Andersons pediatric cardiology 1396
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andlong-termmanagementofKawasakidisease:ascientificstatementforhealth
professionalsfromtheAmericanHeartAssociation.Circulation.
2017;135(17):e927–e999.
InthemostrecentAmericanHeartAssociationguidelinesonthemanagement
ofpatientswithKawasakidisease,coronaryarteryaneurysmsareclassified
usingthez-scoretoaccountforbodysurfacearea.2Theuseofsuchz-scoreshas
suggestedthatthepreviouslyusedJapaneseMinistryofHealthcriteriamay
underdiagnoseandunderestimatethetrueprevalenceofcoronaryarterydilation.
Asmallaneurysmisdefinedasaz-scoreof2.5tolessthan5,mediumaneurysm
asaz-scoreof5tolessthan10andabsolutedimensionlessthan8mm,anda
largeorgiantaneurysmasz-score10orgreaterorabsolutedimension8mmor
greater(Table53.1).2,105–107However,withmostcalculators,z-scoresare
availableonlyfortheleftmaincoronaryarteryandtheproximalsegmentsofthe
leftanteriordescendingandrightcoronaryarteries.Fordistalcoronaries,the
JapaneseMinistryofHealthcriteriashouldstillbeapplied(small,<4mm;
medium,4to8mm;giant,≥8mm;inchildren5yearsofageandyounger,small
if<1.5timestheadjacentsegment,mediumif1.5to4times,andgiantif>4
times).108
Table53.1
ClassificationofCoronaryArteryAbnormalitiesDuringFollow-up
Classification
1
2
3
3.1
3.2
4
4.1
4.2
4.3
5
5.1
5.2
5.3
5.4
Description
Noinvolvementatanytimepoint(z-scorealways<2)
Dilationonly(z-score2to<2.5)
Smallaneurysm(z-score≥2.5to<5)
Currentorpersistent
Decreasedtodilationonlyornormalluminaldimension
Mediumaneurysm(z-score≥5to<10andabsolutedimension<8mm)
Currentorpersistent
Decreasedtosmallaneurysm
Decreasedtodilationonlyornormalluminaldimension
Largeandgiantaneurysm(z-score≥10,orabsolutedimension≥8mm)
Currentorpersistent
Decreasedtomediumaneurysm
Decreasedtosmallaneurysm
Decreasedtodilationonlyornormalluminaldimension
FromMcCrindleBW,RowleyAH,NewburgerJW,etal.Diagnosis,treatmentandlong-term
managementofKawasakidisease:ascientificstatementforhealthprofessionalsfromthe
AmericanHeartAssociation.Circulation.2017;135(17):e927–e999.
Thenumberandlocalizationofaneurysmsshouldbeassessed,aswellasthe
presenceorabsenceofintraluminalthrombiandstenoticlesions.Evaluationfor
freshlyformedthrombuscanbeperformedusingawidergrayscale(Fig.53.6).
However,echocardiographyhaslimitedsensitivityforthedetectionofthrombi
andstenoticlesions.Otherlimitationsofechocardiographyincludedifficultyin
visualizingcoronaryarteriesinolderchildrenaswellasevaluatingmoredistal
segments.Additionalimagingmodalities(computedtomography[CT]
angiography,cardiacmagneticresonanceimaging[MRI],orinvasive
angiography)shouldbeconsideredwhenneeded.
FIG.53.6 Echocardiographicevaluationofintraluminalthrombus.Shortaxisviewdemonstratinggiantaneurysmoftheleftmaincoronaryartery
andleftanteriordescendingcoronaryartery.Thereisanecho-densemass
intheleftanteriordescendinganeurysmsuggestiveofathrombus(arrow).
AssessmentforInducibleIschemia
Childrenwithaneurysmsshouldundergoperiodicstresstesting,withassessment
ofmyocardialperfusionorfunction.Mostmethodsofstresstestingusedinadult
cardiologyhavebeenreportedinsmallseriesofchildrenwithKawasaki
disease.109–119Becausethesensitivityandspecificityofteststoprovoke
myocardialischemiahavebeenexhaustivelystudiedinadultswithcoronary
arterydisease,adultguidelinesshouldbefollowedtochoosethebesttestbased
onpatients’specificcharacteristics.120Additionalfactorsinthechoiceof
modalityfortestingincludeinstitutionalexpertisewithparticulartechniquesand
theageandabilityofthechildtocooperatewithexercise.False-positivetestsare
morelikelyinpatientswithalowpriorprobabilityofcoronaryarterydisease.
Stresstestingisthereforenotrecommendedinpatientswithoutahistoryof
coronaryarteryenlargement.
OtherNoninvasiveMethodsforImagingthe
CoronaryArteries
Inselectedpatients,noninvasiveimagingmethodsotherthanechocardiography
maybeneededtoassessthecoronaryarteryanatomy.Inparticular,ultrafastCT
andMRIandangiographymayprovidevaluabledatainpatientswhosecoronary
arteriescannotadequatelybeimagedbyechocardiography.121–125MRIcan
assessthecoronaryarteriesaswellasventricularfunction,myocardialperfusion,
andscarring(Fig.53.7,Video53.1).Inadditiontoimaging,cardiacresonance
testscanbeperformedtogetherwithpharmacologicstressandmayallow
assessmentofmyocardialinfarctionusingdelayedenhancement.
