Aortic Coarctation & Interrupted Aortic Arch, Congenital

• • Congenital obstructive lesions

Aortic Coarctation

• • 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

Interrupted Aortic Arch

• • 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

Epidemiology

Aortic Coarctation

• • 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

Symptoms and Signs

Aortic Coarctation

• • 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

Interrupted Aortic Arch

• • Symptomatic in first few days of life

Imaging Findings

Aortic Coarctation

• • Chest film: Reversed "3" sign

  • Echocardiography: Diagnostic

  • MRI: Diagnostic

• • Evaluate for other cardiac or extracardiac anomalies

• • Echocardiography is usually sufficient to make diagnosis

  • Arteriography occasionally useful

Aortic Coarctation

• • 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

Interrupted Aortic Arch

• • PGE1 maintains hemodynamics

Surgery

Indications

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

  • Repair interrupted aortic arch early

Complications

• • Operative: Hemorrhage, damage to recurrent laryngeal nerve, ...