Hypoplastic Left Heart Syndrome, Pediatric

Laura Mercer-Rosa, MD, MSCE and Jie Tang, MD, MS Reviewed 10/2018
 


BASICS

DESCRIPTION

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...

DIAGNOSIS

HISTORY

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

  • Postnatal signs and symptoms:

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

    • Cyanosis

    • Cardiovascular collapse and...

TREATMENT

  • 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.

      • Av...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

The admission for the first operation usually lasts for about 3 to 4 weeks after birth. Patients are watched to ensure stable oxygen saturation and weight gain. N...

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 on OvidInsights]

  • Gr...

CODES

ICD9

  • 746.7 Hypoplastic left heart syndrome

  • 758.6 Gonadal dysgenesis

  • 759.89 Other specified congenital anomalies

ICD10

  • Q24.8 Other specified congenital malformations of heart

  • Q96.9 Turner’s syndrome, u...

FAQ

  • Q: What is the significance of an intact or restrictive atrial septum during the initial stabilization of a neonate with HLHS?

  • A: In HLHS patients, restriction to left-to-right flow across the atri...

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