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Congenital Heart Disease, Cyanotic, Emergency Medicine

Reviewed 06/2017



  • Aberrant embryonic development results in mixing of deoxygenated and oxygenated blood returning to systemic circulation by 2 mechanisms:

    • Right-to-left intracardiac shunt

    • Anatomic defec...


  • Most common initial ED presentations of cyanotic congenital heart disease (CHD):

    • Cyanosis

    • CHF

    • Circulatory collapse

  • Physiologic stress triggers cyanosis in older patients with CHD:

    • Cardiac shunt o...


Initial Stabilization/Therapy

  • Maintain warmth (cold ↑ O2 consumption).

  • Treat hypoglycemia and acidosis.

  • Maintain oxygenation.

  • Establish IV access.

  • Prepare for endotracheal intubation.

  • High oxygen ...



Admission Criteria

  • All newborns with suspected CHD:

    • Admit to pediatric ICU.

  • CHD with acute worsening of cyanosis or CHF

  • CHD with symptomatic pneumonia or respiratory syncytial virus

Discharge Criteria

Pearls and Pitfalls

  • Visual appearance of cyanosis requires >3–5 mg/dL deoxygenated hemoglobin.

  • Duct-dependent lesions:

    • Present at 2–3 wk of age

    • Sudden cyanosis or cardiovascular collapse

    • Treat with PGE...

Additional Reading

  • Apitz  C, Webb  GD, Redington  AN. Tetralogy of Fallot. Lancet.  2009;374:1462–1471.

  • Bonow  RO, Mann  DL, Zipes  DP, et al., eds. Congenital heart disease. Braunwald's Heart Dis...



  • 745.2 Tetralogy of fallot

  • 745.4 Ventricular septal defect

  • 746.89 Other specified congenital anomalies of heart

  • 747.21 Anomalies of aortic arch

  • 429.3 Cardiomegaly

  • 745.10 Complete transposition of ...

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