AdultCHD

8/6/2019 AdultCHD

http://slidepdf.com/reader/full/adultchd 1/109

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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8/6/2019 AdultCHD


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8/6/2019 AdultCHD


• 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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8/6/2019 AdultCHD


• 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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8/6/2019 AdultCHD


• 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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8/6/2019 AdultCHD


� 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


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� Earlytreatmentwithprostaglandin� Stagedsurgicalmanagementnowprovideslong-termpalliation

� Thesurvivorsoftheearliestattemptsatstaged

Norwoodpalliationarejustnowenteringadultlife

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8/6/2019 AdultCHD


� 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


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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.


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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


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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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AdultCHD