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Pathology

There are two separate circulation:
The aorta is connected to right ventricle receiving deoxygenated blood
The pulmonary artery is connected to the left ventricle receiving oxygenated   
blood from the lungs 
These children are cyanosed and this defect is incompatible with life unless
there are separate defects to allow mixing of blood (ASD, VSD or PDA) 

Aetiology

Unknown

Signs

Cyanosis at birth or presents with duct closure 


Symptoms

Cyanosis .

Investigations

Antenatal ultrasound: Antenatal diagnosis may be made 
ECG: Often normal
Chest X-Ray: Classical ‘egg on its side’ appearance of the heart shadow 
Echo: To assess for structural abnormality and confirm diagnosis

Treatment

Medical: Resuscitation and prostaglandins keep the ductus arteriosus patent 
Surgical: Balloon atrial septostomy is required to allow mixing of the blood.  
Definitive treatment is arterial switch operation, which is carried out 
in the first 2 weeks of life

Prognosis

Condition is fatal unless treated.
Arrhythmias, heart failure and infective endocarditis

Key Facts

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Key Images

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Key References

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