Management of Heart Failure in Infants and Children

14/10/2015
Managementofheartfailureininfantsandchildren
OfficialreprintfromUpToDate
www.uptodate.com2015UpToDate
Authors
RakeshKSingh,MD,MS
TPSingh,MD,MSc
SectionEditors
JohnKTriedman,MD
DavidRFulton,MD
DeputyEditor
CarrieArmsby,MD,MPH
Alltopicsareupdatedasnewevidencebecomesavailableandourpeerreviewprocessiscomplete.
Literaturereviewcurrentthrough:Sep2015.|Thistopiclastupdated:Feb11,2015.
INTRODUCTIONHeartfailureisestimatedtoaffect12,000to35,000childrenbelowtheageof19yearsinthe
UnitedStateseachyear[1].Itresultsfromanystructuralorfunctionalcardiacdisorderthatimpairstheabilityof
theventricle(s)tofillwithorejectblood.
Themanagementofchildrenwithheartfailurewillbepresentedhere.Theetiology,presentation,diagnosis,and
initialevaluationofthepediatricpatientwithheartfailurearediscussedseparately.(See"Etiologyanddiagnosisof
heartfailureininfantsandchildren".)
OVERVIEWBecauseheartfailureisacommonclinicalconditioninadults,thereisasubstantialamountof
evidencebaseddatabasedonlarge,placebocontrolledclinicaltrialsthatguidemanagement.Overthepast20
years,managementofheartfailureinadultshasshiftedbasedonobservationsthatheartfailureandleft
ventricular(LV)systolicdysfunctionactivatesympatheticnervousandreninangiotensinsystems.Thisresponse
isinitiallyphysiologic(andcompensatory),butpersistentactivationismaladaptiveandcontributestoprogressive
LVdilationanddysfunction(remodeling),andworseningheartfailure.Datafromclinicaltrialshaveshownthat
drugstargetedtoblocktheeffectsofneurohormonalactivationnotonlyreverseLVremodelingbutalsoimprove
survivalinpatientswithheartfailure.(See"Overviewofthetherapyofheartfailurewithreducedejectionfraction",
sectionon'Pharmacologictherapy'.)
However,theabilitytoconductsimilartrialsinchildrenisnotpossiblebecausethemuchlowerprevalencerateof
pediatricheartfailuredoesnotallowforasufficientnumberofpatientstoreplicatethesestudies.Asaresult,
treatmentofheartfailureinchildrenisbasedonresultsprovidedbyadultstudies.Thisapproachisjustifiable
becausechildreninheartfailurehaveneurohormonalchanges[24]andsystemicventricularremodelingsimilarto
thatdescribedinadultswithheartfailure[5].
In2004,theInternationalSocietyofHeartandLungTransplantation(ISHLT)publishedguidelinesforthetreatment
ofheartfailureinchildrenprimarilybasedontheadultliterature[6].Modificationsforspecificpediatricdiagnoses
wererecommendedbasedonexpertconsensusthatwaslargelyinformedbyclinicalexperience,smallcase
series,andphysiologicalstudies.Themanagementapproachforthisreviewisbasedontheseguidelinesand
advancesinheartfailuretherapysincethepublicationoftheseguidelines.
GoalsoftherapyTherapeuticgoalsforchildrenwithheartfailurearetorelievesymptoms,decreasemorbidity
(includingtheriskofhospitalization),slowtheprogressionofheartfailure,andimprovepatientsurvival.
ManagementapproachThemanagementofpediatricheartfailureisdependentonitsetiologyandseverity[6
8].
EtiologyandpathophysiologyManagementbeginswithathoroughassessmentoftheunderlyingcause
ofheartfailure.Thecausesofpediatricheartfailurecanbedividedintopathophysiologiccategories(table1).This
categorizationhelpsguidetheapproachtomanagement.(See"Etiologyanddiagnosisofheartfailureininfants
andchildren",sectionon'Pathophysiologyandetiology'.)
HeartfailureduetoventricularpumpdysfunctionVentricularpumpdysfunctionandreducedventricular
contractilityleadtoimpairedejectionofbloodfromtheventricle.Ventricularsystolicdysfunctionmayoccur
inchildrenwithstructurallynormalheartsorinthosewithcongenitalheartdisease.Medicalpharmacologic
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therapyisgenerallytheprimaryintervention.Drugchoiceisdependentontheseverityofheartfailureand
mayincludeangiotensinconvertingenzyme(ACE)inhibitorsorangiotensinIIreceptorblockers(ARB),
diuretics,aldosteroneantagonists,digoxin,andbetablockers.Inpatientswithadvancedheartfailure
refractorytopharmacologictherapy,interventionssuchaspositivepressureventilation,mechanical
circulatorysupport,andhearttransplantationmaybeconsidered.(See'Pharmacologictherapy'below.)
HeartfailureduetovolumeoverloadwithpreservedventricularpumpfunctionVolumeoverload(increased
preload)mayoccurduetoasignificantlefttorightshuntfromthesystemictothepulmonarycirculation(eg,
ventricularseptaldefectoratrioventricularcanaldefects)or,lesscommonly,duetovalvularinsufficiency.
Surgicalorcatheterbasedinterventionstocorrectthesedefectsleadtoresolutionofheartfailure.Medical
therapymaybeneededforstabilizationorsymptomreliefwhileawaitingamoredefinitiveintervention.
HeartfailureduetopressureoverloadwithpreservedventricularpumpfunctionPressureoverload
(increasedafterload)mayleadtoheartfailureifsevereventricularoutflowobstructionimpedesejectionof
bloodfromtheheart,resultingininadequatecardiacoutput(eg,aorticstenosis),orifventricularhypertrophy
duetoprolongedpressureoverloadresultsinhighfillingpressuresandcongestion(eg,hypertrophic
cardiomyopathy).Surgicalorcatheterbasedinterventionstocorrectthesedefectsleadtoresolutionofheart
failure.Medicaltherapymaybeneededforstabilizationorsymptomreliefwhileawaitingamoredefinitive
intervention.
SeverityofheartfailureThemanagementofpediatricheartfailureisdependentontheseverityofheart
failure,whichisdefinedbyastagingsystem(stagesAtoD)developedbyawritingcommitteeonbehalfofthe
AmericanCollegeofCardiology(ACC)andtheAmericanHeartAssociation(AHA),andmodifiedforchildrenby
theISHLT(table2)[6].Thisschemaidentifiesasymptomaticpatientsintheearlystagesofheartfailure(ie,stage
B)inwhomearlyintervention,suchasACEinhibitors,mayprolongthesymptomfreestate,aswellasdetermine
whichpatientsrequiremoreaggressivetherapy.(See"Etiologyanddiagnosisofheartfailureininfantsand
children",sectionon'StagingoftheprogressionofHF'and'Ourapproach'below.)
COMPONENTSOFTHERAPYComponentsoftherapyforchildrenwithheartfailureincludethefollowing:
Identificationandcorrectionofnoncardiacfactorsthatcontributetocardiacdysfunction.Intheacutesetting,
thesemayincludeacidosisandsepsis.Otherfactorsthatmaybeassociatedwitheitheracuteorchronic
heartfailureincludeanemia,hypertension,andrenalfailure.(See"Approachtoanemiainadultswithheart
failure"and"Treatmentofhypertensioninpatientswithheartfailure"and"Etiologyanddiagnosisofheart
failureininfantsandchildren",sectionon'Noncardiaccauses'and"Etiologyanddiagnosisofheartfailurein
infantsandchildren",sectionon'Structurallynormalheart'.)
Surgicalorcatheterbasedinterventionstocorrecttheunderlyingstructuraldefect(eg,significantlefttoright
shuntorventricularoutflowobstruction).
Pharmacologictherapytorelievesymptoms,slowtheprogressionofventriculardysfunction,andimprove
patientsurvival.
Additionaltherapeuticinterventionsforpatientswithadvancedheartfailurerefractorytopharmacologic
therapyincludepositivepressureventilation,mechanicalcirculatorysupport,andhearttransplantation.
Nonpharmacologictherapyforchronicheartfailureincludesoptimalnutritionandexerciserehabilitation.
Therapeuticinterventionstoreducetheriskofand/ortreatassociatedcomplications(eg,thromboembolism,
arrhythmias,andventriculardyssynchrony).
SurgicalorcatheterbasedinterventionSurgicalorcatheterbasedinterventioncanleadtoresolutionofheart
failureinpatientswitheithervolumeorpressureoverload,andpreservedventricularfunction(table1).Volume
overloadisassociatedwithlesionswithsignificantlefttorightshunting(eg,largeventricularseptaldefectsand
atrioventricularcanaldefects),whereaspressureoverloadisseeninpatientswithventricularoutletobstruction
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(eg,criticalaorticstenosisandcoarctationoftheaorta).(See"Etiologyanddiagnosisofheartfailureininfantsand
children",sectionon'Preservedventricularpumpfunctionwithvolumeoverload'and"Etiologyanddiagnosisof
heartfailureininfantsandchildren",sectionon'Preservedventricularpumpfunctionwithpressureoverload'.)
Pharmacologictherapy
OverviewPharmacologictherapyisprimarilyusedinpatientswithventricularpumpdysfunction.Drug
therapyisalsousedinitiallytostabilizeandrelievesymptomsinpatientswithpreservedventricularfunctionwho
areawaitingcorrectionoftheunderlyingdefectthatresultsineithervolumeorpressureoverload.
Datafromadultswithheartfailurehaveshownthatanumberofdrugsprovidesymptomrelief,andimprovementin
patientoutcomeandcardiacfunctions.(See"Overviewofthetherapyofheartfailurewithreducedejection
fraction",sectionon'Pharmacologictherapy'and"Treatmentofacutedecompensatedheartfailure:Components
oftherapy".)
Improvementinsymptomscanbeachievedbydiuretics,digoxin,angiotensinconvertingenzyme(ACE)
inhibitors,andangiotensinIIreceptorblockers(ARBs).
Prolongationofpatientsurvivalhasbeendocumentedwithbetablockers,ACEinhibitors,ARBs,and
aldosteroneantagonists.
Improvementinleftventricular(LV)functionandreversalofLVdilation,describedasreversalofLV
remodeling,isseenwiththechronicuseofACEinhibitors,ARBs,betablockers,andaldosterone
antagonists.
Hospitalizedpatientswithendstageheartfailureawaitinghearttransplantmayrequireintravenousinotropic
and/ordiureticsbecausetheyarerefractorytooralmedicaltherapy.(See'Drugtherapyforadvancedheart
failure'below.)
Basedonthisevidenceinadults,thesedrugsareusedinchildrenwithheartfailure,ifnocontraindicationstotheir
useareidentified.Thesedrugsarediscussedindetailinthefollowingsections.
DiureticsDiureticsdecreasepreloadbypromotingnatriuresis,andprovidereliefofvolumeoverload
symptomssuchaspulmonaryandperipheraledema.DiureticsareusedtotreatchildrenwithstageCorDheart
failure(table2).
LoopdiureticsLoopdiureticsinhibitsodiumandchloridereabsorptioninthethickascendinglimboftheloop
ofHenle.Furosemideisthemostcommonlyusedloopdiuretic.Astudyof62hospitalizedchildrenwithheart
failureandfluidoverloaddemonstratedtheefficacyandsafetyoffurosemide[9].Bumetanideandtorsemide
aremorepotentdrugs,whichareusedlessfrequentlyandreservedformoresevereorfurosemideresistant
fluidoverload.Sideeffectsofloopdiureticsincludeelectrolyteabnormalities(hyponatremia,hypochloremia,
andhypokalemia),metabolicalkalosis,andrenalinsufficiency.Longtermtherapycanleadto
nephrocalcinosisandototoxicity(usuallywithhighintravenousdosages)[10].
ThiazidediureticsThiazidediureticsinhibitreabsorptionofsodiumandchlorideionsfromthedistal
convolutedtubulesofkidneys.Theygenerallyareusedassecondlineagentsandoftenincombinationwith
aloopdiuretic.Commonlyusedthiazidediureticsarechlorothiazide,hydrochlorothiazide,andmetolazone.
AldosteroneantagonistsAldosteroneantagonistsdecreasesodiumreabsorptionandpotassiumexcretionin
thecollectingductsofkidneys.Theirpotassiumsparingdiureticeffectmakesthemparticularlysuitablefor
useinconjunctionwithloopdiureticsandthiazides.Bothspironolactoneandeplerenonehavebeenshownto
reducemortalityinadultswithheartfailurewhenaddedtostandardtherapy[11,12].Thiseffectis
independentoftheirdiureticeffectandismediatedbyinhibitionofmyocardialfibrosis,animportant
componentofLVremodeling[13].Sideeffectsincludehyperkalemia(withboth)andgynecomastia(with
spironolactone).
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DigoxinDigoxinhasapositiveinotropiceffect(mediatedbyNa+/K+ATPaseinhibitionandincreasein
intracellularCa+),anegativechronotropiceffectthatslowsatrialconduction,andvagotonicpropertiesthatcounter
symptomsandsignsmediatedbytheactivationofthesympatheticnervoussysteminheartfailure[14].Digoxin
usedtobethemainstayofheartfailuremanagementbeforethe1990s,butitsrolebecamecontroversialafterit
wasfoundnottoreducemortalityinadultswithheartfailure[15,16].Althoughdigoxinisnolongerusedinchildren
withasymptomaticventriculardysfunction,itcontinuestobeusedinthetreatmentofinfantsandchildrenwith
stageCheartfailurebecauseofitsphysiologicbenefitandsymptomrelief.Thesebenefitsaregenerallyseenwith
asmallerdose(troughlevel0.5to1ng/mL)thanwasusedinthepast.Potentialadverseeffects(arrhythmias)are
rarewiththislowerlevel.
ReninangiotensinaldosteronesysteminhibitionHeartfailureleadstoactivationoftherenin
angiotensinaldosteronesystem(RAAS)andincreasedsympathetictone.ACEinhibitorsandARBsinhibitthe
RAAS,therebydecreasingafterloadandpromotingreversalofventricularremodelingwithlongtermuse.
AngiotensinconvertingenzymeinhibitorsACEinhibitorsinhibittheformationofangiotensinII,a
potentvasoconstrictorthatalsopromotesmyocytehypertrophy,fibrosis,andaldosteronesecretion[6].Thus,ACE
inhibitorsbenefitpatientsinheartfailurefirstbyreducingafterload,improvingcardiacoutput,and,onchronicuse,
bymediatingreversalofLVremodeling.ClinicaltrialsinadultshaveshownthatACEinhibitorsimprovesurvivalin
patientswithsymptomaticheartfailureandreducetherateatwhichasymptomaticpatientswithsevereLV
dysfunctiondevelopsymptomaticheartfailure.(See"ACEinhibitorsinheartfailurewithreducedejectionfraction:
Therapeuticuse",sectionon'Generalefficacy'and"Angiotensinconvertingenzymeinhibitorsandreceptor
blockersinheartfailure:Mechanismsofaction".)
Prospectivestudieswithsurvival(ormortality)astheirendpointhavenotbeenpossibleinchildrenwithheart
failure.Nevertheless,ACEinhibitorsareacceptedasanimportantcomponentofheartfailuretherapyinchildren.
Themixedresultsinthefollowingpediatricstudiesdiscussedshouldbecautiouslyinterpretedbecauseof
limitationsinstudydesign,includingsmallsamplesize(power),lengthoffollowup,andsurrogateendpoints.
Inonesmallstudyof16patients,ACEinhibitorsimprovedcardiacfunctioninchildrenwithsystemic
ventricularpumpdysfunction[17].
Aretrospectiveanalysisof81childrenwithdilatedcardiomyopathyshowedthattreatmentwithcaptoprilwas
associatedwithbettersurvivalduringthefirstyearoftreatment[18].
Arandomized,controlledstudyofenalaprilin230infantswithsingleventricleanatomyandpredominantly
normalsystemicventricularfunctiondidnotshowanydifferencebetweentheenalaprilandplacebogroupsin
somaticgrowth,ventricularfunction,heartfailureseverity,ordeathafteroneyearoftherapy[19].
ChildrenwithmitraloraorticregurgitationtreatedwithACEinhibitorshaveshowninconsistentimprovement
inLVfunction,dilatation,andhypertrophy[20,21].
Inastudyof17childrentreatedwithanthracyclinechemotherapy,enalaprilwasassociatedonlywith
transientearlybeneficialeffectsonLVfunction[22].Despitecontinuedenalapriltherapy,deteriorationinLV
functionandfractionalshorteningoccurredbetween6and10years.
Inarandomized,doubleblindedtrialinchildrenwithDuchennemusculardystrophy(DMD),althoughgroups
treatedwithperindoprilandplacebohadsimilarLVfunctionafterthreeyears(whenthetrialstopped),those
treatedwithperindoprilhadlatebeneficialeffectsonLVfunctionat5and10years[23,24].
Basedoncurrentevidencefromadulttrialsandpediatricstudies,mostpediatricheartfailureexpertsuseACE
inhibitortherapyinchildrenwithventricularpumpdysfunction(stageBorCheartfailure)(table2).Bloodpressure
andrenalfunctionshouldbecloselymonitored,especiallyinneonates[25].
AngiotensinreceptorblockersInchildrenwithheartfailure,thereisapaucityofdataontheuseof
ARBs,whichblocktheangiotensinreceptor.Thus,ACEinhibitorsarethepreferredclassofdrugsforinhibitionof
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theRAAS.ARBsareusuallyreservedforpatientsunabletotolerateACEinhibitorsduetocoughorangioedema.
BetablockersBetablockerscounteractthemaladaptiveeffectsofchronicsympathicactivationofthe
myocardium.Inadultswithheartfailure,theyimprovepatientsurvival,reverseLVremodeling,anddecrease
myocardialfibrosis.(See"Overviewofthetherapyofheartfailurewithreducedejectionfraction",sectionon'Beta
blocker'.)
Althoughmixedresultshavebeenreportedinthefollowingpediatricstudies,mostexpertsinthefieldusebeta
blockersforchildrenwithstageCheartfailure(table2).Resultsneedtobecautiouslyinterpretedbecauseof
limitationsinstudydesignincludingsmallsamplesize(power),lengthoffollowup,andsurrogateendpoints.
Inaretrospectivemulticenterstudyof15childrenwithchronicheartfailure,metoprololwasassociatedwith
asignificantincreaseinejectionfractionfrom27to41percent[26].
Inseveralsmallobservationalstudies,carvediloltherapyhasbeenassociatedwithimprovedsymptoms,
improvementinventricularfunction,anddelayintimetotransplantordeathinchildrenwithheartfailure[27
30].CarvediloltherapyhasalsobeenshowntopreserveLVfunctionafterexposuretoanthracyclinesatsix
monthsfollowup[31],andtoimproveLVfunctionwhenaddedtoACEinhibitortherapyinpatientswithDMD
anddilatedcardiomyopathy[32].
Inamulticentertrialof161childrenwithheartfailureandventricularpumpdysfunction,therewasno
differenceinthecompositeendpointbetweengroupstreatedwithcarvedilolandplacebo[33].However,the
studywasthoughttobeunderpoweredastheclinicalcourseofallchildrenenrolledwasbetterthan
expected.TherewasatrendtowardsclinicalimprovementinchildrenwithasystemicLV,butnotinthose
withasystemicrightventricle(RV),suggestingthattheresponsetocarvedilolmaybeaffectedbythe
morphologyofthechildssystemicventricle[34].
Inalarge,retrospectivemulticenterreviewofthePediatricHealthInformationSystem(PHIS)database,a
betablockerwasprescribedupondischargein37percentofpediatricpatientsadmittedwithacute
decompensatedheartfailure[35].
A2009Cochranereviewofbetablockertherapyinchildrenwithheartfailureconcludedtherewerenot
enoughdatatorecommendordiscourageitsuse[36].
Basedonthecurrentevidenceinadultandpediatricpatients,mostpediatricheartfailureexpertsusecarvedilolin
childrenwithasystemicLVandsystolicdysfunctioninstageCheartfailurewhoarestableonotherheartfailure
medications(table2).Betablockersareusuallyaddedtoanestablishedregimenofdiuretics,digoxin,andanACE
inhibitor.
Carvediloldosingisinitiatedatalowdose(approximatelyoneeighthoftheeventualtargetdose,usuallyanoral
doseof0.05mg/kgperdosegiventwiceaday)andincreasedeverytwoweekstominimizesideeffects.In
general,thedoseisdoubledafterobservingtheresponsetothenewhighertestdoseinclinictoamaximumoral
doseof0.4mg/kggiventwiceaday.Sideeffectsthatmayprecludedoseincreaseincludedizziness,fatigue,
hypotension,bradycardia,bronchospasm,andhypoglycemia.Betablockersarediscontinuedinpatientswith
decompensatedheartfailure(stageD).
PulmonaryvasodilatorsSildenafil,aphosphodiesterase5inhibitor,isapulmonaryvasodilator.Itsusehas
beenassociatedwithimprovedLVfunction,functionalcapacity,andqualityoflifeinadultswithsystolicLV
dysfunctionandsecondarypulmonaryhypertension[37].Larger,controlledstudiesareunderwayinadultswith
heartfailureandsecondarypulmonaryhypertensiontoassesstheeffectofthesedrugsonpatientsurvival.
Althoughsildenafilwasshowntoimprovesymptomsofheartfailurein13childrenwithfailingFontanphysiology
[38],itremainsaninvestigationaldrug.
DrugtherapyforadvancedheartfailureIntravenousdiureticsandinotropicagentsaregenerallyusedin
hospitalizedpatientswithstageDheartfailure(table2).
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InotropesInotropicagentsareusedduringacuteexacerbationsofheartfailuretoimprovecardiacoutput
andtostabilizepatientsawaitinghearttransplantation.Theireffectismediatedthroughhigherintracellularcyclic
adenylatemonophosphate(cAMP)levels,eitherbyincreasedproduction(catecholamines)orbydecreased
degradation(phosphodiesteraseIIIinhibition).
CatecholaminesSympathomimeticstimulationbycatecholamineagentsimprovesmyocardial
contractilityandmayhaveanadditionalbeneficialeffectonperipheralvascularbeds[39].Dopamineisthe
preferreddrugduringdecompensatedheartfailure(usuallyincombinationwithintravenousmilrinone),although
dobutaminehastheadditiveeffectofreducingafterload.Invasivemonitoringofarterialbloodpressureandcentral
venouspressuremayallowdosetitrationforoptimalendorganperfusion,asmeasuredbyurineoutput,serum
lactate,andmixedvenoussaturations.(See"Useofvasopressorsandinotropes",sectionon'Dopamine'and
"Useofvasopressorsandinotropes",sectionon'Dobutamine'.)
MilrinoneIntravenousmilrinone,aphosphodiesteraseIIIinhibitor,isthepreferreddrugin
decompensatedheartfailure,asitincreasescontractilityandreducesafterloadwithoutasignificantincreasein
myocardialoxygenconsumption[40].Arandomized,doubleblind,placebocontrolledtrialinpediatricpostoperative
cardiacsurgerypatientsdemonstratedthatchildrentreatedwithhighdosemilrinoneinfusion(0.75mcg/kg/min)
wereatalowerriskforthedevelopmentoflowcardiacoutputsyndrome(LCOS)comparedwithchildrentreated
withplacebo(12versus26percent)[41].
Toavoidhypotension,milrinoneisinitiallyadministeredasaninfusionstartingatadoseof0.25mcg/kg/min
(withoutapreinfusionbolus)andtitratedupwardsslowlyasneededtoamaximumdoseof1mcg/kg/min.
Althoughmilrinoneisusuallyusedinhospitalizedchildrenwithheartfailurerefractorytooraldrugtherapyawaiting
hearttransplantation,asmallstudyof14patientsreportedthesafeadministrationofintravenousmilrinoneat
homeinchildrenawaitinghearttransplants[42].Severalcenters(includingours)usehomemilrinoneinselected
childrenawaitinghearttransplantation.Inourpractice,homemilrinoneinfusiontherapyisusedinchildrenwhoare
clinicallystablewithoutendorgandysfunction,withnohistoryofarrhythmias,whogenerallyareonamilrinone
dose0.5mcg/kg/minandastableregimenoforaldiuretictherapy,andwhoareundercontinuousadult
supervision.
NesiritideNesiritideisarecombinantBtypenatriureticpeptidethatreducespreloadandafterloadby
promotingdiuresis,natriuresis,andarterialandvenodilation,therebyimprovingcardiacoutputwithoutadirect
inotropiceffectonthemyocardium.Ithasbeenreportedtobewelltoleratedandefficaciousinchildrenas
illustratedinaprospective,openlabelstudyin63childrenwithrefractoryheartfailurethatshowednesiritidewas
associatedwithimprovedurineoutput,serumcreatinine,andcardiacfunction[43,44].However,trialsinadults
withacutedecompensatedheartfailurehavefailedtoshowthatnesiritideisassociatedwithlowermortalityor
rehospitalizationrate,orthatitimprovesdyspnea.Inaddition,thereisanassociatedincreasedriskof
hypotension.Asaresult,nesiritideisnotrecommendedforgeneraluseinacuteheartfailure.(See"Nesiritidein
thetreatmentofacutedecompensatedheartfailure".)
NonpharmacologicinterventionsforadvancedheartfailureTherapeuticinterventionsforselectedpatients
withadvancedheartfailurerefractorytopharmacologictherapy(stageD)mayinclude:
Positivepressureventilation
Mechanicalcirculatorysupportinpatientswithendstageheartfailure
Hearttransplantation
PositivepressureventilationNoninvasivepositivepressureventilation(NPPV),suchascontinuous
positiveairwaypressure(CPAP)orbilevelpositiveairwaypressure(BiPAP)ventilation,iseffectiveinalleviating
respiratorydistressfromcardiogenicpulmonaryedema.Itpromotesalveolarrecruitment,improveslung
compliance,andleadstodecreasedleftventricularpreloadandafterload[45].Althoughthereishighquality
evidenceofthebenefitofNPPVinadultpatientswithcardiogenicpulmonaryedema,therearenopediatricstudies
evaluatingitsuseinheartfailure.(See"Noninvasivepositivepressureventilationinacuterespiratoryfailurein
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adults".)
MechanicalcirculatorysupportInachildwithdecompensatedheartfailurewithlowcardiacoutput
syndromeunresponsivetomedicaltherapy,mechanicalcirculatorysupport(MCS)canbelifesaving.MCS
maintainsendorganfunctionandreducesmyocardialoxygenrequirements.Itisusedasabridgetorecovery
(extracorporealmembraneoxygenation[ECMO])inpatientswithsecondarycardiomyopathyortoheart
transplantation(ECMOorventricularassistdevice[VAD]).InareportfromtheInternationalSocietyforHeartand
LungTransplantation(ISHLT),25percentofpediatrichearttransplantrecipientsreceivedmechanicalcirculatory
supportasabridgetotransplantation[46].
Theoptionsincludethefollowing:
Extracorporealmembraneoxygenation(ECMO)isatotalheartlungbypassdeviceandisusedinthesetting
ofimminentoractualcardiacarrest,suchaspostcardiotomyshockfollowingcardiacsurgeryandacute
myocarditis.Cannulationcanbeperformedpercutaneously,andECMOcanprovidefullcardiopulmonary
supportfordaystoweeks.Amulticenterregistryreviewof3416neonataland4181pediatriccardiacECMO
casesshowedasurvivaltodischargerateof38and45percent,respectively[47].Ifmyocardialrecovery
doesnotoccurorisnotexpectedtooccurwithintwotothreeweeks,ECMOmaybeusedasabridgetoa
moredurableventricularassistdeviceplacementandsubsequenthearttransplantation.
Ventricularassistdevice(VAD),acardiaconlysupportdevice,canoffereitheruniventricularorbiventricular
support.Multipledevicescurrentlyexistanddifferbyflowdesign(pulsatile,centrifugal,oraxial),pump
locationrelativetopatient(implantable,paracorporeal,orextracorporeal),anddeliverysystem(percutaneous
orcentral)[48].Theyareprimarilyusedinpatientsawaitinghearttransplantationandhaveyieldedfavorable
results[49].Aretrospective,multicenteranalysisof99childrensupportedbyVADasabridgeto
transplantationshoweda77percentsurvivaltotransplant[50].VADoptionsarelimitedinsmallchildren
awaitinghearttransplantationduetobodysizeandanatomicconsiderations,thoughnewdevicesare
currentlyunderdevelopment[51].
Thechoiceofdevicedependsontheetiologyofheartfailure,thepatientscardiacanatomy,theexpectedlengthof
support,theavailabilityofdevices,andtheexpertiseofthecentersclinicians.Seriouscomplicationsassociated
withECMOandVADincludebleeding(eg,gastrointestinalandintracranialhemorrhage),thromboembolism(eg,
stroke),andinfection.(See"Shorttermmechanicalcirculatoryassistdevices".)
HearttransplantationHearttransplantationisrecommendedforendstageheartfailurerefractoryto
medicaltherapy(stageD).Itmayalsobeconsideredforlesssevereheartfailure(stageC)associatedwithsevere
limitationofactivity,significantgrowthfailure,intractablearrhythmias,orrestrictivecardiomyopathy[52].The
currentsurvivalinpediatricrecipients1,5,and10yearsaftertransplantationisapproximately90,80,and60
percent,respectively[46].Earlyreferraltoapediatricheartfailureandtransplantcentershouldbeconsideredto
optimizemedicaltherapyandthetimingoflistingforhearttransplant.Decisiontopursuehearttransplantationis
basedupontheexpectedsurvivalwithmedicaltherapy,qualityoflife,alternativeoptionsfortreatment,and
estimationofsurvivalposttransplantation.(See"Indicationsandcontraindicationsforcardiactransplantation".)
NonpharmacologictherapyforchronicheartfailureNonpharmacologictherapyforchronicheartfailure
includesoptimalnutritionandexerciserehabilitation.
NutritionGrowthfailureiscommonininfantsandchildrenwithheartfailure.Nearlyonethirdofchildren
withcardiomyopathymanifestgrowthfailureduringthecourseoftheirillness[53].Aretrospectiveanalysisof165
childrenwithidiopathicdilatedcardiomyopathyshowedthatnutritionalstatuspositivelyandindependently
correlatedwithsurvival[54].
Caloricintakeandgrowthshouldbecarefullyassessedininfantsandchildrenwithheartfailure.Somechildren
mayneedadailyintakegreaterthan120kcal/kgforoptimalgrowthbecauseofincreasedmetabolicdemandsof
heartfailure.Inordertoprovideadequatecaloricintake,intermittentorcontinuousnasogastricorgastrostomytube
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feedsmayberequired.
Inaddition,saltandfluidrestrictionisrecommendedinchildrenwithsevereheartfailuretoreducevolume
overload.
ExerciserehabilitationAlthoughcardiovascularrehabilitationprogramshavebeenshowntoimprove
exerciseperformance,physicalactivity,andqualityoflifeinadultswithchronicheartfailure[55],datainchildren
arelimited.Inasinglecenterstudy,15of16childrenwithcomplexcongenitalheartdiseasedemonstrated
improvedexerciseperformanceafterparticipationinacardiacrehabilitationprogram[56].Furthermore,astudyof
20hospitalizedchildrenawaitinghearttransplantationdemonstratedthateventhoseoninotropicsupportcan
safelyparticipateinexercisetrainingprogramswithrelativelymoderatetohighcompliance[57].Furtherstudies
areneededtoevaluatethelongtermbenefitsofexerciserehabilitationinchildrenwithheartfailure.
TherapytoreducecomplicationsPatientswithheartfailureareatriskforcomplications,whichincrease
morbidityandmortality.Complicationsincludethromboembolism,arrhythmias,andventriculardyssynchrony.
ThrombiformationChildrenwithheartfailureduetosystemicventriculardysfunctionareatriskforthe
formationofintracardiacthrombi,whichmayresultinpulmonaryembolus,cerebralembolicstrokes,and,insome
cases,death.
Althoughtherearenocontrolledtrialsinchildren,mostexpertsrecommendusingaspirininchildrenwithmoderate
LVdysfunction,andwarfarinorenoxaparinforchildrenwithsevereLVdysfunction.Anticoagulationwithaspirin
shouldalsobeconsideredinthepresenceofmarkedatrialdilationinchildrenwithrestrictivecardiomyopathy.(See
"Antithrombotictherapyinpatientswithheartfailure",sectionon'Roleofantithrombotictherapy'.)
ArrhythmiasInpatientswithdecreasedventricularfunction,sustainedatrialandventricular
tachyarrhythmiascanrapidlyimpairhemodynamics.Inthesepatients,cardioversionordefibrillationmaybe
neededalongwithantiarrhythmictherapy.Ablationtherapyalsohasapotentialroleinthesettingofchronicatrial
tachyarrhythmias.(See"ManagementandevaluationofwideQRScomplextachycardiainchildren"and"Atrial
arrhythmias(includingAVblock)incongenitalheartdisease"and"Managementofsupraventriculartachycardiain
children",sectionon'Radiofrequencyablation'.)
Implantablecardioverterdefibrillatory(ICD)therapyisusedinselectpatientswhoareatriskforsuddencardiac
deathduetoventriculartachycardiaandfibrillation.TheindicationsforICDplacementareabortedsuddencardiac
death,unexplainedsyncope,andrecurrent,sustainedventriculardysrhythmias[58].However,theriskofsudden
deathinchildrenwithendstageheartfailureawaitingtransplantationisonlyabout1percent[59].Unlikeadults,
therearenodatatosupportwhenanICDshouldbeplacedinchildrenwithventriculardysfunction.Risk
stratificationforsuddencardiacdeath(SCD)inchildrenwithdilatedcardiomyopathymaybeusefulinidentifying
childrenwhomaybenefitfromthistherapy[60].(See"Primarypreventionofsuddencardiacdeathinheartfailure
andcardiomyopathy",sectionon'Riskstratificationstrategies'.)
VentriculardyssynchronyIntraventricularconductiondelayorleftbundlebranchblock(LBBB)mayworsen
heartfailurebycausingventriculardyssynchrony.Cardiacresynchronizationtherapy(CRT)usesbiventricular
pacingtominimizeventriculardyssynchronyseeninpatientswithheartfailurewhousuallyhaveaprolongedQRS
onelectrocardiogram.InadultpatientswithLVdysfunction,heartfailure,andLBBB,CRThasbeenshownto
improvehemodynamicsandsymptoms.Pediatricdataarelimitedandincludeamulticenter,retrospectiveanalysis
of103childrenandyoungadultswithcongenitalheartdiseaseandprolongedQRSonelectrocardiogramthat
showedCRTwasassociatedwithimprovementofventricularejectionfractionfrom26to40percent[61].When
CRTisconsideredforapediatricpatient,guidelinesforuseofCRTinadultsareausefulreference[58].(See
"Cardiacresynchronizationtherapyinheartfailure:Indications".)
OURAPPROACHOurmanagementapproachfortreatingpediatricheartfailureisconsistentwiththe2004
InternationalSocietyofHeartandLungTransplantation(ISHLT)publishedguidelines[6].(See'Overview'above.)
Itisbasedontheetiologyoftheheartfailureandtheseverityofheartdisease,asfollows:
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Forpatientswithstructuralheartdiseasewithpreservedventricularpumpfunction,surgicalorcatheterbased
interventionsareusedtocorrecttheunderlyingdefectscausingheartfailure(ie,lefttorightheartshuntsor
ventricularoutflowobstruction).(See'Etiologyandpathophysiology'aboveand'Surgicalorcatheterbased
intervention'above.)
Forpatientswithventricularpumpdysfunctionorthosewhorequirestabilizationbeforesurgicalorcatheter
basedcorrection,therapyisprovidedbasedontheseverityofheartfailure(table2).
StageAPatientsatriskforheartfailurewithnormalcardiacfunctionandsize:Notherapeutic
intervention.
StageBAsymptomaticpatientswithabnormalsystemicventricularfunction:Angiotensinconverting
enzyme(ACE)inhibitors.(See'Angiotensinconvertingenzymeinhibitors'above.)
StageCPatientswithcurrentorpastsymptomsandstructuralorfunctionalheartdisease:ACE
inhibitors,aldosteroneantagonists,betablockers,lowdosedigoxin,andoraldiuretictherapy.(See
'Pharmacologictherapy'above.)
StageDPatientswithendstageheartfailurewhoarerefractorytooralmedicaltherapy:Intravenous
administrationofinotropesanddiuretics.Inaddition,nonpharmacologicinterventionsincludepositive
pressureventilation,mechanicalcirculatorysupport,andsubsequenthearttransplantation.(See'Drug
therapyforadvancedheartfailure'aboveand'Nonpharmacologicinterventionsforadvancedheart
failure'above.)
Identificationandcorrectionofnoncardiacfactorsthatcontributetocardiacdysfunction.Theseinclude
anemia,hypertension,acidosis,renalfailure,andsepsis.
Forchildrenwithchronicheartfailure,nonpharmacologicinterventionsincludeprovisionofadequatecaloric
intakeforgrowth(whichmayentailnasogastricorgastrostomytubefeedings),andanexerciseprogramto
improvephysicalactivitylevel.(See'Nonpharmacologictherapyforchronicheartfailure'above.)
Patientswithheartfailureareatriskforthromboembolicdisease,arrhythmia,andventriculardyssynchrony.
Todecreasetheriskofintracardiacthrombi,weadministerprophylacticaspirintochildrenwith
moderateleftventricular(LV)dysfunction,andwarfarinorenoxaparinforchildrenwithsevereLV
dysfunction.(See'Thrombiformation'above.)
Inpatientswithsustainedatrialorventriculartachyarrhythmias,interventionsincludecardioversion
and/orantiarrhythmictherapyfollowedbyablationtherapyinpatientsdeemedsuitable.Implantable
cardioverterdefibrillatorytherapymaybeconsideredinpatientswhoremainatriskforsuddencardiac
death(SCD)duetoventriculararrhythmias.(See'Arrhythmias'above.)
Inpatientswithventriculardyssynchrony,cardiacresynchronizationtherapymaybeusedtoimprove
ventricularfunction.(See'Ventriculardyssynchrony'above.)
SUMMARYANDRECOMMENDATIONS
Becauseoflimitedpediatricdata,themanagementofchildrenwithheartfailureisprimarilybasedon
evidencefromclinicaltrialsofadultswithheartfailure.(See'Overview'above.)
Thegoalsoftherapeuticinterventionsforchildrenwithheartfailurearetorelievesymptoms,decrease
morbidity(includingtheriskofhospitalization),slowtheprogressionofheartfailure,andimprovepatient
survival.(See'Goalsoftherapy'above.)
Ourmanagementapproachisbasedontheetiologyandseverityofheartfailure,andisconsistentwiththe
2004InternationalSocietyofHeartandLungTransplantation(ISHLT)publishedguidelines.(See
'Managementapproach'aboveand'Ourapproach'above.)
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Forpatientswithstructuralheartdiseaseandpreservedventricularpumpfunction,werecommendsurgicalor
catheterbasedinterventionstocorrecttheunderlyingdefectsresponsibleforheartfailure(ie,lefttoright
heartshuntsorventricularoutflowobstruction)(Grade1A).(See'Etiologyandpathophysiology'aboveand
'Surgicalorcatheterbasedintervention'above.)
Patientswithventricularpumpdysfunctionorthosewhorequirestabilizationbeforesurgicalorcatheter
basedinterventionaretreatedwithacombinationofmedicationsthatalterafterload,preload,andmyocardial
contractility,andmaybeeffectivewithchronicuseinreverseremodelingofthesystemicventricle.Forthese
patients,wesuggestthefollowingapproachbasedontheseverityofheartfailure(table2)(Grade2B):
StageAPatientsatriskforheartfailurewithnormalcardiacfunctionandsize:Notherapeutic
intervention.
StageBAsymptomaticpatientswithabnormalsystemicventricularfunction:Angiotensinconverting
enzyme(ACE)inhibitors,whichdecreaseafterloadbyblockingformationofangiotensinIIandpromote
reversalofventricularremodelingonlongtermuse.(See'Reninangiotensinaldosteronesystem
inhibition'above.)
StageCPatientswithcurrentorpastsymptomsandstructuralorfunctionalheartdisease:ACE
inhibitors,aldosteroneantagonists,betablockers(counteractthemaladaptiveeffectsofchronic
sympathicactivationonthemyocardium),lowdosedigoxin(improvessymptomswithitspositive
inotropiceffectandvagotonicproperties),andoraldiuretictherapy(decreasespreloadbypromoting
natriuresis).(See'Reninangiotensinaldosteronesysteminhibition'aboveand'Betablockers'above
and'Digoxin'aboveand'Diuretics'above.)
StageDPatientswithendstageheartfailurewhoarerefractorytomedicaltherapy:Intravenous
administrationofinotropes(improvemyocardialcontractility)anddiuretics.Inaddition,
nonpharmacologicinterventionsincludepositivepressureventilation,mechanicalcirculatorysupport,
andsubsequenthearttransplantation.(See'Drugtherapyforadvancedheartfailure'aboveand
'Nonpharmacologicinterventionsforadvancedheartfailure'above.)
Inaddition,medicalcareforchildrenwithheartfailureincludesidentificationandcorrectionofnoncardiac
factorsthatcontributetocardiacdysfunctionsuchasanemia,hypertension,renalfailure,andsepsis.(See
'Componentsoftherapy'above.)
Forchildrenwithchronicheartfailure,werecommendprovisionofadequatecaloricintakeforgrowth(which
mayentailnasogastricfeedings)(Grade1B).Wealsosuggestanexerciseprogramtoimprovephysical
activitylevel(Grade2C).(See'Nonpharmacologictherapyforchronicheartfailure'above.)
Additionaltherapeuticinterventionsareusedtopreventortreatcomplicationsassociatedwithheartfailure
suchasintracardiacthrombi,arrhythmias,andventriculardyssynchrony.(See'Therapytoreduce
complications'above.)
UseofUpToDateissubjecttotheSubscriptionandLicenseAgreement.
REFERENCES
1. HsuDT,PearsonGD.Heartfailureinchildren:partI:history,etiology,andpathophysiology.CircHeartFail
20092:63.
2. BaylenBG,JohnsonG,TsangR,etal.Theoccurrenceofhyperaldosteronismininfantswithcongestive
heartfailure.AmJCardiol198045:305.
3. RossRD,DanielsSR,SchwartzDC,etal.Plasmanorepinephrinelevelsininfantsandchildrenwith
congestiveheartfailure.AmJCardiol198759:911.
4. AuerbachSR,RichmondME,LamourJM,etal.BNPlevelspredictoutcomeinpediatricheartfailure
http://www.uptodate.com/contents/managementofheartfailureininfantsandchildren?topicKey=PEDS%2F14520&elapsedTimeMs=11&source=search_re
10/17
14/10/2015
patients:posthocanalysisofthePediatricCarvedilolTrial.CircHeartFail20103:606.
5. SinghTP,SleeperLA,LipshultzS,etal.Associationofleftventriculardilationatlistingforhearttransplant
withpostlistingandearlyposttransplantmortalityinchildrenwithdilatedcardiomyopathy.CircHeartFail
20092:591.
6. RosenthalD,ChrisantMR,EdensE,etal.InternationalSocietyforHeartandLungTransplantation:
Practiceguidelinesformanagementofheartfailureinchildren.JHeartLungTransplant200423:1313.
7. HsuDT,PearsonGD.Heartfailureinchildren:partII:diagnosis,treatment,andfuturedirections.CircHeart
Fail20092:490.
8. SimpsonKE,CanterCE.Canadultheartfailureregimensbeappliedtochildren:whatworksandwhatdoes
not?CurrOpinCardiol201227:98.
9. EngleMA,LewyJE,LewyPR,MetcoffJ.Theuseoffurosemideinthetreatmentofedemaininfantsand
children.Pediatrics197862:811.
10. KantorPF,MertensLL.Clinicalpractice:heartfailureinchildren.PartII:currentmaintenancetherapyand
newtherapeuticapproaches.EurJPediatr2010169:403.
11. PittB,ZannadF,RemmeWJ,etal.Theeffectofspironolactoneonmorbidityandmortalityinpatientswith
severeheartfailure.RandomizedAldactoneEvaluationStudyInvestigators.NEnglJMed1999341:709.
12. PittB,RemmeW,ZannadF,etal.Eplerenone,aselectivealdosteroneblocker,inpatientswithleft
ventriculardysfunctionaftermyocardialinfarction.NEnglJMed2003348:1309.
13. TsutamotoT,WadaA,MaedaK,etal.Effectofspironolactoneonplasmabrainnatriureticpeptideandleft
ventricularremodelinginpatientswithcongestiveheartfailure.JAmCollCardiol200137:1228.
14. ShaddyRE.Optimizingtreatmentforchroniccongestiveheartfailureinchildren.CritCareMed2001
29:S237.
15. PackerM,GheorghiadeM,YoungJB,etal.Withdrawalofdigoxinfrompatientswithchronicheartfailure
treatedwithangiotensinconvertingenzymeinhibitors.RADIANCEStudy.NEnglJMed1993329:1.
16. DigitalisInvestigationGroup.Theeffectofdigoxinonmortalityandmorbidityinpatientswithheartfailure.N
EnglJMed1997336:525.
17. BengurAR,BeekmanRH,RocchiniAP,etal.Acutehemodynamiceffectsofcaptoprilinchildrenwitha
congestiveorrestrictivecardiomyopathy.Circulation199183:523.
18. LewisAB,ChabotM.Theeffectoftreatmentwithangiotensinconvertingenzymeinhibitorsonsurvivalof
pediatricpatientswithdilatedcardiomyopathy.PediatrCardiol199314:9.
19. HsuDT,ZakV,MahonyL,etal.Enalaprilininfantswithsingleventricle:resultsofamulticenter
randomizedtrial.Circulation2010122:333.
20. MoriY,NakazawaM,TomimatsuH,MommaK.Longtermeffectofangiotensinconvertingenzymeinhibitor
involumeoverloadedheartduringgrowth:acontrolledpilotstudy.JAmCollCardiol200036:270.
21. GislerF,KnirschW,HarpesP,BauersfeldU.Effectivenessofangiotensinconvertingenzymeinhibitorsin
pediatricpatientswithmidtosevereaorticvalveregurgitation.PediatrCardiol200829:906.
22. LipshultzSE,LipsitzSR,SallanSE,etal.Longtermenalapriltherapyforleftventriculardysfunctionin
doxorubicintreatedsurvivorsofchildhoodcancer.JClinOncol200220:4517.
23. DubocD,MeuneC,LereboursG,etal.Effectofperindoprilontheonsetandprogressionofleftventricular
dysfunctioninDuchennemusculardystrophy.JAmCollCardiol200545:855.
24. DubocD,MeuneC,PierreB,etal.PerindoprilpreventivetreatmentonmortalityinDuchennemuscular
dystrophy:10years'followup.AmHeartJ2007154:596.
25. LindleKA,DinhK,MoffettBS,etal.Angiotensinconvertingenzymeinhibitornephrotoxicityinneonateswith
cardiacdisease.PediatrCardiol201435:499.
26. ShaddyRE,TaniLY,GiddingSS,etal.Betablockertreatmentofdilatedcardiomyopathywithcongestive
heartfailureinchildren:amultiinstitutionalexperience.JHeartLungTransplant199918:269.
27. BrunsLA,ChrisantMK,LamourJM,etal.Carvedilolastherapyinpediatricheartfailure:aninitial
multicenterexperience.JPediatr2001138:505.
28. AzekaE,FranchiniRamiresJA,VallerC,AlcidesBocchiE.Delistingofinfantsandchildrenfromtheheart
transplantationwaitinglistaftercarvediloltreatment.JAmCollCardiol200240:2034.
29. RusconiP,GmezMarnO,RossiqueGonzlezM,etal.Carvedilolinchildrenwithcardiomyopathy:3year
11/17
14/10/2015
experienceatasingleinstitution.JHeartLungTransplant200423:832.
30. BlumeED,CanterCE,SpicerR,etal.Prospectivesinglearmprotocolofcarvedilolinchildrenwith
ventriculardysfunction.PediatrCardiol200627:336.
31. KalayN,BasarE,OzdogruI,etal.Protectiveeffectsofcarvedilolagainstanthracyclineinduced
cardiomyopathy.JAmCollCardiol200648:2258.
32. KajimotoH,IshigakiK,OkumuraK,etal.Betablockertherapyforcardiacdysfunctioninpatientswith
musculardystrophy.CircJ200670:991.
33. ShaddyRE,BoucekMM,HsuDT,etal.Carvedilolforchildrenandadolescentswithheartfailure:a
randomizedcontrolledtrial.JAMA2007298:1171.
34. O'ConnorMJ,RosenthalDN,ShaddyRE.Outpatientmanagementofpediatricheartfailure.HeartFailClin
20106:515.
35. MoffettBS,PriceJF.Nationalprescribingtrendsforheartfailuremedicationsinchildren.CongenitHeartDis
201510:78.
36. FrobelAK,HulpkeWetteM,SchmidtKG,LerS.Betablockersforcongestiveheartfailureinchildren.
CochraneDatabaseSystRev2009:CD007037.
37. GuazziM,VicenziM,ArenaR,GuazziMD.PDE5inhibitionwithsildenafilimprovesleftventriculardiastolic
function,cardiacgeometry,andclinicalstatusinpatientswithstablesystolicheartfailure:resultsofa1
year,prospective,randomized,placebocontrolledstudy.CircHeartFail20114:8.
38. ReinhardtZ,UzunO,BholeV,etal.SildenafilinthemanagementofthefailingFontancirculation.Cardiol
Young201020:522.
39. SchweigmannU,MeierhoferC.Strategiesforthetreatmentofacuteheartfailureinchildren.Minerva
Cardioangiol200856:321.
40. WesselDL.Managinglowcardiacoutputsyndromeaftercongenitalheartsurgery.CritCareMed2001
29:S220.
41. HoffmanTM,WernovskyG,AtzAM,etal.Efficacyandsafetyofmilrinoneinpreventinglowcardiacoutput
syndromeininfantsandchildrenaftercorrectivesurgeryforcongenitalheartdisease.Circulation2003
107:996.
42. BergAM,SnellL,MahleWT.Homeinotropictherapyinchildren.JHeartLungTransplant200726:453.
43. MahleWT,CuadradoAR,KirshbomPM,etal.Nesiritideininfantsandchildrenwithcongestiveheart
failure.PediatrCritCareMed20056:543.
44. JefferiesJL,PriceJF,DenfieldSW,etal.Safetyandefficacyofnesiritideinpediatricheartfailure.JCard
Fail200713:541.
45. TkacovaR,RankinF,FitzgeraldFS,etal.Effectsofcontinuouspositiveairwaypressureonobstructive
sleepapneaandleftventricularafterloadinpatientswithheartfailure.Circulation199898:2269.
46. KirkR,DipchandAI,EdwardsLB,etal.TheRegistryoftheInternationalSocietyforHeartandLung
Transplantation:fifteenthpediatrichearttransplantationreport2012.JHeartLungTransplant201231:1065.
47. HainesNM,RycusPT,ZwischenbergerJB,etal.ExtracorporealLifeSupportRegistryReport2008:
neonatalandpediatriccardiaccases.ASAIOJ200955:111.
48. AdachiI,FraserCDJr.Mechanicalcirculatorysupportforinfantsandsmallchildren.SeminThorac
CardiovascSurgPediatrCardSurgAnnu201114:38.
49. AdachiI,KhanMS,GuzmnPrunedaFA,etal.Evolutionandimpactofventricularassistdeviceprogram
onchildrenawaitinghearttransplantation.AnnThoracSurg201599:635.
50. BlumeED,NaftelDC,BastardiHJ,etal.Outcomesofchildrenbridgedtohearttransplantationwith
ventricularassistdevices:amultiinstitutionalstudy.Circulation2006113:2313.
51. LortsA,ZafarF,AdachiI,MoralesDL.Mechanicalassistdevicesinneonatesandinfants.SeminThorac
CardiovascSurgPediatrCardSurgAnnu201417:91.
52. CanterCE,ShaddyRE,BernsteinD,etal.Indicationsforhearttransplantationinpediatricheartdisease:a
scientificstatementfromtheAmericanHeartAssociationCouncilonCardiovascularDiseaseintheYoung
theCouncilsonClinicalCardiology,CardiovascularNursing,andCardiovascularSurgeryandAnesthesia
andtheQualityofCareandOutcomesResearchInterdisciplinaryWorkingGroup.Circulation2007115:658.
53. MillerTL,NeriD,ExteinJ,etal.NutritioninPediatricCardiomyopathy.ProgPediatrCardiol200724:59.
12/17
14/10/2015
54. AzevedoVM,AlbanesiFilhoFM,SantosMA,etal.[Theimpactofmalnutritiononidiopathicdilated
cardiomyopathyinchildren].JPediatr(RioJ)200480:211.
55. PiaIL,ApsteinCS,BaladyGJ,etal.Exerciseandheartfailure:AstatementfromtheAmericanHeart
AssociationCommitteeonexercise,rehabilitation,andprevention.Circulation2003107:1210.
56. RhodesJ,CurranTJ,CamilL,etal.Impactofcardiacrehabilitationontheexercisefunctionofchildrenwith
seriouscongenitalheartdisease.Pediatrics2005116:1339.
57. McBrideMG,BinderTJ,ParidonSM.Safetyandfeasibilityofinpatientexercisetraininginpediatricheart
failure:apreliminaryreport.JCardiopulmRehabilPrev200727:219.
58. EpsteinAE,DimarcoJP,EllenbogenKA,etal.ACC/AHA/HRS2008guidelinesforDeviceBasedTherapy
ofCardiacRhythmAbnormalities:executivesummary.HeartRhythm20085:934.
59. RheeEK,CanterCE,BasileS,etal.Suddendeathpriortopediatrichearttransplantation:would
implantabledefibrillatorsimproveoutcome?JHeartLungTransplant200726:447.
60. PahlE,SleeperLA,CanterCE,etal.Incidenceofandriskfactorsforsuddencardiacdeathinchildrenwith
dilatedcardiomyopathy:areportfromthePediatricCardiomyopathyRegistry.JAmCollCardiol2012
59:607.
61. DubinAM,JanousekJ,RheeE,etal.Resynchronizationtherapyinpediatricandcongenitalheartdisease
patients:aninternationalmulticenterstudy.JAmCollCardiol200546:2277.
Topic14520Version7.0
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GRAPHICS
Causesofheartfailureininfantsandchildren
Ventricularpumpdysfunction
Structurallynormalheart
Primarycardiomyopathy
Dilated
Hypertrophic
Restrictive
Noncompaction
Arrhythmogenicrightventriculardysplasia(ARVD)
Secondarycardiomyopathy
Myocarditis
Myocardialinfarction/ischemia
Anomalousleftcoronaryarteryarisingoffthepulmonaryartery(ALCAPA)
Arrhythmogenic
Completeheartblockwithbradycardia
Supraventricularorventriculartachycardia
Drug/toxinexposure
Anthracycline
Noncardiaccauses
Sepsis
Renalfailure
Congenitalheartdisease
Complexcongenitalheartdefectwithconcurrentventriculardysfunction
Complexcongenitalheartdefect,surgicallycorrectedwithlateventriculardysfunction
("burntout"congenitalheartdisease)
Preservedventricularpumpfunction
Volumeoverload(increasedpreload)
Lefttorightshunting
Ventricularseptaldefect
Patentductusarteriosus
Atrialseptaldefect(rare)
Aortopulmonarywindow
Atrioventricularseptaldefect
Singleventriclephysiologywithunobstructedpulmonarybloodflow
Valvularinsufficiency
Aorticregurgitation
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Mitralregurgitation
Pulmonaryregurgitation
Pressureoverload(increasedafterload)
Leftsidedlesions
Aorticstenosis
Aorticcoarctation
Rightsidedlesions
Pulmonarystenosis
Adaptedfrom:HsuDT,PearsonGD.Heartfailureinchildren:partI:history,etiology,and
pathophysiology.CircHeartFail20092:65.
Graphic79989Version3.0
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14/10/2015
Stagesofheartfailureininfantsandchildrenandrecommended
therapy
Stage
Definition
Patientswithincreasedrisk
ofdevelopingHF,butwith
normalcardiacfunction
andchambersize
Example
Exposuretocardiotoxic
agents,familyhistoryof
heritablecardiomyopathy,
univentricularheart,
congenitallycorrected
Therapy
None
transpositionofthegreat
arteries
B
Patientswithabnormal
cardiacmorphologyor
AorticinsufficiencywithLV
enlargement,historyof
ACEinhibitorsforpatients
withsystemicventricular
function,withno
symptomsofHF,pastor
present
anthracyclineexposure
withdecreasedLVsystolic
function
dysfunction
Patientswithstructuralor
Symptomatic
ACEinhibitors,aldosterone
functionalheartdisease,
andpastorcurrent
symptomsofHF
cardiomyopathyor
congenitalheartdefectwith
ventricularpump
dysfunction
antagonistsandbeta
blockersforremodeling
reversallowdosedigoxin
anddiureticsforsymptom
control
PatientswithendstageHF
requiringspecialized
interventions
Markedsymptomsatrest
despitemaximalmedical
therapy
Intravenousdiuretics
and/orinotropes,positive
pressureventilation,
mechanicalcirculatory
support,heart
transplantation
HF:heartfailureLV:leftventricularACEinhibitors:angiotensinconvertingenzymeinhibitors.
From:RosenthalD,ChrisantMR,EdensE,etal.InternationalSocietyforHeartandLung
Transplantation:Practiceguidelinesformanagementofheartfailureinchildren.JHeartLungTransplant
200423:1313.TableusedwiththepermissionofElsevierInc.Allrightsreserved.
Graphic60553Version5.0
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14/10/2015
Disclosures
Disclosures:RakeshKSingh,MD,MSNothingtodisclose.TPSingh,MD,MScNothingtodisclose.JohnKTriedman,MD
Consultant/AdvisoryBoards:BiosenseWebster[Ablation(EPmappingandablationdevices)]BoehringerIngelheim[Anticoagulation
(Dabigatran)].DavidRFulton,MDNothingtodisclose.CarrieArmsby,MD,MPHNothingtodisclose.
Contributordisclosuresarereviewedforconflictsofinterestbytheeditorialgroup.Whenfound,theseareaddressedbyvettingthrougha
multilevelreviewprocess,andthroughrequirementsforreferencestobeprovidedtosupportthecontent.Appropriatelyreferenced
contentisrequiredofallauthorsandmustconformtoUpToDatestandardsofevidence.
Conflictofinterestpolicy
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