cyanotic-congenital-heart-diseases-iap-ug-teaching-slides_compress.ppt

CCHD

1. CYANOTICCONGENITALHEART
DISEASES
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2. INTRODUCTION
•Cyanosisisabluishorpurplishtingetotheskinand
mucousmembranes
•Approximately5g/dLofunoxygenatedhemoglobin
inthecapillariesgeneratesthedarkbluecolor
appreciatedclinicallyascyanosis
•Cyanosisisrecognizedatahigherlevelofoxygen
saturationinpatientswithpolycythemiaandata
lowerlevelofoxygensaturationinpatientswith
anemia
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3. CYANOSIS‐ TYPES
–Central–cyanoticCHD
–Peripheral–hypothermia,CCF
–MixedCyanosis–CHDinShock
–Differentialcyanosis–PDAwithreversal
–Reversedifferentialcyanosis–TGAwithPDAwith
reversal
–IntermittentCyanosis–Ebsteinsanomaly
–Circumoralcyanosis
–Cyclicalcyanosis–Bilateralchoanalatersia
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4. HOWTODIFFERENTIATE?
TrueCyanosis
•Associated
withclubbing
•ABG‐ confirms
Cyanosislikeconditions
•Notassociatedwith
clubbing
•LabestimationofMeth
HbandSulphHb
Confirms
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5. DIFFERENTIALDIAGNOSISFORCYANOSIS
–Methemoglobin
–Sulfhemoglobin
–Pseudocyanosis:isabluishtingetotheskin
and/ormucousmembranesthatisnot
associatedwitheitherhypoxemiaorperipheral
vasoconstrictionMostcausesarerelatedto
metals(eg,silvernitrate,silveriodide,silver,
lead)ordrugs(eg,phenothiazines,amiodarone,
chloroquinehydrochloride).
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6. CYANOTICCHD:CLINICALDIAGNOSTIC
APPROACH
CyanoticCHD
pulmonary
blood flow
pulmonary
blood flow
Normal
Pulmonary flow
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7. CyanoticCongenitalHeartDisease
Cyanosis,Clubbing,Polycythemia
Increased
PulmonaryBlood
Flow
Decreased
Pulmonary
BloodFlow
TranspositionofGreatarteries(3‐5%)
TruncusArteriosus(1‐2%)
SingleVentricle(1‐2%)TAPVC(1‐2%)
HLHS(1‐3%)
TetralogyofFallot(5‐7%)
TricuspidAtersiaEbstein’s
AnomalyPulmonary
Atresia
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8. TETRALOGYOFFALLOT
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9. INTRODUCTION
•In1888,FallotdescribedtheanatomyofTOF
•Incidence10%ofallformsofcongenitalheartdisease
•Themostcommoncardiacmalformationresponsible
forcyanosisafter1yearofage.
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10. PATHOLOGY
•Thefour
components
ofTOFare
–Ventricularseptaldefect
–Obstructiontoright
ventricularoutflow
–Overridingoftheaorta
–Rightventricularhypertrophy
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11. PATHOLOGY–CONT
•Onlytwoabnormalitiesarerequired
–AVSDlargeenoughtoequalizepressuresinboth
ventricles
–Arightventricularoutflowtactobstruction
•RVHissecondarytorightventricularoutflowtract
obstruction(RVOT)andVSD
•Overridingofaortavaries
•VSDisperimembranousdefectwithextensioninto
thesubpulmonaryregion
•VSDisnonrestrictiveandlarge
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12. HEMODYNAMICS
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13. HISTORY
•Appearanceofcyanosisafterneonatal
period
•HypoxemicSpells
•Lowbirthweightordevelopmentdelayor
easyfatigability
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14. GENERALEXAMINATION
•Cyanosis
•Clubbing
•Polycythemia
•Tachypnea
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15. SYSTEMICEXAMINATION
•RVtapinleftsternalborder
•Systolicthrillinupperandmidleftsternalborders
•Ejectionclickwhichoriginatesfromaorta
•S2issingleduetoabsentpulmonarycomponent
•Aloudejectiontypesystolicmurmurheardatthemid
andupperleftsternalborder
•ThismurmuroriginatesfromthePulmonarystenosis
andmaybeconfusedwiththeholosystolicmurmurof
VSD
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16. SYSTEMICEXAMINATION–CONT.
•Intensityofthemurmurdependsoftheseverityof
pulmonarystenosisorRVOTobstruction
•Moreseveretheobstruction,shorterandsofter
murmurwillbeheard
•InPulmonaryatresia,murmuriseitherabsentor
verysoft
•Auscultationofbackisimportanttofindthe
presenceofMAPCAs(MajorAortoPulmonary
CollateralArteries)
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17. INVESTIGATIONS
•Hematology
–Polycythemiasecondarytocyanosis(hematocrit
>65%)
–Anemia–duetorelativeirondeficiency
•Electrocardiography
•X‐ray
•Echocardiography
•Angiogram
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18. XRAY
•Normalsizeheart
•Pulmonaryvascular
markingsaredecreased
•Concavemainpulmonary
arterysegmentwithan
upturnedapex–BOOT
shapedheartorcoeuren
sabot
•Rightatrialenlargement
(25%)
•Rightaorticarch(25%)
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19. ELECTROCARDIOGRAPHY
Rightaxisdeviation,Rightventricularhypertrophy
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20. ECHOCARDIOGRAPHY
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21. DIFFERENTIALDIAGNOSISOFFALLOT’SPHYSIOLOGY
•Fallot’sTetralogy
•Transpositionofgreatarteries
•Tricuspidatresia
•Singleventricle
•Doubleoutletrightventricle
•Correctedtranspositionofgreatarteries
•Atrioventricularcanaldefect
•Malposition's
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22. COMPLICATIONSOFCYANOSIS/CYANOTICCHD
•Clubbing
•CyanoticSpell
•DepressedIQ
•Infectiveendocarditis
•Polycythemia
•Embolicphenomenon
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23. HYPOXEMICSPELL
•HypercyanoticorTetorcyanoticorhypoxicspell
•Mechanism‐ Secondarytoinfundibularspasm
and/ordecreasedSVRwithincreasedright‐to‐left
shuntingattheVSD,resultingindiminished
pulmonarybloodflow
•Peakincidence2‐ 4months
•Usuallyoccursinmorningaftercrying,feeding
ordefecation
•Severespellmayleadtolimpness,convulsion,
cerebrovascularaccidentorevendeath
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24. HEMODYNAMICSOFSPELL
•Increasedactivity
•Increasedrespiration
•Increasedvenousreturn
•Fixedpulmonarybloodflow
•Increased(RV)to(LV)shunt
•Increasedcyanosis
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25. HYPOXEMICSPELL‐ SYMPTOMS
•Suddenonsetofcyanosisordeepeningofcyanosis
•Suddenonsetofdyspnea
•Alterationsinconsciousness,encompassinga
spectrumfromirritabilitytosyncope
•Decreaseinintensityorevendisappearanceof
systolicmurmur
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26. HYPOXEMICSPELL–TREATMENT
•Kneechestpositionorsquatting–decreasessystemic
venousreturnandincreasessystemicvascular
resistanceatfemoralarteries
•Morphinesulphate–0.2mg/kgsubcutaneouslyor
intramuscularly,suppressestherespiratorycentreand
abolisheshyperpnoea
•Oxygenhaslittleeffectofarterialoxygensaturation
•Acidosisshouldbetreatedwithsodiumbicarbonate
1mEq/kgadministeredintravenously
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27. HEMODYNAMICSOFSQUATTING
•Decreasedvenousreturn
•Increasedsystemicvascular
resistance
•Increasedpulmonarybloodflow
•Decreasedcyanosis
•SquattingEquivalent–Knee
Chestposition,childsittingwith
flexedlimbs,mothercarryingthe
childwithfoldedlimbs.
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28. HYPOXEMICSPELL–FOLLOWUP
•Followingtreatment,patientbecomeslesscyanotic,
andheartmurmurbecomelouder
•Indicatesincreasedamountofbloodflowingthrough
stenoticrightventricularoutflowtract
•IfHypoxemicspellnotfullyrespond
–Vasoconstrictor:Phenylephrine0.02mg/kgIV
–Propranolol0.01to0.25mg/kgslowIVpush,
reducestheheartrateandmayreversethespell
–Ketamine1–3mg/kgover60secs,increases
systemicvascularresistanceandsedatesthe
patient
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29. NEUROLOGICALCOMPLICATIONSOFCHD:
TIPOFTHEPROVERBIALICEBERG!
Stroke
Brainabscess
Seizures
Adverse
neuro‐developmental
outcome:
•LowerIQ
•Poormotorskills
•Poorlanguageskills
•Cognitiveimpairment
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30. NEUROLOGICALCOMPLICATIONS
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31. MANAGEMENTPRINCIPLES
MEDICAL SURGICAL
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32. TREATMENTOFTOF–MEDICAL
•PreventionofHypoxemicspell
–OralPropranololtherapy0.5to1.5mg/kgevery6
hours–topreventHypoxemicspell
•Relativeirondeficiencyanemiashouldbedetected
andtreatedsinceanemicchildrenaremoresusceptible
tocerebrovascularcomplications
•Maintenanceofgooddentalhygieneandinfective
endocarditisprophylaxis
•Hematocrithastomaintained<65%,Phlebotomymay
beneededtomanagepolycythemia
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33. INDICATIONSFORSHUNTPROCEDURES
•NeonateswithTOFandpulmonaryatresia
•Infantswithhypoplasticpulmonaryannulus,which
requiresatransannularpatchforcompleterepair
•Childrenwithhypoplasticpulmonaryarteries
•Severelycyanoticinfantsyoungerthan3monthsof
age
•Infantsyoungerthan3to4monthsoldwhohave
medicallyunmanageablehypoxicspells
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34. SHUNTPROCEDURES
Systemic–PulmonaryShunt
•Blalock‐Taussig:
–anastomosedbetweenthesubclavianarteryandipsilateral
PA,preformedininfantsolderthan3months
•Gore‐TexInterpositionshunt:
–PlacedbetweenthesubclavianandipsilateralPA,done
eveninsmallinfantsyoungerthan3months
•Waterston:
–anastomosedbetweenascendingaortarightPA,nolonger
performed
•Potts:
–anastomosedbetweendescendingaortaandleftPA,no
longerperformed
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35. TRICUSPIDATRESIA
•MarkedCyanosis
presentfrombirth
•ECGwithleftaxis
deviation,right
atrialenlargement
andLVH
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36. EBSTEIN’SANOMALY
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37. IAPUGTeachingslides2015‐16
EBSTEIN’SANOMALY–CONT.
•Displacementofabnormal
tricuspidvalveintoright
ventricle
•Anteriorcuspretainssome
attachmenttothevalve
ring
•Otherleafletsareadherent
tothevalveoftheright
ventricle
•IntermittentCyanosis
•MultipleClicks
•Rightatriumishuge‐
ArterializationofRight
Ventricle
•Tricuspidvalveis
regurgitant 37

38. EBSTEIN’SANOMALY–CONT.
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39. PULMONARYATRESIA
•Cyanosisatbirth
•X‐rayChestshowa
concavepulmonary
arterysegmentand
apextiltedupward
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40. TRANSPOSITIONOFGREATARTERIES
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41. TGA:TRANSPOSITIONPHYSIOLOGY
•Oxygenatedbloodcirculates
withinthepulmonarycirculation
andde‐oxygenatedbloodin
systemiccirculation.
•Hypoxiaistheresultofimpaired
mixing.
•Betteradmixture–betteroxygen
saturation
•Earlypresentation
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42. TRUNCUSARTERIOSUS
•EarlyCHF
•MildorNoCyanosis
•Systolicejectionclick
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43. HYPOPLASTICLEFTHEARTSYNDROME
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44. PULMONARYAVFISTULA
•Fistulousvascularcommunicationsinthelungs
maybelargeandlocalizedormultiple,scattered
andsmall
•Themostcommonformofthisunusualcondition
is“Osler–Weber–RenduSyndrome”
•Clinicalfeaturesdependonthemagnitudeof
shunt
•Mildcyanosiswillbepresent
•Routineechowillbenormalbut“Contrast”echo
willbediagnostic
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45. TAPVC
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46. CYANOTICCHD:APPROACH
CLINICAL SUSPICION
ASSIGN PHYSIOLOGY
ASSESS SEVERITY
PRECISE DIAGNOSIS
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47. BEDSIDETOOLS
•Clinicalevaluation
•Chestx‐ray
•ECG
•Measurementofoxygensaturation
•Thehyperoxiatest
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48. STEP1:DETECTIONOFCYANOSIS
Clinicalrecognitionofcyanosishasitspitfalls
•Lighting
•Anemia
•Pigmentation
•Peripheralcyanosis
•Mildcyanosis
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49. IFINDOUBT……..
•Misleadingif
usedincorrectly
•Watchovera
periodof1‐2min
•Stable
waveforms
•Heartrate
display
correlatingwith
actualHR
•Protectprobe
fromlight
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50. THEHYPEROXIATEST
•100%O
2viahood
•~10min..
•TakeABG–pO2
<70
mmHg
CHDvery
likely
PO2
<150mmHg
150to
200
PO2
>200mmHg,
CHDlikely
CHD
unlikely
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51. STEP2:ASSIGNPHYSIOLOGY
CLINICAL ASSESSMENT OFPULMONARY BLOODFLOW
REDUCED INCREASED
NOFTT
1.MORECYANOSIS
2.CYANOTICSPELLS
3.QUIETPRECORDIUM
4.NOHEPATOMEGALY
1.CHF+
2.FTT+
3.MILDERCYANOSIS
4.NOCYANOTICSPELLS
5.HYPERACTIVEPRECORDIUM
6.HEPATOMEGALY
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52. CARDIACEXAMINATION:
CLUESBASEDONS2SPLIT
S2
single
fixed
normal
TOFphysiology
TGA
Mostadmixture
TAPVC
Excludes
Cardiac
cause
lesions
PurePSmayhaveawidesplitS2withsoftlyaudibleP2
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53. TYPEANDLOCATIONOFMURMUR
•EjectionSMinpulmonaryarea–
Mostcases.
•PSMinLLSB–TricuspidAtresia(
VSD)
•Continuousmurmurs:Pulmonary
atresia.
•ToandFroMurmur:TOF‐APV.
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54. CXRINCLASSIFYINGPHYSIOLOGY
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55. STEP3:ASSESSMENTOFSEVERITY
•Earlyonsetofcyanosis(especiallyinneonatalperiod)
•CyanoticSpells
•CyanosiswithCHF
•SevereCyanosiswithno/verysoftmurmurs
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56. STEP4:CONFIRMINGDIAGNOSIS
•Echocardiographyallows
completediagnosisinmajorityof
cases.
•CardiacCatheterizationrequired
inveryselectedsituations.
•AdvancesinCT/MRIobviate
needforcathfurther
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57. REFERRALTOASPECIALIST
•Referassoonasyoumakeadiagnosisofcyanotic
heartdisease.
•Neonatesarelikelytoneedimmediateintervention
•OlderchildrenandthosewithstableCHDfor
diagnosticconfirmationandplanningfurther
management
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58. TIMINGOFINTERVENTION:NEWERTRENDS
•Earlycorrectionofcongenitalheartdiseaseis
desirablebecauseitavoidsanumberofadverse
cardiac,neurodevelopmentandotherconsequences
•Earlycorrectionofavarietyofcongenitalheart
lesionsisfeasibleandrealisticwithexcellentresults
inmostofthedevelopednationsandselected
Indiancenters
58IAPUGTeachingslides2015‐16

59. TIMINGGUIDELINES:TAPVC
•Assoonasdiagnosisismade
•ObstructedTAPVCisasurgicalemergency
•Anydelaymaybecatastrophic
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60. TGA:TIMINGOFSURGERY
•Neonataldiagnosis:ArterialSwitchOperationat10‐
21daysage
•Diagnosisafter1monthage:AtrialSwitch(Senning)
operationat3‐4months
•TGAwithVSD:ArterialSwitchwithVSDclosure
between1‐3months.
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61. TRUNCUSARTERIOSUS
•ElectiveRepairby1–3months
•>3monthshighriskforpulmonaryvasculardisease.
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62. OTHERSITUATIONSWHEREWAITINGMAYBEJUSTIFIED:
•Complex2ventriclestates:waittillcyanosisis
apparent/olderage(4‐5years)
DORVVSDPSTGAVSDPScTGAVSDPS
•BalancedSingleVentriclestates(SaO285‐90%)Intervene
ifsymptoms/cyanosis+
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63. CYANOTICCHD:THEROLEOFTHE
PEDIATRICIANTODAY
Earlydetectionandreferralfortimelyintervention
withaviewtominimizemortalityandmorbidity
fromprolongedhypoxemia
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64. ThankYou
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