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  • • Congenital obstructive lesions

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Aortic Coarctation

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  • • 98% are located near aortic isthmus (proximal to ductus arteriosus)

    • Occurs in 3 contexts

    • -Isolated

      -Associated with ventricular septal defect (VSD)

      -Associated with severe intracardiac anomalies and extensive arch involvement

    • Associated anomalies (occur in 70% of neonates, 15% of older children): patent foramen ovale (PFO), ductus arteriosus, VSD, bicuspid aortic valve (40%), and Shone syndrome

    • Obstruction leads to systolic and diastolic hypertension in upper extremities

    • Collaterals eventually develop

    • Patent ductus arteriosus maintains distal flow

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Interrupted Aortic Arch

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  • Type A (35%): Absence of arch distal to left subclavian artery

    Type B (60%): Between left carotid and left subclavian artery

    Type C (5%): Between innominate and left carotid artery

    • Ductus arteriosus maintains distal flow

    • Almost always has associated VSD, truncus arteriosus, aortopulmonary window, subaortic stenosis, or transposition of great vessels

    • Type B associated with aberrant right subclavian artery

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Epidemiology

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Aortic Coarctation

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  • • Occurs in 0.2-0.6 per 1000 live births

    • 5-8% of congenital cardiovascular anomalies

    • 2-5 times more common in males

    • 15-30% of patients with Turner syndrome have aortic coarctation

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Symptoms and Signs

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Aortic Coarctation

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  • • 2 distinct presentations

    • -Early infancy: Severe congestive heart failure, sudden cardiovascular collapse with duct closure

      -Older children: Many asymptomatic; headache; pain in calves when running; frequent nose bleeds; hypertension of upper extremities; LV hypertrophy; classically, notched ribs (from enlarged intercostals vessels) seen in children older than 4 years

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Interrupted Aortic Arch

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  • • Symptomatic in first few days of life

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Imaging Findings

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Aortic Coarctation

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  • Chest film: Reversed "3" sign

    Echocardiography: Diagnostic

    MRI: Diagnostic

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  • • Evaluate for other cardiac or extracardiac anomalies

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  • • Echocardiography is usually sufficient to make diagnosis

    • Arteriography occasionally useful

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Aortic Coarctation

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  • • In neonate, alprostadil (PGE1 ) to maintain ductus patency, mechanical ventilation, and HCO3

    Surgical options: Resect with end-to-end anastomosis, subclavian flap aortoplasty ± resection, patch aortoplasty, interposition graft, percutaneous balloon

    • Repair performed via left thoracotomy

    • Median sternotomy if concomitant intracardiac anomaly repair

    Neonate: Primary repair or subclavian flap preferred

    Child: Primary repair or interposition graft preferred

    • Recoarctation (gradient > 20 mm Hg), consider balloon or patch aortoplasty

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Interrupted Aortic Arch

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  • • PGE1 maintains hemodynamics

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Surgery

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Indications

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  • • Once diagnosis of aortic coarctation is made, correct when stabilized

    • Repair interrupted aortic arch early

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Complications

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  • • Operative: Hemorrhage, damage to recurrent laryngeal nerve, Horner syndrome, chylothorax, paraplegia

    • Postoperative mesenteric vasculitis can be minimized with good blood ...

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