearprimarypatencyrate.(B)5-yearsecondarypatencyrate.SurvivalrateswereestimatedusingtheKJPkIn-MeiermethodinPerCenQgeand95%G.Primarypatencywasdefinedastheabsenceofacompletestemocclusiononfollow-upcross-sectionalimaging.Patientswithfailureofrecanalisationthatwasdefinedbytheabsenceofpost-procedurestentopacification(failuretostentinsertionorimmediatethrombosisofthestentdespitemaximaldilatation)wereexcludedinper-protocolanalyses.五年内4例PVR失败和6例支架完全闭塞。意向性治疗分析显示五年首次通畅率和二次通畅率分别为63%(45-82%)和66%(4885%)。依从性分析显示五年首次通畅率和二次通畅率分别为73%(5392%)和76%(5895%)o4、五年首次再通相关因素Tjble4.Univjrutraiuly«hofCjctortJitwciatedwithSyerprimarypotencyinIMtienUwithCNCEHPVOwhounderwenttrchnicallyMicceMfuIporUlveinrvc4fuiIsationProCedUrebetween1January20and31December2019.PatientwithHarprinurypatency(n21)Pj(ienuwithoutS-ycarPrifnrNteney(n6)pvalueSarin<t(1L,n(%)111(52)3(50)092O(O)O(O)310(48)3(50)Manxerof.1*n(X)113(62)3(50)0228(38)2(33)3O(O)1(17)Extensionwithinthemainupstreamvdnn(%)Absenceofextension2(10)O(O)05Spknicveinalone(O)O(O)Mescntcncveinalone9(43)4(67)BothsplenicandmesentericVetrtS10(47)2(33)Lengthoftheextensionwithintheinveinsupstream,n3(1-5)2(1-4)05Upstreamextensioninlateralbranden(X)AtxienceofHmlbranchesOerheion2(9)0(0)OS1or2lateralbrandiesoccluded13(62)5(83)>2UterAlbranchesOCdUdCd6(29)1(17)CompleteOCdUSion.n(X)20(95)6(100)05CNC旧CPVOreUtedtoIMOmbOHCdisorder,n(X)8(38)3(50)OfIndicationofrccanalisatk)n.n(X)GIbleeding12(57)O(O)Ooo9Abdominalpain1(5)3(50)Other8(38)3(50)DeljybetweendianossandrecarwIisatioamonth9(1-79)5(2-44)FeatureofPOnoSinUSoidalvascularliverdiseaseatb)sy.n(%)j3(60)3(100)SerumJRxJmin.g/L35(2"38)36(32-47)Serumbtlirb<n.molL10(7-16)13(9M0)SerumALRIUL%(62-169)BI(61-657)ScrumGGTIU/L39(1134)180(44-1j073)SerumAST.IU29(22-37)28(150)ScrumALT.IUL24(15-47)37(25-88)Scrumcreatinine,moll6A(Sg-90)69(676)Prothro>tnrate.%85(75-90)95(69-100)Iuemoglabin.g/Lt04(94-120)B2(116-144)TotalWBC.10L7(4-8)9(4-10)PUtekI>umC/L243(102-350)212(91-282)Long-termantkoagulant<11tiphtclcttreatmentNone2(»)1(17)ck<MguUttreatment15(7»)3(50)Antiphtelectreatment4(19)1(17)Bothan>c<MguUntJndAntipbteletIrUMmenHO(O)1(17)Primjrypatencyw<bdefinedJBtheJtnenCeof<completestrntocclusiononfollow-upcrosssectionalaragin¢.DJtImcxprasrdinmedian(IQR)ornumberJndPerCenUje.CompanionsbetweenpxntswithUCtmQsuccessandUflurcofrccarwltst)nwereperformedusingccMann-WhcncyUtestIbrqiuntKaovevanableortheGv-squaretestforceforiiVtfMbIcspvaluwinbolddcno(cstad33lSignifkancc.CUssifkahonproposedbySannftLnis»follows:type1.onlytrunk：type2.onlybranch(es):type3.trunkandbrnc(esCljssifkatiDnproposedbyMo(rti,gisJSfallows:typeLocclusionKmitedtotheoriginoftheminportalveinJn(VOrtotherightorleftportalbranches:type2.type1plusEOnMoncotheOrWinofSCgmenHbranches:type1type2plusextensionn)dbsulbranches.AlKallubncphohau*:AIT.JUnInCmino(rjnsferjse;CNC-EIIVO.chronicnon-drrhotacCXtQhepaticPoruiveinobstruction：CLgjstrointestinjkGGtglutamyltrjnsfere;VCwhitebloodceil.*Data4vabblefor9patients.在单因素分析中，与五年首次通畅率相关的因素包括：腹痛作为再通指征(P=O.009);和较高的血红蛋白水平(P=O.02)o5 .五年时门静脉高压并发症的结局31例意向性治疗分析的患者中有21例(68%)在五年时门静脉高压相关症状完全缓解。27例依从者分析的患者中有21例(78%)在五年时门静脉高压相关症状完全缓解。6 .以腹痛为PVR主要指征的患者分析TableS3.Baselinecharacteristicsofpatientsaccordingtotheirindication(otherindicationvs.abdominalpain)forportalveinrecanalisation(PVR)inthenlextofchronicnoncirrhoticextrahepaticporta)veinobstruction(CNC-EHPVO)betweenJanUary1s2000and31s,December2019.Patientwithotherindication(*24)Patientwithabdoniimtlpainasindication(N7)pvalueSarinetal,N(%)112(50)3(43)2O(O)O(O)0.2312(50)4(57)MarotetaL,N(%)I14(58)2(29)29(38)3(42)0.093(4)2(29)Extensionwithinthemainupstreamveins,N(%)Absenceofextension2(8)(4)Splenicveinalone(0)O(O)Mcscntcricveinalone10(42)3(43)0.9Bothsplenicandmesentericveins12(50)3(43)LengthoftheextensionWithintheminveinsupstream.Cm3(1-4)3(1-5)0.7LpstrcamextensioninlateralbranchesN(%)Absenceoflateralbranchesocclusion9(38)2(29)1or2lateralbranchesoccluded9(38)1(14)03>2lateralbranchesoccluded6(24)4(57)Completeocclusion.N(%)23(96)6(86)0.5CNC-EHPVOrelatedtothromboticdisorder.N(%)7(29)4(57)0.09Delaybetweendiagnosisandrecanalisation,month9(1-67)15(3-18)0.7Featureofporto-sinusodalvascularIherdiseaseatbiopsy、N(%)5(71)2(100)0.4Age,years52(44-61)31(28-58)0.08Femalr,N(%)6(25)2(29)0.9Scrumalhumin.g/L35(25-37)41(37-47)0.006Srrumbilihin,molL10(7-17)Il(5-17)0.6SerumALP,IUL106(62-197)74(55-126)0.3SerumGGT,IUL53(20-185)71(27-170)0.8ScrumAST,IUL28(21-37)27(20-37)0.5SerumALT,IUL26(16-48)27(20-69)0.3Scrumcreatinine,molL69(58-82)71(64-81)0.5Prothrombinrate,%85(73-97)90(85-110)0.3Hemoglobin,g/L105(89-121)125(123-147)0.004IotalWBcIOVL7(4-9)8(7-10)0.7Plateletcounts.G/L251(113-346)225(108-349)0.7Dataareexpressedinmedian(interquartilerange)ornumberandpercentageComparisonsbeturcenpatientswithsuccessfulandfailureOfrccanalisationwereperformedusingtheMann-Whitney