Congenital Heart Disease

Authors: Fatema Jaffer, Riad Hosein / Editor: Lauren Fraser / Reviewer: Sandi Angus / Codes: / Published: 19/02/2024

This session details the three main presentations of Congenital Heart Disease to the emergency department (ED); shock, cyanosis and congestive heart failure. The lesions causing each presentation and general/ specific management are also discussed. 


After completing this session, you will be able to: 

  • Describe basic foetal and neonatal circulation 
  • Identify the most common presentations of congenital heart disease to the emergency department 
  • Describe the importance of duct dependent lesions 
  • Distinguish between cyanotic and acyanotic forms of congenital heart disease 
  • Formulate a management plan for the infant in shock/cyanosis/congestive heart failure
  • Describe the general management for common CHD lesions 
  • Have an appreciation that many CHD patients survive to adulthood and may present to the ED as an adolescent or adult 


  1. Liu Y, Chen S, Zhlke L, et al. Global birth prevalence of congenital heart defects 1970-2017: updated systematic review and meta-analysis of 260 studies. Int J Epidemiol. 2019 Apr 1;48(2):455-463. doi: 10.1093/ije/dyz009.
  2. Somerville J. MANAGEMENT OF ADULTS WITH CONGENITAL HEART DISEASE: An Increasing Problem. Annual Review of Medicine, 1997. 48(1), pp.283-293.
  3. Judge P, Meckler Mshs G. Congenital Heart Disease In Pediatric Patients: Recognizing The Undiagnosed And Managing Complications In The Emergency Department. Pediatr Emerg Med Pract. 2016 May;13(5):1-28; quiz 27-8.
  4. Bernier PL, Stefanescu A, Samoukovic G, Tchervenkov CI. The challenge of congenital heart disease worldwide: epidemiologic and demographic facts. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2010;13(1):26-34.
  5. Erikssen G, Liestl K, Seem E, Birkeland S, Saatvedt KJ, Hoel TN, Dhlen G, Skulstad H, Svennevig JL, Thaulow E, Lindberg HL. Achievements in congenital heart defect surgery: a prospective, 40-year study of 7038 patients. Circulation. 2015 Jan 27;131(4):337-46; discussion 346.
  6. MacDonald MG, Mullett MD, Seshia MMK, editors. Averys neonatology: Pathophysiology and management of the newborn. 6th ed. Philadelphia, PA: Lippincott Williams and Wilkins; 2005.
  7. Kouchoukos NT, Blackstone EH, Hanley FL. Kirklin/Barratt-Boyes Cardiac Surgery [Internet]. Elsevier Health Sciences; 2012.
  8. Beattie R, Champion M. Essential Revision Notes In Paediatrics For The MRCPH. Knutsford, Cheshire: PasTest Ltd., 2002.
  9. Radiopaedia – The Wiki-Based Collaborative Radiology Resource. [online] Available at: <> [Accessed 12 June 2020].
  10. Kliegman, R., Stanton, B., St. Geme, J., Schor, N., Behrman, R. and Nelson, W., 2016. Nelson Textbook Of Pediatrics. 20th ed. Philadelphia: Elsevier.
  11. Brawn, W., Barron, D. and Jones, T., 2011. Hypoplastic left heart. Paediatrics and Child Health, 21(1), pp.19-24.
  12. Barron, D., Kilby, M., Davies, B., Wright, J., Jones, T. and Brawn, W., 2009. Hypoplastic left heart syndrome. The Lancet, 374(9689), pp.551-564.
  13. Hosein RB, Clarke AJ, McGuirk SP, Griselli M, Stumper O, De Giovanni JV, Barron DJ, Brawn WJ. Factors influencing early and late outcome following the Fontan procedure in the current era. The ‘Two Commandments’? Eur J Cardiothorac Surg. 2007 Mar;31(3):344-52; discussion 353. 
  14. Best KE, Rankin J. Long-Term Survival of Individuals Born With Congenital Heart Disease: A Systematic Review and Meta-Analysis. J Am Heart Assoc. 2016;5(6):e002846. Published 2016 Jun 16. doi:10.1161/JAHA.115.002846

Additional resources

There are some descriptions of murmurs listed in this module. This website has an open access course designed specifically for congenital heart disease murmurs complete with audio which may be useful.

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