Andersons pediatric cardiology 1153
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approachcanbeused,however,forballoonatrialseptostomyifauniventricular
repairisplanned.Oximetryisnotparticularlyhelpful,excepttoshowright-toleftshuntingattheatriallevel.
Astep-upfromrightatriumtoventricleofmorethan6%issaidtobe
indicativeofretrogradeflowintotheRVfromcoronaryarterialfistulous
connections.58Meanrightatrialpressureisusuallyslightlyhigherthanleftatrial
pressure,butalargegradientisnotexpectedbecausetheovalforamenisusually
largeandwidelypatent.Rightventricularpressureistypicallysuprasystemic,
althoughrightventricularpressureislowiftricuspidregurgitationisverysevere
(seeTable43.2).IftheRVissmall,itmaybeenteredonlyafterrepeatedprobing
withanend-holecatheter.
Table43.2
Interrelations(PValues)ofMorphologicVariablesatPresentationof
aUK/IrelandPopulation-BasedStudy(1991–1995)
TVz-score
(increased
valvesize)
RVinletzscore
(increased
inletlength)
Ductalangle
(normal)
Fistulas
(absence)
Typeof
atresia
(membranous)
Partite
(tripartite)
Stenoses
(absence)
Tricuspid
regurgitation
(increased)
RVInletzScore
(Increased
Inlet
Length)
<.0001
R=
0.45
Ductal
Typeof
Tricuspid RV
Fistulae
Partite
Stenoses
Angle
Atresia
Regurg.
Pressure
(Absence)
(Tripartite) (Absence)
(Normal)
(Membranous)
(Increased) (Lower)
.0120
<.0001
<.0001
<.0001
.1763
.0050
.3304
.0011
.0131
<.0001
.5755
.0052
.0095
<.0001
<.0001
.0852
.0743
.3077
<.0001
<.0001
.0055
.0321
>.9999
.0140
.1938
.8409
.0022
.9720
.2090
.0008
<.0001
.0011
R
0.402
.7459
R
0.041
.0143
Thereisconsiderablecovarianceofmorphologicfeatures(e.g.,asmallrightventriclewilltendto
havemuscularatresiaandasmalltricuspidvalve).Asshown,forapopulationofpatientswith
pulmonaryatresiawithintactventricularseptumatpresentation,thePvalueforthepresence,
absence,orotherdenotedstate,ofkeymorphologicfeaturescoexistinginthesameheart.
RV,Rightventricle;TV,tricuspidvalve.
FromDaubeneyPE,DelanyDJ,AndersonRH,etal.Pulmonaryatresiawithintactventricular
septum:Rangeofmorphologyinapopulation-basedstudy.JAmCollCardiol.2002;39:1670–
1679.
Thediagnosiswillusuallyhavealreadybeenmadebyechocardiographyand
canbeconfirmedbyarightventriculogram.Assessmentofrightventricularsize
isfarfromsimpleinoftenbizarrelyshapedRVs(Fig.43.16).59,60Stenosisor
atresiaoftheinfundibulumcanbediagnosed.Ifanimperforatepulmonaryvalve
ispresent,itmaybeseenmovingtoandfroatthetopofapatentinfundibulum.
Tricuspidregurgitationcanbeapproximatelyquantifiedbecausecatheterinducedregurgitationisusuallymild.Coronaryarterialfistulousconnectionsare
alsowelldemonstratedbyrightventriculography.Stenosesin,orinterruptionof,
bothrightandleftcoronaryarteriesshouldbesought.Ifthereisdoubtaboutthe
coronaryarterialanatomyordistributionaftertherightventriculogram,an
injectionshouldbeperformedintheaorticroot.AssummarizedbyFreedomand
colleaguesintheirexcellentreview,2andlistedindecreasingorderofseverity,
coronaryabnormalitiesinclude:
FIG.43.16 Radiofrequency-assistedballoonpulmonaryvavluloplastyof
membranouspulmonaryatresia.(A)Anteroposteriorand(B)lateralstills
fromthepreproceduralrightventricularangiogram.Therightventricleis
small,butwithinlet,apical,andoutletcomponents.(C)Positioningofthe
radiofrequencywire.(D)Successfulradiofrequencyperforationusing5W
andsubsequentpositioningofthewireinthedescendingaorta.(D)Initial
balloonvalvuloplastyusinga3×2mmcoronaryballoon.(F)Finalballoon
valvuloplastyusingan8×2mmTyshakballoon.(G)Anteroposteriorand
(H)lateralstillsfromthepostproceduralrightventricularangiogram.
▪Atresiaoftheaorticorificesofbothcoronary
arteries.41,61–64
▪Atresiaoftheaorticorificeoftheleftcoronary
artery.65
▪Proximalinterruptionorocclusionofthemainstem
oftheleftcoronaryartery,itsanteriorinterventricular
orcircumflexbranches,ortherightcoronaryartery
(seeFig.43.6),combinedwithfistulous
communicationsfromtheRV.
▪Importantstenosisofthemainstemoftheleft
coronaryartery,itsanteriorinterventricularor
circumflexbranches,ortherightcoronaryartery,in
combinationwithfistulouscommunicationsfromthe
RV.Lesssevereabnormalitiescouldalsoprogress
withtime.
▪Presenceofahugefistulouscommunicationfrom
theRVtoacoronaryartery(seeFigs.43.6and43.12).
DecompressionoftheRVinthissettingwouldresult
inamassivesteal,andhenceresultincoronary
arterialinsufficiency.
Aleftventriculogramshouldalsobeperformed,bothforjudgingtherelative
rightventricularsize,andtovisualizetheductandthepulmonarytrunk.
SimultaneousinjectionintotheaortaandintotheRVmayshowtheextentofthe
gapbetweentherightventricularcavityandthepulmonarytrunkinthepresence
