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Pathology

When the ductus arteriosus closes after birth, the ductal tissue surrounds the 
aorta and causes narrowing/obstruction of the aorta; often distal to origin of left subclavian artery

Aetiology

Commoner in girls, associated with Turners Syndrome, bicuspid aortic valve, 
patent ductus arteriosus, Berry aneurysm or transposition of the great arteries

Signs

Hypertension, ejection systolic murmur radiating to the interscapular region, 
radio femoral delay, left ventricular hypertrophy and differences in systolic blood        
pressure between right and left arms

Symptoms

Failure to thrive, heart failure, dyspnoea, headache, epistaxis, claudication

Investigations

Antenatal ultrasound: Antenatal diagnosis may be 
ECG: Right ventricular hypertrophy 
Chest X-Ray: May be normal, signs of heart failure, cardiomegaly and notching    
of ribs from erosion by collateral vessel formation.
Echo: Assess for structural abnormality and confirm defect 
Classification: Classified by the relationship to the remnant of the ductus   
arteriosus, ligamentum arteriosum and insertion into the aorta:
Pre-Ductal – Most common
Ductal
Post-Ductal

Treatment

Medical: Resuscitation and prostaglandins to keep the duct patent 
Surgical: Percutaneous transcatheter balloon dilatation and stenting

Prognosis

Mortality from untreated defects or hypertension is high
Premature coronary artery disease, congestive cardiac failure, hypertensive 
encephalopathy

Key Facts

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Key Images

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Key References

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