Congenital Cyanotic Heart Disease
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Congenital cyanotic heart disease – aka blue baby syndrome accounts for about 25% of all congenital heart defects

Causes

There are lots more! In the exam, you might be asked to examine a child, and be expected to come to a conclusion of ‘congenital cyanotic heart disease’, and then give a few examples of what can cause it:

Presentation

  • Most cases are duct dependent – which means the ductus arteriosus allows blood to flow left to right across the duct, to the lungs. However, the duct may close after 1-2 days, and at this time, symptoms may worsen
  • Pale / central cyanosis
  • Blue baby!
  • Possible impalpable femoral pulses / weak peripheral circulation
  • Acidosis

Diagnosis

  • Echocardiography
  • Blood culture to rule out sepsis

Differentials

Treatment

  • Maintaining the PDA is the key to survival
  • Immediate management
    • Stabilise airway and circulation, using mechanical ventilation if necessary
  • IV prostaglandin (PGE) – to maintain the PDA. Keep a close eye on the therapeutic level, and watch for:
    • Apnoea, seizures, vasodilation (and flushing), hypotension

References

  • Congenital Heart Disease in Children
  • Murtagh’s General Practice. 6th Ed. (2015) John Murtagh, Jill Rosenblatt
  • Oxford Handbook of General Practice. 3rd Ed. (2010) Simon, C., Everitt, H., van Drop, F.
  • Beers, MH., Porter RS., Jones, TV., Kaplan JL., Berkwits, M. The Merck Manual of Diagnosis and Therapy

Read more about our sources

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Dr Tom Leach

Dr Tom Leach MBChB DCH EMCert(ACEM) currently works as a GP Registrar and an Emergency Department CMO in Australia. He is also a Clinical Associate Lecturer at the Australian National University. After graduating from his medical degree at the University of Manchester in 2011, Tom completed his Foundation Training at Bolton Royal Hospital, before moving to Australia in 2013. He started almostadoctor whilst a third year medical student in 2009. Read full bio

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