Andersons pediatric cardiology 1027
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Double-OutletVentricle
ChristophHaller,GlenS.VanArsdell,Shi-JoonYoo,CeciliaS.T.GeorgeHyslop,DianeE.Spicer,RobertH.Anderson
Abstract
Double-outletventriclecomprisesawidevarietyofmalformationswithan
abnormalventriculoarterialconnection.Asvariableasthemorphologycan
be,soisthepathophysiologicpictureandthetherapeuticapproach.
Successfultreatmentofpatientswiththiscongenitalcardiacdefect
mandatesathoroughunderstandingoftheunderlyinganatomyandthe
physiologicchangescharacteristicofthevarioussubtypes.Patient
presentationrangesfrompulmonaryovercirculationtowell-balanced
pulmonaryandsystemicbloodflowtoseverecyanosis.Barelyanyother
malformationcoverssuchawidespectrumofmedicaltherapies,surgical
procedures,andcatheterinterventions.Thischapterguidesthereaderfrom
themorphologytothepathophysiologicaspectsofdouble-outletventricle
andhighlightsitsclinicalpresentation,diagnosis,perioperative
managementaswellassurgicalandinterventionalstrategies.
Keywords
Double-outletrightventricle;DORV;Double-outletleftventricle;DOLV;
Intraventricularcommunication;Subpulmonary;Subaortic;Doubly
committed;Remoteinterventricularcommunication;Intraventricular
streaming;Taussig-Binganomaly
Introduction
Double-outletventricleisnomorethananabnormalventriculoarterial
connection.Thereforethefeaturemayoccurwitheachatrialarrangement,any
atrioventricularconnection,andallpossiblevariationsofventricular
morphology.Themorphologicarrangementcanthenbefurthercomplicatedby
associateddefects,somecommonandothersrare.Theclinicalpictureisas
inconstantastheanatomicpermutationsandassociationssuggest.Thischapter
reviewsthevariouscombinationsinwhichtheoutflowtractsarisefromthesame
ventriclebutinthesettingofconcordantatrioventricularconnections.When
botharterialtrunksarefoundinthesettingofdiscordantormixed
atrioventricularconnections,theabnormalarrangementsateitherthe
atrioventricularorvenoatrialjunctionsdominatethepicture.Double-outlet
ventriclecanalsobefoundinthesettingofthefunctionallyuniventricularheart.
