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

Cyanosis in the newborn

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

pulmonary blood flow

Cyanotic

heart disease with increase

pulmonary blood flow

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

Clinical presentation
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
Clinical presentation
Newborn with severe cyanosis,

respiratory distress, metabolic acidosis

Resuscitation, Prostaglandin infusion
ECG, chest x-ray echocardiography
Radiofrequency pulmonary
valvotomy/balloon dilatation and PDA
stenting

Transposition of the great

arteries
Clinical presentation
Newborn with severe cyanosis,

respiratory distress, metabolic acidosis

Resuscitation, Prostaglandin infusion
ECG, chest x-ray and echocardiography

Urgent balloon atrial septostomy after

stabilization.

Total anomalous pulmonary venous

drainage
Clinical presentation
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

Cyanosis in the newborn

Diagnosis,

appropriate support and

stabilisation, selection and timing for
intervention.
Thank you