TAPVC-完全性肺静脉畸形引流课件1

TAPVC-完全性肺静脉畸形引流课件1第一页，共38页。Definition:

Uncommoncongenitalcardiacdefect(1-3%)inwhichthereisnodirectcommunicationbetweenthepulmonaryvenousdrainageandtheLeftAtrium.Allthepulmonaryveinsconnect/draintotheRightatrium.第二页，共38页。Embryology:Totalanomalouspulmonaryvenousconnection(TAPVC)developswhentheprimordialpulmonaryveinfailstounitewiththeplexusofveinssurroundingthelungbuds.Thisresultsinreturnofpulmonaryvenousbloodtotheheartviaasystemicvein,andsubsequentlytotherightatrium.第三页，共38页。Classification:TAPVCisclassifiedaccordingtothesiteofconnection:Supracardiac:includesconnectionstotheleftInnominatevein,theSVC,ortheAzygosCardiac:

includesconnectionstothecoronarysinusordirectlytotherightatriumInfracardiac:includesconnectionsbelowthediaphragmtotheportalvein,hepaticveins,ductusvenosusorIVC.Mixed:involvesconnectionsoftwoormoreofthesetypes;atleastoneofthemainlobarpulmonaryveinsisdrainingdifferentlyfromtheothersEachcategorycanbefurtherclassifiedasobstructiveornon-obstructive.第四页，共38页。1.SupracardiacTAPVC:MostcommonformofTAPVC---45%Bilatdrainingofpulmonaryveins(PV)toCommonPulmonaryVenousSinus(CPVS).ThisconfluencedrainsviaVerticalVein(VV)toInnominatevein,SVCorAzygos.Stenosiscommon(+/-40-60%): Pulmonaryveinsystemicconnection, Vascularviceor LongthinVV第五页，共38页。

-CPVClocation…

SupracardiacTAPVC-Sitesofstenosis…

第六页，共38页。2.CardiacTAPVC:Accountsfor25%ofTAPVCVVdrainsmostlytoCoronarySinus,seldomdirectlytoRightatrium.EnlargedcoronarysinusactsastheCPVSwithonlyathinwallofmyocardiumseparatingsinusandtheLeftatrium.Rightatrialdraining:SeeninRAisomerism.AssociatedwithabsentCoronarysinus,hugeorabsentintraatrialseptum.VVopensasafibrousmidlineconfluence.STENOSIS:Rare.MayoccurwheretheCPVSjoinstheCoronarysinus,oratthemouthofthecoronarysinus(persistingThebesianvalve)第七页，共38页。第八页，共38页。3.InfracardiacTAPVC:Accountsfor25%ofTAPVCThecommonpulmonaryveindrainsthroughthediaphragmtotheportalvein,ductusvenosusorseldomtotheIVC.HasthegreatestpropensityforSTENOSIS: -MaybecompressedwhereitpenetratestheDiaphragm, -Connectingveinisnarrowedatitsjunctionwiththeportalvein, -VVhasathickenedwallwithintimalproliferation, -portalsinusoids(Liver)offeradditionalobstructiontovenousreturn第九页，共38页。InfracardiacTAPVC:第十页，共38页。4.MixedAPVCInvolvesacombinationofconnectionsoftwoormoreofthesubtypes(atleastoneofthemainlobarpulmonaryveinsisdrainingdifferentlyfromtheothers.)第十一页，共38页。AnatomyRA:Enlargedandthickwalled.Decreasedcompliance.LA:Volume53%lessthanpredicted.LAauricleisnormalinsize,decreaseinLAcanbeexplainedbytheabsenceofthepulmonaryveincomponent.ASD:ASDorPFOmustexistforsurvival.Usuallyofadequatesizeandnotobstructive.ObstructionleadstoadecreasedRtoLshuntwithpulmonaryvenousobstructionandPulm.Hypertension.Presentsasaseverelysickneonate.第十二页，共38页。AnatomyLV:Normalinsize,wallthicknessandmass,butdecreasedLVcavity(duetoleftwarddisplacementofseptumsecondarytorightventriclepressure-volumeoverload.)RV:Variesinsize,dependsonmagnitudeofpulmonarybloodflow,pulmonaryvenousstenosis,pointofPVconnection.(Infracardiacconnection-RVnotdilatedorhypertrophied)PA:Mostinfantshavemarkedpulmonaryhypertension.StructuralchangesareusuallyfoundinthelungsevenintheyoungestinfantsdyingofTAPVC.Increaseinpulm.Arterialmuscularity-increaseinwallthicknessandextensionofmuscleintosmallerandperipheralarteries.Veinwallthicknessisalsoincreased.第十三页，共38页。Pathophysiology:Allpulmonaryvenousbloodreturnstotherightatrium.(commonmixingchamber)Aright-to-leftshuntattheatriallevel(-RVcompl,ASDsize,Rp)Increasedpulmonarybloodflowandpulmonaryvenousobstructionwilleventuallyresultinpulmonaryhypertension.Infradiaphragmaticdraining….第十四页，共38页。Pathophysiology:Pulmonaryveinstenosis:Obstructiontopulmonarydrainingwithincreaseinpulm.venouspressure.Capillaryleakwithinterstitialedema.Reflexpulm.vasoconstrictionandprogressiveincreaseinRp=pulmonaryhypertension.IncreasedPAPleadstoincreasedRVpressures(sometimessuprasystemic)withRVfailure,decreasedpulm.bloodflow,decreasedQp:Qs,decreasedsystemicSAT,peripheralhypoxiaandmetabolicacidosiswithmultiorganfailure.PFOobstruction:IncreasedLAP,impedespulm.venousreturn,producingpulm.hypertension.第十五页，共38页。Burchellprinciple:Adirectrelationshipexistsbetweenthemagnitudeofpulm.bloodflowandsystemicsaturation.TheQp:Qsisdeterminedbymagnitudeofpulmonarybloodflow,pulm.bloodflowisinverselyrelatedtoRp.ThusanincreaseinPAPandRpleadstoadecreaseinpulmbloodflowwithadecreasedsystemicSAT.SATlessthan80%:Qp:Qslikelytobe<1.4andRp>10!!!!!!!第十六页，共38页。Clinicalfeatures:Clinicalfeaturesaredeterminedbythedegreeofpulmonaryvenousobstruction.Ifobstructionissevere,infantwillbecriticallyillwithtachypnea,hypoxemia,andmetabolicacidosis.Cyanosiscanbeunimpressivetosevere,thisisasurgicalemergency.Prognosis:50%diewithin3weeks,75%at5weeksand100%at8-12weeksNoobstruction:Notsocriticallyill.Maypresentlaterwithpulmonaryhypertension,cardiomegaly,largepulmbloodflow.Patientssurvivingfirstyearoflifepresentwithfailuretothrive,severegrowthretardation,mildcyanosis.StablehemodynamicstatewithprogressivepulmonaryhypertensionanddevelopmentofEisenmengerintheirtwenties.

第十七页，共38页。Diagnosis:CXR:Showsgroundglass(diffusealveolarpattern)or"snowman"appearancefrompersistentverticalvein,smallheart=pulmstenosis.Nostenosis:Largeheartwithincreasedpulmbloodflow.

第十八页，共38页。Thereiscardiomegalywithincreasedpulmonaryarterialmarkings.Thereisdilationofboththeleftandrightinnominateveinsandtherightsuperiorvenacavaproducingtheclassical"snowman"or"figureof8"appearance.Thesuperiormediastinumisenlargedsecondarytodilationoftherightvenacava,innominateveinandascendingverticalvein.第十九页，共38页。Neonate–nostenosisNeonatewithstenosis第二十页，共38页。Diagnosis:Echo:Diagnosticinmostinfants.Evaluationofcardiacchambers,EchofreespaceposteriortoLAwithabnormaldrainage第二十一页，共38页。Diagnosis:Angiography第二十二页，共38页。Diagnosis:MRIangiogram:第二十三页，共38页。TREATMENT:PatientswithTAPVCshouldundergooperativerepairwhenthediagnosisismadeObstructedTAPVCisasurgicalemergencyNon-obstructedTAPVCshouldhavepromptrepairaswell,astheclinicalstatusofthesepatientscandeterioraterapidlyEarlyrepairofnon-obstructedTAPVCalsopreventstheadversesquealofcyanosisandvolumeoverloadoftheheartandlungs第二十四页，共38页。Treatment:Preoperatively:AdmitinPICUIntubationandventilationTolazoline,PGE1IVIStabilize,correctmetabolicacidosis,confirmdiagnosisPrepareforemergenttheatre.第二十五页，共38页。OperativeTechniqueCardiopulmonarybypasswithhypothermiccirculatoryarrestisthepreferredapproachincriticallyillinfantsSurgicalgoalsare:eliminatingallanomalousconnections,drainingthepulmonaryveinsintotheleftatrium,andclosingintracardiacshunts,smallPFOisleftforRVdecompressionThePV-LAanastomosismustbelargeandundistorted.Problems:Smallaorta

HypothermicCPBresponse第二十六页，共38页。LeftSVCconnection

·Ligateleft-sidedverticalveinatjunctionwithInnominatevein

·Openleftatriumandincisetheposteriorwall

·Findandincisetheanteriorwalloftheconfluence

·Anastomosethepulmonaryvenousconfluencetotheleftatrium

·ClosePFOorASDthroughleftatriumorthroughseparaterightatrialincisionRightSVCconnection

·Exposepulmonaryvenousconfluenceandanastomosetoleftatriumasabove

·LigateanomalousconnectionsorpatchfromwithinSVC

·AbafflemaybeusedinsteadtochannelflowfromtherightSVCthroughanenlargedinteratrialconnectionAzygosconnection

·Ligateanomalousconnection

·Anastomoseconfluencetoleftatriumasabove第二十七页，共38页。第二十八页，共38页。2.CardiacTypeCoronarysinusconnectionClassicrepair:

·Createcommonlargeinteratrialconnectionbyincisingcoronarysinusseptumandseptumprimum

·Closethisnewdefectwithasinglepatch;allpulmonaryvenousreturnandcoronarysinusreturnnowdrainsintotheleftatrium

VanPraagRightatriumconnection

·Enlargeinteratrialconnection

·Createbaffletodirectflowfrompulmonaryvenousopeningacrossinteratrialconnection第二十九页，共38页。3.InfracardiacType·LigatePDAonceCPBisestablished

·Identifyandligateanomalousdescendingverticalveinatthediaphragm

·Initiatecirculatoryarrest

·Openleftatriumandincisetheposteriorwall

·Findandincisetheanteriorwalloftheconfluence

·Anastomosethepulmonaryvenousconfluencetotheleftatrium

·Closetheinteratrialcommunication第三十页，共38页。4.MixedTypeProblemswithMixedType:

·Nopulmonaryvenousconfluence

·Requires2ormoreanastomosis

·Smalleranastomosispredisposetopulmonaryvenousobstruction

·Riskofmortalityandlatepulmonaryvenousobstructionisincreased

·Operativerepairprobablynotcurative第三十一页，共38页。Postoperative:Pulmonaryhypertensivecrisis….Sedation,ventilation,FentanylanalgesiaPco2(<30mmHg)andhighPO2TNT,PGE1,Dobutamine,Tolazoline,Adrenalineetc…..Results

·Early(hospital)mortalityrangesfrom2-20%

·90%ofpatientsarecured

·5-10%havelatefailureofrepairduetopulmonaryvenousobstructionLAsizeadequateduetoincorporationofPVCintoLA

第三十二页，共38页。Postoperative:RiskFactors:

·Poorpre-operativestatus(acidosis)

·Pulmonaryvenousobstruction,smallPFO·Highpulmonaryvascularresistance,preoperativeSAT<80%

·Youngage

·Smallleftventricle

·Majorassociatedanomalies

·Infracardiacormixedtype第三十三页，共38页。Literature:Bu’LockFAetal.Balloondilatationofveinstenosisinobstructed

TAPVC.PedCardio1998;15:78

Balloonatrialseptostomy:Riskfactorfordeath,notanamelioratingfactor.BalloondilatationofleftverticalveininobstructivesupracardiacTAPVCmayprovideimportantclinicalimprovementandshorttermhemodinamicstabilitybeforeoperationKiziltepeUetal.AcutepulmhypertensivecrisisafterTAPVCrepair…treatment.InternatJofCardio2002:87;107-109Treatmentofrecurrentseverepulmhypertensionrefractivetomedicaltreatmentcanbedealtwithinflowocclusionandcreationoflargeintraatrialconnection.Chestleftopenpostoperatively.

第三十四页，共38页。PostoperativePVstenosis:

StarkJ,DeLevalMR.ManagementofpulmvenousobstructionafterTAPVCrepair.EurJCardio-ThoracSurg.2003:24:28-36Complicatessurgeryin5-10%ClassificationofPVstenosis: