1. Cyanotic congenital heart disease

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

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

4. Right to Left Shunts Tetralogy of Fallot
Tricuspid atresia
Ebstein’s anomaly
Transposition of Great Vessels
Truncus Arteriosus
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. Cyanotic heart disease
bluish or purplish tinge to the skin and mucous membranes.
5 g/dL of unoxygenated hemoglobin in the capillaries 
This usually correspond to oxygen saturation of 70-80%

8. Who is this guy?

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

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

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

13. TOF
Vetricular Septal Defect

14. TOF
Vetricular Septal Defect
Pulmonic Stenosis

15. TOF Vetricular Septal Defect Pulmonic Stenosis
Overriding of dextroposed aorta

16. TOF Vetricular Septal Defect Pulmonic Stenosis
Overriding of dextroposed aorta
Right Ventricular hypertrophy

17. TOF Vetricular Septal Defect Pulmonic Stenosis
Overriding of dextroposed aorta
Right Ventricular hypertrophy
Concentric R ventricular hypertrophy without cardiac enlargement

18. TOF Vetricular Septal Defect Pulmonic Stenosis
Overriding of dextroposed aorta
Right Ventricular hypertrophy
Concentric R ventricular hypertrophy without cardiac enlargement
Increase in right ventricular pressure*

20. RV and LV pressures becomes identical

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

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

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

24. Hence, the more severe the pulmonary stenosis

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

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

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

28. 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!

29. 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

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

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

32. Pulmonary obstruction results in delayed P2

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

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

35. Since P2 is inaudible, hence S2 = A2 + P2 [S2 is single sound]
(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!
P2 become soft or inaudible
Pulmonary artery pressure reduce
Pulmonary obstruction results in delayed P2

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

37. Diastolic interval is clear
No S3
No S4

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

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

40. 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

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

42. Dyspnea on exertion
Exercise intolerance

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

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

45. Rarely, patient remain asymptomatic into adult life.

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

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

48. S1 normal S2 single Murmur Ejection aortic click 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

49. Retinal engorgement
Hemoptysis

50. ECG
ECG

51. ECG
ECG

52. ECG
wiLLiam
moRRow
ECG

53. 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)

55. 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

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

57. Decreased pulmonary vascularity

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

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

60. Course and Complication
Each anoxic spell is potentially fatal
Polycytemia
Cerebrovascular thrombosis
Anoxic infaction of CNS
Neurological complication

61. 4) LUNG is an awesome filter.
Bypassing it may not be a good idea!
TOF, venous blood from gut, peripheral system by pass the lung and re-enter circulation
Hence TOF can cause:
Brain Abcess
Infective endocarditis
Paradoxical embolism

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

63. Vasopressors: Methoxamine IM or IV drip Correct anemia
Propanolol
0.1mg/kg/IV during spells
0.5 to 1.0 mg/kg/ 4-6hourly orally
Vasopressors: Methoxamine IM or IV drip
Correct anemia
GA is the last resort

64. Palliative Surgery Blalock-Taussig shunt Pott procedure
Waterston shunt

65. Blalock Taussig Shunt
Subclavian artery – Pulmonary artery anastomosis

66. Modified Blalock Taussig Shunt
Goretex graft

68. Surgical Palliation

69. Palliative operation prolong life
Increase exercise tolerance

70. 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%

71. 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

72. 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

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

74. 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

75. 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

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

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

78. 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)

79. 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

80. 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

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

82. 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

83. 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

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

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

86. 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

87. Total Anomalous Pulmonary Venous Return (TAPVR)

88. TAPVR- Infracardiac

89. Radiography

90. Infracardiac type

92. Thank You