Andersons pediatric cardiology 1129

patientswithsurgicalinterventionsforpulmonaryvalvestenosishaveariskfor
ventriculararrhythmias,andthereforerequireadedicatedworkupofanyclinical
symptomssuchaspalpitationsorsyncope,assuggestedbyRuckdescheland
colleagues.137

BalloonDilation
Acuteresultsofballoonpulmonaryvalvuloplastyareexcellent,usuallyleading
toasignificantreductioninthetransvalvargradient.Thereis,however,awide
varietyofwhatisconsideredasuccessfulproceduraloutcomewhenrelatedto
theresidualgradient,withreportedsuccesscriteriaranginganywherefromless
than20tolessthan35mmHg.119,138Othercriteriathathavebeenusedtodefine
proceduralsuccessincludeagradientreductionbymorethan50%,ora
reductionoftherightventricletosystemicpressureratiobymorethan50%.120
EarlyresultsfromtheVACAregistry(784pulmonaryvalvuloplasties)
documentedasignificantdecreaseingradientfrom71to28mmHg.117More
recentresultsfromtheImprovingPediatricandAdultCongenitalTreatment
registrydocumentedthatagradientreductiontolessthan20mmHgwas
achievedin73%of268patientsundergoingballoonpulmonaryvalvuloplasty.119
HolzerandcolleaguesreportedontheresultsoftheC3POregistry,where
88%ofpatientsachievedagradientreductionto25mmHgorless,79%a
gradientreductionby50%ormore,and45%areductionintherightventricleto
systemicpressureratioby50%orgreater.120Proceduralsuccessusingeitherof
thosethreecriteriawasachievedin91%ofcases.Inmultivariateanalysis,the
onlyindependentriskfactorsforproceduralfailureweremoderateorsevere
pulmonaryvalvethickening,andthepresenceofsupravalvarpulmonary
stenosis.
Patientswithcriticalpulmonaryvalvestenosispresentingintheneonatal
periodareoftenmoredifficulttomanage,anddependingonthesizeand
morphologyoftherightventricleandtricuspidvalve,theoutcomesandneedfor
additionalproceduresmaymorecloselyresemblethatofpatientswith
pulmonaryatresiaandintactventricularseptum(discussedinChapter43).

Shaathandcolleaguesrecentlyevaluatedtheacuteandshort-termoutcome
comparing23patientswithcriticalpulmonaryvalvestenosisto20patientswith
pulmonaryatresiaandintactventricularseptum.99Reinterventionswere
significantlymorefrequentinpatientswithpulmonaryatresiaandintact
ventricularseptum(55%)whencomparedtopatientswithcriticalpulmonary


valvestenosis(17%).AstudybyYucelandcolleaguesreportedthat38%of
neonatesthatunderwentballoonvalvuloplastyforcriticalpulmonaryvalve
stenosisrequiredpulmonarybloodflowaugmentationafterpulmonary
valvuloplasty.139Karagozandcolleaguesreporteda30%incidenceof
reinterventionwithin8monthsoftheprocedurein50patientswithaweightless
than3kgundergoingballoonpulmonaryvalvuloplasty.140
Althoughmostpediatricpatientswithpulmonaryvalvestenosisare
asymptomatic,adultpatientsoftenexperiencesymptomssuchasfatigueand
dyspneapriortoballoonpulmonaryvalvuloplasty,whichoftenimprovesafter
theprocedure.Taggartandcolleaguesevaluated40adults(78%symptomatic)
andfoundanimprovementinsymptomsin20of24previouslysymptomatic
patientsfollowingballoonpulmonaryvalvuloplasty(meanfollow-up,1.9
years).141
Long-termresultsofballoondilationduringchildhoodareexcellent.131
Follow-updatareportedfromtheVACAregistry(upto8.7years)documenteda
suboptimaloutcomein15%ofpatientswithtypicalvalvarmorphologyand65%
ofpatientswithadysplasticpulmonaryvalve(overall23%).138Suboptimal
outcomeinthiscontextwasdefinedaseitheraresidualrightventricleto
pulmonaryarterypeaksystolicgradientabove35mmHg,ortheneedforrepeat
valvuloplastyorsurgicalintervention.
Fig.42.36showsthefreedomfromreinterventioninaseriesof150children
whounderwentballoonpulmonaryvalvuloplastyforpulmonarystenosis
between1984and1992.Althoughlatepulmonaryincompetencecommonly

occurs,itappearswelltoleratedinthefirstdecadeafterintervention.Forthose
childrenwithseverepulmonaryincompetence,longerfollow-upisnecessaryto
determineiftheydeveloporcanbepredictedtodevelopprogressiveright
ventriculardilatationandrequirereplacementofthevalve.Lifelongfollow-upis
essentialinchildrenwhohavehadballoondilationwherepulmonary
incompetenceispresent.


FIG.42.36 Freedomfromreinterventionafterballoondilationofthe
stenosedpulmonaryvalve;95%confidenceintervalsareindicatedbythe
dottedlines.Numbersabovethex-axisindicatechildrenatrisk.(From
GartyY,VeldtmanG,LeeK,etal.Lateoutcomesafterpulmonaryvalve
balloondilatationinneonates,infantsandchildren.JInvasiveCardiol.
2005;17:318–322.)

Inadults,balloondilationissimilarlyfeasible.72,107,142Long-termfollow-up
hasagainbeenexcellent,withminimalrecurrenceofpulmonarystenosis.74,143
Observedinfundibularhypertrophy(Fig.42.37)andtricuspidincompetence
resolvesovertime.73,144Inalargestudyof127adultswhounderwentballoon
dilation,immediatereliefofpulmonarystenosiswasachieved,withfurther
reductioninthemeasuredgradientfrom6to8yearslater(Fig.42.38).74