Hypoplastic left heart syndrome




  • With a diminutive LV, the RV must perfuse both pulmonary and systemic circulations
  • Survival is dependent on:
    • PDA (for systemic perfusion from RV to the aorta)
    • Nonrestrictive ASD to ensure adequate mixing of oxygenated and deoxygenated blood

Clinical Features

Infant with cyanosis due to hypoplastic left heart syndrome.
  • Asymptomatic at birth because of adequate systemic perfusion through a PDA and initially high pulmonary vascular resistance
  • As the PDA begins to close and pulmonary vascular resistance decreases, may develop:
  • Symptoms can rapidly progress from cyanosis, increased respiratory distress, and poor feeding to heart failure and cardiogenic shock

Differential Diagnosis

Congenital Heart Disease Types



  • Stabilize cardiopulmonary function prior to surgery
  • Maintain PDA to provide sufficient mixing of oxygenated and deoxygenated blood, and adequate systemic perfusion
    • Prostaglandin E1
      • Start infusion at 0.05 mcg/kg/min IV and titrate up to 0.1 mcg/kg/min, monitoring for hypotension and apnea
      • Side Effects: Hypotension, Bradycardia, Seizures and Apnea
  • Staged surgical repair
    • First stage (Norwood procedure) performed in neonates
      • SpO2 ranges in mid 70s-80s
    • Second stage (bidirectional Glenn procedure) performed at 3-6 months
      • SpO2 ranges in mid 70s-80s
    • Third stage (Fontan procedure) performed at 18-30 months
      • Preload dependent with SpO2 ranging from low to high 90s


  • Admit

See Also

External Links


  1. Gordon BM. Decreasing number of deaths of infants with hypoplastic left heart syndrome. J Pediatr. 2008;153(3):354-8.
  2. Knipe K et al. Cyanotic congenital heart diseases. Radiopaedia. http://radiopaedia.org/articles/cyanotic-congenital-heart-disease