Hypoplastic Left Heart Syndrome

Aka: Hypoplastic Left Heart Syndrome, Hypoplastic Left Heart, Hyoplasia of the Aortic Tract Complex, HLHS

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II. Definitions

  1. Hypoplastic Left Heart Syndrome
    1. Under-developed entire left heart (left atrium, left ventricle, aorta, aortic valve, mitral valve)
    2. Severe symptom onset as the ductus arteriosus closes

III. Pathophysiology

  1. Ductus dependent lesion (along with reliance on right ventricle)
  2. Characteristic Anomalies
    1. Left ventricle hypoplasia
    2. Ascending aorta hypoplasia
    3. Aortic Stenosis (or hypoplasia or atresia)
    4. Mitral Stenosis (or hypoplasia or atresia)

IV. Associated Conditions

  1. Atrial Septal Defect (ASD) or Patent Foramen Ovale
  2. Patent Ductus Arteriousus
  3. Aortic Coarctation

V. Management: Surgery

  1. Uniformly fatal without surgical correction
  2. See Single Ventricle Defect Management
    1. Includes Acute decompensation following first Norwood Procedure

VI. References

  1. Civitarese and Crane (2016) Crit Dec Emerg Med 30(1): 14-23
  2. Sloas and Orman in Majoewsky (2013) EM:Rap 13(9): 10-11
  3. Rao in Berger (2013) Pediatric Hypoplastic Left Heart Syndrome, Emedicine
    1. http://emedicine.medscape.com/article/890196-overview#showall

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