Andersons pediatric cardiology 726
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PulmonaryVenousAbnormalities
RachelD.Vanderlaan,RobertH.Anderson,GirishShirali,DianeE.Spicer,
AnthonyM.Hlavacek,ChristopherA.Caldarone
Abstract
Pulmonaryvenousabnormalitiescompriseawidespectrumofcongenital
lesions.Totalanomalouspulmonaryvenousconnectionisthemost
commonandconstitutesaspectrumofcongenitallesionsinwhichthe
pulmonaryveinsremainconnectedtosystemicvenousvesselsoraberrantly
connecttotherightatrium.Definitivemanagementrequiressurgical
intervention;inpatientswithobstructiontopulmonaryvenousflow,urgent
operationisrequired.Theuseoftemporizingcatheter-basedinterventions
allowsforoptimizationinhemodynamicallyunstableneonates.Other
pulmonaryvenousconditions,suchaspartialanomalouspulmonaryvenous
connections,requiresurgicalcorrectionsbutonanelectivebasis.Overall,
survivalforanomalouspulmonaryvenousabnormalitieshassignificantly
improvedoverthepastdecadesthroughbetterperioperativemanagement
andtheevolutionofsurgicalandcatheter-basedapproaches.Postrepairand
congenitalpulmonaryvenousstenosispersistsasmajordeterminantsof
long-termoutcomes.
Keywords
Anomalouspulmonaryveins;pulmonaryveinstenosis;atresia;sutureless
repair;obstructedTAPVC
Introduction
Thischapterconsidersabnormalitiesofthepulmonaryveins,inparticulartheir
anomalousconnectionstothesystemicvenoussystem.Thevariantofpartially
anomalouspulmonaryvenousconnectionassociatedwiththesinusvenosus
interatrialcommunicationisdescribedindetail.Theanomalouspulmonary
venousconnectionssocommonlyassociatedwithisomerismoftheatrial
appendagesarementionedinthischapterbutarediscussedmorefullyinChapter
26,notleastbecauseoftheparticularproblemsofnomenclaturethatariseinthe
settingofisomerism.Althoughdivisionofthemorphologicallyleftatriumwas,
inthepast,1consideredapulmonaryvenousanomaly,thislesion,alongwith
divisionofthemorphologicallyrightatrium,iscoveredinChapter30.
