Hypoplastic left heart syndrome

Background

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Physiology

  • 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

  • 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 hypotension, acidosis, and respiratory distress
  • Symptoms can rapidly progress from cyanosis, increased respiratory distress, and poor feeding to heart failure and cardiogenic shock

Differential Diagnosis

Congenital Heart Disease Types

Evaluation

  • Echocardiography
  • Chest x-ray
    • Cardiomegaly, increased pulmonary vasculature
  • ECG
    • Right axis deviation, RV hypertrophy

Management

  • 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
    • Second stage (bidirectional Glenn procedure) performed at 3-6 months
    • Third stage (Fontan procedure) performed at 18-30 months

Disposition

  • Admit

See Also

External Links

References

  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