Andersons pediatric cardiology 1400
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comparingtheefficacyofdifferentregimens.Itishypothesizedthatlonger
steroidcoursesleadtoimprovedoutcomeduetothesuppressionofpersistent
vascularinflammation.
Smallnonrandomizedstudiesoftheuseofinfliximabinpatientsresistantto
IVIGsuggestaclinicalresponsewithresolutionofinflammationinthemajority
ofpatientsbutwithoutimpactoncoronaryarteryoutcomes.162,163
TheuseofAnakinra,anIL-1receptorantagonist,hasbeendescribedincase
reports,andclinicaltrialsareongoing.164,165
Cyclosporinehasbeenusedinhighlyrefractorypatientswithresolutionof
fever;however,morestudiesareneededtodeterminetheimpactoncoronary
arteryoutcome.166,167
Rarely,cytotoxicagentshavebeenusedtotreatrefractorypatientswithacute
disease,163buttherisksofthesetherapiesaresuchthattheyshouldbeusedonly
inpatientswithexpandinganeurysmswhoareresistanttootheragents.
Plasmaexchangehasbeenreportedtolowertheincidenceofaneurysmsin
uncontrolledstudies.161,168,169Becausethistherapyistechnicallycomplexto
administer,itsuseshouldbereservedforchildreninwhomallothermedical
therapieshavefailed.
PreventionofCoronaryArteryThrombosis
Thromboticocclusionofacoronaryarteryisthemostseriouscomplicationin
patientswithKawasakidisease,precipitatingmyocardialinfarctionorsudden
death.Multiplefactorscontributetotheriskofthrombosis,including
thrombocytosisandincreasedplateletadhesion,inflammation,endothelial
dysfunction,andabnormalflowconditionsthroughareasofseveredilationor
stenosis.Becauserandomizedtrialsofantithromboticregimenshavenotbeen
performed,thechoiceofagentsisderivedprimarilyfromexperienceinadults
withatheroscleroticdiseaseaswellascaseseriesandconsensusofexperts.
Anticoagulationisbasedonthesizeoftheaneurysmatthetimeoftreatmentas
wellasonpriorhistory(Table53.2).Patientswithsmallaneurysmsaretreated
withaspirinatanantiplateletdose(3to5mg/kgperday).Inpatientswith
moderateaneurysms,clopidogrelmaybeaddedtoaspirin,butanticoagulationis
generallynotindicated.Patientswithlargeorgiantaneurysmsareathighestrisk
forthrombosisandareusuallytreatedwithaspirinandanticoagulation(warfarin
orlow-molecular-weightheparin).Patientswitharecenthistoryofthrombosis
areatespeciallyathighriskandmaybenefitfromdualantiplatelettherapyin
additiontoanticoagulation.Inpatientsonwarfarin,aninternationalnormalized
ratio(INR)of2to3isusuallymaintained.Low-molecular-weightheparinis
usuallypreferredininfantsorolderchildrenwhocannotmaintaintheirINRs
withingoals.Trialsofdirectoralanticoagulantsinchildrenareongoing.
Table53.2
ThromboprophylaxisandMedicalTherapyAlgorithm
RiskLevel
1.Noinvolvement
2.Dilationonly
3.1Smallaneurysm;
currentorpersistent
3.2Smallaneurysm;
regressedtonormalor
dilationonly
4.1Mediumaneurysm;
currentorpersistent
4.2Mediumaneurysm;
regressedtosmall
aneurysm
4.3Mediumaneurysm;
regressedtonormalor
dilationonly
5.1Largeandgiant
aneurysm;currentor
persistent
5.2Largeorgiant
aneurysm;regressed
tomediumaneurysm
5.3Largeorgiant
aneurysm;regressed
tosmallaneurysm
5.4Largeorgiant
aneurysm;regressed
tonormalordilation
only
Low-Dose
ASA
4–6weeks
then
discontinue
Indicated
until
regressionto
normal
Indicated
Anticoagulation
DualAntiplateletTherapy
(Warfarinor
βBlocker Statin
(ASA+Clopidogrel)
LMWH)
Notindicated
Notindicated
Not
Not
indicated indicated
Notindicated
Notindicated
Not
indicated
Not
indicated
Notindicated
Notindicated
Maybe
considered
Notindicated
Notindicated
Not
indicated
Not
indicated
Maybe
considered
Maybe
considered
Indicated
Notindicated
Maybeconsidered
Indicated
Notindicated
Maybeconsidered
Not
indicated
Not
indicated
Maybe
considered
Maybe
considered
Reasonably
indicated
Notindicated
Not
indicated
Maybe
considered
Indicated
Reasonably
indicated
Notrecommendedexceptin
thepresenceofinducible
myocardialischemia
Maybeconsideredin
additiontoanticoagulationa
Indicated
Notindicated
Reasonablyindicated
Maybe
Maybe
considered considered
Indicated
Notindicated
Notindicated
Maybe
Maybe
considered considered
Reasonably
Indicated
Notindicated
Notindicated
Not
indicated
Maybe
Maybe
considered considered
Maybe
considered
aMaybeconsideredinadditiontoanticoagulationinthesettingofveryextensiveordistal
coronaryarteryaneurysmsorifthereisahistoryofcoronaryarterythrombosis.
Indicated=ClassIrecommendation;shouldbeperformed.
Reasonablyindicated=ClassIIarecommendation;isreasonabletoperform.
Maybeconsidered=ClassIIbrecommendation;maybeconsidered.
Notindicated=ClassIIIrecommendation;shouldnotbeperformed.
ASA,Acetylsalicylicacid,oraspirin;LMWH,lowmolecularweightheparin.
ModifiedfromMcCrindleBW,RowleyAH,NewburgerJW,etal.Diagnosis,treatmentandlongtermmanagementofKawasakidisease:ascientificstatementforhealthprofessionalsfromthe
AmericanHeartAssociation.Circulation.2017;135(17):e927–e999.
TreatmentofCoronaryArteryThrombosis
Thrombolytictherapyinchildrenwithcoronaryarterythrombosisisprimarily
guidedbystudiesinadultswithacutecoronarysyndromes.Themostcommon
agentusedinchildrenistissueplasminogenactivator,atdosesof0.1to0.5
mg/kgperhourfor6hoursadministeredtogetherwithaspirinandheparinor
low-molecular-weightheparin.2Theburdenofthrombusinagiantaneurysmis
large,andsomechildrenhavereboundofthrombosisafterthecessationof
thrombolytictherapy.Forthisreason,treatmentmayoccasionallyinvolvea
combinationoftissueplasminogenactivatorandabciximab,givenasabolusof
0.25mg/kgover30minutes,followedbyaninfusionof0.125µg/kgperminute
for12hours.Mechanicalrestorationofmyocardialperfusionbymeansof
interventionalcardiologymaybeusedinpatientswhosevesselsarelargeenough
toaccommodateadult-sizedcatheters.
RevascularizationProcedures
Decisionsabouttheneedforrevascularizationproceduresandthepreferredtype
ofrevascularizationareoftendifficultandbasedonexpertconsensusand
experienceinadultsratherthanevidence-baseddatainchildrenwithKawasaki
disease.Intheacute/subacutephaseoftheillness,ischemiaisusuallycausedby
thromboticocclusionofananeurysm.Systemicthrombolysisisusuallythe
preferredchoiceintheacutephase.Duringfollow-up,patientscanpresentwith
ischemiabecauseofnonocclusivethrombusorprogressionofstenosis.
Basedonguidelinesinadults,mechanicalrevascularizationshouldbe
consideredinpatientswithgreaterthan50%stenosisoftheleftmaincoronary
artery(orequivalent)withsymptomsanddocumentationofischemia,172
multivesselcoronarydiseasewithreductioninleftventricularfunction,orhighrisknoninvasiveischemiatesting.2Findingsonnoninvasivetestingthatindicate
highriskincludeearlyinduciblemyocardialischemia,exercise-induced
