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Managementofcoarctationoftheaorta
Authors: BrojendraNAgarwala,MD,EmileBacha,MD,FACS,QiLingCao,MD,ZiyadMHijazi,MD,MPH,FAAP,FACC,MSCAI,
FAHA
SectionEditors: DavidRFulton,MD,HeidiMConnolly,MD,FASE
DeputyEditors: CarrieArmsby,MD,MPH,SusanBYeon,MD,JD,FACC

Alltopicsareupdatedasnewevidencebecomesavailableandourpeerreviewprocessiscomplete.
Literaturereviewcurrentthrough:Dec2016.|Thistopiclastupdated:May06,2016.
INTRODUCTIONCoarctationoftheaortaisadiscretenarrowingofthethoracicaortajustdistaltotheleftsubclavian
artery.Thecareofapatientwithcoarctationdependsupontheseverityofthecoarctation,patientage,andclinical
presentation.
Themanagementofcoarctationoftheaortaincludingcorrectivetreatmentoptionsandcomplicationswillbereviewedhere.
Theclinicalmanifestations,naturalhistory,anddiagnosisofcoarctationoftheaortaarediscussedseparately.(See
"Clinicalmanifestationsanddiagnosisofcoarctationoftheaorta".)
OVERVIEWManagementdecisionsforpatientswithcoarctationoftheaortadependuponpatientage,presentation,
andtheseverityofthelesion.
CriticalcoarctationininfancyInfantswithsevere("critical")coarctationareatriskfordevelopingheartfailureand
deathwhentheductusarteriosuscloses.Identificationofthesepatientsisessentialinordertomaintainpatencyofthe
ductuspriortosurgicalrepair.Inaddition,immediatetreatmentisrequiredtostabilizepatientswithheartfailure.(See
"Clinicalmanifestationsanddiagnosisofcoarctationoftheaorta",sectionon'Neonates'and"Identifyingnewbornswith
criticalcongenitalheartdisease",sectionon'Clinicalfeatures'.)
Medicaltherapyconsistsofthefollowing:
ContinuousintravenousinfusionofprostaglandinE1(alsoknownasalprostadil)tokeeptheductusarteriosusopen.
Dopamineand/ordobutaminetoimprovecontractilityinthosewithheartfailure.
Supportivecaretocorrectmetabolicacidosis,hypoglycemia,respiratoryfailure,andanemiathatmaycontributetoor
beaconsequenceofheartfailure.
Oncethepatientisstabilized,surgicalrepaircanbeperformed.TheintroductionofprostaglandinE1(alprostadil)has
significantlydecreasedthemortalityinneonateswithcriticalcoarctation[1,2].Inaddition,withtheuseofprostaglandin,itis
rareforaffectedneonatestorequireemergencysurgicalrepairorballoonangioplastyasapalliativeemergencyprocedure.
(See'Neonatesandyounginfants'below.)
Indicationsforinterventionfornoncriticalpresentation
AdultsTheunoperatedmeansurvivalrateofadultswithcoarctationoftheaortais35yearsofage,withamortality
rateof75percentby46yearsofage[3].Systemichypertension,acceleratedcoronaryheartdisease,stroke,aortic
dissection,andheartfailurearecommoncomplicationsinadultswhohavenotundergonecorrectionfortheircoarctationor
wereoperatedonlaterinlife.(See"Clinicalmanifestationsanddiagnosisofcoarctationoftheaorta",sectionon'Adults'.)
In2008,theAmericanCollegeofCardiologyandAmericanHeartAssociation(ACC/AHA)guidelinesforadultswith
congenitalheartdiseaserecommendedinterventionforcoarctationforthefollowingindications[3]:
Peaktopeakcoarctationgradient20mmHg.Thepeaktopeakgradientisameasurementderivedfrom
catheterizationdatainwhichthepeakpressurebeyondthecoarctationissubtractedfromthepeakpressureproximal
tothecoarctation.
Peaktopeakcoarctationgradient<20mmHgwithanatomicimagingevidenceofsignificantcoarctationandradiologic
evidenceofsignificantcollateralflow.
ChildrenIndicationsforinterventioninchildrenincludeheartfailure,apeakinstantaneouspressuregradientacross
thecoarctation>20mmHg,and/orradiologicdetectionofcollateralcirculation[46].Therestinggradientalonemaybean
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unreliableindicatorofseveritywhenthereissignificantcollateralcirculation[4].(See"Clinicalmanifestationsanddiagnosis
ofcoarctationoftheaorta",sectionon'Diagnosis'and"Clinicalmanifestationsanddiagnosisofcoarctationoftheaorta",
sectionon'Olderinfantsandchildren'.)
TimingofrepairSystemichypertension,acceleratedcoronaryheartdisease,stroke,aorticdissection,andheartfailure
arecommoncomplicationsinadultswhohavenotundergonecorrectionfortheircoarctationorwereoperatedonlaterin
life[3].Coarctationrepairafterearlychildhooddoesnotpreventpersistenceorlaterecurrenceofsystemichypertension.
Asaresult,correctionofcoarctationshouldbeperformedininfancyorearlychildhoodtopreventthedevelopmentof
chronicsystemichypertension[7].Ifcoarctationescapesearlydetection,repairshouldbeperformedatthetimeof
subsequentdiagnosis.(See'Systemichypertension'below.)
PROCEDURESOptionsforcorrectionofnativediscretecoarctationoftheaortaincludesurgeryandpercutaneous
interventionsthatincludeballoonangioplastyand/orstentplacement.
SurgeryThetypesofsurgicalrepairofcoarctationinclude:
Resectionwithendtoendanastomosis
Subclavianflapaortoplastyininfantswithlongsegmentcoarctation
Abypassgraftacrosstheareaofcoarctationwhenthedistancetobebridgedistoolongforanendtoendrepair[8]
Prostheticpatchaortoplasty,whichisavoidedwheneverpossiblebecauseofthefrequentoccurrenceofaortic
aneurysmorrupture(image1)[912](see'Aorticaneurysm,dissection,andrupture'below)
Perioperativemortalityincludingneonatesandyounginfantsisrare(usuallylessthan1percent)[3,13,14].Thesurvival
ratewasreportedtobe98percentatamedianfollowupof4.8yearsofageforinfantswhoweresurgicallyrepairedfrom
1996to2006[13].Surgicalrepairispossibleinpreterminfantswithbirthweightslessthan2.5kg,withanoverallsurvival
rateof76percentoneyearafterinitialrepair[15].
Earlymorbidityincludespostoperativeparadoxicalhypertension,leftrecurrentlaryngealnerveparalysis,phrenicnerve
injury,andsubclaviansteal[3].Thelattershouldbesuspectedinpatientswithneurologicsymptomsandreducedperfusion
intheleftarmbecauseofcompromiseoftheorificeoftheleftsubclavianarteryaftersubclavianflapangioplasty[16].
Paraplegiaduetospinalcordischemiaandmesentericarteritiswithbowelinfarctionarerarecomplications.
Reboundhypertension(meanincreaseashighas35mmHg)isdueinitiallytoanincreaseincirculatingcatecholamines
andmaybeaccentuatedbyactivationofthereninangiotensinsystem[17,18].Theseresponsesarelesscommonafter
balloonangioplasty[18].Postoperativeincreaseinbloodpressuremaybepreventedorbluntedbyperioperative
administrationofantihypertensiveagentssuchasNiprideandabetablockerinbothadultandpediatricpatients[3,1921].
Inaddition,manypatientsremainhypertensiveandrequireanoralantihypertensiveagent(s)atdischargeasdiscussed
below[21].(See'Systemichypertension'below.)
Recoarctationisanimportantlongtermcomplicationespeciallyinneonatesandyounginfantsinwhomtheriskof
reinterventionisbetween5and10percent[1315,22].Therateappearstobesimilarwiththedifferentsurgicaltechniques
[23].Recurrenceisusuallyduetoinadequateaorticwallgrowthatthesiteofrepairwhensurgeryisperformedbeforethe
aortahasreachedadultsize.(See'Recoarctation'below.)
Aretrospectivestudyof70childrenwithaorticarchobstructionwhounderwentjump(bypass)graftplacementfromthe
ascendingtothedescendingaortafoundthat,amongpatientswhoreceivedabypassgraftof16mmdiameter,results
weregood(ie,noreoperationsat15years)however,amongneonatesandinfantswhoreceivedsmallerbypassgrafts,
outcomeswerelessfavorable(7earlydeaths,6latedeaths,and7hearttransplantations)[8].Theauthorsconcludedthat
thisprocedureshouldbeavoidedinneonatesandinfants,exceptinspecialcircumstances.
BalloonangioplastyBalloonangioplastyisapercutaneousalternativetosurgicalrepairforolderinfantsandyoung
children(greaterthanfourmonths)withnativediscretecoarctation.Itremainsthepreferredinterventionforallpatientswith
isolatedrecoarctationregardlessofage[3,24,25].However,stentplacementhasreplacedballoonangioplastyasthe
procedureofchoiceinolderchildrenandadultswithnativecoarctation.
NativecoarctationBalloonangioplastyhasbeensuccessfullyperformedinbothchildrenandadultswithdiscrete
nativecoarctation.Thiswasillustratedinalargemulticenterretrospectivestudythatincluded422angioplastyprocedures
fornativeaorticcoarctationinpatientswithamedianageof4.2years(range2daysto63years)from1982to1995[26].
Thefollowingfindingswerenoted:
Therewerethreedeaths,allofthemoccurringincriticallyillinfants(ie,9days,6months,and13months).
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In80procedures(19percent),anacuteoutcomewasdeemedsuboptimal(definedasthepresenceofoneormoreof
thefollowing:residualpeaktopeaksystolicpressuregradient20mmHg,residualratioofproximaltodistalpeak
systolicpressure1.33oramajorcomplication,includingdeath,aortictear,orstroke).Theriskofasuboptimal
outcomewaslowerininstitutionsthatperformedmorethan100angioplastyproceduresforbothrecurrentandnative
coarctation.
Overthedurationofthestudyperiod,balloonangioplastywasincreasinglyusedinthetreatmentofnativecoarctation.
Balloonangioplastyfornativecoarctationissafeandsuccessfulininfantsbetweenoneandsixmonthsofagewithdiscrete
narrowingandnoevidenceofarchhypoplasia[24].Balloonangioplastyisnotassuccessfulininfantswitharchhypoplasia,
whichiscommonlyseeninneonatalandinfantileaorticcoarctation[27,28].Recoarctationoccursinmostneonatesafter
balloonangioplasty,evenafteraninitialgoodresult.Repeatballoonangioplastyisgenerallyrequiredwithin5to12weeks
followingtheinitialprocedure.Asaresult,balloonangioplastyisnotrecommendedforinfantslessthanfourmonthsofage
withaorticcoarctation,especiallyifthelesionisaccompaniedbyarchhypoplasia[24].
Palliativeballoonangioplasty,however,maybeconsideredincriticallyillpatients(irrespectiveofage)whohaveheart
failureduetosevereventriculardysfunction,mitralregurgitation,orlowcardiacoutput,orwithsystemicdisease(suchas
Turnersyndrome)thatisaffectedbycardiacfunction[24].(See'Criticalcoarctationininfancy'above.)
Althoughballoonangioplastywasthetreatmentofchoicefordiscretenativecoarctationinadultsinthepast,mostcenters
currentlyperformstentimplantationforolderchildrenandadultswithnativediscreteorlongsegmentcoarctation.(See
'Stentplacement'below.)
Potentialcomplicationsofballoonangioplastyandtheirfrequencyinclude[5,2938]:
Immediateresidualpressuregradients20mmHg(20percent)
Recoarctation(5to25percent)(see'Recoarctation'below)
Aneurysmformation(5to7percent)(image2andimage3)
Femoralarterialcomplications,includingocclusion(upto15percent)
Aorticdissectionandruptureisararecomplication,asitisnotreportedinmostcaseseries.(See'Aorticaneurysm,
dissection,andrupture'below.)
BalloonangioplastyversussurgeryStudieshaveshownthatballoonangioplastyandsurgicalcorrectionare
equallyeffectiveinreducingthepeaksystolicpressuregradientearlyafterintervention[34,39,40].However,therisksof
recoarctationandaneurysmformationweregreaterinpatientswhounderwentballoonangioplastycomparedwiththose
whoweresurgicallyrepaired.
RecoarctationRecoarctationisacommoncomplicationfollowingeithersurgicalorballoonangioplasty,particularly
wheninitialinterventionisperformedininfantsandyoungchildren.Guidelinesfromanumberofprofessionalsocieties
haverecommendedballoonangioplastywithorwithoutstentingasthepreferredinterventionofpatientswithisolated
recoarctation[3,24,25].(See'Recoarctation'below.)
StentplacementInchildrenandadults,stentplacementafterballoonangioplastyorsurgeryreducesthe
complications,resultsinaminimalresidualgradient,improvesluminaldiameter,andsustainshemodynamicbenefit(image
4)[4146].Childrenwithaorticstentplacementaremorelikelytorequireaplannedreinterventionasthestentoftenneeds
tobedilatedasthechildgrows.
Stentplacementisnotgenerallyrecommendedinpatientslessthan25kgduetopotentialinjurytothefemoralarteryfrom
thelargesheathrequiredforstentdelivery.Inaddition,theaortainpatientslessthan25kgissmall.Althoughstentshave
beensuccessfullyplacedinpatientslessthan30kg,implantingastentinsuchpatientswillcommitthemtorepeated
interventionstoexpandthestentastheygrow[47,48].Therisksassociatedwithrepeattranscatheterinterventionsneedto
bebalancedagainstthemoreinvasivesurgicalrepair.Asaresult,inourpractice,wewillonlyconsiderstentplacementin
patientswhoarelargeenoughtoreceiveastentthatcanbeexpandedtoanadultsize[24].(See'Ourmanagement
approachfornativecoarctation'below.)
IntheCOASTItrial,aprospective,multicenter,singlearmclinicaltrialof105childrenandadults(medianage16years)
whounderwentattemptedstentimplantationfortreatmentofnativeorrecurrentcoarctation,stentplacementwas
successfulin99percent[49].Allpatientsexperiencedimmediatereductioninuppertolowerextremitybloodpressure
differencewithsustainedimprovementtotwoyears.Therewerenodeathsorseriousadverseeventsandnopatients
requiredsurgicalintervention.Duringthefirsttwoyearsafterstentplacement,9patientsrequiredreinterventiontoaddress
aneurysmsorforstentredilation(eitheraspartofanintentionallystagedapproachortocompensateforsomaticgrowth).
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Anadditional10patientsrequiredreinterventionaftertwoyears(sevenforredilationofthestentandthreeforredilation
andtoaddressaneurysms).
Retrospectivedatafromamulticentercaseseriesofover500patientsalsodemonstratedtheefficacyandrelativesafetyof
stentplacementforbothnativeandrecurrentcoarctation[43,44].Inthiscohort,thestentplacement(whichwas
predominantlybaremetal)wassuccessfulin98percentofcases,definedasreductioninthegradientto<20mmHgora
ratioofpoststentcoarctationtodescendingaortaof>0.8.Acutecomplicationsoccurredin14percentofpatientsincluding
twoprocedurerelateddeaths.In41of160procedureswithfollowupimaging(25percent),aorticabnormalitieswere
detectedandincludedstentrestenosis(n=16),aneurysms(n=13),anddissections(n=5).
Althoughthecomplicationrateswithbaremetalstentsinthesestudieswerelow,ithasbeensuggestedthattheuseof
coveredstents,whichconsistofabaremetalstentwithapolytetrafluoroethylenesleeve,mayreducetheriskofaneurysms
[5052].IntheCOASTItrial,9ofthe105patientswhounderwentattemptedstentplacementwithabarestenthad
evidenceofaorticwallinjuryandthereforehadcoveredstentsplaced(eitherattheinitialprocedureorafteronetotwo
years).
However,inarandomizedtrialof120adolescentandadultpatients(meanage23.6years,rangefrom12to58years)with
shortsegmentseverenativecoarctation,therewasnodifferenceintheratesofrecoarctationandpseudoaneurysm
formationafter31monthsoffollowupbetweenpatientswhounderwentimplantationusingabarestentandthosewitha
coveredstent[53].Inbothgroups,stentplacementresultedina100percentsuccessrate,butonlythreequartersof
patientsbecamenormotensiveaftertheprocedure.
Theseresultssuggestthatbarestentsmaybesufficientinmany,ifnotmost,patientsthatundergostentplacementand
thatfurtherresearchisneededtodetermineifthereisasubsetofpatientswhotrulybenefitfromtheimplantationofa
coveredversusbarestent.Followupdatawillalsobeimportanttoseeifthereisalongtermbenefitregardingmaintaining
normalbloodpressureusingcoveredstents.IntheUnitedStates,commerciallymadecoveredstentsarenotapprovedby
theFoodandDrugAdministration,butareavailableforclinicaluseinEuropeandotherpartsoftheworld.IntheUnited
States,coveredstentsareavailableinsomecentersconductingspecificclinicaltrialsandthosethatfashionindividual
coveredstents[54,55].
MANAGEMENTAPPROACH
SocietalguidelinesManagementguidelineshavebeendevelopedbasedonreviewoftheavailableliteraturebythe
AmericanHeartAssociation(AHA),AmericanCollegeofCardiology(ACC),andtheCanadianCardiovascularSocietyfor
adultandpediatricpatientswithcongenitalheartdiseaseincludingcoarctationoftheaorta[3,24,25].Allthreepublished
guidelinesrecommendthatcorrectionofcoarctationshouldbeperformedasearlyaspossible(optimallyearlyinchildhood)
toreducethelongtermmorbidityandimprovesurvival,andcorrectivesurgeryandpercutaneousinterventionsare
acceptableoptionsforcoarctationrepair.Thechoiceofinterventionshouldbedeterminedbyamultidisciplinaryteam(ie,
cardiologist,interventionists,andsurgeons)experiencedintreatingpatientswithcongenitalheartdiseaseandisdependent
ontheunderlyingmorphology,ageofthepatient,andthepresenceorabsenceofothercardiaclesions.Ofnote,allthree
guidelinesobservedthatballoonangioplastywithandwithoutstentinghasincreasinglybeenperformedasbothtechnology
andtheexpertiseofinterventionistshasimproved.
OurmanagementapproachfornativecoarctationCorrectiveintervention(ie,surgeryortranscatheterintervention)
shouldbeperformedinallpatientswithcoarctationwitheitherpeaktopeak(ormaximuminstantaneous)coarctation
gradient20mmHg,ORpeaktopeakcoarctationgradient<20mmHginthepresenceofanatomicimagingevidenceof
significantcoarctationwithradiologicevidenceofsignificantcollateralflow.Ourmanagementapproachisbasedontheage
ofthepatientandisconsistentwiththepediatricAHAguidelines,andtheACC/AHAadultguidelinesforthemanagement
ofnativeandrecurrentcoarctation[3,24].(See'Overview'above.)
NeonatesandyounginfantsInneonateswithcriticalcoarctation,aninfusionwithprostaglandinE1(alprostadil)to
maintainpatencyoftheductusarteriosusshouldbeadministereduntilcorrectivesurgerycanbeperformed.Inourcenters,
wegenerallystartwithadoseof0.025to0.05mcg/kgperminuteandincreaseasneededtoamaximumdoseof0.1
mcg/kgperminute.Forpatientswithheartfailure,administrationofinotropicagentsandgeneralsupportivecare(eg,
intubation,correctionofmetabolicacidosis,andmechanicalventilationforrespiratoryfailure)shouldalsobegiven.
Palliativeballoonangioplastymaybeconsideredtostabilizeacriticallyillpatient[24].(See'Criticalcoarctationininfancy'
above.)
Oncethepatientisstabilized,surgicalcorrectionisrecommended,asitisassociatedwithalowerriskforreintervention
comparedwithballoonangioplasty.(See'Nativecoarctation'above.)

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OlderinfantsandyoungchildrenForyoungerpediatricpatients,surgicalcorrectionhasbeentheprimary
treatmentofnativecoarctationatmostcentershowever,therehasbeenanincreaseduseofballoonangioplastyasnoted
bythe2011AHApediatricguidelinesfortranscatheterinterventionforcongenitalheartdisease[24].Thedecisionbetween
balloonangioplastyversussurgicalrepairisdeterminedbythemultidisciplinaryteam,expertiseofthetertiarycenter,and
theunderlyingmorphologyofthecoarctation.
Ourapproachisconsistentwiththe2011AHApediatricguidelinesasfollows:
Ininfantsandchildrenbetweenfourmonthsandfiveyearsofage(ie,weightbelow25kg),wepreferballoon
angioplastyifthelesionisdiscreteandthereisnoevidenceofarchhypoplasia[24].
Inpatientswithcomplexcoarctationanatomyorsystemicdisease(eg,Turnersyndrome),thedecisiontouseballoon
angioplastyversussurgicalrepairismadeonacasebycasebasis.
OlderchildrenandadultsForlargerpatients(weight>25kg),transcatheterinterventionwithstentinghasbecome
thepreferredinterventionfornativecoarctationinmanytertiarycentersincludingourowninstitutions.(See'Stent
placement'above.)
Weperformstentplacementifthestentcanbeexpandedtoanadultsizeinthefollowingsettings[24]:
Coarctationgradient>20mmHgincludingthosethatarelongsegmentlesions(ie,>10to15mminlength)
Coarctationgradientof<20mmHg,butwithsystemichypertensionduetocoarctationorradiologicevidenceof
significantcollateralflow
Failedballoonangioplastyprocedureduetovesselrecoil
Stentingmaybelesssuccessfulinpatientswithsuboptimalanatomywithvesseltortuosityandtransversearchhypoplasia
[25].Forthesepatients,thedecisiontoperformstentplacementversussurgicalcorrectionmustbemadeonacaseby
casedecisionbytheclinicalteam.
LONGTERMCARDIOVASCULAROUTCOMESMajorlongtermcomplicationsfollowingrepairincluderecoarctation,
aorticaneurysm,andsystemichypertension[56,57].
RecoarctationRecurrentcoarctationreferstorestenosisafteraninitiallysuccessfuldilatationoroperativerepair.The
majorfindingsthatsuggestrecurrentstenosisarerestinghypertensionandheadaches(notusuallyfoundinchildren).In
asymptomaticpatients(4of22inonereview),thediagnosisismadebyscreeningimagingstudies[4].
Therateofrecoarctationisapproximately5to14percentaftersurgery[13,15,22].Itisseenprimarilyinchildrenusually
duetoinadequateaorticwallgrowthatthesiteofrepairwhensurgeryisperformedbeforetheaortahasreachedadult
size.
Childrenarealsoatgreaterriskforrecoarctationcomparedwithadultsfollowingballoonangioplasty.
Inonecaseseriesof93patients(medianage4.6years,range3daysto29years)withaninitialsuccessfulresultafter
balloonangioplasty,restenosisoccurredin21patients(23percent)withreinterventionin18[30].
Incontrast,therewasnorestenosisin49patients(meanage22years,range15to55years)whoweretreated
successfullywithballoonangioplasty[29].However,inanothercohortof87adolescentandadultpatients,sixpatients
(7percent)developedrestenosisthatrequiredintervention[50].
Higherratesofrecoarctationoccurinthefollowingsettings:
Neonates(<30daysofage)andtoalesserextentolderinfants(<1yearofage)[23,30,5863]
Whenthecoarctationsegmentis<3.5mmbeforedilationor<6mmafterangioplasty[60]
Patientswithisthmushypoplasia[30,60]
Managementofrecoarctation
IndicationsforinterventionIndicationsforinterventionforrecoarctationincludehypertension,apeak
instantaneouspressuregradientacrossthecoarctation20mmHg,and/orimagingevidenceofcollateralcirculation
[3,4,24].
PercutaneousinterventionConsistentwiththe2008AmericanCollegeofCardiologyandAmericanHeart
Association(ACC/AHA)adultandthe2011AHApediatricguidelines,percutaneouscatheterinterventionisourpreferred
treatmentofchoice[3,24].
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Severalstudieshaveshownsuccessfulrepairofdiscreterecoarctationusingpercutaneousballoonangioplastyinchildren,
includinginfantsasyoungasthreemonthsofage[6,6470].Mortalityforsurgicalreoperationishigherthanforprimary
repair(1to3percentversuslessthan1percent)andcanbeashighas5to10percentiftherearesignificant
comorbiditiesorleftventriculardysfunction.Forthesereasons,catheterbasedinterventionisrecommendedtotreat
discreterecoarctation.(See'Stentplacement'above.)
Surgicalrepairbyasurgeonwithtrainingandexpertiseincongenitalheartdiseaseisindicatedforrecoarctationwhen
confoundingfeaturesarepresentsuchaslongrecoarctationsegment,hypoplasiaoftheaorticarch,oraorticaneurysmor
pseudoaneurysm[3,4].(See'Aorticaneurysm,dissection,andrupture'below.)
Aorticaneurysm,dissection,andruptureAnaorticaneurysmmaydevelopatthesiteofpriorcoarctationfollowing
surgery(especiallyafterpatchangioplasty),balloondilatation,orstentimplantationofnativecoarctation[912,71,72].
Aneurysmstypicallyoccuratthesiteofpreviousrepair(image2andimage1),butmayalsoaffecttheascendingaorta.
Thereportedincidenceofaorticaneurysmaftersurgicalrepairorballoonangioplastyforcoarctationvarieswidely
[11,12,29,72].
Inareviewof65patientswhounderwentsurgeryforcoarctation,ananeurysmattherepairsitewasdetectedby
magneticresonanceimagingin3of14patientstreatedwithpatchangioplastycomparedwithnopatientsofthe51
whoweretreatedwithresectionandendtoendanastomosis,orasubclavianflapprocedure[11].
Inanotherreport,891patientswereevaluated1to24yearsaftercoarctationrepair48patients(5.3percent)
developedaneurysms.However,mostofthepatientsweretreatedwithpatchangioplasty(n=43),andonlyfour
patientsweretreatedwithendtoendanastomosisandonepatientwithaprostheticgraftreplacement[12].
Inoneretrospectiveseriesof58adultandadolescentpatients(meanage24years)fromSaudiArabia,8percentof
patientsdevelopedaneurysmsoneyearafterballoonangioplasty[29].However,inanotherseriesof29patients(age
range15to71years)withsimilarages,therewasnoaneurysmformationnotedatameanfollowupof8.5yearsafter
balloonangioplasty[73].
Aneurysmformationoccursatandaroundthecoarctationsiteduetoaninherentaorticwallmedialabnormality,whichis
characterizedbyfragmentationofelasticfibers,anincreaseingroundsubstance,andareductioninthenumberofsmooth
musclecells[74].Proposedmechanismsfordevelopingaorticaneurysmand/ordissectionaftercoarctationintervention
includefailureofsurgicalrepairtoremoveabnormaltissueordamagefromballoonangioplasty[75,76].Inaddition,an
abnormalaortarelatedtoacoexistingbicuspidaorticvalvemaypredisposetoascendingaorticaneurysmformationor
dissection[77,78].(See"Clinicalmanifestationsanddiagnosisofbicuspidaorticvalveinadults".)
Riskfactorsforpostrepairaneurysmsareageatthetimeofcoarctationrepair(13.5years)andtheuseofpatch
angioplasty(image1)[11,72,79].Theriskofdissectionisincreasedduringpregnancy,whichisassociatedwith
hemodynamic,physiologic,andhormonalchangessuperimposedonthepreexistingaorticwallmedialchanges.(See
'Pregnancy'below.)
Aorticaneurysmorpseudoaneurysmisgenerallytreatedsurgically.Alternatively,endovascularstentgraftshavebeen
usedtorepairaorticaneurysmsatthesiteofpriorcoarctationrepair.(See"Endovascularrepairofthethoracicaorta"and
"Managementofacuteaorticdissection",sectionon'Endovascularrepair'.)
Inonecaseseriesofsixadultpatientswithaorticaneurysmfollowingcoarctationrepair(agerange31to68years),
placementofastentgraftwassuccessfulinallcaseswithnorelatedmorbidityormortalityatoneyearfollowupafter
intervention[80].
Inasecondcaseseriesofninepatients,endovascularstentingwassuccessfullyperformedwithoutanymajor
complicationsatameanfollowupof24months[81].
SystemichypertensionSystemichypertensionisoneofthemajorlongtermproblemsfollowingrepairofcoarctation
oftheaorta.Althoughthebloodpressuretypicallyfallsaftersuccessfulrepair,persistentorrecurrenthypertensionand
disproportionatesystolichypertensionwithexerciseareobserved,especiallyinpatientswhoserepairisperformedlaterin
life.
Hypertensionandleftventricularhypertrophyareamongthefactorsthatcontributetoprematuredeathfromcoronaryand
cerebrovasculardiseaseinpatientswithasurgicallyrepairedcoarctation[82].Asaresult,itisimportanttocontrolelevated
bloodpressure.Asnotedinthe2008ACC/AHAadultcongenitalheartdiseaseguidelines,hypertensionshouldbe
controlledbybetablockers,angiotensinconvertingenzyme(ACE)inhibitors,orangiotensinreceptorblockers[3].The
choiceofagentsmaybeinfluencedbytheascendingaorticsizeandthepresenceofaorticregurgitation.Inpatientswith
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resistantsystemichypertension,acombinationofdrugsmayberequiredtoeffectivelycontrolbloodpressure.(See'Long
termsurvival'belowand"Nonemergenttreatmentofhypertensioninchildrenandadolescents",sectionon
'Antihypertensivedrugs'and"Overviewofhypertensioninadults",sectionon'Drugtreatment'and"Nonemergenttreatment
ofhypertensioninchildrenandadolescents",sectionon'Pharmacologictherapy'.)
Hypertensionismorecommoninpatientswhoserepairwasperformedafter20yearsofagecomparedwiththosewho
werecorrectedinearlychildhood[29,8385].Inareviewofpatientsundergoingsurgicalrepairduringchildhood,blood
pressurewasnormalin90percentofpatientsatfiveyearfollowup,andfellto50and25percentat20and25yearspost
correction,respectively[84].Incontrast,whenrepairwasperformedinearlyadulthood(meanage22years),hypertension
persistedin37percentofpatients[29],andinpatientsafterage40,approximately50percentremainedhypertensive.In
addition,normotensivepatients,especiallythoserepairedatanolderage,oftenhaveanexaggeratedriseinsystolic
pressureinresponsetoexercise[84,86,87].
Thefactorsresponsibleforthepersistentriskofhypertensionaftercoarctationrepairarenotwellunderstood.Amongthe
probablecontributingfactorsare:
Structuralandfunctionalabnormalitiesthatdecreasecomplianceintheprecoarctationarterialwall[88].
Increasedventricularstiffness,leftventricularhypertrophy,andahypercontractilestateinpostrepairpatients[87,89].
OthercardiovasculardiseaseDespitecoarctationrepair,individualswithaorticcoarctationareatincreasedriskfor
thefollowing:
Coronaryarterydiseaseandstroke[82,90,91]Theincreasedprevalenceofcoronaryarterydiseaseappearsto
becausedbytraditionalcardiovascular(CV)riskfactors,asillustratedbyastudyofpatientsfromtheQuebec
CongenitalHeartDiseaseDatabase[90].Ratesofcoronaryarterydisease,heartfailure,stroke,peripheralvascular
disease,hypertension,andhyperlipidemiawerehigherin756individualswithaorticcoarctation(repairedor
unrepaired)comparedwith6481individualswithventricularseptaldefect.Multivariateanalysisdemonstratedthatthe
traditionalCVriskfactorsofhypertension,hyperlipidemia,diabetesmellitus,malesex,andageindependently
predictedthedevelopmentofcoronaryarterydisease,whereasthediagnosisofcoarctationdidnot.Thesefindings
highlighttheimportanceoftreatingtheassociatedcardiovascularriskfactors(eg,hypertensionanddyslipidemia)in
patientswithcoarctationoftheaortatopreventlateCVsequelae.
BicuspidaorticvalvePatientswithcoarctationoftheaortaalsoareatriskforbicuspidaorticvalvedisease(upto
50percent)[92].Thesepatients,despiterepairofthecoarctation,requirelifelongfollowuptoscreenforaorticvalve
diseasebecauseofincreasedriskforstenosisorinsufficiency,anddilatationoftheirascendingaorta.
CardiacarrhythmiasandsuddencardiacdeathPatientswithcoarctationoftheaortaareatriskforcardiac
arrhythmias,whichmayresultinsuddencardiacdeaths[25,93].
LONGTERMMANAGEMENT
MonitoringAllpatientswithcoarctation(repairedornot)shouldbemonitoredwithlifelongcongenitalcardiologyfollow
upandimagingbecauselongtermsurvivalisreducedcomparedwithnormativepopulationsandthereispotentialneedfor
reintervention(see'Longtermsurvival'below)[57].Followupofadultsshouldincludeevaluationbyorconsultationwitha
cardiologistwithexpertiseinadultcongenitalheartdisease[3].Becausecoarctationoftheaortaisassociatedwithan
increasedriskofintracranialaneurysms(ie,berryaneurysms),cranialimagingwitheithermagneticresonanceimaging
(MRI)orcomputerizedtomography(CT)shouldbeperformedinallpatientswithaorticcoarctationtoevaluateintracranial
vessels[94].
Patientswhohaveundergonesurgicalorpercutaneousinterventionforcoarctationshouldreceiveatleastyearlyclinical
followup.Monitoringshouldincludethefollowing[3]:
Closeobservationfortheappearanceorreappearanceofsystemichypertensionatrestorwithexercise.Hypertension
shouldbetreatedaggressively,andrecoarctationshouldbeexcluded.
ImagingofthecoarctationrepairsitebyMRIorCTtodetectlongtermcomplications(eg,aneurysmsand
recoarctation)shouldbeperformedatintervalsoffiveyearsorlessdependingonthespecificanatomicfindingsbefore
andafterrepair,aswellasclinicalstatus[95].(See'Longtermcardiovascularoutcomes'above.)
Periodicechocardiographytoassessbicuspidaorticvalvefunction,ascendingaorta,andventricularfunction.The
frequencywilldependonassociatedcardiovasculardiseases.

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Theroleofroutineexercisetestingforfollowupinadultsisnotwellestablishedhowever,itshouldbeperformedin
patientswhowishtoparticipateincompetitivesports[3].(See'Competitivesports'below.)
ImagingThechoiceofimagingforroutinefollowupdiffersbetweenadultsandchildren.
Ininfantsandyoungerchildren,acousticwindowspermitexcellentechocardiographicimagingsothatMRIorCTscan
isgenerallynotneeded.EchocardiographyisthediagnostictestofchoicesincesedationisrequiredforadequateMRI
imaginginyoungchildren,andthereisconcernaboutcumulativeradiationexposurewithCTscans.
Inchildren,werecommendechocardiographicassessmentoftheareaofcoarctationwithinsixmonthsfrominterventionor
earlierbasedonclinicalfindings(eg,elevatedbloodpressureandweakfemoralpulses).Iftheinitialimagingis
unremarkable,echocardiographyisperformedonayearlybasisuntilthepatientisoldenoughtocooperateforMRI
imagingwithouttheneedforsedation.AlthoughMRIistypicallythepreferredimagingmodality,asechocardiographyisnot
assensitive[96],MRIgenerallyrequiresconscioussedationinyoungchildren.Asaresult,inmostcenters
echocardiographyisusedinitiallyasascreeningtestuntilthepatientisoldenoughtoundergoMRIimagingwithout
conscioussedation.
Inadults,MRImaybemostcosteffectivefordetectingbothrecoarctationandaneurysmformation[97],andavoids
thecumulativeradiationexposureofmultipleCTscans.
Inadults,werecommendthataMRIbedoneoneyearafterintervention.IftheinitialpostinterventionMRIis
unremarkable,followupMRIcanbedoneeveryfiveyearsorasneededforchangeinclinicalstatus.
EndocarditisprophylaxisEndocarditisprophylaxisisNOTrequiredforpatientswithuncomplicatednativecoarctation
orsixmonthsaftersuccessfulrepairofnativeorrecoarctation.Antibioticprophylaxisisindicatedinpatientswithapast
historyofendocarditis,inthosewhoserepairinvolvedinsertionofaconduit,orforsixmonthsafterinterventionifprosthetic
materialorstentwasused.(See"Antimicrobialprophylaxisforbacterialendocarditis".)
PhysicalactivityDataarelackingontheimpactofexerciseonpatientswithcoarctationoftheaorta.
Recommendationshavebeendevelopedbyexpertpanelstoprovideguidanceinthisarea.
CompetitivesportsThe2015scientificstatementoftheAmericanHeartAssociationandAmericanCollegeof
Cardiology(AHA/ACC)providescompetitiveathleticparticipationguidelinesforpatientswithcongenitalheartdisease
(CHD),includingcoarctation[98].Weagreegenerallywiththeserecommendations,butstressthat,aswithanyguidelines,
recommendationsneedtobetailoredtothepatientandacomprehensiveevaluationbyanexperiencedclinicianis
required.Beforeadecisionismaderegardingsportsparticipation,adetailedevaluationshouldbeconducted,whichshould
includeaphysicalexamination,electrocardiography(ECG),chestradiograph,exercisetesting,andcardiac/aorticimaging
(withtransthoracicechocardiogram,MRI,and/orcomputedtomographyangiography[CTA]).Thetimeintervalforrepeating
thisextensivetestingisunclearandshouldbeindividualizedtothespecificpatient.
Patientswithunrepairedcoarctationcanparticipateinallcompetitivesportsiftheymeetallofthefollowingcriteria:
Normalexercisetest
Restingarm/legsystolicbloodpressuregradient<20mmHg
Peaksystolicbloodpressure95thpercentileofpredictedwithexercise
Nosignificantascendingaorticdilation(Zscore3.0)
Patientswhohaveundergonecoarctationrepair(surgeryortranscatheterintervention)mayparticipateincompetitive
sportsthatdonotposeadangerofbodilycollisionanddonotrequirehighintensitystaticexercise(classesIIIA,IIIB,
andIIIC)(figure1)afterthreemonthsfollowingthecorrectiveprocedure,ifthecriterialistedabovearemetandthere
isnoaneurysmatthesiteofcoarctationinterventionandnosignificantconcomitantaorticvalvedisease.
Patientswithanarm/legsystolicbloodpressuregradient>20mmHgorexerciseinducedhypertension(peaksystolic
bloodpressure>95thpercentileofpredictedwithexercise)orwithsignificantascendingaorticdilation(Zscore>3.0)
mayparticipateonlyinlowintensityclassIAsports(figure1).
Patientswithevidenceofsignificantaorticdilation(Zscore>3.0)oraneurysmformation(notyetofasizerequiring
surgicalrepair)mayparticipateonlyinlowintensity(classesIAandIB)(figure1)sports.
Guidelinesforpatientswithbicuspidaorticvalvewithorwithoutaorticrootdilationarediscussedseparately.(See
"Managementofadultswithbicuspidaorticvalvedisease",sectionon'Physicalactivityandexercise'.)

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RecreationalsportsInourpractice,therecommendationforthelevelofsportparticipationisdependentonthe
clinicalstatusofthepatientsasassessedbyechocardiography,andbloodpressuremeasurementsintheupperandlower
extremities.Ifthelevelofactivityiscomparabletothatrequiredbycompetitiveathletes,anexercisestresstestis
performed.
Patientswithnormalbloodpressure,noresidualcoarctation,andnormalascendingaortadiametercanparticipatein
allactivitieswithoutrestriction(ie,normallifeactivity).
Patientswithresidualcoarctationoradilatedascendingaortaaremanagedonacasebycasebasis.Thedegreeof
narrowingofthecoarctationsegmentanddilatationoftheascendingaortadictatesthelevelofsportactivity.
PhysicalactivityandexerciseinpatientswithCHDarediscussedingreaterdetailseparately.(See"Physicalactivityand
exerciseinpatientswithcongenitalheartdisease(CHD)".)
PREGNANCYCoarctationoftheaortaandassociatedlesions,particularlybicuspidaorticvalve,aorticstenosis,and
ascendingaortadilationshouldbeevaluatedbeforepregnancyforappropriatecounselingandadvice[3].
UnrepairedcoarctationMajorcomplicationsassociatedwithunoperatedaorticcoarctationduringpregnancyare
uncommon,butcanbefatal[99]:
Pregnancyincreasestheriskofaorticruptureordissectionatthesiteofnarrowingorintheascendingaortain
patientswithacoexistingbicuspidaorticvalve.(See"Pregnancyinwomenwithabicuspidaorticvalve".)
Intracranialhemorrhagecanoccur,buthypertensionisnotanecessaryprecondition.
Leftventricularfailureisuncommondespitetheincreasedvolumeloadofpregnancyimposedonthealready
pressureloadedleftventricle[100].
Womenwhopresentwithanunrepairedcoarctationduringpregnancyshouldhaveacarefulassessmentoftheadequacy
ofbloodpressurecontrol.Weallowwomenwhoarenormotensivetocarrythepregnancytotermandpursuerepairofthe
coarctationafewweeksafterdelivery.
Incontrast,ifthebloodpressureisnotwellcontrolledduringpregnancy,interventionisrecommended.Bothsurgeryand
stentplacementduringpregnancytoalleviateobstructionshouldbeconsideredbyamultispecialtyteam.Forstent
placement,potentialteratogenicexposurefromradiationcanbelimitedbyperformingtheprocedureafterthesecond
trimesterwithabdominalshielding.(See'Stentplacement'above.)
RepairedcoarctationMostwomenwithsuccessfulcoarctationrepairhaveanuncomplicatedpregnancy,althoughthe
rateofmiscarriage[99,101,102]andpreeclampsiaarehigherthaninthegeneralpopulation[103].
PregnancyoutcomesAliteraturereviewofstudiespublishedbetween1985and2007foundthefollowingratesof
complicationsduringpregnancies(>20weeksgestation)amongwomenwithcoarctationoftheaorta(combinedrepaired
andunrepaired)[103]:
Withrespecttothemother,cardiaccomplicationswereuncommon.Arrhythmiaoccurredinnopregnancies,heart
failurein3of303pregnancies(1percent),andcardiovascularevents(myocardialinfarction,stroke,and
cardiovascularmortality)in1of304pregnancies(0.3percent).
Preeclampsiawasreportedin12of245pregnancies(4.9percentascomparedwithanexpectedrateof2to3
percent).Pregnancyinducedhypertensionwasreportedin27of244(11.1percentascomparedwithanexpected
rateof5percent).
Withrespecttothefetus,prematuredeliveryoccurredin20of253pregnancies(7.9percent),nofetalmortalityin254
pregnancies,perinatalmortalityin6of254pregnancies(2.4percent),andcongenitalheartdisease(ofanytype)in
theoffspringin10of251pregnancies(4percent).
Outcomeslimitedtowomenwhohadundergonecoarctationrepairwerereportedinaretrospectivestudyof54women
with126pregnancies[102].Therewere98successfulpregnancies,22miscarriages,and6abortions.Duringpregnancy
anddelivery,therewerenoseriouscardiovascularcomplications.Hypertensionalonewasreportedin21pregnanciesin14
women,andpreeclampsiain5pregnanciesin4women.Fiveofeightpatientswhohadserialechocardiographic
assessmenthadanincreaseingradient15mmHg.
Inanotherstudy,seriouscomplicationswereuncommoninwomenwithahemodynamicallysignificantgradient(20
mmHg)afterrepair[99].Thesewomenweremorelikelytohavesystemichypertensionrelatedtotheincreasedcoarctation
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gradient.However,therearecasereportsofaorticruptureordissectionthatoccurwithpregnancyaftercoarctationrepair
duetothehemodynamicandaorticmedialchangesofpregnancy[104].
LONGTERMSURVIVALThelargestsinglecenterseriesdescribinglongtermoutcomeincluded819patients(mean
ageatrepair17.213.6years)whounderwentisolatedoperativerepairofcoarctationatMayoClinicbetween1946and
2005[57].Thesurvivalrateswere93,86,and74percentat10,20,and30yearsafterprimaryrepair,respectively.Inthis
report,thecauseofdeathwasnotknownasnoautopsywasperformedinthemajorityofpatients.Inapreviousreport
fromthiscenter,themostcommoncauseoflatedeathwascoronaryarterydisease,followedbysuddendeath,heart
failure,cerebrovascularaccident,andrupturedaorticaneurysm[82].
Otherreportsdemonstratesimilarsurvivalrates.
DatafromtheUnitedKingdomNorthernCongenitalAbnormalitysurveyofchildrenbornbetween1985and2003
demonstratedanestimated20yearsurvivalrateof90percentforpatientswithcoarctationoftheaorta[105].
AreportfromtheEuropeanHeartSurveyonadultcongenitalheartdiseasedescribed551patientswithcoarctation
90percenthadapreviousrepairwithameanageof26yearsatthebeginningofthestudyperiod[106].Attheendof
thestudy,thefiveyearmortalityratewas0.7percent.
Survivalimproveswithyoungerageatthetimeofprimaryrepair[57,82,84,85,107].IntheMayocaseseries,multivariate
analysisshowedthatolderageatrepairwastheonlyfactorassociatedwithincreasedmortality[57].Similardataonlong
termsurvivalafterpercutaneousinterventionarenotavailable.
INFORMATIONFORPATIENTSUpToDateofferstwotypesofpatienteducationmaterials,"TheBasics"and"Beyond
theBasics."TheBasicspatienteducationpiecesarewritteninplainlanguage,atthe5thto6thgradereadinglevel,and
theyanswerthefourorfivekeyquestionsapatientmighthaveaboutagivencondition.Thesearticlesarebestforpatients
whowantageneraloverviewandwhoprefershort,easytoreadmaterials.BeyondtheBasicspatienteducationpieces
arelonger,moresophisticated,andmoredetailed.Thesearticlesarewrittenatthe10thto12thgradereadinglevelandare
bestforpatientswhowantindepthinformationandarecomfortablewithsomemedicaljargon.
Herearethepatienteducationarticlesthatarerelevanttothistopic.Weencourageyoutoprintoremailthesetopicsto
yourpatients.(Youcanalsolocatepatienteducationarticlesonavarietyofsubjectsbysearchingon"patientinfo"andthe
keyword(s)ofinterest.)
Basicstopics(see"Patienteducation:Aorticcoarctationinadults(TheBasics)"and"Patienteducation:Aortic
coarctationinchildren(TheBasics)")
SUMMARYANDRECOMMENDATIONS
Werecommendcorrectiveinterventionforallpatientswithcoarctationwhofulfilloneofthefollowingcriterion(Grade
1A)(see'Overview'aboveand'Longtermsurvival'above):
Neonateswhopresentwithcriticalcoarctationthatisdependentonductalpatencyforsurvival.
Infant,children,andadultpatientswithcoarctationwithpeaktopeak(ormaximuminstantaneous)gradient20
mmHgoragradient<20mmHgwithanatomicimagingevidenceofsignificantcoarctationandcollateralflow.
Surgeryandballoonangioplastyarebothreasonableoptionstocorrectdiscretecoarctationandshouldbeperformed
inearlychildhoodforoptimalresultsandsurvival.Inourcenters,thefollowingapproachforrepairofdiscreteaortic
coarctationisbasedontheageofthepatientandthemorphologyoftheunderlyinglesion(see'Ourmanagement
approachfornativecoarctation'above):
InfantsyoungerthanfourmonthsofageWerecommendsurgicalcorrectionforinfantswithcoarctationofthe
aorta(Grade1B).Inneonates,werecommendinitialadministrationofacontinuousinfusionofprostaglandinE1
(alprostadil)tomaintainductalpatencyandstabilizethepatientpriortosurgicalrepair(Grade1B).(See
'Neonatesandyounginfants'aboveand'Surgery'above.)
InfantsandchildrenbetweenfourmonthstofiveyearsofageThedecisionregardingballoonangioplastyversus
surgicalrepairisdeterminedbytheexpertiseofthecenterandtheunderlyingmorphologyofthecoarctation.In
ourcenter,balloonangioplastyisthepreferredprocedureifthelesionisdiscreteandthereisnoevidenceofarch
hypoplasia.However,surgicalrepaircontinuestobethepreferredapproachinmanycentersworldwidefor
patientsyoungerthanfiveyearsofage.Inpatientswithcomplexcoarctationanatomyorsystemicdisease(such

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asTurnersyndrome),thedecisiontoperformballoonangioplastyversussurgicalrepairismadeonacaseby
casebasisbythemultidisciplinaryteam.
PatientsolderthanfiveyearsofageIfthepatientislargeenoughtoallowuseofanadultsizestent,wesuggest
transcatheterinterventionwithstentplacementfordiscretecoarctationoftheaorta(Grade2B).Forpatientswith
suboptimalanatomy,thedecisiontoperformstentplacementversussurgicalcorrectionismadeonacaseby
casebasisbytheclinicalteam.
Complicationsfollowingeithersurgeryorpercutaneousinterventionincludesystemichypertension,recoarctation,
aorticaneurysm(image2andimage3andimage1),andaorticdissection.Manypatientswithaorticcoarctationalso
haveabicuspidaorticvalve,whichusuallyrequiressubsequentintervention.(See'Longtermcardiovascular
outcomes'above.)
Forpatientswithrecoarctationwesuggestcatheterintervention(ie,balloonangioplastywithorwithoutstent
placement)withapeaktopeakgradientofatleast20mmHg(Grade2B).Surgicalrepairofrecoarctationisindicated
ifconfoundingfeatures(eg,longcoarctationsegmentorconcomitanthypoplasiaoftheaorticarch)arepresent.(See
'Managementofrecoarctation'above.)
Patientswitheitheruncorrectedorcorrectedaorticcoarctationshouldreceiveregularcardiovascularfollowup
includingbloodpressuremonitoring.
Periodiccranialimagingisrecommendedinpatientswithcoarctationoftheaortatoevaluateintracranialvessels
astheyareatriskforintracranialaneurysms.(See'Monitoring'above.)
Periodicaorticimagingisrecommendedfollowinginterventiontodetectpotentialcomplicationssuchas
recoarctationoraneurysm.
Hypertensionshouldbetreatedaggressively,andthepresenceofrecoarctationshouldbeexcludedbyimaging
studies.
Periodicechocardiographicimagingforassessmentandfollowupofdysfunctionalbicuspidaorticvalve.
Althoughdataarelacking,expertpanelrecommendationsareusedtoguidepatientswithcoarctationregarding
participationincompetitivesportsandphysicalactivity.(See'Physicalactivity'above.)
Pregnancyincreasestheriskofaneurysm,dissection,andintracerebralhemorrhagebothinwomenwhohave
undergonecorrectionandthosewhohavenot.Inaddition,theriskofpreeclampsia,prematurebirth,andmaternal
hypertensionisgreaterinpregnantwomenwithcoarctationcomparedwiththegeneralobstetricalpopulation.
Interventionmaybenecessaryinpregnantwomenwithpoorlycontrolledbloodpressure.(See'Pregnancy'above.)
UseofUpToDateissubjecttotheSubscriptionandLicenseAgreement.
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GRAPHICS
Echocardiographydemonstratingananeurysmatthesiteofsurgicalrepair
forcoarctationoftheaorta

Echocardiographicimagedemonstratingalargeaneurysmofthedescendingthoracicaortaina19year
oldpatientwhounderwentsurgicalrepairwithaGoreTexpatchattwoyearsofage.
%:percentDAO:descending(thoracic)aorta.
Graphic90999Version1.0

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Angiogramoflargeaneurysmafterballoondilation

Anascendingaortaangiogramina20yearoldfemalewhounderwentballoonangioplastyofnative
coarctationsevenyearspriortothisstudy.Thisangiogramrevealsalargeaneurysm(arrow).
Graphic90939Version1.0

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Aneurysmformationandresidualcoarctationafterballoondilationfor
coarctationoftheaorta

(A)Ascendingaortaangiogramdemonstratingaseverecoarctationoftheaortainvolvingtheoriginof
thesubclavianartery(arrow)beforeballoondilation.
(B)Repeatangiogramafterballoondilationofthecoarctationshowingacuteaneurysmformation(arrow)
andresidualcoarctation.
Graphic90940Version1.0

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Preandpoststentaortogramofapatientwithcoarctationoftheaorta

(A)Aortogramdemonstratingcoarctationoftheaortapriortostentplacementina16yearold
patient.Thearrowindicatesthediscretenarrowingoftheaorta.
(B)Thearrowshowsthattheareaofnarrowinghasdisappearedafterstentplacement.
Graphic88831Version1.0

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Classificationofsports

Thisclassificationisbasedonpeakstaticanddynamiccomponentsachievedduringcompetition.Itshouldbenoted,however,that
highervaluesmaybereachedduringtraining.TheincreasingdynamiccomponentisdefinedintermsoftheestimatedpercentofMax
O 2 achievedandresultsinanincreasingcardiacoutput.TheincreasingstaticcomponentisrelatedtotheestimatedpercentofMVC
reachedandresultsinanincreasingbloodpressureload.Thelowesttotalcardiovasculardemands(cardiacoutputandblood
pressure)areshowningreenandthehighestinred.Blue,yellow,andorangedepictlowmoderate,moderate,andhighmoderate
totalcardiovasculardemands.
MaxO 2 :maximaloxygenuptakeMVC:maximalvoluntarycontraction.
*Dangerofbodilycollision.
Increasedriskifsyncopeoccurs.
Reproducedwithpermissionfrom:MitchellJH,HaskellW,SnellP,VanCampSP.TaskForce8:classificationofsports.JAmCollCardiol
200545:1364.Copyright2005AmericanCollegeofCardiologyFoundation.
Graphic64493Version6.0

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ContributorDisclosures
BrojendraNAgarwala,MD Nothingtodisclose EmileBacha,MD,FACS Nothingtodisclose QiLingCao,MD Nothing
todisclose ZiyadMHijazi,MD,MPH,FAAP,FACC,MSCAI,FAHA Consultant/AdvisoryBoards:NuMEDInc[Coarctation
oftheaorta(Angioplastyballoons)]. DavidRFulton,MD Nothingtodisclose HeidiMConnolly,MD,FASE Nothingto
disclose CarrieArmsby,MD,MPH Nothingtodisclose SusanBYeon,MD,JD,FACC Nothingtodisclose
Contributordisclosuresarereviewedforconflictsofinterestbytheeditorialgroup.Whenfound,theseareaddressedby
vettingthroughamultilevelreviewprocess,andthroughrequirementsforreferencestobeprovidedtosupportthe
content.AppropriatelyreferencedcontentisrequiredofallauthorsandmustconformtoUpToDatestandardsofevidence.
Conflictofinterestpolicy

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