Cyanotic Heart Disease, Congenital, Paediatrics, Medicine, Cardiology, Chan En Ze,

1. Cyanotic congenital
heart disease

2. Classification of congenital heart
diseases
Group I :
Group II:
Group III:
Left to right shunts
Right to lefts shunts
Obstructive lesions

3. Left to right shunts
• Atrial Septal Defect
• Ventricular Septal Defect
• Patent Ductus Arteriosus

4. Right to Left Shunts
1) Tetralogy of Fallot
2)
3)
Tricuspid atresia
Ebstein’s anomaly
4) Transposition of Great Vessels
5) Truncus Arteriosus
6) Total Anomalous Pulmonary Venous
Return (TAPVR)

5. Obstructive Lesions
• Aortic stenosis
• Coarctation of the Aorta
• Pulmonic Stenosis

6. Cyanotic heart disease
Right to Left Shunt

7. Who is this guy?

8. ÉTIENNE-LOUIS ARTHUR FALLOT!
•
a French physician,
1888 Fallot
accurately described
in detail the four
anatomical
characteristics of
tetralogy of Fallot.

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

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

12. TOF
1) Vetricular Septal Defect

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

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

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

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

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

19. RV and LV pressures
becomes identical

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

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

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

23. Hence, the
more severe
the pulmonary
stenosis

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

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

26. 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

27. 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 cynosis because of less flow to the lung!

28. 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

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

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

31. Pulmonary obstruction results in delayed P2

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

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

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

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

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

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

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

39. Clinical Picture
• Symptomatic any time after birth
• Paroxysmal attacks of dyspnea
–
–
–
–
–
–
–
–
Anoxic spells
Predominantly after waking up
Child cry
Dyspnea
Blue
Lose conscious
Convulsion
Frequency varies from
once a few days to many
attack everyday

40. “tet spell”
• lethal,
• unpredictable episodes
• The mechanism
– spasm of the
infundibular septum,
which acutely
worsens the RV outlet
obstruction.

41. • Dyspnea on exertion
• Exercise intolerance

42. • Sitting posture – squatting
– Compensatory mechanism
– Squatting increases the peripheral vascular
resistance,
– which diminishes the
right-to-left shunt
– increases pulmonary
blood flow.

43. • Cyanosis during feeding
– Poor feeding
– fussiness, tachypnea, and agitation.
– Birth weight is low.
– Growth is retarded.
– Development and puberty may be delayed.

44. • Rarely, patient remain asymptomatic into
adult life.

45. Physical examination
•
•
•
•
Clubbing + Cyanosis (Variable)
Squatting position
Scoliosis – Common
bulging left hemithorax

46. • Prominent “a” waves JVP
• Normal heart size
– Mild parasternal impulse
• Systolic trill (30%)

47. • 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

48. • Retinal engorgement
• Hemoptysis

49. ECG
• ECG

50. ECG
• ECG

51. ECG
• ECG
wiLLiam
moRRow

52. 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)

54. • 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

55. • Normal heart size due
to the lack of
pulmonary blood flow
and congestive heart
failure

56. • Decreased pulmonary
vascularity

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

58. Echocardiography
• Reveals a large VSD
• overriding aorta
• variable degrees of right ventricular
outflow tract (RVOT) obstruction

59. Course and Complication
1) Each anoxic spell is potentially fatal
2) Polycytemia
1) Cerebrovascular thrombosis
3) Anoxic infaction of CNS
1) Neurological complication

60. 4) LUNG is an awesome 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
3) Hence TOF can cause:
1) Brain Abcess
2) Infective endocarditis
3) Paradoxical embolism

61. Management of anoxic spell
1) Knee chest position
2) Humified O2
3) Be careful not to provoke the child
1) Especially you are bad at gaining IV access
2) Ask for help from someone more experience
3) Permit the baby to remain with mother
4) Morphine 0.1 -0.2 mg/Kg Subcutaneous
5) Correct acidosis – Sodium Bicarb IV

62. 6) Propanolol
1) 0.1mg/kg/IV during spells
2) 0.5 to 1.0 mg/kg/ 4-6hourly orally
7) Vasopressors: Methoxamine IM or IV
drip
8) Correct anemia
9) GA is the last resort

63. Palliative Surgery
• Blalock-Taussig shunt
• Pott procedure
• Waterston shunt

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

65. Modified Blalock Taussig Shunt
• Goretex graft

67. Surgical Palliation

68. • Palliative operation prolong life
• Increase exercise tolerance

69. Definitive operation
• Closing the VSD
• Resecting infundibular
• 90% can return almost normal life after operation
• Complication:
–
–
–
–
–
RBBB
Residual VSD
Residual Pulmonary stenosis
Pulmonary regurgitation (pulmonary valve excised)
Risk 5%

70. Transposition of Great Areries
(TGA)
• Aorta originating
from the right
ventricle, and
pulmonary artery
originating from
the left ventricle
• Accounts for 5-7%
of all congenital
heart disease

71. TGA
• Survival is dependent on the presence of mixing
between the pulmonary and systemic circulation
• Atrial septal defect is essential for survival
• 50% of patients have a VSD
• Usually presents in the first day of life with
profound cyanosis
• More common in boys

72. TGA
• Exam :
• cyanosis in an
otherwise healthy
looking baby
• Loud S2 ( aorta is
anterior )
• CXR :
• Egg on side
• Narrow
mediastinum

73. TGA .. Acute Management
• PGE-1 with no supplemental O2
Maintain ductus arteriosus patency, this will
increase the effective pulmonary blood flow,
and thence increase the left atrial pressure,
therefore inhance the left to right shunt at the
atrial level
• Balloon atrial septostomy
Life saving procedure in the presence of
inadequate atrial septal defect

74. TGA .. Surgical Management
• Arterial switch
– with re-implantation of the coronary artery to the
new aortic site.
• Atrial switch :
– the old style surgery
– Redirecting the pulmonary and systemic venous
return to result in a physiologically normal state
– The right ventricle remains the systemic ventricle
– Rarely needed

75. Truncus Arteriosus
• The presence of a
common trunk that
supply the systemic,
pulmonary and
coronary circulation
• Almost always
associated with VSD
• 1.2-2.5% of all
congenital heart
disease

76. Truncus Arteriosus
• There are
different
anatomical tupes
of truncus
arteriosus
• This is relevant for
surgical repair

77. Truncus Arteriosus
• Generally patients have increased
pulmonary blood flow
• Degree of cyanosis is mild and may not be
evident clinically until late stage with
pulmonary vascular disease
• Presenting feature is congestive heart
failure (tachypnia, hepatomegally)

78. Truncus Arteriosus
• Exam is significant for
– Single S2
– Ejection click of the abnormal truncal valve
– Systolic murmur of truncal valve stenosis if
present
– Diaastolic murmur of truncal valve
insufficiency
– Gallop
• CXR : Cardiomegally , increased
pulmonary circulation

79. Managment
• Acute management
– No O2 to minimize pulmonary blood flow
– Diuretics
– Afterload reduction to inhance systemic blood flow
•Surgical management:
complete repair with VSD
closure and conduit placement
between the right ventricle and
pulmonary arteries
•Long term problems :
–truncal valve dysfunction
–RV conduit obstruction

80. Trcuspid Atresia
• Complete absence of
communication
between the right
atrium and right
ventricle
• About 3 % of
congenital heart
disease

81. Tricuspid Atresia
• There is an obligate interatrial communication
• Usually associated with VSD
• The pulmonary blood flow is dependent on the
size of the VSD
• Pulmonary blood flow can be increased or
decreased causing variable presenting symptoms
• If there is no VSD ( also called Hypoplastic right
ventricle) the pulmonary blood flow is
dependent on the PDA

82. Tricuspid Atresia- presentation
• The presentation will depend on the
amount of pulmonary blood flow
– If the PBF is decreased, the main presenting
symptom is cyanosis
– If the PBF is increased the presentation is that
of congestive heart failure
• CXR will also reflect the amount of
pulmonary blood flow

83. Tricuspid Atresia- EKG
Very characterestic : Left axis deviation

84. Management
PBF
Decreased
PGE-1, and minimal
supplemental O2 to
maintain ductal patency
Increased
No O2
Afterload reduction
Diuretics

85. Surgical Management
Single ventricle paliation
• First stage : to establish a reliable source of
PBF
– Aorta to pulmonary artery shunt ( BT shunt)
– Pulmonary arterial banding in cases of increased
PBF
• Second stage: Glenn Anastomosis ( superior
vena cava to pulmonary artery
• Third stage : Fontan anastomosis ( Inferior
vena cava to pulmonary artery

86. Total Anomalous Pulmonary Venous
Return (TAPVR)

87. TAPVR- Infracardiac

88. Radiography

89. Infracardiac type

91. Thank You