Cyanotic congenital heart disease is characterized by a right-to-left shunt and decreased pulmonary blood flow, causing hypoxemia and cyanosis. Tetralogy of Fallot is a common cyanotic heart defect where there is a ventricular septal defect, right ventricular outflow tract obstruction, overriding aorta, and right ventricular hypertrophy. Without surgical intervention, most patients would not survive beyond childhood. Complete repair aims to relieve obstruction and close defects, but may require multiple staged procedures. Preoperative evaluation focuses on assessing cyanosis, growth, polycythemia, and identifying other anomalies to optimize timing of repair.

1. PATHOPHYSIOLOGY &PATHOPHYSIOLOGY &
MANAGEMENT OFMANAGEMENT OF
CYANOTIC HEARTCYANOTIC HEART
DISEASEDISEASE
DR SREEJITH HDR SREEJITH H

2. • Tracheoesophageal fistula
• Cleft lip and palate
• Anorectal anomalies
• Skeletal anomalies
Congenital heart disease: Scope of theCongenital heart disease: Scope of the
problemproblem
Commonest birth defect
1 in 125 live births
30% of children have extra
cardiac anomalies

5. foramen ovaleforamen ovale
 Blood is shunted fromBlood is shunted from
right atrium to leftright atrium to left
atrium, skipping theatrium, skipping the
lungs.lungs.
 More than one-third ofMore than one-third of
blood takes this route.blood takes this route.
 Is a valve with twoIs a valve with two
flaps that preventflaps that prevent
back-flow.back-flow.

6. ductus arterioususductus arteriousus
 The blood pumpedThe blood pumped
from the rightfrom the right
ventricle enters theventricle enters the
pulmonary trunk.pulmonary trunk.
 Most of this blood isMost of this blood is
shunted into the aorticshunted into the aortic
arch through thearch through the
ductus arteriousus.ductus arteriousus.

7. Foramen ovaleForamen ovale Closes shortly after birth,Closes shortly after birth,
fuses completely in firstfuses completely in first
year.year.
Ductus arterioususDuctus arteriousus Closes soon after birth,Closes soon after birth,
becomes ligamentumbecomes ligamentum
arteriousum in about 3arteriousum in about 3
months.months.
Ductus venosusDuctus venosus Ligamentum venosumLigamentum venosum
Umbilical arteriesUmbilical arteries Medial umbilical ligamentsMedial umbilical ligaments
Umbilical veinUmbilical vein Ligamentum teresLigamentum teres

8. ClassificationClassification

9. Physiologic classificationPhysiologic classification
Increased pulmonary
blood flow
Normal pulmonary
blood flow
Increased pulmonary
blood flow
Decreased pulmonary
blood flow

10. 10

11. Cyanotic heart diseaseCyanotic heart disease
Decreased
pulmonary
blood flow
Mixing of systemic
venous and
pulmonary venous
blood

12. CYANOTIC CONGENITAL HEARTCYANOTIC CONGENITAL HEART
DISEASESDISEASES
Characterized byCharacterized by
a right-to-left intracardiac shunt with associateda right-to-left intracardiac shunt with associated
decrease in pulmonary blood flow and thedecrease in pulmonary blood flow and the
development of arterial hypoxemia.development of arterial hypoxemia.
Chronic hypoxemia results in erythrocytosis andChronic hypoxemia results in erythrocytosis and
thromboembolism.thromboembolism.
Secondary erythrocytosis may cause coagulationSecondary erythrocytosis may cause coagulation
defect.defect.
Risk of CVA and brain abscess.Risk of CVA and brain abscess.
Without surgical treatment patient can not survive toWithout surgical treatment patient can not survive to
adulthood.adulthood.

13. Cyanotic with increased PBFCyanotic with increased PBF
 TruncusTruncus
arteriosusarteriosus
 TGATGA
 TAPVRTAPVR
 HLHSHLHS
Complex shunts

14. Cyanotic with decreasedCyanotic with decreased
PBFPBF
 TOFTOF
 Ebstein’sEbstein’s
anomalyanomaly
 Tricuspid atresiaTricuspid atresia
 PulmonaryPulmonary
atresiaatresia
Simple right to
left shunts

15. The Five Ts of Cyanotic CongenitalThe Five Ts of Cyanotic Congenital
Heart DiseaseHeart Disease
 Tetralogy of FallotTetralogy of Fallot
 Transposition of the great arteriesTransposition of the great arteries
 Truncus arteriosusTruncus arteriosus
 Total Anomalous PulmonaryTotal Anomalous Pulmonary
Venous ReturnVenous Return
 Tricuspid AtresiaTricuspid Atresia
First and last have decreased
pulmonary blood flow

16. TETROLOGY OF FALLOT
TETROLOGY OF FALLOT

17. DefinitionDefinition
 10% of all congenital10% of all congenital
heart defectheart defect
 Four characteristicsFour characteristics
 Large VSDLarge VSD
 RVOT obstructionRVOT obstruction
 Overriding aortaOverriding aorta
 RVHRVH
 Spectrum of TOFSpectrum of TOF
 TOF with PATOF with PA
 TOF with PS ( the classicTOF with PS ( the classic
form)form)
 TOF with InfundibularTOF with Infundibular

18. PathophysiologyPathophysiology
 Pathophysiology of TOF depends onPathophysiology of TOF depends on
 The degree ofThe degree of RVOTORVOTO
 Ratio of SVR to PVRRatio of SVR to PVR

19. Cyanotic with decreased PBFCyanotic with decreased PBF
Right to left
shunting of blood
due to
obstruction to
pulmonary blood
flow
Hypoxemia
and
Cyanosis
Polycythemia
Altered
hemostasis
Microvascular
thrombosis
Growth
retardation
Myocardial
dysfunction
Poor tissue
perfusion
Renal and
cerebral
thrombosis
Acidosis

20. R – L shunt: Balance between SVR and PVRR – L shunt: Balance between SVR and PVR
Pulmonar
yvascular
resistance
Systemic
vascular
resistance
Pulmonary
vascular
resistance
Systemic
vascular
resistance
Low
Hig
h
Right to left shunt
increases

21. R – L shuntR – L shunt
Avoid increase in PVRAvoid increase in PVR Avoid decrease in SVRAvoid decrease in SVR
 HypoxiaHypoxia
 HypercapniaHypercapnia
 AcidosisAcidosis
 High airway pressuresHigh airway pressures
 PEEPPEEP
 High hematocritHigh hematocrit
 Inadequate anesthesiaInadequate anesthesia
 HypothermiaHypothermia
 Anesthetic agents whichAnesthetic agents which
cause hypotensioncause hypotension
 HypovolemiaHypovolemia
Avoid increase in
systemic oxygen demand

22. Clinical manifestationClinical manifestation
 Pink tetPink tet
 TOF with adequate PBFTOF with adequate PBF
 Minimal RVOTO with a net LMinimal RVOTO with a net L  R shuntR shunt
 Acyanotic TOFAcyanotic TOF
 TOF with pulmonary atresia/ severe stenosisTOF with pulmonary atresia/ severe stenosis
with PATENT DUCTUS ARTERIOSUSwith PATENT DUCTUS ARTERIOSUS
 Normal saturationNormal saturation
 even show signs of CHFeven show signs of CHF
 Medical MX- diuretics, digoxin,ACE inhibitorsMedical MX- diuretics, digoxin,ACE inhibitors

23.  Hypercyanotic or tet spellsHypercyanotic or tet spells
 Paroxysmal episodes of acutely worsensParoxysmal episodes of acutely worsens
cyanosiscyanosis
 Usually in response to crying, feeding,Usually in response to crying, feeding,
defecating, agitation or frightdefecating, agitation or fright
 ↑↑R to L shuntingR to L shunting
 ↑↑PVRPVR
Hyperventilation with 100% O2
Bicarbonate administration

24.  Hypercyanotic or tet spellsHypercyanotic or tet spells
 Paroxysmal episodes of acutely worsensParoxysmal episodes of acutely worsens
cyanosiscyanosis
 Usually in response to crying, feeding,Usually in response to crying, feeding,
defecating, agitation or frightdefecating, agitation or fright
 ↑↑R to L shuntingR to L shunting
 ↑↑PVRPVR
 ↓↓SVRSVR
 During induction of anesthesiaDuring induction of anesthesia
Squatting position
Flexing the legs or
compressing abdominal aorta
Volume administration
α-adrengeric agonist

25.  Hypercyanotic or tet spellsHypercyanotic or tet spells
 Paroxysmal episodes of acutely worsensParoxysmal episodes of acutely worsens
cyanosiscyanosis
 Usually in response to crying, feeding,Usually in response to crying, feeding,
defecating, agitation or frightdefecating, agitation or fright
 ↑↑R to L shuntingR to L shunting
 ↑↑PVRPVR
 ↓↓SVRSVR
 Dynamic outflow obstructionDynamic outflow obstruction
(infundibular spasm )(infundibular spasm )
 Tachycardia, hypovolemia and increasedTachycardia, hypovolemia and increased
myocardial contractilitymyocardial contractility
volume expansion
β-blockers
Deepening anesthesia
Morphine

26. Natural historyNatural history
 Without surgeryWithout surgery
 25~35% die in the first year of life25~35% die in the first year of life
 40~50% die by the age of 440~50% die by the age of 4
 70% by 10 years70% by 10 years
 95% by 40 years95% by 40 years
 Chronic hypoxemia lead to polycythemiaChronic hypoxemia lead to polycythemia
 With complete repairWith complete repair
 ＞＞ 85% survive to adulthood85% survive to adulthood

27. Surgical ApproachSurgical Approach
Palliative proceduresPalliative procedures
Principle is to increase PBF by creating aPrinciple is to increase PBF by creating a
shunt B/W systemic & pulmonary circulationshunt B/W systemic & pulmonary circulation
 Balloon dilatationBalloon dilatation
 systemic-pulmonary arterial shuntssystemic-pulmonary arterial shunts
 Classic Blalock-Taussig shuntClassic Blalock-Taussig shunt
 Pott’s procedurePott’s procedure
 Waterston shuntWaterston shunt
 Modified Blalock-Taussig shunt (MBTS)Modified Blalock-Taussig shunt (MBTS)

29. Thomas-Blalock-Taussig Shunt
Vivien Thomas, Partners of the Heart, 1998 and
Something the Lord Made - Best Made-for-TV Movie, 2004
Helen Taussig
Alfred Blalock
Vivien Thomas

30. November 29, 1944
Thomas-Blalock-Tuassig

31. Surgical ApproachSurgical Approach
 Complete repairComplete repair
 GoalsGoals
 Maximal relief of RVOTOMaximal relief of RVOTO
 Closure of VSDClosure of VSD
 Preservation of RV functionPreservation of RV function
 Full correction between age ofFull correction between age of 2 to 10 months2 to 10 months
 Primary total repairPrimary total repair
 Staged surgeryStaged surgery
 Presence of coronary abnormalitiesPresence of coronary abnormalities
 Multiple VSDsMultiple VSDs
 Inadequate pulmonary artery anatomyInadequate pulmonary artery anatomy
 The RV pressure at least half of SBP followingThe RV pressure at least half of SBP following
correctioncorrection

32. Preoperative Evaluation andPreoperative Evaluation and
PreparationPreparation

33. Preoperative informationPreoperative information
 Presence of hypercyanotic spellsPresence of hypercyanotic spells
 Weight loss, growth, developmentWeight loss, growth, development
and level of activityand level of activity
 Prevent problems associated withPrevent problems associated with
polycythemiapolycythemia
 Identify other congenital anomaliesIdentify other congenital anomalies

34. HistoryHistory
 Assess exercise toleranceAssess exercise tolerance
 Frequent respiratory infectionsFrequent respiratory infections
 An optimal “window”An optimal “window”
 Cyanotic spellsCyanotic spells
 MedicationMedication
 Previous surgical interventionsPrevious surgical interventions

35. Physical examinationPhysical examination
 TachypneaTachypnea
 CyanosisCyanosis
 ClubbingClubbing

36. Physical examination of CVSPhysical examination of CVS

37. CNSCNS
 ConvulsionsConvulsions
 Signs of raised intracranial tensionSigns of raised intracranial tension
 An unsettling fever – cerebralAn unsettling fever – cerebral
abscessabscess
 Residual defects? (if operated earlier)Residual defects? (if operated earlier)

38. Airway examinationAirway examination
 Airway abnormalities commonAirway abnormalities common
 Examine child from front and sideExamine child from front and side
 Pierre Robin, Treacher Collins, Down’sPierre Robin, Treacher Collins, Down’s
 Tracheal stenosisTracheal stenosis
 Previous prolonged intubation afterPrevious prolonged intubation after
cardiac surgerycardiac surgery
 Vascular ringsVascular rings
 Compression by enlarged CVSCompression by enlarged CVS
structures as well as artificial conduitsstructures as well as artificial conduits

39. Establish room air
saturation in all cyanotics
Check for intravenous
access in all children

40. Laboratory dataLaboratory data
 Hb-polycythemiaHb-polycythemia
 Coagulation profile-Platelet dysfnCoagulation profile-Platelet dysfn
 ElectrolytesElectrolytes
 Arterial blood gases- metabolicArterial blood gases- metabolic
acidosis,hypoxemia,normal co2 retentionacidosis,hypoxemia,normal co2 retention
 X ray chest-boot shaped heartX ray chest-boot shaped heart
 ECG-RBBB,RVHECG-RBBB,RVH
 ECHOECHO
 Cardiac catherisation – gold standardCardiac catherisation – gold standard

41. Cadiac CatheterisationCadiac Catheterisation
 Location size & direction of shuntsLocation size & direction of shunts
 Pulmonary & systemic arterial pressuresPulmonary & systemic arterial pressures
 Ventricular & arterial pressure specifically L & R endVentricular & arterial pressure specifically L & R end
diastolic pressurediastolic pressure
 Oxygen saturation dataOxygen saturation data
 Cardiac chamber sizeCardiac chamber size
 PVRPVR
 VALVE FN & ANATOMYVALVE FN & ANATOMY
 Anatomy location & fn of previously created shuntsAnatomy location & fn of previously created shunts
 Anatomic distortion of systemic & pulmonary arterialAnatomic distortion of systemic & pulmonary arterial
vesselsvessels
 Coronary artery anatomyCoronary artery anatomy

42. TOF (BootTOF (Boot
shape)shape)


43. Preoperative preperationPreoperative preperation

44. NPO guidelines andNPO guidelines and
PremedicationPremedication
 NPO guidelinesNPO guidelines
 Solid food and particulate fluid: 6 hrsSolid food and particulate fluid: 6 hrs
 Clear fluid: 2 hrsClear fluid: 2 hrs
 PremedicationPremedication
 Recommend for patients withRecommend for patients with
hypercyanotic spellshypercyanotic spells
 Propranolol should be continued up toPropranolol should be continued up to
and including the day of surgeryand including the day of surgery

45. Infective endocarditis prophylaxisInfective endocarditis prophylaxis
 Unrepaired cyanotic CHDUnrepaired cyanotic CHD, including, including
palliative shunts and conduitspalliative shunts and conduits
 Completely repairedCompletely repaired congenital heartcongenital heart
defect with prosthetic material or device,defect with prosthetic material or device,
whether placed by surgery or by catheterwhether placed by surgery or by catheter
intervention, during theintervention, during the first 6 monthsfirst 6 months afterafter
the procedurethe procedure
 Repaired CHD with residual defectsRepaired CHD with residual defects at theat the
site or adjacent to the site of a prostheticsite or adjacent to the site of a prosthetic
patch or prosthetic device (which inhibitpatch or prosthetic device (which inhibit
endothelialization)endothelialization)

46. IE prophylaxisIE prophylaxis
IndicatedIndicated Not indicatedNot indicated
 Dental procedures withDental procedures with
bleeding or manipulationbleeding or manipulation
of gingival tissueof gingival tissue
 Incision or biopsy of theIncision or biopsy of the
respiratory mucosarespiratory mucosa
 TonsillectomyTonsillectomy
 Rigid bronchoscopyRigid bronchoscopy
 Procedures on infectedProcedures on infected
skin, skin structures orskin, skin structures or
musculoskeletal tissuemusculoskeletal tissue
 Dental proceduresDental procedures
without bleedingwithout bleeding
 Endotracheal intubationEndotracheal intubation
 Flexible bronchoscopyFlexible bronchoscopy
 Diagnostic GI scopyDiagnostic GI scopy

47. Situation Agent Regimen: Single
dose 30 – 60 min
before procedure
Oral Amoxicillin 50 mg/kg
Unable to take oral
medication
Ampicillin
OR
Cefazolin or Ceftriaxone
50 mg/kg IM or IV
50 mg/kg IM or IV
Allergic to
penicillins or
ampicillin (oral)
Cephalexin
OR
Clindamycin
OR
Azithromycin or
Clarithromycin
50 mg/kg
20 mg/kg
15 mg/kg
Allergic to
penicillins or
ampicillin and
unable to take oral
medication
Cefazolin or Ceftriaxone
OR
Clindamycin
50 mg/kg IM or IV
20 mg/kg IM or IV

48. Intraoperative ManagementIntraoperative Management

49.  Standard monitoringStandard monitoring
 ECGECG
 Pulse oximetryPulse oximetry
 Oppose to the side of proposed shuntOppose to the side of proposed shunt
 BPBP
 Site of arterial cannulationSite of arterial cannulation
 ETCO2ETCO2
 Rectal and esophageal temperatureRectal and esophageal temperature
 Urine outputUrine output
 Meticuloous care to make sure syringes &Meticuloous care to make sure syringes &
tubings are free from air bubblestubings are free from air bubbles

50. Avoid air bubblesAvoid air bubbles

51. Avoid dehydration,
especially if polycythemic
Maintain adequate tissue oxygenation
1.Avoid increasing O2 demand
2.Maintain SVR, systemic BP
3.Minimize PVR
Oral premed/induction
midazolam + ketamine

52. Free written board answer:
Speed of induction:
R->L shunt
• Inhalational: slower
• IV: faster
L->R shunt
• Inhalational: maybe faster
• IV: slower
But probably not clinically important
Tanner et al. Anesth Analg 64:101, 1985

53. TETRALOGY OFTETRALOGY OF
FALLOTFALLOT
Induction of AnesthesiaInduction of Anesthesia
 Induce with Ketamine 1-2 mg/kg i/v.Induce with Ketamine 1-2 mg/kg i/v.
 Decrease rate of muscle rexalant dose.Decrease rate of muscle rexalant dose.
 Induction with volatile anesthetics is slow.Induction with volatile anesthetics is slow.
 Sevoflurane and Halothane can be used but with cautionSevoflurane and Halothane can be used but with caution
and careful monitoring of oxygenation.and careful monitoring of oxygenation.
 Hypercyanotic attacks may occur.Hypercyanotic attacks may occur.

54. Tammy
Minimize RVOT obst & PVR
•oxygen
•beta blocker ready
Maybe:
•nitroglycerin
•phentolamine
•tolazoline
•prostaglandin E1
•nitric oxide

55. Maintenance ofMaintenance of
anesthesiaanesthesia
 Can be achieved by Ketamine.Can be achieved by Ketamine.
 Nitrous oxide but not more than 50% can be used but disadvantageNitrous oxide but not more than 50% can be used but disadvantage
is mild increase in PVR and decrease in FIOis mild increase in PVR and decrease in FIO2.2.
 Opiods and benzodiazepine can be used in low dose to avoidOpiods and benzodiazepine can be used in low dose to avoid
decrease in SVR and BP.decrease in SVR and BP.
 Muscle relaxation by pancuronium to maintain SVR and BP.Muscle relaxation by pancuronium to maintain SVR and BP.
 IPPV – avoid increase in airway pressure and peep.IPPV – avoid increase in airway pressure and peep.
 Maintain intravascular volume.Maintain intravascular volume.
 Avoid infusion of air.Avoid infusion of air.
 PhenylephrinePhenylephrine must be available to treat decrease in BP due tomust be available to treat decrease in BP due to
decrease in SVR.decrease in SVR.

56. Tammy
Minimize R->L Shunt
MAINTAIN SVR
•ketamine
•phenylephrine

57. Cardiopulmonary bypassCardiopulmonary bypass
 ThoracotomyThoracotomy v.sv.s. median sternectomy. median sternectomy
 Sudden decompensation duringSudden decompensation during
anesthesiaanesthesia
 FluidFluid
 VasopressorsVasopressors
 Ventilation adjustmentsVentilation adjustments
 Once open the shuntOnce open the shunt
 Saturation improves immediatelySaturation improves immediately
 BP may drop significantlyBP may drop significantly
 Diastolic hypotension may cause MIDiastolic hypotension may cause MI

58.  Assessment of the shunt flowAssessment of the shunt flow
 O2 saturation≒80%O2 saturation≒80%
 balanced pulmonary and systemic bloodbalanced pulmonary and systemic blood
flowflow
 Higher saturationHigher saturation
 Pulmonary over-circulationPulmonary over-circulation
 Unilateral pulmonary edema or hemorrhageUnilateral pulmonary edema or hemorrhage
shunt size may be reducedshunt size may be reduced
 Low saturationLow saturation
 Inadequate PBFInadequate PBF

59.  For shunt patencyFor shunt patency
 Low dose heparin infusion (8~10Low dose heparin infusion (8~10
U/kg/hr)U/kg/hr)
 Shift to Aspirin after enteral intakeShift to Aspirin after enteral intake
 Avoid platelet transfusionsAvoid platelet transfusions

60.  Problems when weaning from CPBProblems when weaning from CPB
 RV dysfunctionRV dysfunction
 Fluid loadingFluid loading
 Inotropic supportInotropic support
 Epinephrine 0.05~0.5 μg/kg/minEpinephrine 0.05~0.5 μg/kg/min
 Dopamine 1~20 μg/kg/minDopamine 1~20 μg/kg/min
 Milrinone 0.325~0.75 μg/kg/minMilrinone 0.325~0.75 μg/kg/min
 ↓↓RV afterloadRV afterload
 Ventilation adjustmentVentilation adjustment
 NTG 2 μg/kg/minNTG 2 μg/kg/min

61.  Arrhythmia and heart blockArrhythmia and heart block
 Common after VSD repairsCommon after VSD repairs
 Heart blockHeart block
 Epicardial pacingEpicardial pacing
 Permanent pacing if not resolved after 7~10Permanent pacing if not resolved after 7~10
daysdays
 Junctional ectopic tachycardiaJunctional ectopic tachycardia
 AmiodaroneAmiodarone
 ProcainamineProcainamine
 Post-CPB bleedingPost-CPB bleeding

62. ExtubationExtubation
 Elective shunt procedureElective shunt procedure
 In the OR or soon after arrival ICUIn the OR or soon after arrival ICU
 Usually within 4 hrsUsually within 4 hrs
 Emergency shunt placementEmergency shunt placement
 After resolution of hemodynamic,After resolution of hemodynamic,
metabolic and pulmonary problemsmetabolic and pulmonary problems
 Complete repairComplete repair
 same as elective shunt proceduresame as elective shunt procedure

63. Uncorrected patient forUncorrected patient for
noncardiac surgerynoncardiac surgery
 Prevention of hypercyanotic spellsPrevention of hypercyanotic spells
 Maintain SVR and improve PBFMaintain SVR and improve PBF
 MonitoringMonitoring
 The location of shunts and arterial linesThe location of shunts and arterial lines

64. TGATGA

65. 22NDND
COMMON CYANOTICCOMMON CYANOTIC
HEART DISEASEHEART DISEASE
 Results fromResults from failure of Truncus arteriosus tofailure of Truncus arteriosus to
spiralspiral
 Aorta arises from anterior portion of right ventricleAorta arises from anterior portion of right ventricle
& pulmonary artery from left ventricle& pulmonary artery from left ventricle
 Complete seperation of systemic & pulmonaryComplete seperation of systemic & pulmonary
circulationcirculation
 Survival is possible only if there is communicationSurvival is possible only if there is communication
B/W two circulationsB/W two circulations
 VSD PDA ASDVSD PDA ASD

67. Signs & symptomsSigns & symptoms
 Persistent cyanosis & tachypneaPersistent cyanosis & tachypnea
 Congestive heart failureCongestive heart failure
 Ecg – RBBB,RVHEcg – RBBB,RVH
 Chest Xray – egg shapped with aChest Xray – egg shapped with a
narrow stalknarrow stalk

68. TGA (egg on a string)TGA (egg on a string)

69. TreatmentTreatment
 Immediate MxImmediate Mx
Creating intracardiac mixing or increasing theCreating intracardiac mixing or increasing the
degree of mixing bydegree of mixing by
infusions ofprostaglandin E to maintaininfusions ofprostaglandin E to maintain
patency of the ductus arteriosuspatency of the ductus arteriosus
and/or balloon atrial septostomy (Rashkindand/or balloon atrial septostomy (Rashkind
procedure)procedure)
 Administration of oxygen may decrease pulmonaryAdministration of oxygen may decrease pulmonary
vascular resistance and increase pulmonary bloodvascular resistance and increase pulmonary blood
flow.flow.
 Diuretics and digoxin are administered to treatDiuretics and digoxin are administered to treat
congestive heart failurecongestive heart failure

70. Surgical correction –Surgical correction –
ARTERIAL SWITCHARTERIAL SWITCH
 the pulmonary artery and ascending aorta arethe pulmonary artery and ascending aorta are
transected above the semilunar valvestransected above the semilunar valves
 and re anastomosed with the right and leftand re anastomosed with the right and left
ventricles, and coronary arteries are thenventricles, and coronary arteries are then
reimplanted,reimplanted,
 so the aorta is connected to the left ventricle andso the aorta is connected to the left ventricle and
the pulmonary artery is connected to the rightthe pulmonary artery is connected to the right
ventricleventricle..

71. ANAESTHETICANAESTHETIC
MANAGEMENTMANAGEMENT
 doses and rates of injection of intravenouslydoses and rates of injection of intravenously
administered drugs may have to be decreased.administered drugs may have to be decreased.
 the onset of anesthesia produced by inhaled drugsthe onset of anesthesia produced by inhaled drugs
is delayedis delayed
 induction and maintenance of anesthesia-induction and maintenance of anesthesia-
ketamine combined with muscle relaxants toketamine combined with muscle relaxants to
facilitate tracheal intubationfacilitate tracheal intubation
 Ketamine + benzodiazepines/opiodsKetamine + benzodiazepines/opiods
 Dehydration must be avoided during theDehydration must be avoided during the
perioperative periodperioperative period

72. TRICUSPIDTRICUSPID
ATRESIAATRESIA

73. Tricuspid Atresia
3rd most common cyanotic CHD
Characterised byCharacterised by
Arterial hypoxemiaArterial hypoxemia
Small rt ventricleSmall rt ventricle
Large lt ventricleLarge lt ventricle
Marked decrease in pulmonary blood flowMarked decrease in pulmonary blood flow
Poorly oxygenated blood from rt atrium-Poorly oxygenated blood from rt atrium-
through ASD – Lt atrium- mixes with oxygenatedthrough ASD – Lt atrium- mixes with oxygenated
blood- Lt ventricle- systemic circualtionblood- Lt ventricle- systemic circualtion
PBF is via a VSD , PDA or BronchialPBF is via a VSD , PDA or Bronchial
vesselsvessels

74. TreatmentTreatment
 Fontan procedureFontan procedure
anastomosis of RT atrialanastomosis of RT atrial
appendage to RT pulmonary arteryappendage to RT pulmonary artery

75. Management of AnaesthesiaManagement of Anaesthesia
 Opiods / volatile anaaestheticsOpiods / volatile anaaesthetics
 In early post op Maintain increasedIn early post op Maintain increased
RT atrial pressure (16-20mm hg) toRT atrial pressure (16-20mm hg) to
facilitate PBFfacilitate PBF
 Early extubation is desirableEarly extubation is desirable
 Dopamine with or without vasodilatorsDopamine with or without vasodilators
are required to maintain CO & loware required to maintain CO & low
PVRPVR

76. Ebstein’sEbstein’s
AnomalyAnomaly

77.  Abnormality of the tricuspid valveAbnormality of the tricuspid valve
 The valve leaflets are malformed or displacedThe valve leaflets are malformed or displaced
downward into the right ventricledownward into the right ventricle..
 Usually regurgitant but may be stenotic alsoUsually regurgitant but may be stenotic also
 Most pts will hv a interatrial communication (ASD ,Most pts will hv a interatrial communication (ASD ,
PFO) through which RT to LT shunting occursPFO) through which RT to LT shunting occurs

78. Signs & symptomsSigns & symptoms
 severity of the hemodynamic derangements in patientsseverity of the hemodynamic derangements in patients
depends ondepends on
the degree of displacementthe degree of displacement
the functional status of the tricuspid valve leafletsthe functional status of the tricuspid valve leaflets
 Neonates - cyanosis and congestive heart failureNeonates - cyanosis and congestive heart failure
 older children incidental murmur(systolic murmur of TRolder children incidental murmur(systolic murmur of TR
in the LT sternal border)in the LT sternal border)
 Adults presents as supraventricular dysrhythmias thatAdults presents as supraventricular dysrhythmias that
lead to congestive heart failure, worsening cyanosis,lead to congestive heart failure, worsening cyanosis,
and occasionally syncopeand occasionally syncope..

79.  HEPATOMEGALY due to increased RT atrialHEPATOMEGALY due to increased RT atrial
pressurepressure
 ECG-tall & broad P wave(RBBB)ECG-tall & broad P wave(RBBB)
AV blockAV block
PSVT & ventricular dysarrythmiasPSVT & ventricular dysarrythmias
Ventricular preexcitation(WPW)Ventricular preexcitation(WPW)
 ECHO- to assess RT atrial dilatation & distortion ofECHO- to assess RT atrial dilatation & distortion of
tricuspid leafletstricuspid leaflets
To assess severity of tricuspid regurgitation /To assess severity of tricuspid regurgitation /
stenosisstenosis

80. Hazards of pregnancy in ptsHazards of pregnancy in pts
with EBSTEIN’S ANOMALYwith EBSTEIN’S ANOMALY
 Deterioration in RT ventricular Fn due toDeterioration in RT ventricular Fn due to
increased blood volume & COincreased blood volume & CO
 Increased RT to LT shunt & arterialIncreased RT to LT shunt & arterial
hypoxemia if ASD is presenthypoxemia if ASD is present
 Cardiac dysrythmiasCardiac dysrythmias
 PIH may cause Congestive Heart FailurePIH may cause Congestive Heart Failure

81. TreatmentTreatment
 Arterial shunt from systemic to pulmonaryArterial shunt from systemic to pulmonary
circulationcirculation
 Glen shunt & Fontan procedure to createGlen shunt & Fontan procedure to create
Univentricular heartUniventricular heart
 Repair /replacement of tricuspid valve along withRepair /replacement of tricuspid valve along with
closure of interatrial communicationclosure of interatrial communication
 Diuretics / Digoxin for Mx of Congestive heartDiuretics / Digoxin for Mx of Congestive heart
failurefailure
 IE prophylaxisIE prophylaxis

82. Anaesthetic managemnetAnaesthetic managemnet
 Hazards during anaesthesiaHazards during anaesthesia
Accentuation of arterial hypoxemia due to increasedAccentuation of arterial hypoxemia due to increased
RT to LT shuntRT to LT shunt
Development of supraventricular tachydysrhythmiasDevelopment of supraventricular tachydysrhythmias
 Increased RT atrial pressure indicates RT ventricularIncreased RT atrial pressure indicates RT ventricular
failurefailure
 Unexplained hypoxemia or air embolism during intraopUnexplained hypoxemia or air embolism during intraop
may be due to shunting thr previously closed foramenmay be due to shunting thr previously closed foramen
ovaleovale
 Delayed onset of iv drugs due to pooling & dilution inDelayed onset of iv drugs due to pooling & dilution in
RT atriumRT atrium
 Epidural analgesia has been used safe for labor &Epidural analgesia has been used safe for labor &
deliverydelivery

83. Ebstein’s anomalyEbstein’s anomaly