Andersons pediatric cardiology 1156
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GreatOrmondStreet
UKandIrelandpopulationbased
CongenitalHeartSurgeons
Toronto
UCLA,LosAngeles
1976–
1989a
1991–
1995
1987–
1997
1992–
1998a
1982–
2001
135
72%b
49%b
Bulletal.115
183
71%c
64%c
Daubeneyetal.9
408
68%
60%
Ashburnetal.116
210
75%
67%
106
88%b
86%
Dyamenahalliet
al.118
Odimetal.121
a
Usesmostrecenteradatafromthestudy.
bEstimateofmortalityat1and5years.
cSurvivalofthosewhounderwentaprocedure.
FIG.43.17 Kaplan-Meiersurvivalcurvesforsubgroupsofpatientswith
pulmonaryatresiaandintactventricularseptum.RV,Rightventricle;RVOT,
Rightventricularoutflowtract.(DatafromDaubeneyPEF,WangD,Delany
DJ,etal.Pulmonaryatresiawithintactventricularseptum:predictorsof
earlyandmedium-termoutcomeinapopulation-basedstudy.JThorac
CardiovascSurg.2005;130:1071.)
Whythislesionshouldhavesuchapooroutcomeisnotwellidentified,and
optimalstrategiesfortreatmentremaintobeelucidated.Thelargestfourstudies
haveidentifiedriskfactorsforpooroutcome.Theseincludefactorsrelatingto
thesmallsizeoftheRV,dilatedrightventricularsize,lowbirthweightand
prematurity,presenceofrightventricular-to-coronaryarteryfistulous
connections,andanRV-dependentcoronaryarterialcirculation.55,114–118As
moreislearnedaboutadverseriskfactors,cliniciansaretailoringtheirapproach;
forexample,bynotdecompressingRVswherethereisthoughttobeanRVdependentarterialcirculation.Freedomandcolleaguesstatedin2005that
“ventriculo-coronaryconnections,andarightventriculardependentcoronary
circulation,animportantriskfactorinourearliersurgicalexperience,117,119
therefore,hadbeeneffectivelyneutralizedbytheintroductionofthefunctionally
univentricularpalliation.”2
Catheterperforationoftheimperforatepulmonaryvalveisnowawellestablishedtechniqueforprimaryintervention.Itaccountedfor60%ofall
valvotomiesperformedwithoutconcomitantconstructionofashuntinthestudy
carriedoutintheUnitedKingdomandIreland.55Whenanalyzedonthebasisof
intentiontotreat,survivalwassimilarbetweenthosetreatedatcatheterizationor
surgically.Arecentreportnotesongoingtechnicalimprovement,witha
mortalityofonly10%.107Successfulperforationoftheatreticpulmonaryvalve
isnotguaranteed,butpublishedratesofsuccessrangefrom85%to90%.104–106
Longer-termoutcomes(median,9.2years[range,2.2to21years])ofthis
procedurehavealsobeenreported,indicatinganearlymortalityof21%,butno
latedeathsafterthefirst35days.111
