Cyanosis in the Newborn

Ductus arteriosus

Fetal circulation
Present of PFO and ductus arteriosus; shunting R L

Foramen ovale Eustachian flap valve

High peripheral pulmonary resistance Upper limbs/brain receive more oxygenated blood

Ductus venosus Umbilical veins

Umbilical arteries

Changes at birth
PVR falls (with first breath and lung expansion pulmonary vasoconstriction ceases) System vascular resistance rises (removal of placental circulation) PDA closes (o in aortic oxygen saturation) PFO closes (o in pulmonary venous return) Ductus venosus closes Note: PA pressure qqq 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

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%)

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%)

Obstructive lesions : (11.6%)

Pulmonary stenosis Aortic stenosis Coarctation of the aorta

Patent ductus arteriosus (21%)

Pulmonary atresia with ventricular septal defect

Ductus Collateral vessel

Large overriding aorta Blocked pulmonary valve

Ventricular septal defect

Thick right ventricle

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 Right atrium Enlarged right atrium Coronary sinus Enlarged right ventricle

Left vertical vein

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 p + 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 hypertrophy Dilated 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.

Cyanosis in the newborn

Diagnosis, appropriate support and stabilisation, selection and timing for intervention.