This document discusses congenital heart disease, including cyanotic heart lesions such as transposition of the great arteries and tetralogy of Fallot. For transposition of the great arteries, the aorta arises from the right ventricle and pulmonary artery from the left, requiring a mixing defect like VSD to allow blood flow. Clinical symptoms depend on anatomy and range from profound hypoxia without mixing to cyanosis with mixing. Management includes prostaglandins and atrial septostomy. For tetralogy of Fallot, the four defects cause right ventricular outflow obstruction, leading to cyanosis during spells that can be treated with positioning, sedation, and propranolol. Later treatments include palliative shunts

1. Please help my family to treat me ifeel always sever pai?

2. Congenital Heart Disease
Dr.Wahid Helmi .,
Pediatric consultant .
Zarka hospital Demiate

3. Normal Cardiac PressuresNormal Cardiac Pressures
120/<8
25/<5
<5
<8
120/80
25/15

4. EchocardiographyEchocardiography
 Describe the defect .
 Site of the defect .
 Location of the defect .
 Pressure gradient across the defect .
 Blood flow ,chamre enlargement.
 Coplications if occured

5. Congestive Cardiac FailureCongestive Cardiac Failure
 Tachycardia
 Tachypnea
 Tender enlarged liver
 Cardiomegaly.
 FAILURE TO THRIVE
 Treatment :-
 ACE-inhibitors - arterial vasodilator / afterload
reducing agent
 Digoxine – increases contractility and decreases
heart rate. .
 Diuretics - enhance renal secretion of sodium
and water by reducing circulating blood
volume and decreasing preload.
 Beta Blocker - increases contractility

6. Congenital Heart Disease-Congenital Heart Disease-
StructuralStructural
PINK
Shunts ( L to
R) :
 ASD
 VSD
 PDA
Stenosis:
 AS
 PS
 Coarctation
 HLHS
BLUE
 TOF
 TGA
 Tricuspid atresia
 Truncus Arteriosus
 TAPVR
 Ebstein’s
 Single ventricle

7. Cyanotic Heart LesionsCyanotic Heart Lesions

8. CyanosisCyanosis
 Arterial saturation less than 90% and a PO2 less
than 60 torr
 In all cyanotic heart lesions the amount of
cyanosis seen is dependent on the amount of
pulm blood flow
Decreased PBF- increased
cyanosis
Increased PBF- minimal
cyanosis but CHF may develop
 With 100% oxygen
PO2 <100 is cardiac disease

9. 5 “T’s”5 “T’s”
Most common cyanotic lesions of
the newborn
Tetralogy of Fallot
Transposition of the Great
Arteries
Truncus Arteriosus
Total Anomalous Venous

10. Transposition of the Great ArteriesTransposition of the Great Arteries
5% of all CHD
Boys 3:1
Most common cyanotic condition that
requires hospitalization in the first
two weeks of life

11. Transposition of Great Vessels
 Aorta arises from the right
ventricle, and the
pulmonary artery arises
from the left ventricle -
which is not compatible
with survival unless there
is a large defect present in
ventricular or atrial
septum.
artery
aorta

12. T G AT G A
Aorta arises from the right ventricle &
Pulmonary artery arises from the left
ventricle → two isolated circulation .
After birth there must be amixing defect
usually present ( PDA , VSD , ASD) to
maintain life .
VSD is present in 40% of cases .

13. T G AT G A
What is the result of
separation of the 2 circuits?
Hypoxemic blood circulating
in the body .
Hyperoxemic blood
circulating in the pulmonary
circuit .

15. T G AT G A
Defect to permit mixing of 2Defect to permit mixing of 2
circulations :-circulations :-
- ASD, VSD, PDA.- ASD, VSD, PDA.
VSD is present in 40% ofVSD is present in 40% of
casescases
Necessary for survivalNecessary for survival..

17. Clinical SymptomsClinical Symptoms
Depend on anatomy present
No mixing lesion and restrictive
PFO
Profound hypoxia.
Rapid deterioration .
Death in first hours of life.
Absent respiratory symptoms or

18. Clinical SymptomsClinical Symptoms
 Mixing lesion present (VSD or large
PDA)
Large vigorous infant .
Cyanotic .
Little to no resp distress.
Most likely to develop CHF
in first 3-4 months of life,
excessive sweating (a cold, clammy

19. Chest x-rayChest x-ray
 Egg-shaped heart in TGA

20. ManagementManagement
 Prostaglandin to establish patency of the
ductus arteriosus
Increases shunting from aorta into the
pulmonary artery
Increases pulmonary venous return
distending the left atrium
Facilitates shunting from the left to the
right atrium of fully saturated blood

21. ManagementManagement
Urgent operation (Rashkind balloon
atrial septostomy Procedure) if surgery
is not going to be performed immediately.
Total correction ( arterial switch ) at 1yea
of life .

22. Tetralogy of Fallot
Four defects are:
1. 2.
3.
4.

23. Tetraology of FallotTetraology of Fallot
1. VSD
2. RVOT Obstruction
3. RVH
4. Overriding aorta

24. Tetralogy of Fallot (TOF(

25. HaemodynamicHaemodynamic
When the RV contract in
pesence of PS blood is shunted
to the overriding aorta leading
to central cyanosis .
Mild RVD due to pulmonary
stenosis .
No shunt through the VSD ,

26. Clinical Presentation of Cyanotic TOFClinical Presentation of Cyanotic TOF
 Gradually the mother notice
cyanosis (1-2 months), squatting&
hypoxic spells.
Cyanosis increases with crying &
infection .
Cyanosis may appear in neonatal
period & may be absent (pink
fallot) .

27. Clinical Presentation of Cyanotic TOFClinical Presentation of Cyanotic TOF
Ejection systolic murmur heard at
the pulmonary area (from day 1) .
During the hyper cyanotic spells the
murmur will be very short or
inaudible .

28. Hypoxic SpellHypoxic Spell
(“TET Spell”)(“TET Spell”)
Peak incidence of 2-4 months
Characterized by:
Hyperapnea (Rapid and deep
respirations) .
Irritability and prolonged crying .
Deep attack of cyanosis with
crying during fedding .

29. Please help my family to treat me ifeel always sever pai?

30.  Coeur en sabot in
tetralogy of Fallot
Chest x-rayChest x-ray

31. TET Spell TreatmentTET Spell Treatment
1. Hold infant in knee-chest position
2. Sedation &pain relief .
3. Sodium bicarbonate for aidosis .
4. Propranolol (IV):- relief spasm of
the infundibulum & causes
peripheral vasoconstriction .

32. TreatmentTreatment
Iron therapy ,
Palliative shunt operation
( modified Blalock
Taussing operation).
Total correction at 1 year .

33. Good Luck
And
Thank you