Skip to main content

Abstract

Congenital heart disease is very common in persons with Down syndrome. Today, with the survival of persons with Down syndrome reaching the age of 50–60 years it is important to ensure ongoing evaluation and follow-up. Some research studies have found a large number of adults with Down Syndrome in residential centers to have mild to moderate regurgitation of heart valves. Identifying valvular insufficiency and mitral valve prolapse is highly likely even in patients with no known congenital heart problems. Monitoring for pulmonary hypertension is important especially in those with known snoring and sleep apnea. Appropriate diet and exercise is essential as it is for us all.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 389.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 499.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Campbell RM, Adatia I, Gow RM, Webb GD, Williams WG, Freedom RM. Total cavopulmonary anastomosis (Fontan) in children with Down’s syndrome. Ann Thorac Surg. 1998;66(20):523–6.

    Article  CAS  PubMed  Google Scholar 

  2. Perfume F, Presti F, Thilaganathan B, Carvalho JS. Association between increased nuchal translucency and second trimester cardiac echogenic foci. Obstet Gynecol. 2003;101(5 pt 1):899–904.

    Google Scholar 

  3. Murphy Jr DJ. Ayrioventricular canal defects. Curr Treat Options Cardiovasc Med. 1999;1(4):323–34.

    Article  Google Scholar 

  4. Cope JT, Fraser GD, Kouretas PC, Kron IL. Complete versus partial atrioventricular canal: equal risks of repair in the modern era. Ann Surg. 2002;236(4):514–21.

    Article  PubMed  PubMed Central  Google Scholar 

  5. van Son JA, Phoon CK, Silverman NH, Haas GS. Predicting feasibility of biventricular repair of right dominant unbalanced atrioventricular canal. Ann Thorac Surg. 1998;66(1):310.

    Google Scholar 

  6. Drinkwater Jr DC, Laks H. Unbalanced atrioventricular septal defects. Semin Thorac Cardiovasc Surg. 1997;9(1):21–5.

    PubMed  Google Scholar 

  7. Majdalany DS, Burkhart HM, Connolly HM, Abel MD, Dearani JA, Warnes CA, et al. Adults with Down syndrome: safety and long-term outcome of cardiac operation. Congenit Heart Dis. 2010;5(1):38–43.

    Article  PubMed  Google Scholar 

  8. Lamberti JJ, Jensen TS, Grehl TM, Oury JH, Waldman JD, Kirkpatrick SE, et al. Late reoperation for systemic atrioventricular valve regurgitation after repair of congenital heart defects. Ann Thorac Surg. 1989;47(4):517–22.

    Article  CAS  PubMed  Google Scholar 

  9. Prevention of infective endocarditis guidelines. Circulation 2007;116:1736–54.

    Google Scholar 

  10. Marcus CL, Greene MG, Carroll JL. Blood pressure in children with obstructive sleep apnea. Am J Respir Crit Care Med. 1998;157:1098–103.

    Article  CAS  PubMed  Google Scholar 

  11. Tal A, Leiberman A, Margulis G, Sofer S. Ventricular dysfunction in children with obstructive sleep apnea: radionuclide assessment. Pediatr Pulmonol. 1988;4:139–43.

    Article  CAS  PubMed  Google Scholar 

  12. Fitzgerald DA, Paul A, Richmond C. Severity of obstructive apnoea in children with Down syndrome who snore. Arch Dis Child. 2007;92:423–5.

    Article  PubMed  PubMed Central  Google Scholar 

  13. Chopo GR, Lazaro MA, Ucles P. Obstructive sleep apnea syndrome in childhood. Rev Neurol. 2001;32(1):86–91.

    CAS  PubMed  Google Scholar 

  14. Brouillette RT, Fernbach SK, Hunt CE. Obstructive sleep apnea in infants and children. J Pediatr. 1982;100:31–40.

    Article  CAS  PubMed  Google Scholar 

  15. Gozal D. Sleep disordered breathing and school performance in children. Pediatrics. 1998;102:616–20.

    Article  CAS  PubMed  Google Scholar 

  16. Clinical practice guidelines. Diagnosis and management of childhood obstructive sleep apnea syndrome. Pediatrics 2002;109(4):704–12.

    Google Scholar 

  17. Crepaz R, Romeo C, Montanaro D, De Santis S. Long-term results of treatment with Bosentan in adult Eisenmenger’s syndrome patients with Down’s syndrome related to congenital heart disease. BMC Cardiovasc Disord. 2013;13:74.

    Article  PubMed  PubMed Central  Google Scholar 

  18. Pueschel SM, Werner JC. Mitral Valve prolapse in persons with Down syndrome. Res Dev Disabl. 1994;15(2):91–7.

    Article  CAS  Google Scholar 

  19. Geggel RL, O’Brien JE, Feingold M. Development of valve dysfunction in adolescents and young adults with Down syndrome and no known congenital heart disease. J Pediatr. 1993;122(5pt1):821–3.

    Article  CAS  PubMed  Google Scholar 

  20. Pueschel SM, Craig WY, Haddow JE. Lipids and lipoproteins in persons with Downs syndrome. J Intellect Disabil Res. 1992;36(Pt4):365–9.

    PubMed  Google Scholar 

  21. Vis JC, De Bruin-Bon HACM, Bouma BJ, Huisman SA, Imschoot L, van den Brink K, et al. Adults with Down syndrome have reduced cardiac response after light exercise testing. Neth Heart J. 2012;20:264–9.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  22. Galsson EJ, Sulllivan S, Hussain R, Petterson B, Montgomery P, Bittles A. The survival profile of people with Down’s syndrome: implications for genetic counselling. Clin Genet. 2002;62:390–3.

    Article  Google Scholar 

  23. Vis JC, de Bruin-Bon RH, Bouma BJ, Huisman SA, Imschoot L, van den Brink K, et al. Congenital heart defects are under-recognized in adult patients with Down’s syndrome. Heart. 2010;96(18):148–84.

    Article  Google Scholar 

  24. Weijerman ME, de Winter JP. Clinical practice. The care of children with Down syndrome. Eur J Pediatr. 2010;169(12):1445–52.

    Article  PubMed  PubMed Central  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Kenneth J. Dooley M.D. .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2016 Springer International Publishing Switzerland

About this chapter

Cite this chapter

Dooley, K.J. (2016). Congenital Heart Disease and Down Syndrome. In: Rubin, I.L., Merrick, J., Greydanus, D.E., Patel, D.R. (eds) Health Care for People with Intellectual and Developmental Disabilities across the Lifespan. Springer, Cham. https://doi.org/10.1007/978-3-319-18096-0_105

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-18096-0_105

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-18095-3

  • Online ISBN: 978-3-319-18096-0

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics