Andersons pediatric cardiology 1131

FIG.42.39 Kaplan-Meiercurvesillustratingfreedomfromreinterventionin
isolatedpulmonaryvalvestenosismanagedbysurgeryin54patientsand
balloondilationin92patients.(FromPetersonC,SchilthuisJJ,DodgeKhatamiA,etal.Comparativelong-termresultsofsurgeryversusballoon
valvuloplastyforpulmonaryvalvestenosisininfantsandchildren.Ann
ThoracSurg.2003;76:1078–1082.)

Whatcanbesaidisthatballoonvalvuloplastyhasaslightlyhigherincidence
ofreinterventionsforresidualpulmonaryvalvestenosiswhencomparedto
surgicaltherapy.However,longer-termdataareneededtocomparetheincidence
ofreinterventionsforpulmonaryregurgitationbetweensurgicalandtranscatheter
therapy.Furthermore,oneshouldconsiderthatpotentialsurgicalrepairofthe
pulmonaryvalvemaybefeasibleinselectedpatientsthathaveundergone
balloonpulmonaryvalvuloplasty,whichisusuallynotanoptionaftersurgical
repairusingatransannularpatch.152
Nevertheless,inmostcenters,thelessinvasive,lessexpensive,andshorter
hospitalstaycurrentlyfavortheroutineuseofballoondilationoversurgeryfor
pulmonarystenosis.


RecommendationsforLong-Term
Follow-up
Oncepulmonarystenosishasbeentreated,usuallybyballoondilation,follow-up
ismandatory.Thereis,however,afairlywidevariationasmanyunitswillhave
theirownprotocolsforfollow-up,laiddownbyseniormembersofthe
department.Whereassomealgorithmsforfollow-upofmanycongenitalcardiac
malformationshavebeenpublished,153manyofthosedonottakeintoaccount
thespecificneedtofollowpulmonaryregurgitation.
Withtheknowledgethatsomedegreeofpulmonaryregurgitationisvirtually
universallypresentafterballoonpulmonaryvalvuloplasty,butthedegreenot
necessarilybeingimmediatelyapparent,areasonableapproachthathadbeen
adoptedbyourgroupistofollowpatientsaftertranscatheterinterventionat1,6,

12,and24monthsaftertheprocedure.Ateachfollow-upvisit,historyand
physicalexamareofcoursemandatory,complementedbyechocardiographic
evaluationtoassessthedegreeofpulmonaryregurgitationandthedegreeof
residualpulmonaryvalvestenosis.Echocardiographyisnotneededatthe1monthfollow-upvisit,providedthepatienthasundergoneechocardiographic
evaluationatthetimeofdischarge.Furtherfollow-upalwaysdependsonthe
presenceofclinicalsymptoms,possiblearrhythmias,aswellastheresidual
gradientanddegreeofpulmonaryinsufficiency.Asymptomaticpatientswithno
morethanmildpulmonaryinsufficiency,aresidualgradientoflessthan30mm
Hg,andanondilatedrightventriclebyechocardiographycouldbefollowedat5yearlyintervals,whilepatientswitharesidualgradientof40mmHgorgreater
orclinicalsymptomsrequireanindividualizedapproachtofollow-up.Patients
whodonotfallintoeitherofthesecategoriesshouldprobablybefollowedona
yearlybasis.Pediatricpatientswithmild-moderateormorepulmonary
regurgitationcanbefollowedbyechocardiographyaloneinitially,providedthat
therearenoconcerningrightventricularappearancesorprogressivedilatation.
Ourgroupwould,however,recommendperformingserialintervalcardiac
MRIandcardiopulmonaryexercisetestingtoassesstheeffectsofpulmonary
incompetenceontheanatomy,physiologyandfunctionalstateofthepatient,and
helptodeterminewhetherfurtherinterventionswillbenecessaryinanypatient
withmorethanjustmildpulmonaryregurgitation.Providedthereareno
additionalconcernsrelatingtotherightventricle,thefirstMRIcanprobablybe


delayeduntilthepatienthasmaturedenoughtotoleratethetestwithouttheneed
foranesthesia,usuallyaround8to10yearsofage,whichwouldalsobeagood
timingforacardiovascularexercisetest.Patientsthatwereolderatthetimeof
pulmonaryvalvuloplastyshouldideallyundergocardiovascularMRI(and
exercisetesting)within2yearsoftheprocedure.Thefrequencyofrepeatingthe
MRIsubsequentlydependsonthefindingsoftheinitialMRI,theageofthe
patient,andanyprogressivechangesseenbyechocardiography.Inmostpatients
thoughitwouldbeconsideredagoodpracticetorepeattheMRIwithin3years.

ProphylaxisforEndocarditis
Althoughendocarditishasbeendescribedinpatientswithpulmonaryvalve
stenosisthathaveundergoneballoonpulmonaryvalvuloplasty,154–156its
occurrenceisrare.Themostrecentguidelinesfortheuseofantibioticsto
preventinfectiveendocarditisnolongerconsiderpulmonaryvalvestenosistobe
aconditionathighriskforendocarditis,eitherbeforeorafterballoon
dilation.157,158ThecurrentguidelinespublishedbytheAmericanHeart
Associationrecommendthatthosewithrepairedmalformationsbutwithresidual
defectsatthesiteofaprostheticpatchorprostheticdevice,whichmayinhibit
endothelialization,arestilltobeconsideredtohaveanindicationfor
prophylaxis.Prophylaxisshouldberecommended,therefore,forpatientswith
prostheticmaterialatthesiteofrepair―forinstance,atransannularpatch,andin
thepresenceofresidualhemodynamiceffectssuchaspulmonarystenosisor
incompetence.

RecommendationsforExercise
PriortoIntervention
Therecommendationsforparticipationinexercisebypatientswithpulmonary
stenosisisbasedonanalysisoftheknownfeaturesofthisdiseasebyataskforce
ofexperts.159Fortheserecommendations,theclassificationofexerciseintensity
isalsoimportant(Fig.42.40).160