Andersons pediatric cardiology 1838

judgement.
ANONYMOUS
HalfofwhatI'mteachingyouiswrong.TheproblemisthatIdon'tknow
whathalfitis.
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Introduction
Someofthemostchallengingformsofcongenitalheartdisease(CHD)fallinto
thecategoryofthosewithfunctionallyonepumpingchamber,colloquially
knownas“singleventricle”defects,althoughwepreferthetermfunctionally
univentricularheart(fUVH).AfUVHisdefinedasaheartinwhichdivision
intoseparatepulmonaryandsystemicventricularpumpingchambersisnot
possible.Thisincludesmalformationssuchashypoplasticleftheartsyndrome,
tricuspidatresia,double-inletleftventricle,severeformsofpulmonaryatresia
andintactseptum,unbalancedatrioventricular(AV)septaldefectsinwhichone
oftheventriclesisprohibitivelyhypoplasticorabsent,aswellasother
malformationswheretwopumpingchambersarepresentbutdivisionisnot
practicalduetoAVvalvestraddlingorarrangementofthegreatvesselsand
remotelocationofaventricularseptaldefect.
Inthepast3decadessurvivalbeyondtheneonatalperiodhasbecomeareality
duetoabroadrangeofinnovations,includingtheuseofprostaglandinto
maintainductalpatency;improveddiagnosticcapabilities,particularlywith
echocardiography;innovationsinsurgerysuchastheNorwoodprocedure;
improvementsinpostoperativemanagement;improvedpostdischarge
management;andthedevelopmentofastandardizedthree-stageapproachto
completionoftheFontanprocedure.Favorableshort-andlong-termoutcomes
arenowtheexpectationandsurvivalintoadulthoodisanticipated.With
improvedsurvival,thefocusisnowbeingdirectedatoptimizing
neurodevelopmentaloutcome,functionaloutcome,andqualityoflifeamong
survivorsofwithfUVHanatomy.

TheultimateanatomicgoalforthepatientwithafUVHisaFontancirculation
inwhichthevenousreturnthroughthesuperiorandinferiorcavalveinsis
connecteddirectlytothepulmonaryarteries.Withthisarrangement,blood
circulatesascloselyaspossibletothenormalpattern.AsuccessfulFontanis
morelikelyinindividualswithnormalpulmonaryarteriolarresistance,no
pulmonaryarterystenoses,noobstructiontopulmonaryvenousreturn,good
systemicventricularfunction,andadequatefunctionoftheAVandsemilunar
valvesandAVsequentialrhythm.Thegoalofsurgicalpalliationoftheinfant
withafUVHisthecreationofagoodFontancandidate.Overtime,basedonthe
increasingknowledgeofriskfactorsfortheFontanprocedure,anumberof
surgicaltenetshaveemerged(Box71.1;seealsoChapter70).



Box71.1

GoalsofNeonatalSurgicalReconstruction
■Unobstructedsystemicbloodflow
■Approximatelylimitedpulmonarybloodflow
■Undistortedpulmonaryarteries
■Unobstructedpulmonaryvenousreturn
■Unobstructedsystemicvenousreturn
Thischapterpresentsthecombinedexperienceofmultiplecentersinthe
UnitedStates(theCincinnatiChildren'sHospitalMedicalCenter,Texas
Children'sHospital,BostonChildren'sHospital,Children'sHospitalof
Philadelphia,andChildren'sNationalMedicalCenter).Theyarenotmeanttobe
all-inclusive,andstrategiesthataresuccessfulinoneinstitutionmaynotbeas
successfulinothers.Multiplereportshaveshownconsiderablevariabilityin
practice;thesearebeyondthescopeofthischapter.1–24Wherecontroversies
exist,weattempttodescribeareasoffutureresearchthatmaydifferentiatebest

practices.