Cyanosis in The Newborn

Cyanosis in the Newborn
Dr.Hasri Samion
Ductus
arteriosus
Fetal circulation
Present of PFO and
ductus arteriosus;
shunting R
L
Foramen
ovale
High peripheral
pulmonary resistance
Eustachian
flap valve
Ductus
venosus
Umbilical
veins
Upper limbs/brain
Umbilical
arteries
receive more
oxygenated blood
Changes at birth
PVR falls (with first breath and lung expansion

pulmonary vasoconstriction ceases)
System vascular resistance rises (removal of

placental circulation)
PDA closes ( in aortic oxygen saturation)
PFO closes ( in pulmonary venous return)
Ductus venosus closes
Note: PA pressure in the first 2 - 3 days of life

gradual fail to normal adult level by 2 weeks
Normal circulation
Left ventricle (systemic)

with high systolic
pressure
Right ventricle (venous)

lower systolic pressure
L
R shunt in septal
or arterial defects
Cyanosis in the newborn

Obstructive lesions/Ductal dependent
pulmonary blood flow
Critical pulmonary artery stenosis
Pulmonary atresia with ventricular septal defect
Pulmonary atresia; intact ventricular septum
Tricuspid atresia with restrictive VSD
Single ventricle with pulmonary atresia
Severe Ebsteins anomaly

Other possible underlying causes
Complete transposition with intact
ventricular septum
Common mixing (univentricular heart,
TAPVD)
Functional pulmonary atresia in severe
Ebsteins anomaly.
Lung diseases
Bristol congenital heart disease register 1966-87

Incidence of lesion comprising more than 1% of total
Lesion
1966-76
1977-87
1966-87
Ventricular septal defect

Atrial septal defect
Pulmonary stenosis
Patent ductus arteriosus
Coarctation
Aortic stenosis
Tetralogy of Fallot
Transposition
Atrioventricular septal defect
Pulmonary atresia
Primitive ventricle
Tricuspid atresia
Mitral regurgitation
Aortic atresia
Hypertrophic cardiomyopathy
Total anomalous pulmonary
venous connection
Ebsteins anomaly
29.4
9.4
9.1
6.4
6.2
5.4
6.2
4.5
2.7
3.0
1.2
1.3
1.1
1.2
36.1
8.2
6.9
7.9
5.9
5.7
4.6
3.8
3.6
2.6
2.2
1.5
1.4
1.4
1.3
33.3
8.7
7.8
7.3
6.0
5.6
5.3
4.1
3.2
2.8
1.9
1.4
1.4
1.3
1.3
1.3
1.2
1.2
-
1.2
1.0
Total
89.6
94.3
93.6
What are the types of congenital

heart disease? (IJN 1996-1997 n =3284)
Acyanotic defect (78.2%)
Septal defects : (45%)
Ventricular septal defect

Atrial septal defect
Atrioventricular septal
defect
Cyanotic defect (18.3%)
Obstructive lesions : (11.6%)
Pulmonary stenosis
Aortic stenosis
Coarctation of the aorta
Patent ductus arteriosus

(21%)
Tetralogy of Fallot (9%)

Transposition of the great
arteries (2.7%)
Pulmonary atresia;
ventricular septal defect
(2.1%)
Complex univentricular heart
(2.2%)
TAPVD (0.7%)
PAIVS (0.7%)
Pulmonary atresia with ventricular septal

defect
Ductus
Collateral vessel
Large overriding aorta

Blocked
pulmonary valve
Thick right
ventricle
Ventricular septal
defect
Figure 9.6 : Blood supply to the lungs in pulmonary atresia with

ventricular septal defect.
- low resistance vascular bed. (a) the
central pulmonary arteries are present and supply to the lungs is
through the patent ductus arteriosus only. (b) The central
pulmonary arteries are absent and supply to the lungs is through
major aorto-pulmonary collateral arteries which arise from the
aorta and have stenosis on them as they enter the lungs
Simple transposition
Aorta
Pulmonary artery
Transposition of great arteries
2 separate circulation
Survival depends on mixing of blood between the

pulmonary circulation of oxygenated blood and
systemic deoxygenated blood through the PDA or
foramen ovale
Severe cyanosis develops with closure of PDA and PFO
Total anomalous pulmonary venous

drainage
Right superior
vena cava
Left vertical vein
Right atrium
Enlarged right atrium
Coronary sinus
Enlarged right ventricle
Hepatic vein
Cyanotic heart disease in the newborn:

Clinical presentation
Anatomical physiologic entities

Cyanotic
heart disease with reduced

Cyanotic
heart disease with increase

Cyanotic heart disease in the newborn:

clinical presentation
The critical ill newborn/neonates
Onset of symptoms correlates with ductal closure
Rapid deterioration + severe hypoxia,
hypotension, metabolic
acidosis and renal failure
Severe right heart obstruction - pulmonary atresia
with/without VSD
Univentricular with pulmonary atresia
Ductus dependent pulmonary circulation
TGA
Electrocardiography (ECG)
QRS
axis
Ventricular
Dilated
hypertrophy
right atrium
Chest x-ray
Cardiac position - possible dextrocardia

Heart size, great vessels (pulmonary artery size, position of
aortic arch)
Cardiac chambers
Lung fields
plethora - excessive pulmonary blood flow
oligemia - reduced pulmonary blood flow
Characteristic features
Bootshaped heart TOF/PAVSD
Egg on side - TGA
Narrow pedicle
Snow man heart - total anomalous pulmonary venous drainage
(TAPVD)
TGA
Cardiomegaly
with egg on side

appearance
Diagnosis of CHD
Symptoms
Clinical examination
ECG, Chest x-ray

Echocardiography
CT scan, MRI
Cardiac catheterization
The critically ill neonate

Acyanotic
Coarctation of aorta
Interrupted aortic arch
Hypoplastic left heart
syndrome
Severe critical AS
Cyanotic
Severe critical PS
Pulmonary atresia, intact
septum
Pulmonary atresia, VSD
TGA
Obstructed TAPVD
Complex univentricular heart
Cardiovascular collapse, acidosis + hypoxia

May be PDA dependent systemic/pulmonary circulation
Treatment: Prostaglandin, correction of acidosis,
stabilize haemodynamics definitive treatment depending
on the lesion
Critical Pulmonary Stenosis

Newborn with severe cyanosis,
respiratory distress, metabolic acidosis

Resuscitation, Prostaglandin infusion
ECG, chest x-ray and echocardiography
Urgent valvuloplasty after stabilization.
Pulmonary atresia with intact

septum

ECG, chest x-ray echocardiography
Radiofrequency pulmonary
valvotomy/balloon dilatation and PDA
stenting
Transposition of the great

arteries

ECG, chest x-ray and echocardiography
Urgent balloon atrial septostomy after
stabilization.
Total anomalous pulmonary venous

drainage
Respiratory distress, heart failure
cyanosis and hypotension

ECG and chest x-ray
Echocardiography
Urgent relief of the obstruction
Issues?
Correct
diagnosis and associated

problems.
Possible options for either catheter
intervention or surgery
Age and weight at diagnosis
Ideal timing for intervention or surgery
Result and long-term problems.
Balloon atrial septostomy
PDA stenting

Diagnosis,
appropriate support and

stabilisation, selection and timing for
intervention.
Thank you

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Cyanosis in the Newborn

PVR falls (with first breath and lung expansion

System vascular resistance rises (removal of

PDA closes ( in aortic oxygen saturation)

PFO closes ( in pulmonary venous return)

Ductus venosus closes

Note: PA pressure in the first 2 - 3 days of life

Left ventricle (systemic)

Right ventricle (venous)

Cyanosis in the newborn

Cyanosis in the newborn

Bristol congenital heart disease register 1966-87

Ventricular septal defect

What are the types of congenital

Septal defects : (45%)

Ventricular septal defect

Cyanotic defect (18.3%)

Obstructive lesions : (11.6%)

Patent ductus arteriosus

Tetralogy of Fallot (9%)

Pulmonary atresia with ventricular septal

Large overriding aorta

Figure 9.6 : Blood supply to the lungs in pulmonary atresia with

Transposition of great arteries

Survival depends on mixing of blood between the

Severe cyanosis develops with closure of PDA and PFO

Total anomalous pulmonary venous

Left vertical vein

Cyanotic heart disease in the newborn:

Anatomical physiologic entities

heart disease with reduced

heart disease with increase

Cyanotic heart disease in the newborn:

Cardiac position - possible dextrocardia

with egg on side

ECG, Chest x-ray

The critically ill neonate

Cardiovascular collapse, acidosis + hypoxia

Critical Pulmonary Stenosis

respiratory distress, metabolic acidosis

Pulmonary atresia with intact

respiratory distress, metabolic acidosis

Transposition of the great

respiratory distress, metabolic acidosis

Urgent balloon atrial septostomy after

Total anomalous pulmonary venous

cyanosis and hypotension

diagnosis and associated

Balloon atrial septostomy

Cyanosis in the newborn

appropriate support and

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