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Clinicalmanifestationsanddiagnosisofcoarctationoftheaorta
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:Sep21,2016.
INTRODUCTIONCoarctationoftheaortaisanarrowingofthedescendingaorta,whichistypicallylocatedat
theinsertionoftheductusarteriosusjustdistaltotheleftsubclavianartery(figure1).Thisdefectgenerally
resultsinleftventricularpressureoverload.
Theclinicalmanifestationsanddiagnosisofcoarctationoftheaortawillbereviewedhere.Themanagementof
coarctation,includingcorrectivetreatmentoptionsandoutcome,suchastheriskofrecurrentcoarctation,is
discussedseparately.(See"Managementofcoarctationoftheaorta".)
EPIDEMIOLOGYCoarctationoftheaortaaccountsfor4to6percentofallcongenitalheartdefectswitha
reportedprevalenceofapproximately4per10,000livebirths[1,2].Itoccursmorecommonlyinmalesthanin
females(59versus41percent)[3].Mostcasesaresporadic.
PATHOGENESISANDETIOLOGYAlthoughthereareacquiredcauses,theetiologyofcoarctationofthe
aortaisgenerallycongenital.
CongenitalThevastmajorityofcoarctationcasesarecongenital.Althoughtheprecisepathogenesisis
unknown,thetwomaintheoriesforthedevelopmentofcongenitalcoarctationoftheaortaare:
Reducedantegradeintrauterinebloodflowcausingunderdevelopmentofthefetalaorticarch[4]
Migrationorextensionofductaltissueintothewallofthefetalthoracicaorta[57]
Pathologicexaminationofpatientswithcongenitalcoarctationoftheaortarevealsmedialthickeningandintimal
hyperplasiaatthecoarctationsiteformingaposterolateralridgethatencirclestheaorticlumen.Thereisalso
increasingevidenceofavascularwalldefectintheascendingaortaofindividualswithcongenitalcoarctation.
Reportedaorticwallabnormalitiesinneonateswithcoarctationoftheaortainclude[810]:
IncreasedaorticwallstiffnessanddecreasedaorticdistensibilityInastudythatevaluatedtheelastic
propertiesoftheaorticwall,newbornswithcoarctationoftheaorta,bothbeforeandaftersurgicalrepair,
hadincreasedaorticwallstiffnessanddecreasedaorticdistensibilitycomparedwithneonateswithout
coarctation[8].
Increasedcollagenanddecreasedsmoothmusclemassintheprestenoticcomparedwithpoststenotic
segmentsinpatientswithcoarctationnotedbypathologicanalysisfromsurgicalspecimens[9].
Cysticmedialnecrosishasbeennotedbyhistologicexaminationofsurgicalorautopsyspecimens[10].In
somecases,thefindingswerepresentatornearbirth,suggestingthatthesechangesoccurredinutero.
Theunderlyingmechanismforthesearterialwallabnormalitiesisunknown.Geneticdefectsand/orintrauterine
insultssuchasimpairedbloodflowthatalterendothelialdevelopmentmayplayaroleandresultindisturbances
intheelasticpropertiesandnarrowingoftheaorta.Theintrinsicdefectintheaorticwallappearstopredispose
todissectionorruptureintheascendingaortaorintheareaofthecoarctationrepair.

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GeneticfactorsAgeneticpredispositionissuggestedbyreportsofcoarctationoccurringinfamily
members[1113]andbyitsassociationwithTurnersyndrome.
FamilialriskThereisevidenceofanincreasedfamilialriskforcongenitalleftventricularoutflowtract
(LVOT)obstructionmalformationsincludingcoarctationoftheaorta[11,12].Thiswasshowninastudyof
familymembersof124patientswithLVOTobstruction[11].Thirtyofthe351(9percent)relativeswhowere
evaluatedhadasymptomaticLVOTobstructionstructuralheartdefectsthatincludedabnormalitiesinthe
aorticarch(three),leftventricle(five),andaorticvalve(21).Segregationanalysissuggeststhesefindings
maybetheresultofoneormoreminorlociwithraredominantalleles.
TurnersyndromeApproximately5to15percentofgirlswithcoarctationhaveTurnersyndrome(lossof
anXchromosome)[14,15].MostgirlswithTurnersyndromehaveadditionalassociatedclinicalfindings
(eg,congenitallymphedema,webbedneck,growthfailure,renalanomalies(table1andpicture1))
however,ingirlswithmosaicism,otherclinicalfindingsmaybeabsent[14].Upto30percentofpatients
withTurnersyndromehavecoarctation.GenetictestingforTurnersyndrome(ie,karyotypeanalysis)
shouldthereforebeperformedinfemalepatientsdiagnosedwithcoarctationoftheaorta.(See'Turner
syndrome'belowand"ClinicalmanifestationsanddiagnosisofTurnersyndrome".)
AcquiredInadditiontoacongenitaletiology,aorticnarrowingcanbeanacquiredabnormalitydueto
inflammatorydiseasesoftheaorta,suchasTakayasuarteritisor,rarely,severeatherosclerosis[1618].The
midthoracicorabdominalaortaisoftenthesiteofinvolvementinTakayasu'sarteritis[16,17].(See"Clinical
featuresanddiagnosisofTakayasuarteritis".)
ANATOMY
AnatomicalvariantsAlthoughmostpatientshaveadiscretenarrowingofthedescendingaortaatthe
insertionoftheductusarteriosus,thereisaspectrumofaorticnarrowingthatencompassestheusualdiscrete
thoraciclesions,longsegmentaldefects,tubularhypoplasia,and,rarely,coarctationlocatedintheabdominal
aorta.
OthercardiaclesionsCoarctationoftheaortaisusuallyaccompaniedbyanothercardiaclesion[1921].
Therelativefrequencyofassociatedcardiaclesionsdifferssomewhatbasedupontheageofthepopulation
studied.
Inalargepediatriccaseseriesof1892patientsfromBostonChildren'sHospital,approximatelyonethirdof
patientshadothercomplexcardiacdefects(includingsingleventriclevariants[eg,hypoplasticleftheart
syndrome(figure2)],atrioventricular[AV]canaldefect(figure3),orDtranspositionofthegreatarteries[d
TGA](figure4))18percenthadaventricularseptaldefect(VSD)[20].Inthe806patientswhowereclassified
asuncomplicatedcoarctation,othercardiacabnormalitiesincludedbicuspidaorticvalve,atrialseptaldefector
patentforamenovale,mitralregurgitation,aorticstenosis,aorticregurgitation,andmitralstenosisonly17
percentofuncomplicatedcaseshadnoothercardiacproblems.
Inacaseseriesof216infants(<1yearold),48percentofpatientswerediagnosedwithcomplexcoarctation
duetothepresenceofothercardiacdefectsincludingVSD,aorticandsubaorticstenosis,AVcanaldefect,and
dTGA[21].Theremaining113patientswerediagnosedwithsimplecoarctationhowever,PDAoccurredin48
patients(42percent).Asaresult,approximatelyonethirdofaffectedinfantsdidnothaveanotherdetected
cardiacanomaly.Inthisseries,itisunclearwhetheranevaluationtodetectthepresenceofbicuspidaortic
valvewasperformed.
Inacaseseriesof500primarilyadultpatientsevaluatedwithmagneticresonanceimaging,17percenthadno
additionalcardiovascularanomalies[19].Inthiscohort,bicuspidaorticvalves,archhypoplasia,VSD,andPDA
weredetectedin60,14,13,and7percentofpatients,respectively.
PATHOPHYSIOLOGYCoarctationoftheaortadoesnotcauseahemodynamicprobleminutero,astwo
thirdsofthecombinedcardiacoutputflowsthroughthepatentductusarteriosus(PDA)intothedescending
thoracicaorta,bypassingthesiteofconstrictionattheisthmus(figure5).
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Duringtheneonatalperiod,whenthePDAandforamenovale(betweentherightandleftatria)begintoclose,
thecardiacoutputthatmustcrossthenarrowedaorticsegmenttoreachthelowerextremitiessteadily
increases.Withthesechangesthehemodynamicchangesmayrangefrommildsystolichypertensiontosevere
heartfailuredependingupontheseverityofthecoarctationanduponthepresenceofotherassociatedlesions.
Atbirth,theleftventricularafterloadincreasesbecauseofoutflowtractobstructionresultinginincreased
systolicpressureintheleftventricleandproximalaorta.Incasesofsevereobstruction,thesystolicpressure
gradientmayreach50to60mmHgatrest.
Severalcompensatorymechanismsarisetosurmounttheleftventricularoutflowtractobstruction.These
includeleftventricularmyocardialhypertrophy,whichmaintainsnormalsystolicfunctionandejectionfraction,
andthedevelopmentofcollateralbloodflowinvolvingtheintercostal,internalmammary,andscapularvessels,
whichcircumventsthestenoticlesion[20,21].However,inneonateswithseverelesions,heartfailuremay
developbecausethereisinsufficienttimeforthedevelopmentofmyocardialhypertrophyorcollateralblood
flow.
Othercardiaclesionsmayaddfurtherstrainonventricularfunction.Valvarandsubvalvaraorticstenosismay
furtherincreaseleftventricularsystolicpressureandafterload,whereasalargeventricularseptaldefect,PDA,
ormitralregurgitationwillincreaseleftventricularenddiastolicvolumeandpreload,whichsubsequentlyleads
toincreasedleftventricularenddiastolicpressureandtopulmonaryarteryhypertension.Thesepatientswith
complexcoarctationarelikelytodevelopheartfailureandpulmonaryarteryhypertension.
CLINICALMANIFESTATIONS
ManifestationsaccordingtoageTheclinicalmanifestationsofcoarctationvaryindifferentagegroups.
(See"Clinicalmanifestationsanddiagnosisofpatentductusarteriosusinterminfants,children,andadults".)
NeonatesThenewborninfantmayremainasymptomaticifthereisapersistentpatentductusarteriosus
(PDA)orifthecoarctationisnotsevere.Onphysicalexamination,aclinicaldiagnosisismadeifthereisan
absentordelayedfemoralpulse(whencomparedwiththebrachialpulse).Amurmurmaybeassociatedwith
othercardiacdefects,suchasPDA,aorticstenosis,orventricularseptaldefect(VSD).Asystolicclickmaybe
heardduetoabicuspidaorticvalve.Differentialcyanosisisseeninaneonatewithseverecoarctationofthe
aorta,andwithalargePDAwitharighttoleftshuntintothedescendingthoracicaorta.
Aneonatewithseverecoarctationmaypresentwithheartfailureand/orshockwhenthePDAcloses(image1).
Thesepatientsarepale,irritable,diaphoretic,anddyspneicwithabsentfemoralpulsesand,often,
hepatomegaly.Thepulsesmaybepoorinallfourextremities.Intheclinicalsettingofneonatalshock,important
diagnosestoconsiderincludeseverecoarctation,sepsis,andmetabolicabnormalities.(See"Managementof
coarctationoftheaorta",sectionon'Criticalcoarctationininfancy'and"Heartfailureinchildren:Etiology,clinical
manifestations,anddiagnosis",sectionon'Clinicalmanifestations'and"Inbornerrorsofmetabolism:
Epidemiology,pathogenesis,andclinicalfeatures"and"Inbornerrorsofmetabolism:Metabolicemergencies".)
OlderinfantsandchildrenDiagnosisisoftendelayedinolderinfantsandchildrenbecausephysical
findingsaresubtleandbecausemostpatientsareasymptomatic.Withcarefulhistorytaking,somepatientswill
reportchestpain,coldextremities,andclaudicationwithphysicalexertion.However,theseareoftennotedafter
thediagnosisofcoarctationismade.Carefulmeasurementofbloodpressureandpalpationofpulsesinallfour
extremitiessuggesttheclinicaldiagnosiswithlowersystolicbloodpressureinthelowerextremitiescompared
withupperextremitiesandbrachialorradialarterytofemoralarterypulsedelay.Inyoungchildren,coarctation
oftheaortamaypresentwithhypertensionand/ormurmursresultingfromcollateralsorassociatedheart
defects.Heartfailurerarelyoccursbeyondtheneonatalperiod.
AdultsInpreviouslyundiagnosedadults,theclassicpresentingsignishypertension.Despitethe
variabilityinbloodpressureintheupperandlowerextremities,regionalbloodflowisgenerallymaintained
withinnormallimitsbyautoregulatoryvasoconstrictioninthehypertensiveareasandbyvasodilationinthe
hypotensiveareas[22].Mostpatientsareasymptomaticunlessseverehypertensionispresent,whichmaylead
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toheadache,epistaxis,heartfailure,oraorticdissection.Inaddition,claudicationofthelowerextremitiescan
occurduetoreducedflow,especiallywithphysicalexertion.
NaturalhistoryDataonthenaturalhistoryofcoarctationoftheaortaarelargelyderivedfromhospital
postmortemrecordsandfromcaseseriespriortotheavailabilityofoperativerepair(introducedin1945)[23].
Theaveragesurvivalageofindividualswithunoperatedcoarctationwasapproximately35yearsofage,with75
percentmortalityby46yearsofage[24].Commoncomplicationsinunoperatedpatientsorinthoseoperated
onduringlaterchildhoodoradulthoodweresystemichypertension,acceleratedcoronaryarterydisease,stroke,
aorticdissection,andheartfailure.Causesofdeathincludeheartfailure,aorticrupture,aorticdissection,
endocarditis,endarteritis,intracerebralhemorrhage,andmyocardialinfarction[23,24].Patientswithan
associatedbicuspidaorticvalvemayalsodevelopsignificantaorticstenosis,aorticregurgitation,anddilated
ascendingaortafrommyxomatousdegenerationofthemedialwalloftheaorta.(See"Clinicalmanifestations
anddiagnosisofbicuspidaorticvalveinadults".)
PhysicalfindingsThefindingsofreducedsystolicbloodpressureinthelowerextremitiescomparedwith
upperextremitiesandradialarterytofemoralarterypulsedelaysuggestadiagnosisofcoarctationoftheaorta,
whichisusuallyconfirmedbyechocardiographyoralternateimagingmodalities.(See'Diagnosis'below.)
BloodpressureandpulsesTheclassicfindingsofcoarctationoftheaortaaresystolichypertensionin
theupperextremities,diminishedordelayedfemoralpulses(brachialfemoraldelay),andloworunobtainable
arterialbloodpressureinthelowerextremities(figure6)[25].(See"Evaluationofsecondaryhypertension"and
"Definitionanddiagnosisofhypertensioninchildrenandadolescents",sectionon'TechniqueofBP
measurement'.)
Inourpractice,wefollowtherecommendationsoutlinedbythe2008AmericanCollegeofCardiology
(ACC)/AmericanHeartAssociation(AHA)guidelinesforadultswithcongenitalheartdiseasetoscreenfor
coarctationinbothhypertensivechildrenandadults[24]:
Everypatientwithsystemicarterialhypertensionshouldhavetheradial/brachialandfemoralpulses
palpatedsimultaneouslytoassesstimingandamplitudetosearchforthe"brachialfemoraldelay"of
significantaorticcoarctation(figure6).(See"Examinationofthearterialpulse",sectionon'Unequalor
delayedpulses'.)
Upperandlowerextremitybloodpressuremeasurementshouldalsobeperformedclassicallyitis
suggestedthatsupinebilateralarm(brachialartery)bloodpressuresandpronerightorleftsupineleg
(popliteal)bloodpressuresbemeasuredtosearchfordifferentialpressure.However,inclinicalpracticeit
maynotbefeasibletoobtainpronepoplitealbloodpressure,inthiscaseananklebloodpressuremaybe
analternative.
Thesiteoforiginoftheleftsubclavianarteryandtheseverityofthecoarctationdeterminethepatternofpulse
andbloodpressurefindings:
Inmostcases,theoriginoftheleftsubclavianarteryisproximaltothecoarctation,resultinginhypertension
inbotharms(figure1).
Lessoften,theoriginoftheleftsubclavianarteryisjustdistaltothecoarctation,sotheleftbrachialpulseis
diminishedandisequaltothefemoralpulse.Asaresult,inthissetting,comparingthebloodpressurefrom
theleftarmandlegscanbemisleading,asthecoarctationisproximaltotheoriginoftheleftsubclavian
artery.
Inapproximately3to4percentofcases,boththerightandleftsubclavianarteriesoriginatebelowthearea
ofcoarctation,resultinginthebloodpressuresandpulsesthatareequallydecreasedinallfourextremities.
Inmildcoarctationoftheaorta,allthepulsesmaybeeasilypalpable,buttheremaybeadelayinthe
femoralpulsecomparedwiththebrachialpulse.

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Iftheoriginoftherightsubclavianarteryisanomalouslylocateddistaltothecoarctation,comparingblood
pressuremeasurementsbetweentherightarmandlegmaybemisleadingastheywillbediminishedtoa
similardegree.
Themechanicalobstructiontoflowislargelyresponsiblefortheelevationofbloodpressureintheupper
extremities.Inaddition,renalhypoperfusionmayleadtoenhancedreninsecretionandtosubsequentvolume
expansion[26].Volumeexpansionproducesafurtherelevationinbloodpressure,restoringrenalperfusionand
reninsecretiontowardnormal.
Becauseotherconditionscanalsocauseunequalpulsesandbloodpressures(eg,atheroscleroticdisease,
aorticdissection),thesedisordersshouldalsobeconsideredwhenpulseandbloodpressurediscrepanciesare
found.(See'Differentialdiagnosis'belowand"Examinationofthearterialpulse",sectionon'Unequalor
delayedpulses'.)
Whencoarctationoftheaortaisaccompaniedbysubstantialcollaterals,thefemoralpulsesmaybeless
diminished,andthegradient(differentialsystolicbloodpressures)acrossthecoarctationmaybelesssevere
thanexpectedforthedegreeofobstruction[24].
CardiacexaminationCardiacauscultationmaybenormaliftherearenoassociatedcardiac
abnormalities[25].
Thefirstandsecondheartsoundsareusuallynormal.Rarely,pulmonaryhypertensionispresentwhen
thisoccurs,thepulmoniccomponentofthesecondheartsoundisaccentuated(movie1).(See"Approach
totheinfantorchildwithacardiacmurmur",sectionon'Heartsounds'.)
Theremaybeanejectionsystolicclickandasystolicejectionmurmurfromabicuspidaorticvalve(movie
2),whichisheardbestattheapexorleftsternalborder.(See"Approachtotheinfantorchildwitha
cardiacmurmur",sectionon'Othersounds'and"Auscultationofheartsounds",sectionon'Ejection
sounds'and"Auscultationofcardiacmurmursinadults",sectionon'Midsystolicejectionmurmurs'.)
Asystolicmurmurcanextendbeyondthesecondheartsound,attheleftparavertebralinterscapulararea,
duetoflowacrossthenarrowcoarctationarea.
Continuousmurmursmaybecausedbyflowthroughlargecollateralvessels.(See"Auscultationofcardiac
murmursinadults",sectionon'Continuousmurmurs'.)
Systolicmurmursmaybepresentduetocoexistingcardiacdefects(eg,PDA(movie3),VSD(movie4),or
aorticstenosis(movie5)).
Ininfantswithheartfailure,aprominentrightventricularimpulseistypical.Inolderchildrenandadults,theleft
ventricularimpulseispalpableandsustained,andpulsationsmaybepalpableintheintercostalspacesfrom
largecollateralarteries.(See"Examinationoftheprecordialpulsation"and"Approachtotheinfantorchildwith
acardiacmurmur",sectionon'Palpationofthechest'.)
Noncardiacmanifestations
IntracranialaneurysmsAdultpatientswithcoarctationoftheaortaareatincreasedriskforintracranial
aneurysms(IA)[2730].Severalstudiesusingmagneticresonanceangiography(MRA)orcomputed
tomographyangiographyhavedemonstratedIAinapproximately10percentofadultpatients,whichis
substantiallyhigherthanthe2to3percentriskreportedinthegeneralpopulation.TheriskofIAisgreatestin
olderpatientsandthosewithhypertension[29,30].(See"Unrupturedintracranialaneurysms",sectionon
'Epidemiology'.)
Incontrast,theredoesnotappeartobeanincreasedriskofIAamongchildrenwithcoarctationwhoundergo
treatmentearlyinlife.Inastudyof80childrenwithcoarctation,treatedwithsurgicalorendovascular
interventioninearlychildhood(meanageof2.6years),MRAperformedatmeanageof15.7yearsfoundno
evidenceofIAinanyoftheenrolledpatients[31].
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Inadditiontotheriskofintracranialaneurysms,dilatedcollateralarterieswithinthespinalcanalmay
accompanycoarctation.Thesevesselscancompressthespinalcordorcanrupture,causingaclinicalpictureof
subarachnoidhemorrhage[3234].
TurnersyndromeFemalepatientsshouldbeexaminedforthedysmorphicfeaturesofTurnersyndrome
(see"ClinicalmanifestationsanddiagnosisofTurnersyndrome",sectionon'Clinicalmanifestations'):
Neonatesmayhavecongenitallymphedemaofthehandsandfeet,awebbedneck,andalowhairline.
Oldergirlsandwomenaretypicallyshortwithabroad,shieldshapedchestandwithwidelyspacednipples
(picture1).
GenetictestingforTurnersyndrome(ie,karyotypeanalysis)shouldbeperformedinallgirlsdiagnosedwith
coarctationbecauseofthehighrateofassociation(approximately5to15percentoffemalepatients)and
becauseclinicalfindingssuggestiveofTurnersyndromemaybeabsentingirlswithmosaicism[14,15].(See
"ClinicalmanifestationsanddiagnosisofTurnersyndrome",sectionon'Diagnosis'.)
WomenwithTurnersyndromearegenerallyinfertilehowever,pregnancycanbeachievedthroughoocyte
donation.Comprehensivecardiovascularevaluationshouldbeperformedbeforeinitiatingfertilitytherapy
becausethesepatientsareatincreasedriskofdeathfromruptureordissectionoftheaortaduringpregnancy.
(See"ManagementofTurnersyndrome",sectionon'Managementoffertilityandpregnancy'.)
ECGandXrayfindingsMostpatientswillundergoinitialtestingthatincludeselectrocardiography(ECG)
andchestradiography.However,thediagnosisisgenerallyconfirmedbyechocardiography.Insomecases,
particularlyadultsandthosewithcomplexconditions,magneticresonanceimaging(MRI)orcomputerized
tomographyareusedtoconfirmthediagnosis,delineatethelengthofcoarctation,andhelpplanintervention.
(See'Echocardiography'belowand'Magneticresonanceimaging/computedtomography'below.)
ElectrocardiogramECGvarieswithageofthepatientandseverityofthelesion.Eveninneonatesand
younginfantswithaseveredefect,theECGmaybenormalandmaydisplaytheageappropriateright
ventricularhypertrophy.Sometimesbiventricularhypertrophyisseen.Inolderchildrenandadults,theECG
eithermaybenormalormayshowleftventricularhypertrophy,withincreasedvoltageandSTandTwave
changesintheleftprecordialleads.TheECGwilloccasionallyshowrightventricularconductiondelay[24].
(See"Electrocardiographicdiagnosisofleftventricularhypertrophy".)
ChestradiographThechestradiographvarieswithageandseverityofthecoarctation.
Ininfantswithheartfailure,thechestradiographshowsgeneralizedcardiomegalywithincreased
pulmonaryvascularmarkingsduetopulmonaryvenouscongestion(image1).
Inolderchildrenandadults,theheartsizemaybenormal,butthefollowingabnormalitiesareoften
present:
Notchingoftheposterioronethirdofthethirdtoeighthribsduetoerosionbythelargecollateral
arteries.Ribnotchingincreaseswithageandusuallybecomesapparentbetweentheagesof4and12
years(image2).Notchingisnotseenintheanteriorribsbecausetheanteriorintercostalarteriesare
notlocatedincostalgrooves[25].
Indentationoftheaorticwallatthesiteofcoarctationwithpreandpostcoarctationdilatation,which
canproducea"3"sign(image3).Bariumswallowandcardiacangiogram,whicharenolonger
performedroutinely,mayshowareverse"3"or"E"sign(image4).
DIAGNOSIS
PrenataldiagnosisItischallengingtodetectcoarctationbyantenatalultrasoundbecauseonly10percent
ofthefetalcardiacoutputflowsacrossthedefect(figure5)[3537].Inaddition,thepresenceofthepatent
ductuslimitstheabilitytodetectanypressuregradientatthecoarctationsite,andmayalsomaketheanatomic
narrowinglessmarked.Ifthediagnosisismade,itcanbemadeasearlyasat16to18weeksofgestational
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age.Thepresenceofalongsegmentcoarctationorothercardiacfindings(eg,smallleftventricle,smallmitral
annulus,ordilatedrightventricle)improvesdetection.
Inastudyof90infantswithisolatedcriticalcoarctationborninSwedenbetween2003and2012,onlythree
caseswerediagnosedprenatallydespite97percentofpregnantwomeninSwedenhavingasecondtrimester
ultrasound[38].Theultrasoundassessmentincludedafourchamberviewofthefetalheartinallcases
whereasthepracticeofobtainingviewsoftheoutflowtractwassteadilyintroducedduringthestudyperiod,with
65percentofunitsroutinelyapplyingthispracticebytheendofthe2012.
Antenataldetectionbyechocardiographyappearstobeimprovedbyserialstudiesthatusetransverseviewsof
theaorticandductalarches,whichcomparetheratiooftheaorticandductalarcheswithnormativedata[39].
Fetuseswithcoarctationcomparedwiththosewithoutcoarctationweremorelikelytohavealowerratioofthe
distalaorticisthmustoarterialductdiameters.(See"Fetalcardiacabnormalities:Screening,evaluation,and
pregnancymanagement",sectionon'Advancedfetalcardiacevaluation'.)
PostnataldiagnosisTheclinicaldiagnosisofcoarctationoftheaortaisbaseduponthecharacteristic
findingsofsystolichypertensionintheupperextremities,diminishedordelayedfemoralpulses(brachial
femoraldelay),andloworunobtainablearterialbloodpressureinthelowerextremities.Thediagnosisis
confirmedbynoninvasiveimagingmethods,particularlyechocardiography.
EchocardiographyInmostpatients,highqualitytwodimensionalandDopplerechocardiographycan
establishthediagnosisandseverityofcoarctationoftheaorta,includinginneonateswithapatentductus
arteriosus[24,4042].Echocardiographycanalsodetectassociatedcardiacdefects,includingaortichypoplasia,
andcanbeusedforfollowupafterrepair[40,41].
Inthehighparasternalorsuprasternallongaxisview,adiscreteareaofnarrowing(posteriorshelf)withinthe
lumenoftheproximaldescendingthoracicaortaistypicallyvisualized(image5).ColorandpulsedDoppler
echocardiographycanlocalizetheareaofcoarctationbydemonstratingincreasedvelocitiesandturbulence,as
wellasforwarddiastolicflow.
ContinuouswaveDopplercanestimatetheseverityofcoarctationbaseduponthemaximalflowvelocityacross
thenarrowarea,bycalculatingthepressuregradientacrossthecoarctationwithappropriatecorrectionfor
velocityproximaltothesiteofcoarctation.Theseverityofcoarctationcanalsobeestimatedbycalculatingthe
ratioofthemaximalvelocityacrossthecoarctation(inthesuprasternalview)tothepeakvelocityinthe
abdominalaorta(inthesubcostalview)[40].Ofnote,thepresenceofcollateralbloodflowmaydiminishthe
gradientacrossthecoarctationthegradientmaybelessseverethanexpectedforthedegreeofobstruction
[24].Therefore,indicationsforinterventionarenotbasedsolelyupongradient.(See"Managementof
coarctationoftheaorta".)
Identificationofcharacteristicdiastolicrunoffshouldbeincludedintheroutineevaluationforcoarctation
becauselowamplitude,undulating,continuousDopplerflowwithinthedescendingthoracicaortabelowthe
areaofcoarctationandwithintheabdominalaortaprovidesindirectevidenceofcoarctation.Abnormalflowin
collateralvesselsmayalsobedetectedbycolorflowandpulsedwaveDoppler.PulsedwaveDoppler
assessmentoftheabdominalaortainpatientswithcoarctationgenerallydemonstratesreducedanddelayed
systolicamplitudewithpersistentflowduringdiastole.Thisfindingmaybethefirstcluetotheechocardiographic
diagnosisofcoarctation.
Becausemostpatientswithcoarctationwillalsohaveassociatedcardiacanomalies,echocardiographic
evaluationshouldincludedetailedaorticandcardiacchambermeasurements,identificationofaorticvalve
anatomy,andidentificationofotherpotentialassociatedlesionssuchasventricularseptaldefect,subvalvular
aorticstenosis,andmitralvalvedeformity.
Magneticresonanceimaging/computedtomographyMagneticresonance(MR)orcomputed
tomography(CT)angiographyclearlydefinesthelocationandseverityofcoarctationoftheaorta,aswellas
collateralvessels(image6)[40].MRimaging(MRI)alsomayaccuratelyidentifypatientswithsignificant
pressuregradientacrossthecoarctation[43,44].
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Inchildrenwithcoarctation,echocardiographyoftenprovidesadequateanatomicandhemodynamicinformation
forthesurgeonorinterventionalcardiologistwithouttheneedforafurtherimagingstudy.However,MRIorCT
isgenerallyusedasacomplementarydiagnostictoolinadolescentandadultpatients,andprovidesimportant
anatomicdatapriortointervention.Inthe2008AmericanCollegeofCardiologyandAmericanHeart
Association(ACC/AHA)adultcongenitalheartdiseaseguidelines,itisrecommendedthateveryadultpatient
withcoarctation(repairedornot)shouldhaveatleastonecardiovascularMRIorCTforcompleteevaluationof
thethoracicaorta[24].MRIcanalsodetectassociatedcardiacabnormalitiesandcanbeusedforserialfollow
upaftersurgicalrepairorballoonangioplasty[41].IfMRIiscontraindicated,CTimagingmaybeusedto
diagnosecoarctation.MRIisgenerallypreferredtoCTtodecreasethelifetimeradiationburden.
Inadults,cranialMRangiography(orCTangiography)isalsoappropriatetosearchforintracranialaneurysms.
(See'Intracranialaneurysms'above.)
CardiaccatheterizationGiventheaccuracyofnoninvasivemethodsfordiagnosisanddeterminationof
severity[45],cardiaccatheterizationforanisolatedcoarctationoftheaortainchildrenisgenerallyperformedin
conjunctionwithatherapeuticintervention(eg,stentplacement)(image4andimage7andimage8)[45].
Catheterizationmayalsobenecessarywhencoarctationisassociatedwithcomplexcardiacdefects.
Inadults,cardiaccatheterizationisindicatedwhenassociatedcoronaryarterydiseaseissuspected.
DIFFERENTIALDIAGNOSISThedifferentialdiagnosisforcoarctationvariesbasedupontheclinical
presentation:
Inneonatalheartfailure,severecoarctationshouldbeinthedifferentialdiagnosis.Otherpossiblecauses
includesevereobstructiontooutflowoftheleftheart(eg,hypoplasticleftheartsyndromeandsevere
criticalaorticvalvestenosis),sepsis,myocarditis,andperinatalhypoxia.Echocardiographydistinguishes
thesedisordersfromcoarctation.
Inpatientswithunequalpulsesandbloodpressures,thedifferentialdiagnosisincludesobstructive
peripheralarterialdiseases(eg,fromatherosclerosis,orarterialthrombosisfrompriorcatheterization),
priorsurgicalligation(eg,historyofclassicBlalockTaussigshunt),aorticdissection,andsupravalvaraortic
stenosis.Echocardiographydistinguishescoarctationfromthesedisorders.
INFORMATIONFORPATIENTSUpToDateofferstwotypesofpatienteducationmaterials,"TheBasics"and
"BeyondtheBasics."TheBasicspatienteducationpiecesarewritteninplainlanguage,atthe5thto6thgrade
readinglevel,andtheyanswerthefourorfivekeyquestionsapatientmighthaveaboutagivencondition.
Thesearticlesarebestforpatientswhowantageneraloverviewandwhoprefershort,easytoreadmaterials.
BeyondtheBasicspatienteducationpiecesarelonger,moresophisticated,andmoredetailed.Thesearticles
arewrittenatthe10thto12thgradereadinglevelandarebestforpatientswhowantindepthinformationand
arecomfortablewithsomemedicaljargon.
Herearethepatienteducationarticlesthatarerelevanttothistopic.Weencourageyoutoprintoremailthese
topicstoyourpatients.(Youcanalsolocatepatienteducationarticlesonavarietyofsubjectsbysearchingon
"patientinfo"andthekeyword(s)ofinterest.)
Basicslinks(see"Patienteducation:Aorticcoarctationinadults(TheBasics)"and"Patienteducation:
Aorticcoarctationinchildren(TheBasics)")
SUMMARYANDRECOMMENDATIONS
Coarctationoftheaortaisacommonmalformationaccountingfor4to6percentofallcongenitalheart
defectswithaprevalenceof4per10,000livebirths.(See'Epidemiology'above.)
Coarctationoftheaortaisgenerallycongenitalinorigin.Theunderlyingpathogenesisisunknown.There
maybeageneticpredispositionbaseduponfamilialriskofleftventricularoutflowtractobstructive
malformations,includingcoarctation,andupontheassociationofTurnersyndromewithcoarctation.There
arerareacquiredcauses.(See'Pathogenesisandetiology'above.)
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Thereisaspectrumofanatomicvariantsfromtheusualdiscretethoraciclesionstolongsegmental
defects,tubularhypoplasia,and,rarely,coarctationoftheabdominalaorta.Coarctationoftheaortais
usuallyaccompaniedbyanothercardiaclesionincludingbicuspidaorticvalve,ventricularseptaldefect,or
patentductusarteriosus(PDA).(See'Anatomy'above.)
Thepostnatalpresentationvariesdependingupontheageofthepatientandtheseverityofthelesion.
(See'Manifestationsaccordingtoage'above.)
NeonatesmaybeasymptomaticifthereisapersistentPDAorifthecoarctationisnotsevere.
However,patientswithseveredefectsmaypresentwithheartfailureand/orshockwhenthePDA
closes(image1).
Olderinfantsandchildrenareoftenasymptomaticandpresentwithhypertension,murmurscausedby
collateralbloodfloworassociatedheartdefects,orsymptomsofchestpainorclaudication.
Hypertensionisthetypicalpresentingsigninadults.Claudicationandheadachesmayalsobenotedin
adultswithunrepairedcoarctation.
Theclassicalphysicalfindingsaresystolicbloodpressurewhichislowerinthelowerextremitiescompared
withtheupperextremitiesand/orradial(orbrachial)arterytofemoralarterypulsedelay.(See'Physical
findings'above.)
Everyindividualwithsystemicarterialhypertensionshouldbeassessedforthepresenceofcoarctationby
simultaneouspalpationofthebrachialorradialandfemoralpulsestodetectthepresenceofa"radial
femoraldelay,"andbybilateralarmandlegbloodpressurestodetectalowerextremitybloodpressure
reduction.(See'Bloodpressureandpulses'aboveand"Examinationofthearterialpulse",sectionon
'Unequalordelayedpulses'.)
Adultpatientswithcoarctationoftheaortaareatincreasedriskforintracranialaneurysms.Theriskofis
greatestinolderpatientsandthosewithhypertension.(See'Intracranialaneurysms'aboveand
"Unrupturedintracranialaneurysms".)
GenetictestingforTurnersyndrome(ie,karyotypeanalysis)shouldbeperformedinallgirlsdiagnosedwith
coarctationbecauseofthehighrateofassociation(approximately5to15percentoffemalepatients)and
becauseclinicalfindingssuggestiveofTurnersyndromemaybeabsentingirlswithmosaicism.(See
'Turnersyndrome'aboveand"ClinicalmanifestationsanddiagnosisofTurnersyndrome",sectionon
'Diagnosis'.)
Chestradiographicfindingsvarywithageandseverityofthecoarctation.Ininfantswithheartfailure,the
chestradiographusuallyshowsgeneralizedcardiomegalywithincreasedpulmonaryvascularmarkingsdue
topulmonaryvenouscongestion(image1).Inolderchildrenandadults,theheartsizemayremainnormal,
butotherfindingsincluderibnotchingandthe"3"sign(indentationoftheaorticwallatthesiteof
coarctationwithpreandpostcoarctationdilatation)(image2andimage3).(See'Chestradiograph'
above.)
Theprenataldiagnosisofcoarctationischallengingasitisdifficulttodetectaorticnarrowingbecauseonly
10percentofthefetalcardiacoutputflowsthroughthethoracicaorta.(See'Prenataldiagnosis'above.)
ThediagnosisofcoarctationisgenerallyconfirmedbytwodimensionalandDopplertransthoracic
echocardiography.Inadolescents,adults,andsomepediatriccases,magneticresonanceimaging(MRI)or
computerizedtomography(CT)(image6)isusedasacomplementarydiagnostictool.MRIorCTdefine
thelocationandlengthofobstructionandidentifycollateralvesselsandotherassociatedlesionssuchas
aorticdilatation.(See'Postnataldiagnosis'aboveand"Managementofcoarctationoftheaorta",sectionon
'Monitoring'.)
UseofUpToDateissubjecttotheSubscriptionandLicenseAgreement.
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REFERENCES
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Atlanta,19982005.JPediatr2008153:807.
2.HoffmanJI,KaplanS.Theincidenceofcongenitalheartdisease.JAmCollCardiol200239:1890.
3.ReportoftheNewEnglandRegionalInfantCardiacProgram.Pediatrics198065:375.
4.RudolphAM,HeymannMA,SpitznasU.Hemodynamicconsiderationsinthedevelopmentofnarrowingof
theaorta.AmJCardiol197230:514.
5.WielengaG,DankmeijerJ.Coarctationoftheaorta.JPatholBacteriol196895:265.
6.RussellGA,BerryPJ,WattersonK,etal.Patternsofductaltissueincoarctationoftheaortainthefirst
threemonthsoflife.JThoracCardiovascSurg1991102:596.
7.HoSY,AndersonRH.Coarctation,tubularhypoplasia,andtheductusarteriosus.Histologicalstudyof35
specimens.BrHeartJ197941:268.
8.VogtM,KhnA,BaumgartnerD,etal.Impairedelasticpropertiesoftheascendingaortainnewborns
beforeandearlyaftersuccessfulcoarctationrepair:proofofasystemicvasculardiseaseofthe
prestenoticarteries?Circulation2005111:3269.
9.NiwaK,PerloffJK,BhutaSM,etal.Structuralabnormalitiesofgreatarterialwallsincongenitalheart
disease:lightandelectronmicroscopicanalyses.Circulation2001103:393.
10.IsnerJM,DonaldsonRF,FultonD,etal.Cysticmedialnecrosisincoarctationoftheaorta:apotential
factorcontributingtoadverseconsequencesobservedafterpercutaneousballoonangioplastyof
coarctationsites.Circulation198775:689.
11.McBrideKL,PignatelliR,LewinM,etal.Inheritanceanalysisofcongenitalleftventricularoutflowtract
obstructionmalformations:Segregation,multiplexrelativerisk,andheritability.AmJMedGenetA2005
134A:180.
12.WesselsMW,BergerRM,FrohnMulderIM,etal.Autosomaldominantinheritanceofleftventricular
outflowtractobstruction.AmJMedGenetA2005134A:171.
13.SehestedJ.Coarctationoftheaortainmonozygotictwins.BrHeartJ198247:619.
14.EckhauserA,SouthST,MeyersL,etal.TurnerSyndromeinGirlsPresentingwithCoarctationofthe
Aorta.JPediatr2015167:1062.
15.WongSC,BurgessT,CheungM,ZacharinM.Theprevalenceofturnersyndromeingirlspresentingwith
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16.D'SouzaSJ,TsaiWS,SilverMM,etal.Diagnosisandmanagementofstenoticaortoarteriopathyin
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17.PagniS,DenataleRW,BoltaxRS.Takayasu'sarteritis:themiddleaorticsyndrome.AmSurg1996
62:409.
18.SheikhzadehA,GiannitsisE,GehlHB,etal.Acquiredthromboatheromatouscoarctationoftheaorta:
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19.TeoLL,CannellT,BabuNarayanSV,etal.Prevalenceofassociatedcardiovascularabnormalitiesin500
patientswithaorticcoarctationreferredforcardiovascularmagneticresonanceimagingtoatertiary
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20.KeaneJF,FlyerDC.Coarctationoftheaorta.In:Nadas'PediatricCardiology,2nded,KeaneJF,LockJE,
FylerDC(Eds),SaundersElsevier,Philadelphia2006.p.627.
21.BeekmanRHIII.CoarctationoftheAorta.In:MossandAdams'HeartDiseaseinInfants,Children,and
Adolescents,6thed,AllenHD,DriscollDJ,ShaddyRE,FeltesTF(Eds),WKLippincottWillamsand
Wilkins,Philadelphia2008.Vol2,p.987.
22.TobianLJr.Aviewpointconcerningtheenigmaofhypertension.AmJMed197252:595.
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23.JenkinsNP,WardC.Coarctationoftheaorta:naturalhistoryandoutcomeaftersurgicaltreatment.QJM
199992:365.
24.WarnesCA,WilliamsRG,BashoreTM,etal.ACC/AHA2008GuidelinesfortheManagementofAdults
withCongenitalHeartDisease:areportoftheAmericanCollegeofCardiology/AmericanHeart
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25.BricknerME,HillisLD,LangeRA.Congenitalheartdiseaseinadults.Firstoftwoparts.NEnglJMed
2000342:256.
26.AlpertBS,BainHH,BalfeJW,etal.Roleofthereninangiotensinaldosteronesysteminhypertensive
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27.HODESHL,STEINFELDL,BLUMENTHALS.Congenitalcerebralaneurysmsandcoarctationofthe
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28.ConnollyHM,HustonJ3rd,BrownRDJr,etal.Intracranialaneurysmsinpatientswithcoarctationofthe
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29.CurtisSL,BradleyM,WildeP,etal.Resultsofscreeningforintracranialaneurysmsinpatientswith
coarctationoftheaorta.AJNRAmJNeuroradiol201233:1182.
30.CookSC,HickeyJ,MaulTM,etal.Assessmentofthecerebralcirculationinadultswithcoarctationofthe
aorta.CongenitHeartDis20138:289.
31.DontiA,SpinardiL,BrighentiM,etal.FrequencyofIntracranialAneurysmsDeterminedbyMagnetic
ResonanceAngiographyinChildren(MeanAge16)HavingOperativeorEndovascularTreatmentof
CoarctationoftheAorta(MeanAge3).AmJCardiol2015116:630.
32.BannaMM,RosePG,PearceGW.Coarctationoftheaortaasacauseofspinalsubarachnoid
hemorrhage.Casereport.JNeurosurg197339:761.
33.WatsonAB.Spinalsubarachnoidhaemorrhageinpatientwithcoarctationofaorta.BrMedJ19674:278.
34.ChadduckWM,CatheySL,GearhartAT,etal.Paraplegiacausedbycoarctationoftheaortaand
hydrocephalus.ChildsNervSyst19862:162.
35.HeadCE,JowettVC,SharlandGK,SimpsonJM.Timingofpresentationandpostnataloutcomeofinfants
suspectedofhavingcoarctationoftheaortaduringfetallife.Heart200591:1070.
36.WrenC,ReinhardtZ,KhawajaK.Twentyyeartrendsindiagnosisoflifethreateningneonatal
cardiovascularmalformations.ArchDisChildFetalNeonatalEd200893:F33.
37.BrownKL,RidoutDA,HoskoteA,etal.Delayeddiagnosisofcongenitalheartdiseaseworsens
preoperativeconditionandoutcomeofsurgeryinneonates.Heart200692:1298.
38.LanneringK,BartosM,MellanderM.LateDiagnosisofCoarctationDespitePrenatalUltrasoundand
PostnatalPulseOximetry.Pediatrics2015136:e406.
39.MatsuiH,MellanderM,RoughtonM,etal.Morphologicalandphysiologicalpredictorsoffetalaortic
coarctation.Circulation2008118:1793.
40.TeienDE,WendelH,BjrnebrinkJ,EkelundL.EvaluationofanatomicalobstructionbyDoppler
echocardiographyandmagneticresonanceimaginginpatientswithcoarctationoftheaorta.BrHeartJ
199369:352.
41.GreenbergSB,BalsaraRK,FaerberEN.Coarctationoftheaorta:diagnosticimagingaftercorrective
surgery.JThoracImaging199510:36.
42.LuCW,WangJK,ChangCI,etal.Noninvasivediagnosisofaorticcoarctationinneonateswithpatent
ductusarteriosus.JPediatr2006148:217.
43.NielsenJC,PowellAJ,GauvreauK,etal.Magneticresonanceimagingpredictorsofcoarctationseverity.
Circulation2005111:622.
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44.MuzzarelliS,MeadowsAK,OrdovasKG,etal.Predictionofhemodynamicseverityofcoarctationby
magneticresonanceimaging.AmJCardiol2011108:1335.
45.MarekJ,SkovrnekJ,HucnB,etal.Sevenyearexperienceofnoninvasivepreoperativediagnosticsin
childrenwithcongenitalheartdefects:comprehensiveanalysisof2,788consecutivepatients.Cardiology
199586:488.
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GRAPHICS
Criticalcoarctationoftheaorta

Coarctationoftheaortaisanarrowingofthedescendingaorta.Thenarrowing
typicallyisattheisthmus,thesegmentjustdistaltotheleftsubclavianartery.In
criticalcoarctation,thenarrowingissevereandbloodflowtothedescending
aortaisdependentonapatentductusarteriosus(PDA).WhenthePDAcloses,
neonateswithcriticalcoarctationdevelopheartfailureand/orshock.Onphysical
exam,femoralpulsesareweakorabsent.
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ApproximateincidenceofmajorclinicalabnormalitiesinTurnersyndrome
Frequency
(percent)

Abnormalities

Skeletalgrowthdisturbances
Shortstature

95to100

Growthfailure

90to95

Increaseduppertolow
segmentratio

>90

Defectivedental
development,
malocclusion

upto75

Characteristicfacieswith
micrognathia

Frequency
(percent)

Abnormalities
Otherfeatures
Cardiacmalformations

upto50

Aorticvalveabnormalities(primarily
bicuspidaorticvalve)

15to30

Elongatedtransverseaorticarch

40to50

Coarctationoftheaorta

upto17

Ventricularseptaldefects

1to4

60

Atrialseptaldefects

1to2
8to13

Cubitusvalgus

50

Systemicvenousabnormalities(suchas
persistentleftsuperiorvenacava)

Kyphosis

50

Pulmonaryvenousabnormalities

13to15

Shortneck

40

Renalandrenovascularanomalies

>30

Genuvalgum

35

Hypertension

30

Higharchedpalate

35

Ocularabnormalities

Widelyspacednipples,
broadchest

30to35

Shortmetacarpals

Myopiaorhyperopia

20to50

Strabismus

15to30

35

Amblyopia

>15

Scoliosis

10to20

Ptosis

10to30

Madelungdeformity

Lymphaticobstruction
Lowposteriorhairline

40

Edemaofhands/feet

20to30

Characteristic
dermatoglyphics

30

Webbedneck

25

Earlobeanomalies(eg,
rotated)

15to20

Naildysplasia

10

Germcellchromosomaldefects
Infertility

95

Ovarianfailure

90

Gonadaldysgenesis

85to90

Gonadoblastoma

Earsandhearing

Recurrentotitismedia

50to70

Sensorineuralhearingloss

50(by
adulthood)

Conductivehearingloss

10to40

Cholesteatoma

Skin

Multiplepigmentednevi

25

Vitiligo

Alopecia

Autoimmune
Thyroiditis

15to30

(rateincreaseswithage)
Celiacdisease

Inflammatoryboweldisease

References:
1.GravholtCH,JuulS,NaeraaRW,HansenJ.MorbidityinTurnersyndrome.JClinEpidemiol199851:147.
2.SylvnL,HagenfeldtK,BrndumNielsenK,vonSchoultzB.MiddleagedwomenwithTurner'ssyndrome.Medical
status,hormonaltreatmentandsociallife.ActaEndocrinol(Copenh)1991125:359.
3.LippeB.Turnersyndrome.EndocrinolMetabClinNorthAm199120:121.
4.GtzscheCO,KragOlsenB,NielsenJ,etal.Prevalenceofcardiovascularmalformationsandassociationwith
karyotypesinTurner'ssyndrome.ArchDisChild199471:433.
5.KimHK,GottliebsonW,HorK,etal.CardiovascularanomaliesinTurnersyndrome:spectrum,prevalence,and
cardiacMRIfindingsinapediatricandyoungadultpopulation.AJRAmJRoentgenol2011196:454.
6.MortensenKH,AndersenNH,GravholtCH.CardiovascularphenotypeinTurnersyndromeintegratingcardiology,
genetics,andendocrinology.EndocrRev201233:677.
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ClinicalfeaturesofTurnersyndrome

Elevenyearoldwithclassicalappearanceof45,XTurnersyndrome,including
shortstature,lackofbreastdevelopment,andshieldchestwithwidelyspaced
nipples.Additionalfeaturesmayincludewebbedneck,cubitusvalgus,and
shortenedfourthmetatarsals.
Reproducedwithpermissionfrom:RebarRW,PaupooAAV.Puberty.In:Berekand
Novak'sGynecology,Berek,JS(Ed),Philadelphia:LippincottWilliams&Wilkins,
2012.Copyright2012LippincottWilliams&Wilkins.www.lww.com.
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Spectrumofhypoplasticleftheartsyndrome

Thespectrumofhypoplasticleftheartsyndrome(HLHS).
(PanelA)AorticatresiawithmitralatresiaisthemostextremeformofHLHS.Theleftventricle(LV)is
diminutive.Theascendingaortaandarchareextremelyhypoplastic,andflowisretrograde.Systemic
outputisductaldependent.
(PanelB)Aorticatresiawithapatentmitralvalve.Asinaorticatresia,theascendingaortaandarchare
hypoplasticandallsystemicoutputisductaldependent.Thereisinflowwithoutoutflow.Asaresult,the
leftventricleishypertensivewithhypertrophyandendocardialfibroelastosis.Theleftventricularmass
canbegreaterthannormalandresultindistortionoftheinflowoftherightventricle,resultingin
tricuspidvalveinsufficiency.
(PanelC)Aorticvalvestenosiswithapatentmitralvalve.Theleftventricleishypoplastic,but
antegradeflowthroughtheaorticvalvepersists.Thedegreeofaorticandarchhypoplasiaislessthan
thatobservedwithaorticatresia.ThisendofthespectrumofHLHSblendssmoothlyintocriticalaortic
stenosis,anddecisionmakingconcerningsuitabilityfortwoventriclerepaircanbechallenging.
Reproducedwithpermissionfrom:TweddellJ,HoffmanG,GhanayemN,etal.Hypoplasticleftheart
syndrome.In:MossandAdams'HeartDiseaseinInfants,ChildrenandAdolescents:IncludingtheFetusand
YoungAdult,7thed,AllenH,DriscollD,ShaddyR,etal(Eds),LippincottWilliams&Wilkins,Philadelphia
2008.Copyright2008LippincottWilliams&Wilkins.www.lww.com.
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Differentformsofatrioventricularcanaldefects

Anatomicandphysiologicsimilaritiesbetweenthedifferentformsofatrioventricularseptaldefect
(AVSD)areillustrated.CompleteAVSDshaveoneannuluswithlargeinteratrialand
interventricularcommunications.Intermediatedefects(oneannulus,twoorifices)areasubtype
ofcompleteAVSD.CompleteAVSDshavephysiologyofventricularseptaldefects(VSD)and
atrialseptaldefects(ASD).Incontrast,partialAVSDshavephysiologyofASDs.Transitional
defectsareaformofcompleteAVSDinwhichasmallinsignificantinletVSDispresent,andasa
resultthephysiologyismoresimilartothatofapartialdefect.Partialdefectsandthe
intermediateformofcompleteAVSDshareasimilaranatomicfeature:atongueoftissuedivides
thecommonatrioventericularvalveintodistinctrightandleftorifices.
LA:leftatriumLPV:leftpulmonaryveinLV:leftventricleRA:rightatriumRPV:rightpulmonary
veinRV:rightventricle.
Reproducedwithpermissionfrom:CettaF,MinichLL,EdwardsWD,etal.Atrioventricularseptal
defects.In:MossandAdams'HeartDiseaseinInfants,Children,andAdolescentsIncludingtheFetus
andYoungAdult,7thed,AllenHD,ShaddyRE,DriscollDJ,FeltesTF(Eds),LippincottWilliams&
Wilkins,Philadelphia2007.Copyright2007LippincottWilliams&Wilkins.www.lww.com.
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Diagramofthedextrotypeoftranspositionofthegreat
arteries

Diagramofthedextrotypeoftranspositionofthegreatarteriesshowingthe
abnormalpositionoftheaorta(Ao)andthepulmonaryartery(PA).
Ao:aortaLA:leftatriumLV:leftventriclePA:pulmonaryarteryRA:rightatrium
RV:rightventricle.
Reproducedwithpermissionfrom:MultimediaLibraryofCongenitalHeartDisease,
Children'sHospital,Boston,editorRobertGeggelMD,
www.childrenshospital.org/mml/cvp.
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Fetalcirculation

Thedegreeofoxygensaturationisindicatedbyshading,asexplainedinthefigurekey.
Graphic66765Version4.0

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Chestradiographyofaninfantwithcriticalcoarctationoftheaortaand
heartfailure

Thisisachestradiographofanewborninfantwhopresentedwithrespiratorydistress.ImageAisa
frontalradiographofthechestdemonstratingpulmonaryedemacausedbycriticalcoarctationofthe
descendingaorta,whichwasdiagnosedbyechocardiography.ImageBisthelateralchestradiographfrom
thesamepatient.
CourtesyofLachlanSmith,MD.
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Brachialandfemoralarteryrecordingsfromanadult
withcoarctationofaorta

Tracingsofthepulsepressureofthebrachialandfemoralarteriesdemonstrate
thedelayinthepeaksystolicpressureofthefemoralarterycomparedwiththe
peakpulsepressureinthebrachialartery.
CrawfordMH.Inspectionandpalpationofvenousandarterialpulses.In:
ExaminationoftheHeart.Part2.AmericanHeartAssociationNewYork1990.
Reprintedwithpermission.Copyright1990AmericanHeartAssociation,Inc.
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Ribnotchingcoarctationoftheaorta

Thischestradiographofanadultpatientwithcoarctationoftheaortashowsribnotching.ImageAshows
ribnotchingintheposteriorandinferioraspectsoftherightsidedribsstartingatthethirdribandismost
pronouncedinribs7and8(arrow).ImageBisamagnifiedviewofthenotchingofribs7and8.
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Chestradiographofapatientwithcoarctationoftheaorta

Posterioranterior(PA)chestradiographofapatientwithcoarctationoftheaorta(A)
showssofttissuefullnessintheregionoftheectaticsubclavianartery(arrow)andwith
subtleribnotchingbestappreciatedinthefourthrightrib(arrowhead).Thelateral
examination(B)showsthecharacteristic3signconsistingoftheenlargedanddistorted
aorticknobandsubclavianarteryformingtheupperportionofthe3,thewaistatthesite
ofthecoarctation(arrow),andthelowerportionformedbythepoststenoticdilatationof
theproximaldescendingaorta.
Graphic87857Version2.0

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Aortogramcoarctationshowingaorta"3"sign

Theaortogramshowsthe"3"signconsistingoftheenlargedanddistorted
aorticknobandsubclavianarteryformingtheupperportionofthe3,thewaist
atthesiteofthecoarctation(arrow),andthelowerportionformedbythepost
stenoticdilatationoftheproximaldescendingaorta.
Graphic87858Version1.0

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Transthoracicechocardiographic(TTE)imageswithDopplerassessmentinapatient
withcoarctationoftheaorta

(A)TwodimensionalsuprasternalTTEimagedemonstratingseverenarrowinginthedescendingthoracicaorta(arrow).
(B)ColorflowDopplerdemonstratingmarkedflowaccelerationatthenarrowedsite.
(C)ParasternalshortaxisTTEimageshowingabicuspidaorticvalve(doubleasterisk),whichfrequentlyisseenin
patientswithcoarctation.
(D)PulsedwaveDoppleroftheabdominalaortademonstratingalowantegradesystolicvelocitywithpersistentflowin
diastole(arrow).
(E)ContinuouswaveDopplerdemonstratingsevereobstructionatthesiteofcoarctationwithapeaksystolicgradientof
65mmHgandameangradientof33mmHg.Notepersistentforwardflowduringdiastole,consistentwithpersistent
pressuregradientacrosstheobstruction.
AAO:ascendingaortaDAO:descendingaortaTTE:transthoracicechocardiographic.
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Computedtomographicthreedimensionalreconstructionofcoarctation
oftheaorta

Computedtomographicthreedimensionalvolumerenderedreconstructionofthethoracicaorta
demonstrating:
(A)Severeaorticcoarctation(arrow)ina22yearoldpatientwithhypertensionandanewdiagnosis
ofaorticcoarctation.
(B)Demonstratesextensivecollateralvesselsinthesamepatient.
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Angiogramsofcoarctationsoftheaorta

(A)AngiogramofatwomonthfemaleinfantwithTurnersyndrome.Arrowshowingdiscreteareaof
narrowing.Arrowheadisshowinghypoplasticaorticarch.Dashedarrowisshowingaverysmallpatent
ductusarteriosus(PDA).
(B)Transverseaorticarchangiogramimageina31yearoldmalepatientwhopresentedwith
hypertensionandwasfoundtohaveseverecoarctation.Theangiogramrevealspresenceofsevere
narrowingdistaltotheleftsubclavianartery(arrow).
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Preandpoststentaortogramofapatientwithcoarctationoftheaorta

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

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ContributorDisclosures
BrojendraNAgarwala,MD Nothingtodisclose EmileBacha,MD,FACS Nothingtodisclose QiLingCao,
MD Nothingtodisclose ZiyadMHijazi,MD,MPH,FAAP,FACC,MSCAI,FAHA Consultant/AdvisoryBoards:
NuMEDInc[Coarctationoftheaorta(Angioplastyballoons)]. DavidRFulton,MD Nothingtodisclose HeidiM
Connolly,MD,FASE Nothingtodisclose CarrieArmsby,MD,MPH Nothingtodisclose SusanBYeon,MD,
JD,FACC Nothingtodisclose
Contributordisclosuresarereviewedforconflictsofinterestbytheeditorialgroup.Whenfound,theseare
addressedbyvettingthroughamultilevelreviewprocess,andthroughrequirementsforreferencestobe
providedtosupportthecontent.Appropriatelyreferencedcontentisrequiredofallauthorsandmustconformto
UpToDatestandardsofevidence.
Conflictofinterestpolicy

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