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23May2011
PeerapatThaisiam
YossavadeeRuamcharoen
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• IncidenceofmoderateandsevereformsofCHDis6per1000livebirths
• Includebicuspidaorticvalves->19per1000livebirths
• Withoutearlymedicalorsurgicaltreatment,themajorityofpatientswithcomplexCHDwouldnotsurvivetoadulthood
• Resultfromalteredembryonicdevelopmentofanormalstructure
• Genderpreponderance
• PDA,Ebsteinanomaly,ASD:morecommoninfemales• Aorticvalvestenosis,coarctationoftheaorta,hypoplasticleft
heartsyndrome,pulmonaryandtricuspidatresia,andTGA:morecommoninmales
• Extracardiacanomaliesoccurinabout25%
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• Geneticdisorder
• Singlegenemutation:familialformsofASDwithprolongedAVconduction;mitralvalveprolapse;VSD;congenitalheartblock;situsinversus;pulmonaryhypertension;andthesyndromesofNoonan,LEOPARD,Ellis–vanCreveld,andKartagener
• Severaldefects:long-QTsyndrome,Holt-Oramsyndrome,Marfansyndrome,HCMsupravalvularaorticstenosis
• <15%ofallcardiacmalformations:chromosomalaberrationsorgeneticmutationsortransmission
• Environmentaltoxin• Maternalrubella,thalidomideandisotretinoinearlyduringgestation,
andchronicmaternalalcoholabuse
• Multifactorialgeneticandenvironmental
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• Hemodynamics-CHF
• Cyanosis• Pulmonaryhypertension-Eisenmenger
• Infectiveendocarditis• Cardiacarrhythmia-assopreviously
repairedCHD
• Pregnancy
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• Progressivemyocardialdysfunction:pressureorvolumeoverload
• Presentwithleft,right,biventricular,oruniventricularfailure
• Intrinsicmyocardialabnormalities:restrictivediastolic
• Chroniclowcardiacoutputstate,• Volumeoverload,
• Congestivehepatopathy,ascites,andproteinlosingenteropathy
• CHFisnotcommoninadultcongenitalheart
• Presenceofasubstrate:myocardialdysfunction,valvular
regurgitation• Precipitant:sustainedarrhythmia,pregnancy,hyperthyroidism
• Renin–angiotensinsystem:NOTplayarole
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• Arterialoxygendesaturation:shuntingormixingofsystemicvenousbloodintothearterialcirculation
• Clinicalfeatures
• Hyperviscositysyndrome
• Hematologic
• Bleedingtendency:Elevatedprothrombinandpartialthromboplastintimes,decreasedfactorlevels(factorsV,VII, VIII,andIX),qualitativeandquantitativeplateletdisorders,increasedfibrinolysis,andsystemicendothelialdysfunction
• Neurological:cerebralhemorrhage,paradoxicalemboli
• Renal:proteinuria,hyperuricemia,orrenalfailure
• Rheumatologic:hypertrophicosteoarthropathy
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• Pulmonaryvascularobstructivedisease:consequenceofalargepreexistingleft-to-rightshunt
• Exerciseintolerance• 3rddecade:slowlyprogressivedeclinein
theirphysicalabilities
• After40yr:CHF
• Mostcommonmodesofdeatharesuddendeath,CHF,andpulmonaryhemorrhage
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• HIGHrisk:
• prostheticvalves,cyanosis,systemicorpulmonaryarteryconduits,andpreviousendocarditis
• MODERATErisk:otherCHD
• LOWrisk
• surgicalrepairofASD,VSD,PDA(withoutresidua>6months
• Onlymod-to-highrisk:needATBprophylaxisfordental,respiratory,genitourinary,orgastrointestinalprocedures
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• Arrhythmiasandconductiondefects
• SND:extensiveatrialreconstruction• Atrialtachyarrhythmia:
• Atrioventricularvalvediseaseorshunts• Scar-mediatedreentrantatrialflutter
• Ventriculartachyarrhythmia:afterTOFrepair
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• Pregnancycounselingismandatoryforallpatients
• Obstructivelesionsarelesswelltoleratedthanregurgitantlesionsorshunts
• Heartfailureandarrhythmiaaremajorcomplications
• Theincidenceofearlyspontaneousabortionisproportionaltotheseverityofcyanosis
• Tissueabnormalitiesoftheaorta:increaseriskruptureor
dissection• Prostheticvalves:teratogenic
• Vaginaldelivery
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� Lefttorightshunt� Atrialseptaldefects� Patentforamenovale
� Atrioventricularseptaldefect� Ventricularseptaldefect� Patentductusarteriosus
� Cyanoticheartdisease� TetralogyofFallot� TricuspidAtresia� HypoplasticLeftHeartSyndrome� Double-InletLeftVentricle� TotalAnomalousPulmonaryVenousConnection
� TranspositionComplexes� EbsteinAnomaly
� LVOT� Coarctationoftheaorta
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• Ostiumsecundumdefects
• Excessiveresorptionoftheseptumprimumor
• Deficientgrowthoftheseptumsecundum
• Sinusvenosus–IVCdefectsareveryuncommon
• PATHOPHYSIOLOGY
• Degreeofshunting:sizeofthedefectandtherelativediastolicfilling2ventricles
• Increaseshunt:anyconditioncausing
• reducedleftventricularcompliance(e.g.,systemichypertension,car-diomyopathy,myocardialinfarction)or
• increasedleftatrialpressure(mitralstenosisorregurgitation)
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• Naturalhistory
• largeASD=Qp/Qs>2.0:1.0,CHFandFTT
• sig.ASD=Qp/Qs>1.5:1.0,symptomsinadolescenceoradulthood
• Effortdyspnea:30%in3rddecade,>75%in5thdecade
• Supraventriculararrhythmiasandright-sidedHF:40years
• Paradoxicalembolism
• Pulmonaryhypertension
• Clinicalfeatures
• Exerciseintoleranceandpalpitations(typicallyfromAflutter, Afibrillation,orSSS)
• Rightventricularfailure:presentingsymptominolder
• Cyanosis:shuntreversalandEisenmengersyndrome
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• “leftatrialization”ofJVP(Awave=Vwave)
• Hyperdynamicrightventricles
• WideandfixedsplitofS2ishallmark
• SEM,usuallygr.2:2ndleftICS,andamid-diastolicrumbleLLPSB,fromincreasedflowthroughthetricuspidvalve,
• PSMofTR:Rt.ventricularfailure
• ELECTROCARDIOGRAPHY.
• SinusrhythmorAflutterorAfibrillation
• TheQRSaxisistypicallyrightwardinsecundumASD
• NegativePwavesintheinferiorleads->lowatrialpacemaker->sinusvenosus–superiorvenacavadefects
• CompleteRBBB
• TallRorR ′wavesinV1oftenindicatepulmonaryhypertension.
• CHESTRADIOGRAPHY.
• CardiomegalyRAE,RVH
• Prominentpulmonarytrunkandincreasepulmonaryflow
• Smallaorticknuckle
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• Presenceofright-sidedheartdilationandasignificantASD(>5mm)
• Qp/Qs<1.5donotrequireclosure,withthepossibleexceptionoftryingtopreventparadoxicalemboliinolderpatientsafterastroke
• “Significant”ASDs(Qp/Qs>1.5,orASDsassociatedwithrightventricularvolumeoverload)
• pulmonaryhypertension:netleft-to-rightshuntofatleast1.5:1orevidenceofpulmonaryarteryreactivityonchallengewithapulmonaryvasodilator
• DEVICECLOSURE
• onlyforsecundumASDwithastretcheddiameterof<41mmandwithadequaterimstoenablesecuredeploymentofthedevice
• majorcomplications<1%andclinicalclosureachievedin>90%
• SURGERY
• sinusvenosusorostiumprimumdefects
• secundumdefectswithunsuitableanatomy
• Afterdeviceclosure,6moofaspirinandendocarditisprophylaxis
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• Tunnel-likespacebetweentheoverlyingseptumsecundumandseptumprimum
• Typicallyclosesin75%atbirth
• Associatedwith
• atrialseptalaneurysms
• eustachianvalves(aremnantofthesinusvenosusvalve)
• Chiarinetworks(filamentousstrandsintherightatrium)
• Conduitforparadoxicalembolization
• Stagnantflow,nidusforinsituthrombusformation
• Cryptogenicstroke,theusualcausesofstrokemustfirstbeeliminated
• DiagnosisbyTTE,TEE->microbubblesareseenintheleft-sided
• THERAPY:antiplateletoranticoagulantagents,percutaneousdeviceclosureandsurgicalPFOclosure
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“atrioventricularcanaldefect”
“endocardialcushiondefect”
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• Spectrumoflesionsinvolving
• atrialandventricularseptum
• atrioventricularvalves
• leftventricularoutflowtract
• "partial"form=primumASDbutnoVSD
• "complete"=primumASDandaninletVSD
• Deficiencyoftheinletventricularseptum+abnormalitiesoftheAVvalves->elongatedLVOT
"gooseneck"• Subaorticstenosis:chordalattachmentsofthecleft
anteriormitralvalvetotheleftventricularoutflowseptum
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• IsolatedprimumASD~largesecundumASDs
• +/-significantleftAVvalveregurgitation
• Asymptomaticuntil30-40yr,CHF,atrialarrhythmias,CHB,andpulmonaryhypertension
• CompleteAVcanal~sizeofASDandVSD,AVvalves
• Largeleft-to-rightshunt->heartfailureorpulmonary vasculardisease,palpitationsfromatrialarrhythmias
• 35%ofDown’ssyndrome:AVcanaldefect(morecomplete)
• Echo:4-chamberview,theAVvalvesappearatthesamelevel
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• UnrepairedornewlydiagnosedAVseptaldefectandsignificanthemody-namicdefects
• PersistentleftAVvalveregurgitationcausingsymptoms
• Significantsubaorticobstruction(meangradient>50mmHg
atrest)• IncaseofseverePHT,netleft-to-rightshuntof>1.5:1or
evidenceofpulmonaryarteryreactivityonchallengewithapulmonaryvasodilator
• ASDprimum->Pericardialpatchclosurewithsuture“cleft”leftAVvalve+/-MVR
• Complete->primaryintracardiacrepairininfancy>stagedapproach
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• RestrictiveVSD
• pulmonary-to-aorticsystolicpressureratio<0.3
• Qp/Qs≤1.4:1
• ModeratelyrestrictiveVSD
• pulmonary-to-aorticsystolicpressureratio<0.66
• Qp/Qsof1.4to2.2:1
• Largeornon-restrictiveVSD
• pulmonary-to-aorticsystolicpressureratio>0.66
• Qp/Qs>2.2
• Cyanosisinadult“Eisenmenger”,signofPHT,clubbingoffinger
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• Notcausesignificanthemodynamicderangement
• Mayclosespontaneouslyduringchildhoodandsometimesinadultlife
• Aperimembranousdefectinanimmediatelysubaorticposition~progressiveaorticregurgitation
• Asymptomatic
• Harshorhigh-frequencyPSM,usuallygrade3to4/6,heardattheleftsternalborder3rd-4thintercostalspace
• ECG-normaltracing• Treatment:IEprophylaxis,followupBUT...SurgeryinSmall
defectswithaorticvalveprolapseandAR
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• HemodynamicburdenLV->LAE,LVHanddysfunction
• Increaseinpulmonaryvascularresistance
• ECG:notchedPwave,leftventricularvolumeoverload,deepQandtallRwithtallTinV5andV6
• Indicatesurgery
• symptomaticpatientQp/Qs>1.5:1,
• pulmonaryarterysystolicpressure>50mmHg,
• increasedleftventricularandleftatrialsize,ordeteriorating
leftventricularfunction• absenceofirreversiblepulmonaryhypertension
• Directsuturewithpatch
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• Theductusarteriosus~Lt.6thprimitiveaorticarchandconnectstheproximalleftpulmonaryarterytothedescendingaorta,justdistaltotheleftsubclavianartery
• Silent:tinyPDAdetectedonlybynonclinicalmeans(usuallyechocardiography)
• Small:continuousmurmurcommon;Qp/Qs<1.5:1
• Moderate:continuousmurmurcommon;Qp/Qsof1.5to2.2:1
• Large:continuousmurmurpresent;Qp/Qs>2.2:1
• Eisenmenger:continuousmurmurabsent;substantialpulmonaryhypertension,differentialhypoxemia,anddifferentialcyanosis(pinkfingers,bluetoes)
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• SilentPDA:nolongtermcomplication
• Smallductusmaypredisposetoendarteritis
• Moderate-sizedductandshuntposeavolumeloadonLAandLV,Afibrillation
• Largeductresultsinitiallyinleftventricularvolumeoverloadbutdevelopsaprogressiveriseinpulmonaryarterypressures
• “machinery”murmur->widesystemicpulsepressurefromaorticdiastolicrunoffintothepulmonarytrunk
• ECG:moderateductshowleftventricularvolumeoverload
• CXR:moderate-sizedduct,cardiomegalywithleft-sidedheartenlargement,aprominentaorticknuckle,andincreasedpulmo-naryperfusion
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• INTERVENTION• Hemodynamicsignificant
• DebateininaudibleorsmallPDAstrictlytoreducetheriskofendarteritis
• Transcathetertreatment:ductssmallerthan8mm
• Surgical:ductalligation,toolargefordeviceclosure
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� Double-outletrightventricle(aorticoverrid>50%)� MaycoexistwithanAVseptaldefect
� RVOTisvariable:atresia,stenotic,bicuspidpulmonaryvalvewithsupravalvularhypoplasiaexists
� Thedominantsiteofobstructionisusuallyatthesubvalvelevel
� Majoraortopulmonarycollateralarteries
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� Rightaorticarch� Abnormalitiesofthecourseofthecoronaryarteries:theanteriordescendingarteryoriginatesfromtherightcoronaryartery
� Absentpulmonaryvalvesyndrome
� Thepulmonaryarteriesareusuallymarkedlydilatedoraneurysmal
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� cyanosis� paroxysmalhypoxemia
� rightventricularimpulse
� systolicthrillareoftenpalpablealongtheLSB� earlysystolicejectionsoundatthelowerLSBandapex� thesecondheartsoundisusuallysingle� theintensityanddurationofthesystolicejectionmurmur varyinverselywiththeseverityofsubvalveobstruction
� continuousmurmurfaintlyaudibleovertheanteriororposteriorchestreflectsflowthroughaortopulmonarycollateralvesselsoraduct.
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� EKG:RAD,RAE,RVH,RBBB[postrepair],QRSwidthmayreflectthedegreeofrightventriculardilation>>maybeariskfactorforsustainedVTandsuddendeath
� CXR:anormal-sized,bootshapedheartwithprominenceoftherightventricleandpulmonary vascularmarkingsarediminished,andtheaorticarchmaybeontherightside,ascendingaortais
prominent� Echocardiography:
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� systemictopulmonaryshunt:MBTS� Surgicalrepair
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� AbsenceoftherightAVconnection� mustbeanASD
� hypoplasiaofthemorphologicrightventriclewhichcommunicatestothedominantventricleviaaVSD
� subdividedintothosewithconcordant ventriculoarterialconnectionsandnormallyrelatedgreatarteries(70%to80%ofcases)andthosewithdiscordantconnections
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� subaorticstenosisandaorticarchanomalies[discordant]
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� Allpatientshave“mixing”ofatrialblood� concordantventriculoarterialconnectionstendtobemorecyanosed(dependingonthesizeoftheVSD)
� discordantconnectionsarepinkerandtendtodevelopheartfailure(becausetheunobstructedpulmonarycirculationarisesdirectlyfromtheleft ventricle
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� EKG:LAD,RAE,LVH� CXR:situssolitus,levocardiaandaleft-sidedaorticarch,theheartsizeandpulmonaryvascularmarkings varywiththeamountofpulmonarybloodflow
� Echocardiography:
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� Concordant:systemictopulmonaryshunt[bidirectionalGlennprocedure]
� Discordant:pulmonaryarterybandingtoreducepulmonarybloodflow
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� underdevelopmentoftheleftcardiacchambers� atresiaorstenosisoftheaorticorthemitralorificeandhypoplasiaoftheaorta
� Ductdependentsystemicbloodflow
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� Anyinfantwiththesuddenonsetofcirculatorycollapseandseverelacticacidosis
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� EKGRAD,RAE,RVH,STandTwaveabnormalitiesintheleftprecordialleads
� CXR:cardiomegalyandincreasedpulmonaryvenousandarterialvascularmarkings
� Echocardiography:
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� Earlytreatmentwithprostaglandin� Stagedsurgicalmanagementnowprovideslong-termpalliation
� Thesurvivorsoftheearliestattemptsatstaged
Norwoodpalliationarejustnowenteringadultlife
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� thedominantventricleisaleftventricle[75%]� separatedfromtherightventriclebyaVSD
� ventriculoarterialconnectionisdiscordant� theaortaarisesfromthesmallrightventricleandisfedviatheVSD
� unobstructedpulmonaryarteryarisesfromtheleft ventricle
� Subaorticstenosis,aortichypoplasia,andarchanomaliesarecommon
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� severecyanosisorwithduct-dependentpulmonarybloodflow
� heartfailurebecauseofincreasedpulmonarybloodflow
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� EKG:variable� CXR:variable� Echocardiography:
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� surgicalinterventiontoestablishthepulmonaryvalveastheunobstructedsystemicoutflowtract
� Pulmonaryarterybandingisofferedonlytothoseinfantswithpulmonaryovercirculation,heartfailure,
andunobstructedsystemicoutflow
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� situationinwhichallpulmonaryveinsfailtoconnectdirectlytothemorphologicleftatrium
� allofthesystemicandpulmonaryvenousreturnusuallydrainstotherightatrium
� subdividedaccordingtothepathoftheabnormaldrainage
� connectingviaaverticalveintotheleftbrachiocephalicvein,directlytotherightatrium,
directlytothesuperiorvenacava,belowthediaphragm[10%]
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� signsofheartfailureorcyanosis� clinicalpicturedependonthesizeoftheinteratrialcommunicationandthedegreeofobstruction
� afixed,widelysplitsecondheartsoundwithanaccentuatedpulmoniccomponent
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� EKG:RAD,RAE,RVH
� CXR:cardiomegaly[RA,RV]withincreasedpulmonarybloodflow,pulmonarya.segmentisenlarged.Theso-called“figure-of-8or“snowman”
heartisduetoenlargementoftheheartandthepresenceofadilatedrightsuperiorvenacava,innominatevein,andleftverticalvein
� Echocardiography:
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� balloonatrialseptostomyisoccasionallyrequiredtoincreasesystemicbloodflowbeforesurgery
� Surgicalrepair
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� ventriculoarterialdiscordance� AVconcordance:completeTGAorD-TGA
� AVdiscordance:congenitallycorrectedTGAorL-TGA� clinicalmanifestationsaredyspneaandcyanosis
frombirth,progressivehypoxemia,andcongestiveheartfailure
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� Theoriginoftheaortafromthemorphologicright ventricleandthatofthepulmonaryarteryfromthemorphologicleftventricle
� communicationbetweenthetwocirculationsmust
existafterbirthtosustainlife:ASD,VSD,PDA
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� EKG:variable
� CXR:anarrowvascularpediclewithanoblongcardiacsilhouette(“eggonside”)istypicallyseeninpatientsaftertheatrialswitchprocedure.
� Echocardiography:
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� balloonatrialseptostomy
� Surgicalrepair
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� systemicvenousbloodpassesfromtherightatriumthroughamitralvalvetoaleftventricleandthentotheposteriorlylocatedpulmonaryartery
� Pulmonaryvenousbloodpassesfromtheleftatriumthroughatricuspidvalvetoaleft-sidedrightventricle
andthentoananterior,leftsidedaorta� Associatedanomalies:VSD(75%),pulmonaryorsubpulmonarystenosis(75%),andleft-sidedtricuspidandoftenEbstein-like)valveanomalies(>75%)
� inherentlyabnormalconductionsystem:completeheartblock
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� Asymptomatic
� Dyspnea,exerciseintolerancefromdevelopingcongestiveheartfailure
� palpitationsfromsupraventriculararrhythmias
� VSD&PS:emboliorcyanosis� TheA2isoftenpalpableinthesecondLt.ICS� AsingleS2(A2)isheard,withP2oftenbeingsilentbecauseofitsposteriorlocation
� murmurofanassociatedVSDorofleftARmaybeheard
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� EKG:reversaloftheprecordialQwavepattern,1st-degreeAVblock,completeAVblock,atrialarrhythmias
� CXR:convexityoftheleftsupracardiacborderproducedbytheleft-sidedascendingaorta,mainpulmonarytrunkismediallydisplacedandabsentfromthecardiacsilhouette,rightpulmonaryhilumisoftenprominentandelevatedcomparedwiththeleft,
producingaright-sided“waterfall”appearance.� Echocardiography:
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� Medicaltherapy:ACEinhibitororbetablockertherapyforpatientswithsystemicventriculardysfunction
� Surgicalrepair
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� apicaldisplacementoftheseptaltricuspidleafletinconjunctionwithleafletdysplasia
� “atrialization”(functioningasanatrialchamber)oftheinflowtractoftherightventricleand
consequentlyproducesavariablysmallfunctionalrightventricle
� AssociatedanomaliesincludePFOorASD,accessoryconductionpathways,RVOT,VSD,aorticcoarctation,
PDA,ormitralvalvedisease� tricuspidregurgitation,rightatrialenlargement
� Right-to-leftshuntingthroughaPFOorASD
i i l
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� exerciseintolerance� palpitationsofsupraventricularorigin� cyanosisfromaright-to-leftshuntatatriallevel
� rightsidedcardiacfailurefromsevereTRandright ventriculardysfunction
� unimpressivejugularvenouspressure
� widelysplitS1withaloudtricuspidcomponent
� widelysplitS2fromaRBBB,andaright-sidedS3
� PSMincreasingoninspirationfromTRisbestheardattheLLPSB
EKG RAD RAE AV bl k RBBB AF/fl
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� EKG:RAD,RAE,AVblock,RBBB,AF/flutter� CXR:rightwardconvexityfromanenlargedrightatriumandatrializedrightventriclecoupledwithaleftwardconvexityfromadilatedinfundibulumgives
thehearta“waterbottle”appearance,pulmonary vasculatureisusuallynormaltoreduced
� Echocardiography:
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b t ti l i
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� substantialcyanosis � right-sidedheartfailure � poorfunctionalcapacity� paradoxicalemboli
� recurrentsupraventriculararrhythmias[uncontrol]� asymptomaticsubstantialcardiomegaly(cardiothoracicratio>65%)
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� discrete narrowing at the ligamentum arteriosum
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� discretenarrowingattheligamentumarteriosum
� diffuseformsmayinvolvethearchoristhmus
� descendingaortadistaltothesegmentofcoarctationisoftenaneurysmal
� intracranialaneurysms[10%]� systemicarterialhypertensionintheupperextremities
� associationwithgonadaldysgenesis(Turnersyndrome)andbicuspidaorticvalve,VSD,MR,MS
� EKG : RAD RVH
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� EKG:RAD,RVH� CXR:cardiomegalyandpulmonaryarterialand venousengorgement,ribnotching
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� Excision of the narrowed segment and end-to-end
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� Excisionofthenarrowedsegmentandend to endanastomosis
� prostheticgrafts� Percutaneousballoonangioplastywithstenting
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