TAPVC-完全性肺静脉畸形引流课件.ppt

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1、TOTALANOMALOUSPULMONARYVENOUSCONNECTIONCJJORDAAN29/01/04Definition:Uncommoncongenitalcardiacdefect(1-3%)inwhichthereisnodirectcommunicationbetweenthepulmonaryvenousdrainageandtheLeftAtrium.Allthepulmonaryveinsconnect/draintotheRightatrium.Embryology:Tot

2、alanomalouspulmonaryvenousconnection(TAPVC)developswhentheprimordialpulmonaryveinfailstounitewiththeplexusofveinssurroundingthelungbuds.Thisresultsinreturnofpulmonaryvenousbloodtotheheartviaasystemicvein,andsubsequentlytotherightatrium.Classification:TA

3、PVCisclassifiedaccordingtothesiteofconnection:Supracardiac:includesconnectionstotheleftInnominatevein,theSVC,ortheAzygosCardiac:includesconnectionstothecoronarysinusordirectlytotherightatriumInfracardiac:includesconnectionsbelowthediaphragmtotheportalve

4、in,hepaticveins,ductusvenosusorIVC.Mixed:involvesconnectionsoftwoormoreofthesetypes;atleastoneofthemainlobarpulmonaryveinsisdrainingdifferentlyfromtheothersEachcategorycanbefurtherclassifiedasobstructiveornon-obstructive.1.SupracardiacTAPVC:Mostcommonfo

5、rmofTAPVC---45%Bilatdrainingofpulmonaryveins(PV)toCommonPulmonaryVenousSinus(CPVS).ThisconfluencedrainsviaVerticalVein(VV)toInnominatevein,SVCorAzygos.Stenosiscommon(+/-40-60%):Pulmonaryveinsystemicconnection,VascularviceorLongthinVV-CPVClocation…Suprac

6、ardiacTAPVC-Sitesofstenosis…2.CardiacTAPVC:Accountsfor25%ofTAPVCVVdrainsmostlytoCoronarySinus,seldomdirectlytoRightatrium.EnlargedcoronarysinusactsastheCPVSwithonlyathinwallofmyocardiumseparatingsinusandtheLeftatrium.Rightatrialdraining:SeeninRAisomeris

7、m.AssociatedwithabsentCoronarysinus,hugeorabsentintraatrialseptum.VVopensasafibrousmidlineconfluence.STENOSIS:Rare.MayoccurwheretheCPVSjoinstheCoronarysinus,oratthemouthofthecoronarysinus(persistingThebesianvalve)3.InfracardiacTAPVC:Accountsfor25%ofTAPV

8、CThecommonpulmonaryveindrainsthroughthediaphragmtotheportalvein,ductusvenosusorseldomtotheIVC.HasthegreatestpropensityforSTENOSIS:-MaybecompressedwhereitpenetratestheDiaphragm,-Connectingveinisnarrowedatitsjunctionwiththeportalve