最新TAPVC-完全性肺静脉畸形引流课件(1)ppt课件.ppt

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1、TAPVC-完全性肺静脉畸形引流课件(1)Definition:Uncommoncongenitalcardiacdefect(1-3%)inwhichthereisnodirectcommunicationbetweenthepulmonaryvenousdrainageandtheLeftAtrium.Allthepulmonaryveinsconnect/draintotheRightatrium.Embryology:Totalanomalouspulmonaryvenousconnection(TAPVC)developswhentheprimordialpulmona

2、ryveinfailstounitewiththeplexusofveinssurroundingthelungbuds.Thisresultsinreturnofpulmonaryvenousbloodtotheheartviaasystemicvein,andsubsequentlytotherightatrium.2.CardiacTAPVC:Accountsfor25%ofTAPVCVVdrainsmostlytoCoronarySinus,seldomdirectlytoRightatrium.EnlargedcoronarysinusactsastheCPVSwith

3、onlyathinwallofmyocardiumseparatingsinusandtheLeftatrium.Rightatrialdraining:SeeninRAisomerism.AssociatedwithabsentCoronarysinus,hugeorabsentintraatrialseptum.VVopensasafibrousmidlineconfluence.STENOSIS:Rare.MayoccurwheretheCPVSjoinstheCoronarysinus,oratthemouthofthecoronarysinus(persistingTh

4、ebesianvalve)3.InfracardiacTAPVC:Accountsfor25%ofTAPVCThecommonpulmonaryveindrainsthroughthediaphragmtotheportalvein,ductusvenosusorseldomtotheIVC.HasthegreatestpropensityforSTENOSIS:-MaybecompressedwhereitpenetratestheDiaphragm,-Connectingveinisnarrowedatitsjunctionwiththeportalvein,-VVhasat

5、hickenedwallwithintimalproliferation,-portalsinusoids(Liver)offeradditionalobstructiontovenousreturnInfracardiacTAPVC:4.MixedAPVCInvolvesacombinationofconnectionsoftwoormoreofthesubtypes(atleastoneofthemainlobarpulmonaryveinsisdrainingdifferentlyfromtheothers.)AnatomyRA:Enlargedandthickwalled

6、.Decreasedcompliance.LA:Volume53%lessthanpredicted.LAauricleisnormalinsize,decreaseinLAcanbeexplainedbytheabsenceofthepulmonaryveincomponent.ASD:ASDorPFOmustexistforsurvival.Usuallyofadequatesizeandnotobstructive.ObstructionleadstoadecreasedRtoLshuntwithpulmonaryvenousobstructionandPulm.Hyper

7、tension.Presentsasaseverelysickneonate.AnatomyLV:Normalinsize,wallthicknessandmass,butdecreasedLVcavity(duetoleftwarddisplacementofseptumsecondarytorightventriclepressure-volumeoverload.)RV:Variesinsize,dependsonmagnitudeofpulmonarybloodflow,