Hypoplastic Left Heart Syndrome, Pediatric

Reviewed 06/2017



Hypoplastic left heart syndrome (HLHS) is a continuum of congenital cardiac defects resulting from severe underdevelopment of the structures of the left side of the heart (left atriu...



  • In the current era, HLHS is often diagnosed prenatally.

  • Postnatal signs and symptoms:

    • Respiratory distress (tachypnea, grunting, flaring, retractions)

    • Cyanosis

    • Cardiovascular collapse and...


Initial Stabilization

During initial resuscitation and stabilization of a newly diagnosed infant: 
  • Prostaglandin E1 therapy should be initiated as soon as possible to maintain ductal patency.

  • A...

Additional Reading

  • Alsoufi  B, Bennetts  J, Verma  S, et al. New developments in the treatment of hypoplastic left heart syndrome. Pediatrics.  2007;119(1):109–117. [View Abstract]

  • Grossfeld  P. H...



  • 746.7 Hypoplastic left heart syndrome

  • 758.6 Gonadal dysgenesis

  • 759.89 Other specified congenital anomalies


  • Q24.8 Other specified congenital malformations of heart

  • Q96.9 Turner’s syndrome, ...


  • Q: What should the differential diagnosis include when an infant with HLHS who’s undergone stage I palliation presents with cyanosis and respiratory distress?

  • A: Modified Blalock-Taussig shunt thro...

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