Cyanotic congenital

heart diseases
Cyanosis: is blue discoloration of the
skin & mucous membranes & requires
a concentration of deoxygenated
haemoglobin of >5g/dl; of 2 types
 (1) central: cyanosis of mucous membranes &
tongue ; often due to circulatory or ventilatory
problems (hyperoxia test)
 Common causes :
 Respiratory

 CVS

 CNS

 Haematological
 Peripheral : blue tint in finger &
extremities
 Causes
 All of central Causes of central
 Hypothermia
 Raynuads phenomenon
 HF , hypovolemia
 Hypo glycaemia
 CHD produce cyanosis due to deoxygenated blood
bypassing the lungs & entering the systemic
circulation , this can be caused by:
– Rt ventricular out flow obstruction with Rt – to -
Lt shunt
– Bidirectional shunt
– Malposition of the great arteries
 CCHD account for 25% of all CHDs include :
1. 1 tetra logy of fallot (T O F)
2. Total anomalous pulmonary venous connection
3. Transposition of great arteries
4. Truncus arteriosus
5. Tricuspid atresia
6. Pulmonary atresia
7. Interrupted aortic arch
8. Hypoblastic Lt heart syndrome
9. Epstein's anomalies
Tetra logy of fallot (T O F )

 Consist of:
1. Pulmonary stenosis( Rt V outflow obstruction)
2. VSD
3. Over-riding of the aorta )
4. Rt V hypertrophy

The most common CCHD representing 55-70% of

CCHD.
 Pathophysiology:
 Determined by the degree of Rt V outflow
obstruction (the degree of cyanosis, the timing of
the onset of symptoms &the degree of hypertrophy
 Is usually at both Rt V infndibulum (sub aortic
area),& pulmonary valve
 Infundibular muscle is hypertrophied gradually with
age
 Complete obstruction of Rt V ( P Artesia with VSD )
is classified as sever form of TOF.
 Systemic venous return to Rt A & Rt V is normal
 When the V contracts in presence of stenosis the
blood shunted through the VSD into the aorta
 Persistent arterial desaturation occur& cyanosis
result .
 When the obst. Is mild to moderate a balanced
shunt is present across the VSD & the Pt. may not
be cyanosed ( a cyanotic [pink] TOF)
 Clinical manifestations :-
 Mild may present initially with HF due to shunt .
 Often cyanosis is not present at birth , with
increase hypertrophy occurs latter in the first year
of life .
 In sever cases it is noted immediately .
 In these cases when DA closed circulatory collapse
may occur .
 Clubbing ,dyspnoea ,paroxysmal hyper cyanotic