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Interrupted
aortic arch (Clinical
presentation (Cyanosis, Shock,…
Interrupted
aortic arch
Definition
Congenital structural heart lesion
where there is no connection between
the proximal aorta and aorta distal to
the ductus arteriosus
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Pathophysiology
Defect
No connection between proximal aorta and aorta distal to the ductus arteriosus
Patency of ductus arteriosus allowing some cardiac output to the body via the pulmonary artery
Often co-exists with a VSD, allowing mixing of ox and deox blood
Mechanism
Blood from LV can enter aorta and supply the carotids to the brain, but cannot connect to the descending aorta to supply the body
Duct dependent system (R-L shunt via duct)
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Diagnosis
Examination
Cardio
Cyanosis, shock
Absent femoral and L brachial pulse
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History
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POH
Antenatal: scans, bloods, growth
Perinatal: gestation, delivery, weight
Postnatal: growth, complications
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Management
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Definitive
Surgical
Corrective surgery
Indication: ASAP first few days of life
MOA: closure of VSD, repair of arch
Conservative
Information, advice, support
Medical
Prostaglandins
Indication: asap cyanosed neonate
E.g. aloprostil
MOA: maintains duct in duct-dependent circulation
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