Case Presentation on Tetralogy of Fallot

1. Dr Bhagirath S N
TETRALOGY OF FALLOT
CASE PRESENTATION

3. • Name- deepak
• Age – 4 years
• Informant- mother
• Place- Bihar
Presenting complaints
• Bluish discolouration of lips since 1 month of age
• Breathlessness on exertion since 6 months of age

4. • H/o present illness
• Asymptomatic in the first month of life
• Gradual, bluish discolouration of lips and skin worsens with crying and
suckling.
• H/o episodes of increasing bluish discolouration and rapid breathing on
exertion (playing, excess crying) since 2-3 months of age
• h/o episodes of loss of consciousness before which child becomes limp. 5 -6
episodes per month during exertion.
• Episodes lasted for 3-5 minutes
• Resolved with squatting posture or on calming by mother
• increased frequency of such episodes over last 1 year

5. • H/o breathlessness on exertion x last 1½ years NYHA II
Ordinary activity like playing with other children, climbing stairs
cause shortness of breath .Relieved on taking rest
• No H/o frequent respiratory tract infections
• No H/o feeding difficulties
• No H/o fever, headache, vomiting, convulsions, lethargy or focal
motor weakness
• No H/o headache, dizziness, blurred vision, somnolence, fatigue,
paresthesia of fingers, toes, lips

6. Treatment history
• No history of previous surgery
• Receiving T. propranolol (10 mg) OD x last 6 months (irregular)
Birth history
• Full term normal delivery
• Birth weight 2.3 kg
• No significant maternal illness in antenatal period
• No h/o bluish discolouration/jaundice/cyanosis in the neonatal period

7. • Skeletal abnormalities like
• 6 fingers in (R) hand
• 4 fingers in (L) hand, noticed since birth
• No other obvious deformity noticed since birth
Developmental history
• Developmental milestones attained within normal limits
Family history
• No history of similar complaints in the family
7 years 4 years

8. PHYSICAL EXAMINATION
General physical examination
• Weight – 10 kg
• Height – 95 cm
• Afebrile
• Pallor – Nil
• Cyanosis – (+), (lips, tongue, fingers)
• Icterus – Nil
• Clubbing – (+) 3°
• Oedema – Nil
• Lymphadenopathy – Nil
• Neck veins – not engorged

9. • Pulse – 88/min, regular, no radio-radial or radio femoral
delay
• All peripheral pulses palpable
• BP – 84/60 mmHg (left arm, supine position)
• Peripheral venous access – adequate
• Room air saturation calm child 82%

10. CARDIOVASCULAR SYSTEM
Inspection
• Precordium normal on inspection
• No visible apical impulse
• No visible pulsation
• No scar mark visible
• Parasternal heave
Palpation
• Apex
• Palpable at (L) 4th ICS, 1 cm lateral to the mid-clavicular line
• Thrill palpable at (L) 2nd and 3rd intercostal space along the sternal border

11. Auscultation
• S1, S2 audible
• Systolic murmur, grade IV, best heard at left upper sternal border harsh in
quality. Not radiating to back/axilla

12. Respiratory system
• No chest wall deformity on inspection
• Respiratory rate 20/min,
• regular, accessory muscles not working
• Auscultation:
• B/L air entry equal
• No added sounds
Central nervous system
• Higher functions – normal
• Cranial nerves, cerebellum, motor and sensory examination – within normal
limits

13. Airway assessment
• Mouth opening > 4 cm
• Neck movement adequate
• MP class I
• No facial deformity noted
Spine examination
• No abnormality detected

14. Provisional diagnosis
• Cyanotic congenital heart disease with no evidence of infective
endocarditis or congestive cardiac failure in sinus rhythm.

15. Investigations
• Hb – 16.4
• TLC – 8,900
• Na+/K+ - 139/4.4meq
• Urea – 42mg/dl
• Creatinine- 0.8mg/dl
• Bd. group B (+ve)

16. CXR:
• Heart size – (N)
• RV type apex
• Pulmonary vascularity ¯
ECG
• RAD
• 100/min, regular
RV apex
Pulmonary
oligaemia

17. Echo
• Severe infundibular + valvular PS
• Confluent good sized pulmonary arteries
• Large VSD with aortic override (perimembranous,
R  L)
• No ASD, coarctation of aorta, PDA
• (N) RV/LV function
Impression
• CCHD, ¯ pulmonary blood flow, confluent good sized pulmonary
arteries.TOF

18. CYANOTIC CONGENITAL HEART
DISEASE

19. ÉTIENNE-LOUIS ARTHUR FALLOT!
• a French physician, 1888 Fallot
accurately described in detail the
four anatomical characteristics of
tetralogy of Fallot. …’la maladie
bleue
• First anatomic description…Danish
anatomist Niels Stensen, in 1672...

20. • Prevalence … 0.26 to 0.48 per 1,000 live births
• Genes identified : NKX2.5, [4%]; JAG1 in Alagille syndrome.; TBX5 in
Holt-Oram syndrome.
• Syndromes - DiGeorge/Velocardiofacial syndrome, Down syndrome,
Alagille syndrome, cat's-eye syndrome, recombinant chromosome (or San
Luis Valley) and Kabuki syndromes, and CHARGE and VATER/VACTERL
associations.
• Microdeletion on 22q11….seen in 15–35% of TOF patients;
• Sibling recurrence rate …2.5% to 3%
• Environmental factors
• maternal diabetes retinoic acids, maternal phenylketonuria

21. Developmental abnormality
Defective embryonic neural crest migration abnormal conotruncal
development.
• Incomplete rotation and faulty partitioning of the conotruncus during
septation….
• Malrotation of truncal-bulbar ridges results in misalignment of the outlet
and trabecular septum and consequent straddling of the aorta over the
malaligned VSD.
• Abnormally anterior septation of the conotruncus by the bulbotruncal
ridgessubpulmonic obstruction
• Tetrad …right ventricular outflow obstruction, aortic override, ventricular
septal defect, and right ventricular hypertrophy

22. PS
Most characteristic …. subpulmonic stenosis
• Obstruction along the entire course of the RVOT and pulmonary arteries
(LPA commonly) can occur.
• more severe the proximal obstruction, the greater the likelihood of distal
areas of obstruction
• Obstruction within the RV body :
• 1)hypertrophy of the septoparietal muscle bundles ;
• 2)anatomic displacement of the normal moderator band attachment

23. Pulm valve….bicuspid in 40%
VSD : nonrestrictive…..Few ptsVSD restrictive d/t the accessory TV
prolapsing thru the defect
Coronary Anomalies
• LAD from RCA (5%) & coursing in front of infund (Sx imp)
• Single coronary (4%)
So coronary evaluation before Sx imp -
• Echo….proximal coronary
• If needed … root angio / CAG….. MRI/CT

24. • Right Aortic A in 25%......Twice more common in TOF+PA
• Aortopulmonary Collateral Arteries..
• Stenosis of LPA in 40%
Associated cardiac abnormalities
• PFO or a true atrial defect in 83% of hearts with TOF… AVSD
(downs)…LSVC(11%)
• TOF with Absence of PA…almost always LPA….PS murmur radiates
to Rt chest…CXR..Lt hemithorax small,Lt lung hypovasc, Lt
hemidiaphragm elevated

25. Tetralogy OF Fallot
• Most common cyanotic heart disease!
• components
75%!

26. TOF
4 component!
Imagine this is a HEART!

27. TOF
1) Vetricular Septal Defect

28. TOF
1) Vetricular Septal Defect
2) Pulmonic Stenosis

29. TOF
1) Vetricular Septal Defect
2) Pulmonic Stenosis
3) Overriding of dextroposed aorta

30. TOF
1) Vetricular Septal Defect
2) Pulmonic Stenosis
3) Overriding of dextroposed aorta
4) Right Ventricular hypertrophy

31. TOF
1) Vetricular Septal Defect
2) Pulmonic Stenosis
3) Overriding of dextroposed aorta
4) Right Ventricular hypertrophy
Concentric R ventricular
hypertrophy without
cardiac enlargement

32. TOF
1) Vetricular Septal Defect
2) Pulmonic Stenosis
3) Overriding of dextroposed aorta
4) Right Ventricular hypertrophy
Concentric R ventricular
hypertrophy without
cardiac enlargement
Increase in right ventricular pressure*

34. RV and LV pressures
becomes identical

35. RV and LV pressures
becomes identical
There is little or
no L to R shunt

36. RV and LV pressures
becomes identical
There is little or
no L to R shunt
Hence, VSD is silent

37. RV and LV pressures
becomes identical
There is little or
no L to R shunt
Hence, VSD is silent
Right ventricle
into pulmonary
artery across
pulmonic stenosis
producing ejection
systolic murmur

38. Hence, the
more severe
the pulmonary
stenosis

39. Hence, the
more severe
the pulmonary
stenosis
The BIGGER
the Left to
RIGHT shunt

40. Hence, the
more severe
the pulmonary
stenosis
The BIGGER
the Left to
RIGHT shunt
Less flow into
the pulmonary
artery

41. Hence, the
more severe
the pulmonary
stenosis
The BIGGER
the Left to
RIGHT shunt
Less flow into
the pulmonary
artery
Shorter the
ejection
systolic murmur

42. Hence, the
more severe
the pulmonary
stenosis
The BIGGER
the Left to
RIGHT shunt
Less flow into
the pulmonary
artery
Shorter the
ejection
systolic murmur
More cyanosis because of less flow to the lung!

43. Hence,
• Severity of cyanosis is directly proportional to the severity of pulmonic
stenosis
• Intensity of the systolic murmur is inversely related to the severity of
pulmonic stenosis

44. Congestive failure never occurs* because…
Right ventricle is effectively decompressed because of the ventricular septal
defect.
* exception

45. Congestive failure never occur* because…
Right ventricle is effectively decompressed because of the ventricular septal
defect.
* exception
1)Anemia
2)Infective Endocarditis
3)Systemic hypertension
4)Unrelated myocarditis
complicating TOF
5)Aortic or pulmonary valve
regurgitation

46. Pulmonary obstruction results in delayed P2

47. Pulmonary obstruction results in delayed P2
Pulmonary artery pressure
reduce

48. Pulmonary obstruction results in delayed P2
Pulmonary artery pressure
reduce
P2 become soft or
inaudible

49. Pulmonary obstruction results in delayed P2
Pulmonary artery pressure
reduce
P2 become soft or
inaudible
(Second Sound) S2= A2 + P2
Since P2 is inaudible, hence S2 = A2 + P2
[S2 is single sound]
Aorta is displace anteriorly too, A2 become
LOUD!

50. • Ascending aorta in TOF is large, results aortic ejection click

51. • Diastolic interval is clear
• No S3
• No S4

52. Concentric right
ventricular hypertrophy
reduce the
distensibility of the
right ventricle during
diastole

53. Concentric right
ventricular hypertrophy
reduce the
distensibility of the
right ventricle during
diastole
“a” waves become
prominent in JVP*
*but not too tall

54. Clinical Picture
• Symptomatic any time after birth
• Paroxysmal attacks of dyspnea
• Anoxic spells
• Predominantly after waking up
• Child cry
• Dyspnea
• cyanosis
• Loss of consciousness
• Convulsion
• Frequency varies from
once a few days to many
attack everyday
• Dyspnea on exertion
• Exercise intolerance

55. • Sitting posture – squatting
• Compensatory mechanism
• Squatting increases the peripheral vascular resistance,
• which diminishes the right-to-left shunt
• increases pulmonary blood flow.
• Cyanosis during feeding
• Poor feeding
• fussiness, tachypnea, and agitation.
• Birth weight is low.
• Growth is retarded.
• Development and puberty may be delayed.

56. Physical examination
• Clubbing + Cyanosis (Variable)
• Squatting position
• Scoliosis – Common
• bulging left hemithorax
• Prominent “a” waves JVP
• Normal heart size
• Mild parasternal impulse
• Systolic trill (30%)

57. • S1 normal
• S2 single
• only A2 heard
• P2 soft & delayed: INAUDIBLE
• Murmur
• Shunt murmur (VSD) absent
• Flow murmur: Ejection systolic,
the smaller the flow the shorter
the murmur
• Ejection aortic click

58. ECG
• QRS axis … same as that of a normal newborn
• RVH…Tall monophasic R in V1 with an abrupt change to an
rS pattern in V2 (Tall R extends into adj precordial leads in
TOF + APV)
• Reduced PBF+ underfilled LVrS in V2-V6
• Balanced shunt …qR in V5,V6
• L-R shuntQR in V5,V6
• LAD with counterclockwise depolarisn TOF+AVSD

59. ECG
• Right axis deviation (+120° to +150°)
• Right or combined ventricular hypertrophy
• Right atrial hypertrophy
• Partial or complete right bundle branch block (especially true of patients
after surgical repair)

60. ECG
• ECG

61. ECG
• ECG wiLLiam
moRRow

63. CXR:
• Normal sized heart; [may be large in PA]
• upturned apex; attenuated & concave left heart border (infundibular and PA
hypoplasia)….boot-shaped heart, or coeur en sabot…small underfilled LV that
lies above horiz IVS, inferior to which is a concentric hypertrophied nondilated
RV
• Diminished pulmonary vascularity in proportion to the degree of cyanosis.
• Absent thymic shadow in the newborn may indicate associated chromosome
22q11.2 microdeletion (DiGeorge syndrome).
• RAA in roughly 25%...accompanied by Rt DA on Rt side
• In PA..lacy reticular pattern (d/t the anast b/w lobar/segm PAs & CAs)
• Syst arterial collaterals rarely cause rib notching as they do not run in
intercostal grooves.

64. • Coeur en sabot
(boot-shaped heart)
secondary to uplifting of the
cardiac apex from RVH
and the absence of a normal
main pulmonary artery
segment

65. • Decreased pulmonary
vascularity
• Right atrial enlargement
• Right-sided aortic arch (20-
25% of patients) with
indentation of leftward-
positioned tracheobronchial
shadow

66. Cardiac Catheterization
• Less often needed ;
• Diagnostic
• Therapeutic
• foremost goal …. clarification or better definition of anatomic
characteristics, such as pulmonary arterial or coronary arterial
anatomy
• coronary artery anatomy … either by aortic root angio, selective
coronary artery injection, or a combination of both.
• Definition of any aortopulmonary collaterals ….usually originate
from the descending aorta.

67. Course and Complication
1) Each anoxic spell is potentially fatal
2) Polycytemia
1) Cerebrovascular thrombosis
3) Anoxic infaction of CNS
1) Neurological complication
LUNG is an very good filter.
1) Bypassing it may not be a good idea!
2) TOF, venous blood from gut, peripheral system by pass the lung and
re-enter circulation

68. CNS complications
• Paradoxic embolus
• Cerebral thrombosis
• Cerebral abcess
• Seizures
• Hypoxic damage
• Endocarditis & vegetations
• Postoperative strokes

69. HYPERCYANOTIC SPELLS OR TETRALOGY SPELLS :
• best described in TOF;
• can occur with other forms of structural heart disease
• mediated, in part, by dynamic changes (acute increase)in subpulmonic
obstruction….
• . changes in contractility due to ‘endogenous catecholamines or exacerbated
by hypovolemia’
• other mechanisms - decrease in systemic vascular resistance.
• Child may assume squatting posture (instinctive) during spells …
Pathogenetic mechanisms : Vulnerable respiratory control centres ;Increase in
HR ; Increase in CO & VR ;Increase in R L shunt; Infundibular contraction
may reinforce,but does not initiate.

70. TYPICAL SPELL :
• The child becomes distressed and inconsolable, without apparent
reason, most often in the morning.
• Peak incidence2nd-6th m ;few after 2 yrs
• Crying is associated with progressively deeper cyanosis and hyperpnea
(not tachypnea).
• Spells are self-aggravating;
• During the spell  diminished/absent murmur
• Not infrequently,the spell terminates with unconsciousness and,
rarely,convulsions. If the hypoxemia is extreme, permanent neurologic
sequelae and even death may ensue. True hypercyanotic spells are rare
in neonates, although cyanosis may increase with crying.

71. Hypercyanotic spells …Rx aims @ lowering impedance to pulmonary
flow and further increasing systemic vascular resistance.
Refractory Transfusion of whole blood/red cells
• Balloon angioplasty of pulm annulus
• Emergent surgical palliation or repair

72. Management of anoxic spell
1) Calm the baby
2) increase SVR
3) Knee chest position
4) Humified O2
5) Morphine 0.1 -0.2 mg/Kg Subcutaneous
6) Correct acidosis – Sodium Bicarb IV
6) Inj phenyelphrine
7) Propranolol
1) 0.1mg/kg/IV during spells
2) 0.5 to 1.0 mg/kg/ 4-6hourly orally
7) Vasopressors:
8) Correct anemia

73. MANAGEMENT

74. • Infants with very severe RVOT stenosis and those with TOF + PA,
• with SaO2<70%, should have surgery within a few weeks after birth.
• Infants with moderately severe stenosis and marked cyanosis (SaO2 70–
90%) should have corrective surgery by 2–4 months.
• Corrective surgery should be performed in all other infants with tetralogy
of Fallot by 6 months.
Contraindications for repair in early infancyPalliation initially
• LAD from RCA crossing infundibulum
• Severely hypoplastic PAs
• Pulmonary atresia

75. Palliative Procedures
Classic BT shunt[1945]…SCAPA on side opposite AA
Modified BT… esp in small infants <6 months…side to side anastom
with interposition graft of PTFE or Gore-Tex b/w SCA & PA [on the same
side of AA]
Waterston shunt : Side-side anastomosis of RPA to AA
Potts : Side - side anastomosis of LPA to DA
Waterston/Potts shunts : complications
• Excessive PBF  HF [20%] & PHTN
• Difficulty taking shunt down at time of correction
• Distortion of Rt/Lt PA ; Right/Left PA aneurysm

76. BT shunt-advantages :
(a)low incidence of problems from excess PBF
(b)No pericardial adhesions as pericardium is not entered
(c)Easy to close @ time of complete repair..ligating its distal part just proximal to
anastom with PA
(d)Less distortion of PAs
CENTRAL SHUNT :connecting a short tubular graft of Teflon or GoreTex from the
aorta to the MPA.
advantages vs other shunts:
• the size of the communication could be controlled by selecting a tube with a diameter
appropriate for the patient;
• branch PAs are not disturbed so that reconstruction is not required at the time of
corrective surgery.

77. Blalock Taussig Shunt
• Subclavian artery – Pulmonary artery anastomosis

78. Modified Blalock Taussig Shunt
• Goretex graft

80. Surgical Palliation

81. CLASSIC BT shunt

82. POTTS SHUNT WATERSTON SHUNT

83. Interventional procedures :
In patients with severe annular hypoplasia….. palliation of significant
cyanosis by balloon valvuloplasty or RVOT stent placement can be
done… Improvement in antegrade flow is thought to simultaneously
enhance pulmonary arterial growth by augmenting PBF
Balloon angioplasty of the pulmonary valve annulus is preferable to a
shunt procedure as it is
• less traumatic,
• it avoids a thoracotomy,
• reduces the likelihood of distortion of the pulmonary arteries
Coil embolization of APCs ….Coiling of vessels that perfuse
pulmonary segments already supplied by pulmonary arterial flow
serves to reduce LV volume loading as well as to eliminate runoff into
the pulmonary arterial bed during CPB

84. Surgery aims @
• relieving all possible sources of RVOTO;
• If possible, pulmonary valve function is preserved by avoiding a transannular patch
• Closure of VSD (dacron patch)
• To relieve RVOTOpulmonary valvotomy, the insertion of an outflow tract patch
or a transannular patch are often required.
• Surgery during early infancy, when the pulmonary annulus is markedly stenotic,
frequently requires the insertion of a long and wide transannular patch.
• Consequently, most patients acquire PR as a result of the repair. PR may be well
tolerated by many in the early postoperative years, but in the long term chronic PR
is associated with reduced exercise capacity, RV dilatation, ventricular
arrhythmias, and sudden death.

85. PVR : Early PVR in selected patients results in beneficial remodelling of the right
ventricle
Optimal timing is critical for preserving RV function (not too late) and avoiding the
need for early re-operation (not too early). Amelioration of RV function following
PVR has to be weighed against the risk of subsequent re-operation for homograft
failure.
Studies…RV end-diastolic volume may become a helpful indicator for defining both
a lower limit (150 mL/m2) and an upper limit for re-intervention
(200 mL/m2).[normal 60-100]
CMR is the gold standard for evaluation of RV volumes and quantification of the
degree of PR & TR.

86. SURGERY in TOF contd…
VSD closure : Done thru’ RA approach whether or not a trans-annular patch is used,
as this approach allows to minimise the length of the right ventriculotomy
(length only necessary to relieve the RVOT obstruction and not for the VSD
exposure).
Efficacy of the RA approach ….
• Preserves the right ventricular function, …..
• Resultant PR after limited transannular patching is less severe than that which
occurs after transventricular repair
• Less incidence of ventricular/atrial arrhythmias is
• Easier to preserve the integrity & function of the tricuspid valve.
Concerns of right ventriculotomy (classical RV approach)
• Low cardiac output in the early postoperative period,
• a higher incidence of arrhythmias,
• Risk of late sudden death

87. In case of a major coronary artery crossing the RVOT…an external conduit or
homograft would be necessary;
Risk factors for early death after repair:
1) very young age
2) older age
3) severity of annular hypoplasia
4) small size of pulmonary arteries
5) need for transannular patch (debatable)
6) high peak RV to LV pressure ratio
7) previous palliative operations
8) multiple VSDs
9) co-existing cardiac anomalies

88. Advantages of early total correction
• Prevents cerebral hypoxia,cerebral embolism, abscess and hematological changes
• Decrease RVPprevent persistent myocardial hypertrophy and probably reduce
the risk of fibrosis of the RV
• Providing adequate PBF will optimize the opportunity for normal growth of the
main and branch PAs…also normal pulmonary circulation may be important for
lung development.
• Tendency for progressive hypertrophy of the RV infundibular region is largely
abolished by early repair.
• Early separation of MAPCAs from aorta reduces the risk of PVOD

89. SURGERY IN TOF + PA :
• PA anatomy not favourable? ….Palliative procedures… Central shunt vs
reconstruction of RVOT using a patch/conduit while leaving open the VSD
• If PA anatomy appears amenable to reconstruction, procedures leading to complete
repair are indicated.
Such procedures include RV outflow reconstruction for inducement of central
pulmonary artery growth using a valved conduit / aortic or pulmonary homograft
• If there is a connection between RV & PT  correction can be achieved with a
patch reconstruction

90. PA anatomy assessment
1) McGoon ratio: (Diameter of RPA/DAo + Diameter of LPA/DAo)
• Normal 2.1
• Adequate for VSD closure 1.2
• Inadequate <0.8 for VSD closure
2) Nakata Index:(CSA of RPA + CSA of LPA)/BSA
• Normal value > 200 mm2/m2
> 150 mm2/m2 is adequate.
(Not usable preoperatively when MAPCAs are the major source of PBF & one-
stage unifocalization + full repair is planned).

91. 3) Total Neo-Pulmonary Artery Index (TNPAI) = APC index + Nakata Index
• APC index is the sum of CSA of all usable APCs/BSA
>250 - suitable for one-stage repair including VSD closure (These pts. have low
RV/LV pressure ratio postoperatively).
Critique of all these indices: These indices consider only the size of proximal vessel
and not consider the condition of distal parts of the vessels (which may be stenosed).
Recently the value of all these has been questioned

92. MAPCAs : management : 2 options
1)Obliterate them by Sx ligation/coil embolisation
2)Surgical unifocalisation : if it is the sole supply to many segments …..connecting
all MAPCAs, as well as the small native pulmonary arteries, to one source of blood
flow from the RV [ie to a central PA confluence or prosthetic PA confluence]….. If
performed early,it avoids the dvpt of PVOD changes, as well as stenoses in the
MAPCAs.
If the pulmonary vascular morphology and resistance are such that adequate PBF
can be accommodated…then RVP after correction will be low enough to close the
VSD.

93. • To assess whether this is likely, surgeons at the University of California in San
Francisco perfuse blood from the perfusion system through the pulmonary artery at
a rate of 2.5 L/min per m. If PA mean pressure is <25 mmHg, it is considered safe
to close the VSD. If it is not closed, the patient is followed; the PVR may decrease
over time, allowing later closure. Also, if stenoses in pulmonary vessels are noted,
relief by balloon angioplasty, with stenting if necessary, may permit RVP to fall
and permit closure of the defect.
• Post surgery if the RVP/LVP is >0.75….RVF may result…avoid closure VSD /
fenestrated patch closure

94. Hypoplastic PAs … intervene early …. Encourage them to grow
• Reconstruction of RVOT with a patch or valveless conduit
• Placement of a central AP shunt
• If MPA,RPA & LPA are present, even though very small (diameter 3 mm), they are
capable of considerable enlargement if blood flow through them is increased.
Creating an AP window early in infancy sufficient enlargement of the pulmonary
arterial tree to later perform successful repair using the normal pulmonary arteries
and a unifocalization procedure can be avoided.

95. Dvpt of small pulmonary arteries
(A) Before central shunt was
performed, the Nakata index was
64 and the McGoon ratio was
0.82. (B) One hundred seventy-
four days after operation, the
Nakata index rose to 89 and the
McGoon ratio to 1.1.

96. Post Repair for TOF in general
Current surgical survival, even for symptomatic infants <3 months of age, is
excellent.
Hospital and 1-month survival rates of 100% have been reported.
Earlier age at repair (<1 year of age) did not adversely affect the rate of
reintervention; so primary repair should be regarded as the preferred management
strategy.
Twenty-year survival for hospital survivors, irrespective of management strategy,
was 98% for patients who have TOF with PS and slightly lower for patients with
PA, reflecting the overall excellent long-term survival of these patients.

97. COURSE
In unrepaired TOF….increasing cyanosis due to progressive infundibular
obstruction. Beyond the neonatal period, they are also at increasing risk for
developing hypercyanotic spells.
Older children with unrepaired or palliated TOF … complications a/w chronic
cyanosis ,polycythemiastroke, brain abscess, and pigment gallstones.
Now rare …
‘infant repair is the rule’
PVOD in TOF : rare
• large surgical systemic-to-pulmonary artery shunts
• persisting large aortopulmonary collaterals

98. REPAIRED TOF :
Outcomes : excellent…recent reports…mortality <3%
Problems that may develop include
a)Residual VSDs
• Persistent defects at the patch margin or
• Previously unrecognized or underestimated additional defects in the muscular
septum.
• Partial patch dehiscence
b)Recurrent RVOTO :
• Muscle bundles obstruct the os infundibulum in 3%
• Nontransannular patches…annulus becomes restrictive as child grows
• Transannular patch…restriction at the distal insertion of the patch into the branch
pulmonary arteries.
• Stenosis owing to compression from an aneurysmal RVOT patch …. should be
approached surgically with revision of the patch and repair of the proximal
obstruction.
• may dvp additional sites of peripheral PS over time.

99. c)Progressive aortic root dilation &AR :
• Intrinsic dvptal abnormalities of aortic valve/root
• Palliative shunts or significant APCs left-to-right shunt volume contribute to
aortic dilation.
Progressive dilation of aorta correlate with longer time between palliation & repair ;
Pulmonary atresia ; right AA & male gender.
d) RV dilation;syst/diast dysfunction later RHF
• excess volume load from pulmonary regurgitation
• pressure load from outflow obstruction,
• Hypoxemic coronary perfusion,
• surgical incision, patch and scarring, and post–cardiopulmonary bypass ischemia
reperfusion injury—possibly superimposed on tetralogy associated congenital
abnormalities of the myocardium.

100. e) LV dysfunction … consequence of adverse ventricular interaction.
f) Rhythm disturb./SCD : Long-term mortality …. 3% to 6%.
A review of 125 adult patients with TOF suggested that a greater degree of PR, a
history of sustained VT, QRS duration >180 ms, or LV dysfunction was a predictor
of sudden death
g) Lower IQ
h) Life time increased risk for IE
i)Membranous subaortic stenosis …..may be seen years after the initial repair in a
few patients…may require surgical excision.
j)Small coronary-to-RV fistulas, thought to relate to the excision of muscle in the
right ventricular outflow tract.

101. Post repair TOF + PA :
• Need for reoperation/ transcatheter balloon dilation and stenting of an obstructed
conduit in RV to PA conduit surgery patients.
• Ongoing risk for recurrent peripheral PS…serial catheterizations are indicated for
ongoing pulmonary artery rehabilitation
• higher risk for progressive aortic root dilation and AR

102. TOF + AVCD :
• risk for atrioventricular valve incompetence …. TR right ventricular dilation,
and dysfunction.

103. Congenital Absence of Pulmonary Valve Syndrome
• Chever in 1847
• incompletely formed, rudimentary pulmonary valve that typically is both stenotic and
regurgitant ; aneurysmally dilated PA, and a large malaligned outlet VSD.
• PDA is always absent….has been postulated as being responsible for its pathogenesis
and PA dysplasia.
• Other clinical features fairly typical in this disorder include the common association of
airway abnormalities that may lead to severe respiratory failure .
• The conal septal abnormalities and infundibular obstruction, however, in distinction to
typical tetralogy, are less severe or absent, and much of the PS results from annular
hypoplasia.

104. • Typically present in the neonatal period, and are diagnosed based on the presence
of their characteristic murmur and the presence of cyanosis.
• A significant proportion of patients will present with associated respiratory distress
or frank respiratory failure, often requiring mechanical ventilation.
• harsh to-and-fro murmur of PS followed by the diastolic murmur of PR….sound of
’sawing wood’
• CXR…Massively dilated Pas ; Infundibular dilation project leftward as a hump
shaped shadow ; Pulmonary vascularity is normal

105. • Some neonates present with severe bronchial obstruction and require immediate
tracheal intubation and mechanical ventilation, followed by early surgical repair.
• Moderate resp obstruction … lying prone may help … by relieving the anterior
vascular compression of bronchi
• Mild or no airway obstruction may require no additional support in the neonatal
period and go on to elective repair later in infancy.
• The surgical repair….. in addition to ventricular septal defect closure and right
ventricular outflow reconstruction, involves reduction of the aneurysmal
mediastinal pulmonary arteries to relieve bronchial compression.

106. Outcome of TOF + APV depends largely on the severity of airway disease.
Even after Sx,some pts continue to have bronchial obstruction
• Residual airway hypoplasia and deformity
• Abnormally branching segmental pulmonary arteries compressing the
intraparenchymal bronchi
As these patients grow, however, pulmonary function tends generally to improve as
PAP fall and the maturing tracheobronchial tree develops less compressible walls
and larger caliber.

107. ANAESTHETIC MANAGEMENT

108. Congenital Cardiac Surgery
• Perioperative concerns
• Increase in PVR or decrease in SVR leading to Right to Left shunt
• Tet Spells pre induction (crying/anxiety)
• Polycythemia and bleeding , coagulation abnormalities
• Air embolus
• RV failure

109. Congenital Cardiac Surgery
• Preoperative Preparation
• Heavy premedication
• Consider IM ketamine or inhalation induction but get rapid control of
airway.
• Keep SVR up and PVR down, maintain heart rate
• Intraoperative TEE

110. Congenital Cardiac Surgery
• Weaning from CPB, ratio RV : LV pressure should be < 0.8
• May need to keep PVR low with NTG, milrinone, dobutamine
phentolamine, PGE1
• May need RV inotrope post op
• May need temporary pacing wire.

111. THANK YOU