TAPVC-完全性肺静脉畸形引流课件讲解学习.ppt

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1、TAPVC-完全性肺静脉畸形引流课件Embryology:Totalanomalouspulmonaryvenousconnection(TAPVC)developswhentheprimordialpulmonaryveinfailstounitewiththeplexusofveinssurroundingthelungbuds.Thisresultsinreturnofpulmonaryvenousbloodtotheheartviaasystemicvein,andsubsequentlytotherightatrium.Classification:TAPVCisclass

2、ifiedaccordingtothesiteofconnection:Supracardiac:includesconnectionstotheleftInnominatevein,theSVC,ortheAzygosCardiac:includesconnectionstothecoronarysinusordirectlytotherightatriumInfracardiac:includesconnectionsbelowthediaphragmtotheportalvein,hepaticveins,ductusvenosusorIVC.Mixed:involvescon

3、nectionsoftwoormoreofthesetypes;atleastoneofthemainlobarpulmonaryveinsisdrainingdifferentlyfromtheothersEachcategorycanbefurtherclassifiedasobstructiveornon-obstructive.1.SupracardiacTAPVC:MostcommonformofTAPVC---45%Bilatdrainingofpulmonaryveins(PV)toCommonPulmonaryVenousSinus(CPVS).Thisconflue

4、ncedrainsviaVerticalVein(VV)toInnominatevein,SVCorAzygos.Stenosiscommon(+/-40-60%):Pulmonaryveinsystemicconnection,VascularviceorLongthinVV-CPVClocation…SupracardiacTAPVC-Sitesofstenosis…2.CardiacTAPVC:Accountsfor25%ofTAPVCVVdrainsmostlytoCoronarySinus,seldomdirectlytoRightatrium.Enlargedcorona

5、rysinusactsastheCPVSwithonlyathinwallofmyocardiumseparatingsinusandtheLeftatrium.Rightatrialdraining:SeeninRAisomerism.AssociatedwithabsentCoronarysinus,hugeorabsentintraatrialseptum.VVopensasafibrousmidlineconfluence.STENOSIS:Rare.MayoccurwheretheCPVSjoinstheCoronarysinus,oratthemouthofthecoro

6、narysinus(persistingThebesianvalve)3.InfracardiacTAPVC:Accountsfor25%ofTAPVCThecommonpulmonaryveindrainsthroughthediaphragmtotheportalvein,ductusvenosusorseldomtotheIVC.HasthegreatestpropensityforSTENOSIS:-MaybecompressedwhereitpenetratestheDiaphragm,-Connectingveinisnarrowedatitsjunctionwithth

7、eportalvein,-VVhasathickenedwallwithintimalproliferation,-portalsinusoids(Liver)offeradditionalobstructiontovenousreturnInfracardiacTAPVC:4.MixedAPVCInvolvesacombinationofconnectionsoftwoormoreofthesubtypes(atleastoneofthemainloba