Congenital cyanotic Heart Disease

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