Cynotic conginital heart disease ( tetralogy of fallot & transposition of great arteries ) - Ref:. Nelson Pediatric

1. C.CHD
By:
Abdullah Taskin Abdulabri

2. Introduction

3. Non‐cyanotic CHD
(L  R)
• Atrial septal defects (ASD)
• Ventricular septal defects (VSD)
• Patent ductus arteriosus (PDA)
• Obstruction to blood flow
• Pulmonic stenosis (PS)
• Aortic stenosis (AS)
• Aortic coarctation
Cyanotic CHD
(R L)
• Tetralogy of Fallot (TOF)
• Transposition of the great
vessels
• Tricuspid atresia (TA)
• Total anomalous pulmonary
venous return (TAPVR)
• Truncus arteriosus
• Hypoplastic left heart syndrome
• Pulmonary atresia (PA)
• Double outlet right ventricle
Introduction

4. cyanosis • Cyanosis is a bluish or purplish tinge to the skin and mucous
membranes.
• Peripheral cyanosis (blueness of the hands and feet) .
• Central cyanosis, seen on the tongue as a slate blue colour, is
associated with a fall in arterial blood oxygen tension.
• It can only be recognized clinically if the concentration of reduced
haemoglobin in the blood exceeds 5 g/dl.
• Persistent cyanosis in infant is nearly always CHD .

5. cyanosis

6. cyanosis

7. cyanosis Hyperoxia (nitrogen washout) test
• cardiac VS respiratory causes of cyanosis.
• Infant placed in 100% oxygen for ten minutes.
• It works on the assumption that if there is right to left
shunting in cyanotic heart disease, no amount of
oxygenation in the pulmonary circulation will alter the
desaturation effect of the shunt.
• However, if there is a pulmonary defect causing cyanosis
this may be corrected by increasing the inspired oxygen.

8. cyanosis Hyperoxia (nitrogen washout) test
• If the infant remains cyanotic after this period, the cyanosis
is said to be secondary to cyanotic heart disease.
• This can be defined in blood gases as follows:

9. TOF • Most common cause of C.CHD .
• Characterized by :
Problem Of Small Heart

10. TOF

11. TOF • Key problem : RV outflow obstruction
• VR > RH > LH > body , without going to lung !
• Degree of PS > severity of disease .
• Presentation :
• Murmur in 1st 2 month of life . ( inc. severity > shorter)
• Cyanosis & softer
• Typically :
( Single S.2 + RV impulses at Left sternal border )

12. TOF • Key problem : RV outflow obstruction
• VR > RH > LH > body , without going to lung !
• Degree of PS > severity of disease .
• Presentation :
• Murmur in 1st 2 month of life . ( inc. severity > shorter)
• Cyanosis & softer
• Typically :
( Single S.2 + RV impulses at Left sternal border )
•Investigation :
• ECG : R‐axis deviation + RV hypertrophy
• CX‐ray : boot shaped heart & ABG
• Echo : PS , coronary A > LAD arise from R‐coronary A( 5%)

13. TOF

14. TOF Tet spells
• hypercyanotic episodes precipitated by a sudden increase
in righ‐to‐left shunting of blood.
• lf sever > loss of murmur , unconsciousness , convulsion ,
hemiparesis & death .
• They can be elicited by activity (e.g. eeding, crying), or they
may occur without warning.
• The classic description is of a patient who becomes cya
notic and then assumes a squatting position to relieve the
cyanosis and hypoxia.

15. TOF • Complication :
• cerebral thromboembolism & cerebral abscess
•Treatment :
• Initially is medical
• Definitive surgical repair at 5 month .
• If sever cyanosed > artificial tube btw subclavian art. & PA
or balloon dilatation of RV outflow .
• Spells : mostly self limiting & followed by period of sleep
• If > 15 min , need treatment :

16. TOF • If > 15 min , need treatment :
1. sedation and pain relief ( morphine )
2. IV propranolol or a‐adreno‐receptor agonist
3. IV volume
4. Bicarbonate for acidosis
5. Muscle paralysis
6. Ventilation

18. TGA •Transposition of the Great Arteries (TGA)
•the aorta arises from the right ventricle and the
pulmonary artery arises from the left ventricle,
creating two parallel circuits.
• body to body circulation without lung .
•Lung to lung circulation without body .
•Fortunately : naturally ASD , VSD & PDA or therputic
short term .

19. TGA

20. TGA • Clinically :
• cyanosis “ductal closure”
• S2 loud and single
• Usually No murmur “but may +”
•Investigation :
• CX‐ray :
classic CXR presentation features a heart with an “egg on a
string” appearance
• ECG : normal
• Echo : essential for Dx .

21. TGA

22. TGA •Management :
• in sever key is > mixing ( ASD , VSD , PDA )
• Maintain PDA by prostaglandin
• Balloon atrial septostomy
• Surgery of arteries switching in neonate period
• Coronary .A transferred to new aorta .

23. TGA&TOF

24. References:• Nelson essential 7th E.
•Illustrated pediatric 4E