ACYANOTICCONGENITALHEART

DISEASE

IAPUGTeachingslides201516 1
ATRIALSEPTALDEFECT(ASD)

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 ATRIALSEPTALDEFECT(ASD)

IsolatedanomalyIn10%

M:Fratio:1:2

3050%ofchildrenhaveASDaspartofcardiac
defects.

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 ASD

Increasinglyreferredasmurmuranddetectedin

infancy

AuscultatoryFindingshelpfulindetection

ECGquiteuseful

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 ASDTYPES

Threetypes
Secundum (70%)
Primum (20)
SinusVenosus (10)

ThePatentForamenOvale(PFO)doesnotordinarily
produceintracardiacshunts.

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 ASDTypes

PrimumASD Secundum Sinus

ASD VenosusASD
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 ASD
PretricuspidLRshunt
Asymptomatic
DetectedinLateinfancy&Childhood

Pulse N
BP N
JVP A=V

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 ASD

Auscultation

Wide&FixedSplitofS2NochangewithRespiration/

Standing.P2canbeloudbutnoPAH

EjectionsystolicmurmuratPulmonaryArea

MidDiastolicmurmuratLLSB
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 ASD

ECG:RAD/rSR:RSR:rRinV1orV3R(85%)
CXR: VariableCardiacsize
Rightatrialenlargement
PBF
Echocardiography: LocationofASD/Size
DilatationofRV/RA/PA
ParadoxicalmotionofIVS

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 NATURALHISTORYOFASD

Spontaneousclosure?

ASD<4mmby18months>90%closure.
ASD48mmby18m75%closure.
ASD>8mmrarelyclosespontaneously.

IfuntreatedCHFandPAHdevelopsinadults
SBEprophylaxisisnotrequiredunlessassociateddefects
present.

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 ATRIALSEPTALDEFECT(ASD),OTHERTHAN
PRIMUMTYPE

Modeofdiagnosis:
Physicalexamination,ECG,XrayChest,
transthoracicechocardiography

Spontaneousclosure:Rareifdefect>8mmatbirth.
Rareafterage2years.VeryrarelyanASDcan
enlargeonfollowup.

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 PATENTFORAMENOVALE

Patentforamenovale:

Echocardiographic detection of a small defectin

fossa ovalis region with a flap with no evidenceof
right heart volume overload (dilatation ofright
atriumandrightventricle).
Patent foramen ovale is a normal findingin
newborns.

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 INDICATIONFORCLOSURE:
ASDASSOCIATEDWITHRIGHTVENTRICULAR
VOLUMEOVERLOAD
(i) In asymptomatic child: 24 yrs. (For sinusvenosus
defect45yrs..)
(ii)SymptomaticASDininfancy(CCF,severePAH):seenin
about8%10%ofcases.Ruleoutassociatedlesions
(e.g., total anomalous pulmonary venous drainage,left
ventricular inflow obstruction,aortopulmonary
window).
Earlyclosureisrecommended.

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 INDICATIONFORCLOSURE

(iii) If presenting beyond ideal age: Electiveclosure

irrespective of age as long as there is rightheart
volume overload and pulmonary vascularresistance
isinoperablerange.

Methodofclosure:
Surgical:Establishedmode.
Device closure: More recent mode, may be usedin
childrenweighing>10kgandhavingacentralASD.

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VENTRICULARSEPTALDEFECT
(VSD)

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 VSDTYPES(location)

Locationofthedefect:
TypeI:Subarterial
(outlet,subpulmonic,supracristalorinfundibular)
TypeII:Perimembranous(subaortic)
TypeIII:Inlet
TypeIV:Muscular

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 VSDTYPES(SIZE)
Large(nonrestrictive):
Diameterofthedefectisapproximatelyequaltodiameterofthe
aorticorifice
Rightventricularsystolicpressureissystemic
Degreeoflefttorightshuntdependsonpulmonaryvascular
resistance
Moderate(restrictive):
Diameterofthedefectislessthanthatoftheaorticorifice
Rightventricularpressureishalftotwothirdsystemic
Lefttorightshuntis>2:1
Small(restrictive):
Diameterofthedefectislessthanonethirdthesizeoftheaortic
orifice
Rightventricularpressureisnormal
lefttorightshuntis<2:1 IAPUGTeachingslides201516 17
VSDHEMODYNAMICS

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 CLINICALPRESENTATION

WithsmallVSDasymptomatic.

WithlargeVSD,delayedgrowthanddevelopment,
repeatedpulmonaryinfectionsandCHF.

Withlongstandingpulmonaryhypertension,a
historyofcyanosisandadecreasedactivity.

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 MODEOFDIAGNOSIS

Physicalexamination
ECG
Xraychest
Echocardiography

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 VSDAUSCULTATION

S1Normal.S2Loud(P2)

WidesplitwithvariableP2

CloselysplitwhenPAHdevelops

PSMatLLSB/MSBorder

MDMatApex.[EDMatAorticArea]

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 VSDAUSCULTATION

SmallModerateLarge

Normalsplit Nsplit Closelysplit

S2 P2loud
P2+loud
S3 Physiologic + +

Murmur PSMorESM PSM PSMorESM

MDM 0 + +

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VSDMURMUR

ModerateVSD

Small,ClosingVSD

(harsh)

Large,unrestrictiveVSD

(lessharsh)
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 NATURALHISTORY

About10%oflargenonrestrictiveVSDsdieinfirst
year,primarilyduetocongestiveheartfailure.
SpontaneousclosureisuncommoninlargeVSDs.
30%40%ofmoderateorsmalldefects(restrictive)
closespontaneously,majorityby35yearsofage.
DecreaseinsizeofVSDisseenin25%.

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 VSDTIMINGOFCLOSURE

LargeVSDwithuncontrolledcongestiveheartfailure:Assoonas
possible.
LargeVSDwithseverepulmonaryarteryhypertension:36months.
ModerateVSDwithpulmonaryarterysystolicpressure50%66%of
systemicpressure:Between12yearsofage,earlierifone
episodeoflifethreateninglowerrespiratorytractinfectionorFTT.
SmallsizedVSDwithnormalpulmonaryarterypressure,leftto
rightshunt>1.5:1:Closureby24yrs..
SmalloutletVSD(<3mm)withoutaorticvalveprolapse:12yearly
followuptolookfordevelopmentofaorticvalveprolapse.
SmalloutletVSDwithaorticvalveprolapsewithoutaortic
regurgitation:Closureby23yearsofageirrespectiveofthesize
andmagnitudeoflefttorightshunt.
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 VSDTIMINGOFCLOSURE

SmalloutletVSDwithanydegreeofaorticregurgitation:
Surgerywheneveraorticregurgitationisdetected.
SmallperimembranousVSDwithaorticvalveprolapsewithno
ormildaorticregurgitation:12yearlyfollowuptolookfor
anyincreaseinaorticregurgitation
SmallperimembranousVSDwithaorticcuspprolapsewith
morethanmildaorticregurgitation:Surgerywheneveraortic
regurgitationisdetected.
SmallVSDwithmorethanoneepisodeofinfective
endocarditis:EarlyVSDclosurerecommended.
SmallVSDwithonepreviousepisodeofinfectiveendocarditis:
EarlyVSDclosurerecommended
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 VSDMODEOFCLOSURE

Surgicalclosure.

DeviceclosureformuscularVSDinthoseweighing
>15Kg&ForperimembranousVSD.

Pulmonaryarterybandingisindicatedformultiple
(Swisscheese),orverylargeVSD,almostsingle
ventricle,infantswithlowweight(<2Kg),andthose
withassociatedcomorbiditylikechestinfection.

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 PDA

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 PDA

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 MODEOFDIAGNOSIS

Physicalexamination
ECG
Xraychest
Echocardiography.

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 PDA
SmallModerateLarge

Pulse ++ +++ +++

BP WidePP WidePP WidePP
CE 0 + +
Apex N LV LV
Continuousm + + +
Thrill 0 + +
MDM 0 + +
Systoliccompofm 0 0 +

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 PDA

ModeratePDA

LargePDA.PAH(H)

LargePDA.SeverePAH

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 CONTINUOUSMURMUR,
PINKCHILD

4.CAVFistula
1.PDA
5.SAVFistula
2.RSOV
6.AorticRAFistula
3.APWindow

7.ALCAPA
8.Lutembacher
9.PABstenosis
10.Coarctation
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 VENOUSHUM

Softblowingmurmur
IIIIMediumpitched
HighR/LSternalborderorboth
NoPeakingaroundS2
onsittingupwithneckflexed
onlyingdown,changeinneckposition

D.D:PDA,AVM,PAVfistula
Collaterals

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 SIZEOFPDA

LargePDA:Associatedwithsignificantleftheartvolume
overload,CCF,severePAH.PDAmurmurisunlikelytobe
loudorcontinuous.
ModeratePDA:Somedegreeofleftheartoverload,mildto
moderatePAH,no/mildCCF.Murmuriscontinuous.
SmallPDA:Minimalornoleftheartoverload.NoPH/CCF.
Murmurmaybecontinuousoronlysystolic
SilentPDA:Nomurmur,noPH.Diagnosedonlyonecho
Doppler.
Spontaneousclosure:SmallPDAsinfulltermbabymay
closeupto3moofage,largePDAsareunlikelytoclose.
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 TIMINGOFCLOSURE

Large/moderatePDA,withcongestiveheartfailure,
pulmonaryarteryhypertension:Earlyclosure(by36
months).
ModeratePDA,nocongestiveheartfailure:6
months1year.Iffailuretothrive,closurecanbe
accomplishedearlier.
SmallPDA:At1218months.
SilentPDA:Closurenotrecommended.

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 MODEOFCLOSURE

Individualized.
Deviceclosure,coilsocclusionorsurgicalligationin
children>6monthsofage.
Surgicalligationif<6monthsofage.
Device/coilsin<6months.
Indomethacin/ibuprofennottobeusedintermbabies
.

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 PDAINAPRETERMBABY

Interveneifbabyinheartfailure(smallPDAsmay
closespontaneously).
IndomethacinorIbuprofen(20)(ifno
contraindication).
Surgicalligationifabovedrugsfailorare
contraindicated
Prophylacticindomethacinoribuprofentherapy:Not
recommended.

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 CONGENITALFORMSOF
LVOTOBSTRUCTION

Subvalvular
Discretemembranousstenosis,Fibromusculartunnel

Valvular
Unicuspid,Bicuspid,QuadricuspidandDysplastic

Supravalvular
Discrete(membranousorhourglass)
Aortichypoplasiaoratresia
Interruptedaorticarch
CoarctationofAorta

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DIFFERENTTYPESOFAORTICVALVES

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 AVSTENOSIS

Obstructivelesion.Usuallyasymptomatic
SCD/Syncope/Anginapossible
PulseAbnormal
JVPNormal
BPNearNormal

NoCardiomegaly.Heavingapex
RtUSB
Thrill
Suprasternal
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 ASAUSCULTATION

S1NS2NParadoxicSplit?

S4.S3rare(ominous)

EjectionClick(constant)

EjectionSystolicmurmurRUSB

EDM+

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 ASSEVERITYASSESS
PulseLowvolume
HeavingApex
S1EclickDistance
S4
Murmur Length
Harshness
LatePeaking
SuprasternalThrill
Thrill

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 NATURALHISTORYOFAS

MildASandModerateASasymptomatic.

SevereASheartfailureinnewborns,chestpain,
syncope&suddendeath.

Pressuregradientincreaseswithgrowth.
WorseningofARmayoccurinsubaorticstenosis.
SBEis4%invalvarAS.

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 TIMINGOFINTERVENTION:VALVULARAS
Forinfantsandolderchildren:
Leftventriculardysfunction:
Immediateinterventionbyballoondilatation,irrespectiveof
gradients.
Normalleftventricularfunction:
Balloondilatationifanyofthesepresent:
(i)gradient>80mmHgpeakand50mmHgmeanbyechoDoppler;
(ii)STTchangesinECGwithpeakgradientof>50mmHg;
(iii)symptomsduetoASwithpeakgradientof>50mmHg.I
(iv)ncaseofdoubtaboutseverity/symptoms,anexercisetestmay
bedoneforolderchildren.
Forneonates:Balloondilatationifsymptomaticorthereis
evidenceofleftventriculardysfunction/mildleftventricular
hypoplasia,orifDopplergradient(peak)>75mmHg.
IAPUGTeachingslides201516 45
COARCTATIONOFTHEAORTA

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COARCTATIONOFTHEAORTA

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 COARCTATIONOFAORTA(COA)

8%ofallCHD.
M:F=2:1.30%ofTurnerSyndrome.
85%ofCOAhavebicuspidvalve.
Poorfeeding,dyspnea&poorweightgain,&acute
circulatoryshockinfirst6weeks.
2030%ofCOAdevelopCHFby3months

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 COARCTATIONOFAORTA

Stenoticlesion.Asymptomaticinmany
InfancytoAdulthood

Pulsediscrepancy
BPdiscrepancy

NormalJVP

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 COA

Radiofemoraldelay
Strongradials;WeakFemorals
TouchtheFeetofEachInfant
Upperlimbhypertension;NormotensiveLowerlimb
SBPofLowerlimb10mmormore
LessthanSBPofUpperlimb

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 COAAUSCULTATION

S1S2N

S3S4notusual

EjClick+

Ejectionmurmur/continuousmurmur

Nomurmur
Clinical:Radiofemoraldelay;Pulsediscrepancy
Nevermindthemurmur!

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 COACXR
Infant
Cardiomegaly.PVH
Aorta+
Child
NoCardiomegaly.PVH+
Aorta++&3signs
Ribnotching

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 COARCTATIONOFTHEAORTA

Diagnosticfinding

Aorticlumenisnarrowed,
typicallydistaltotheleft
subclavianartery.
Hypoplasticaorticarch
Poststenoticdilatationof
theaorta.
Bicuspidaorticvalve.
Dopplerwillshowthe
severityofobstruction.
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 NATURALHISTORYOFCOA

Bicuspidvalvemaycausestenosisorregurgitation
withage.
SBEmayoccuroneitheraorticvalveoron
coarctation.
LVfailure,ruptureofaorta,ICH,hypertensive
encephalopathymaydevelopduringchildhood.

IAPUGTeachingslides201516 54
 TIMING&MODEOFINTERVENTION
Timing
Withleftventriculardysfunction/congestiveheartfailureorsevere
upperlimbhypertension(forage):Immediateintervention.
Normalleftventricularfunction,nocongestiveheartfailureand
mildupperlimbhypertension:
Interventionbeyond36monthsofage.
Nohypertension,noheartfailure,normalventricularfunction:
Interventionat12years
Modeofintervention
Balloondilatationorsurgeryforchildren>6moofage.
Surgicalrepairforinfants<6moofage.
Balloondilatationwithstentdeploymentcanbeconsideredin
children>10yearsofageifrequired.
Electiveendovascularstentingofaortaiscontraindicatedfor
children<10yearsofage
IAPUGTeachingslides201516 55
THANKYOU

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