Andersons pediatric cardiology 1920

Managementofthe“Well”Fontan
RoutineSurveillanceandTesting:theRoleof
PracticeGuidelines
TheFontanpopulationisheterogenouswithawidespectrumoffunctional
performance.Themorbidityprofileishighlyvariabledependingonanumberof
factorsincludingunderlyinganatomyandventricularmorphology,timefromthe
Fontanprocedure,andtheerainwhichFontansurgerywasperformed.Hence
practiceguidelinesmustbeadaptabletothesefeaturesandmustalsotakeinto
accountthelifestageofthepatient.Forexample,thepediatricpopulationhas
careprioritiesthatdiffertothoseofadults.Moreover,thetransitionsbetween
lifestages,lifestyles,andphysiologicandpsychologicalstatesareimportant
milestonesthatneedtobeanticipated,anddiscussedandplannedin
advance.71,246Thereisapaucityofpublishedpracticeguidelinesthatadequately
traversethelifetimecareofaFontanpatient.247Thisislargelyduetothelackof
ademonstrableadvantageofanyparticulartreatmentoptionsormanagement
strategies.Consequently,thereissignificantpracticevariation.248,249Thereare
fewpracticeguidelinesforpediatriccare,especiallyforthe“well”Fontan,11in
partduetoageneralperceptionthatthesepatientshavealowresource
requirement.Moreover,thereisnoevidencebasetoassesstheimpactofamore
structuredapproachtoongoingsurveillanceonoutcomeinthisgroup.The
majorityofpublishedguidelinesareforadultpatients,oftenwithafocusonthe
failingFontan.250–253Nevertheless,astructuredapproachtosurveillancein
childhoodislikelytoleadtolowerresourceuseandabetterunderstandingasto
whichinvestigationsareproductiveatagiventime.Inaddition,specifictiming
ofdiscussionandcounselinginrelationtoexerciseparticipation,teenagerisk
taking,contraception,andtransitiontoadultcareislikelytoimproveoutcomes
intheseareas.Thelatterisespeciallyimportantgiventhemortalityand
morbidityriskassociatedwithdropoutfromcardiologysurveillanceatthetime
oftransition.254–257
TheFontanpatientrequiresregularsurveillanceoverhisorherlifetime(Fig.
73.23).Thecorerequirementisaregularclinicalreviewwithapediatric

cardiologistduringchildhoodandanadultcongenitalcardiologistthereafter.The
frequencyofreviewinpediatricpracticeisdebatable;however,Americanand


Europeanguidelinesforadultcongenitalheartdiseaserecommendannual
review.250,253Echocardiographyformsthemainstayofimaginggivenitsrelative
easeofaccessandnoninvasiveproperties.Usefulinformationprimarilyrelates
tothefunctionoftheventricleandtheassessmentofvalveregurgitationandthe
outflowtracts,althoughadditionalinformationincludingthequantificationof
thegradientbetweentheFontanpathwayandthepulmonaryvenousatrium
throughDopplerinterrogationofapatentfenestrationorthediagnosisof
thrombosiscanbeuseful.Theprimarylimitationsofechocardiographyincludea
relianceongeometricindicestoassessventriclesizeandfunction,whichisoften
problematicgiventheheterogeneousventricularmorphologyencounteredinthe
Fontanpopulationandtheincreasinglychallengingacousticpropertiesinolder
patients.Nevertheless,itcontinuestohaveaplaceinsurveillanceforolder
patientswhoareMRIincompatible.Itsusefulnesswouldincreaseif
nongeometricindicesofdiastolicandsystolicperformancewereabletopredict
outcome.48,49,258–263Smallstudieshaveshowninconsistentperformancetodate
inthisregard,althoughdeformationassessmentappearstoholdthemost
promise.49,260,264–266CardiacMRIissuperiortoechocardiographyinthe
assessmentofFontanflowdynamicsandthesizeandsystolicfunctionofthe
systemicventricle,especiallywhenitisarightventricle.Itisausefuladjunctto
surveillanceparticularlyintheadultwithaFontancirculation,251,260,267–269
althoughcurrentguidelinesleaveitsinclusioninregularsurveillanceto
individualassessment.250,253TheabilitytoperformexerciseMRImayleadtoits
inclusioninfuturesurveillancealgorithms.270TheutilityofcardiacMRIis
limitedinthepediatricpopulationgiventhefrequentneedforgeneralanesthesia.
CardiacCTandcardiaccatheterizationareusefulwheretherearespecific
questionsnotansweredbyechocardiographyorMRI.

FIG.73.23 LifelongpracticeguidelinesforthecareoftheFontanpatient.
PracticeGuidelinescanbebrokendownintoUniversalRecommendations,
SuggestedSerialAdditionalSurveillance,andtheIndividualized
Component.ThelargestpartofanyFontanpracticeguidelineisthe
IndividualizedComponentduetothespectrumofthepopulationandthe
changeinneedsovertime.*Underuniversalguidelines“regular”follow-up
hasbeendefinedasatleastyearly.(FromBaumgartnerH,BonhoefferP,
DeGrootNM,etal.ESCGuidelinesforthemanagementofgrown-up
congenitalheartdisease[newversion2010].EurHeartJ.
2010;31[23]:2915–2957;andWarnesCA,WilliamsRG,BashoreTM,etal.
ACC/AHA2008GuidelinesfortheManagementofAdultswithCongenital
HeartDisease:ExecutiveSummary:areportoftheAmericanCollegeof
Cardiology/AmericanHeartAssociationTaskForceonPracticeGuidelines
[writingcommitteetodevelopguidelinesforthemanagementofadultswith
congenitalheartdisease].Circulation.2008;118[23]:2395–451.)