Cardiovascular Complications of Down Syndrome: Scoping Review and Expert Consensus

Author:

Dimopoulos Konstantinos12ORCID,Constantine Andrew12ORCID,Clift Paul3,Condliffe Robin4,Moledina Shahin56,Jansen Katrijn78,Inuzuka Ryo9,Veldtman Gruschen R.10,Cua Clifford L.11ORCID,Tay Edgar Lik Wui12,Opotowsky Alexander R.13ORCID,Giannakoulas George14ORCID,Alonso-Gonzalez Rafael1516ORCID,Cordina Rachael17ORCID,Capone George1819,Namuyonga Judith2021,Scott Charmaine H.22,D’Alto Michele23ORCID,Gamero Francisco J.24,Chicoine Brian25ORCID,Gu Hong26,Limsuwan Alisa27,Majekodunmi Tosin28,Budts Werner29ORCID,Coghlan Gerry30,Broberg Craig S.31ORCID,Dimopoulos KonstantinosORCID,Constantine AndrewORCID,Clift Paul,Condliffe Robin,Moledina Shahin,Jansen Katrijn

Affiliation:

1. Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, Royal Brompton and Harefield Hospitals, Guy’s and St Thomas’ NHS Foundation Trust, London, United Kingdom (K.D., A.C.).

2. National Heart and Lung Institute, Imperial College London, United Kingdom (K.D., A.C.).

3. Department of Cardiology, Queen Elizabeth Hospital Birmingham, United Kingdom (P.C.).

4. Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield, United Kingdom (R.C.).

5. National Paediatric Pulmonary Hypertension Service UK, Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom (S.M.).

6. Institute of Cardiovascular Science, University College London, United Kingdom (S.M.).

7. Adult Congenital and Paediatric Heart Unit, Freeman Hospital Newcastle Upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, United Kingdom (K.J.).

8. Population Health Sciences Institute, Newcastle University, Newcastle upon Tyne, United Kingdom (K.J.).

9. Department of Pediatrics, The University of Tokyo Hospital, Japan (R.I.).

10. Scottish Adult Congenital Cardiac Service, Golden Jubilee Hospital, Glasgow, Scotland, United Kingdom (G.R.V.).

11. The Heart Center, Nationwide Children’s Hospital, Columbus, OH (C.L.C.).

12. Department of Cardiology, National University Hospital Singapore (E.T.L.W.).

13. The Heart Institute, Department of Pediatrics, Cincinnati Children’s Hospital, University of Cincinnati College of Medicine, OH (A.R.O.).

14. Department of Cardiology, AHEPA University Hospital School of Medicine, Aristotle University of Thessaloniki, Greece (G.G.).

15. Division of Cardiology, Toronto General Hospital, University Health Network, Peter Munk Cardiovascular Center, University of Toronto, Canada (R.A.-G.).

16. Toronto Adult Congenital Heart Disease Program, Canada (R.A.-G.).

17. Department of Cardiology, Royal Prince Alfred Hospital and Sydney Medical School, University of Sydney, New South Wales, Australia (R.C.).

18. Down Syndrome Clinical and Research Center, Kennedy Krieger Institute, Baltimore, MD (G. Capone).

19. Johns Hopkins School of Medicine, Baltimore, MD (G. Capone).

20. Department of Paediatric Cardiology, Uganda Heart Institute, Kampala (J.N.).

21. Department of Paediatrics and Child Health, Makerere University College of Health Sciences, Kampala, Uganda (J.N.).

22. University Hospital of the West Indies, Kingston, Jamaica (C.H.S.).

23. Department of Cardiology, University “L. Vanvitelli”–Monaldi Hospital, Naples, Italy (M.D.).

24. Department of Cardiovascular Surgery, Benjamin Bloom Children’s Hospital, El Salvador (F.J.G.).

25. Advocate Medical Group Adult Down Syndrome Center, Park Ridge, IL (B.C.).

26. Department of Pediatric Cardiology, Beijing Anzhen Hospital, Capital Medical University, China (H.G.).

27. Division of Pediatric Cardiology, Department of Pediatrics, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand (A.L.).

28. Department of Cardiology, Euracare Multi-specialist Hospital, Nigeria (T.M.).

29. Division of Congenital and Structural Cardiology, University Hospitals Leuven, and Department of Cardiovascular Science, Catholic University Leuven, Belgium (W.B.).

30. Department of Cardiology, Royal Free Hospital, London, United Kingdom (G. Coghlan).

31. Knight Cardiovascular Institute, Oregon Health and Science University, Portland (C.S.B.).

Abstract

Cardiovascular disease is a leading cause of morbidity and mortality in individuals with Down syndrome. Congenital heart disease is the most common cardiovascular condition in this group, present in up to 50% of people with Down syndrome and contributing to poor outcomes. Additional factors contributing to cardiovascular outcomes include pulmonary hypertension; coexistent pulmonary, endocrine, and metabolic diseases; and risk factors for atherosclerotic disease. Moreover, disparities in the cardiovascular care of people with Down syndrome compared with the general population, which vary across different geographies and health care systems, further contribute to cardiovascular mortality; this issue is often overlooked by the wider medical community. This review focuses on the diagnosis, prevalence, and management of cardiovascular disease encountered in people with Down syndrome and summarizes available evidence in 10 key areas relating to Down syndrome and cardiac disease, from prenatal diagnosis to disparities in care in areas of differing resource availability. All specialists and nonspecialist clinicians providing care for people with Down syndrome should be aware of best clinical practice in all aspects of care of this distinct population.

Publisher

Ovid Technologies (Wolters Kluwer Health)

Subject

Physiology (medical),Cardiology and Cardiovascular Medicine

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