Relevant physical signs

  • Peripheries
    • Stigmata of infective endocarditis (association with bicuspid aortic valve)
    • Radio-radial delay
      • Coarctation proximal to the left subclavian artery
      • If thoracotomy scar present:
        • Repair of coarctation (left subclavian artery used in repair)
        • Blalock-Taussig shunt
    • Radio-femoral delay
    • Bounding carotid pulsation
    • Jugular venous pulsation – elevated in pulmonary hypertension
  • Precordium
    • Apex beat
      • Usually not displaced
      • Heaving in character if left ventricular hypertrophy present
      • May be displaced with thrusting character with concomitant aortic regurgitation
    • Parasternal heave – pulmonary hypertension
    • Palpable thrills
    • Auscultation
      • Ejection systolic murmur over left sternal edge radiating to the back
      • Ejection systolic murmur over the aortic area, radiating to carotids – aortic stenosis
      • Early diastolic murmur over lower left sternal edge – aortic regurgitation
  • Underlying Turner’s syndrome
    • Short stature
    • Shield-like chest
    • Wide-spaced nipples
    • Webbed neck
    • High-arched palate

Investigation

  • Electrocardiogram: left ventricular hypertrophy
  • Chest radiograph
    • Rib notching (collaterals from dilated, tortuous posterior intercostal arteries)
    • Prominent aortic knuckle
    • Cardiomegaly and pulmonary congestion
  • Echocardiogram to confirm diagnosis and assess severity, LVEF
    • Look for and assess severity of co-existent valvular heart disease

Complications

  • Hypertension
  • Hypoplastic limbs (legs if distal to left subclavian artery)
  • Left ventricular failure

Management

  • Surgical repair with excision of coarctation
  • Balloon angioplasty with stent insertion