Coarctation of the aorta

Background

• Narrowing of aorta, most commonly at ductus arteriosus insertion site just distal to left subclavian artery
  • Less common: diffuse arch hypoplasia with long segment of narrowing proximal to left subclavian
• ~7% of live births with congenital heart disease
• Associated with bicuspid aortic valve (50%), VSD, PDA

Clinical Features

• Systolic murmur, often conducted to back
• Weak or absent femoral pulses, leg claudication, exercise intolerance
• Upper limb hypertension
• Usually diagnosed within first year of life, when presentation typically dramatic, particularly after closure of PDA in ductal-dependant critical coarctations.
  • Consider in all critically ill neonates, particularly with poor perfusion to lower extremities
  • Heart failure, shock
• Less severe coarctations may go undiagnosed for years, but can still lead to complications long-term:
  • Dysrhythmias
  • Heart failure
  • ICH
  • Aortic dissection
  • Infective endocarditis

Differential Diagnosis

Sick Neonate

THE MISFITS

• Trauma
• Heart
  • Congenital heart disease
  • Hypovolemia
• Endocrine
  • hypoglycemia
  • Hypothyroidism
  • Congenital adrenal hyperplasia
• Metabolic
  • Sodium
  • Calcium
  • Glucose
• Inborn errors of metabolism
• Seizure
• Formula / feeding problems
  • Hyponatremia
  • Hypocalcemia
  • FPIES / Food protein-induced enterocolitis syndrome
• Intestinal Disasters
  • Duodenal atresia
  • Midgut volvulus
  • Necrotizing enterocolitis
  • Intussusception
• Toxin
• Sepsis

Evaluation

• CXR
  • Rib notching due to collateral blood vessels
  • "Figure 3" sign seen in 50% of patients
  • Abnormal aortic knob shadow due to coarct and dilation of left subclavian
• EKG: LVH +/- LA enlargement
• Echocardiography
• Cardiac MRI

Management

Neonate in Shock (duct-dependent lesion)

• PGE1 0.1mcg/kg/min IV/IO
  • Side Effects:
    • Apnea (intubate)
      • Hypotension
      • Bradycardia
      • Flushing
• NS 10cc/kg
• Dobutamine

CHF

• O2 (give only if SpO2 <95%)
• Furosemide 1-2mg/kg IV
• Dopamine 5-10mcg/kg/min
• Dobutamine 5-10mcg/kg/min

Disposition

• Admit

See Also

• Congenital heart disease
• Neonatal resuscitation

External Links

• ACEP - Coarctation of the Aorta in the Older Child