Hostname: page-component-cd9895bd7-dk4vv Total loading time: 0 Render date: 2024-12-23T13:53:18.098Z Has data issue: false hasContentIssue false

Congenital cardiovascular lesions in children with trisomy 21 at the Bustamante Hospital for Children

Published online by Cambridge University Press:  22 March 2010

Tamra W. Tomlinson
Affiliation:
Bustamante Hospital for Children, Arthur Wint Drive, Kingston 5, Jamaica
Charmaine H. Scott
Affiliation:
Bustamante Hospital for Children, Arthur Wint Drive, Kingston 5, Jamaica
Helen L. M. Trotman*
Affiliation:
Department of Obstetrics, Gynaecology and Child Health, University of the West Indies, Mona, Jamaica
*
Correspondence to: Dr Helen Trotman Department of Obstetrics, Gynaecology and Child Health, University of the West Indies, Mona, St Andrew, Jamaica. Tel: 876-970-0329; Fax: 876-927-1446; E-mail: [email protected].

Abstract

Objective

To describe the cardiac lesions seen in children with trisomy 21, the outcome of these children and rates of access to corrective surgery at the Bustamante Hospital for Children.

Methods

A 10-year retrospective review of the records of trisomy 21 patients with cardiac lesions referred to the Bustamante Hospital for Children was conducted.

Results

A total of 76 patients were enrolled in the study, 30 (40%) males and 46 (60%) females; among these 110 cardiac lesions were detected. A total of 20 (26%) patients died, 48 (63%) survived, and for 8 (11%) the status was unknown. The most common lesion was the atrioventricular septal defect, which accounted for 41 (37%) of all the lesions and occurred as a single defect in 24 (53%) patients. At presentation, 33 (46%) patients had one or more medical complication; 30 (91%) had cardiac failure, 10 (30%) had pneumonia and 5 (15%) had evidence of systemic pulmonary arterial pressures. Cardiac catheterisation was recommended for 43 (56%) patients but only 10 (23%) had the procedure done. Surgery was recommended for 60 (79%) patients; of these 6 (10%) patients had the procedure done. The median time of survival was 88 months (7.3 years). The age of presentation was not found to significantly affect outcome.

Conclusion

Trisomy 21 patients with cardiac lesions have high morbidity and mortality. This morbidity and mortality could be reduced if surgical intervention was offered routinely.

Type
Original Articles
Copyright
Copyright © Cambridge University Press 2010

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1. Leshin, L. The Story of Down Syndrome, 2003. Available at URL: http://www.ds-health.com (accessed February, 2005).Google Scholar
2. Benke, PJ, Carver, V, Donahue, R. Risk and Recurrence Risk of Down Syndrome. University of Miami school of Medicine, 1995. Available at URL: http://www.ds-health.com/benke.htm (accessed February, 2005).Google Scholar
3. Jones, KL (ed.). Smith’s Recognizable Patterns of Human Malformation, 5th edn, Saunders Philadelphia, Pennsylvania, 1997, pp 810.Google Scholar
4. Huret, JL, Sinet, PM. Trisomy 21. Atlas Genet Cytogenet Oncol Haematol August, 2000. Available at URL: http://AtlasGeneticsOncology.org/Educ/PolyTri21Eng.html (accessed February, 2005).Google Scholar
5. Park, MK. Pediatric Cardiology for Practitioners, 4th edn, Mosby, St. Louis, Misouri, 2002, pp 10.Google Scholar
6.The Heart Center Encyclopedia, Cincinatti Children’s posting. Heart Related Syndromes: Down Syndrome (Trisomy 21), 1999. Available at URL: http://www.cincinnatichildren's.org/health/heart-encyclopedia (accessed February, 2005).Google Scholar
7. Hayes, C, Johnson, Z, Thornton, L, et al. Ten year survival of Down syndrome births. Int J Epidemiol 1997; 26: 822829.CrossRefGoogle ScholarPubMed
8. Park, SC, Matthews, RA, Zuberbuhler, JR, Rowe, RD, Neches, WH, Lennox, CC. Down syndrome with congenital heart malformation. Am J Dis Child 1977; 131: 2933.Google Scholar
9. Lo, NS, Leung, PM, Lau, KC, Yeung, CY. Congenital cardiovascular malformations in Chinese children with Down’s syndrome. Chin Med J (Engl) 1989; 102: 382386.Google ScholarPubMed
10. Grech, V, Gatt, M. Syndromes and malformations associated with congenital heart disease in a population-based study. Int J Cardiol 1999; 68: 151156.Google Scholar
11. Vida, VL, Barnoya, J, Larrazabal, LA, de Maria Garcia, F, Castaneda, AR. Congenital cardiac disease in children with Down’s syndrome in Guatemala. Cardiol Young 2005; 15: 286290.Google Scholar
12. Matthew, P, Moodie, D, Sterba, R, Murphy, D, Rosenkranz, E, Homa, A. Long term follow up of children with Down syndrome with cardiac lesions. Clin Pediatr (Phila) 1991; 30: 128.Google Scholar
13.Ministry of Health, Jamaica. Bustamante Hospital for Children Monthly Hospital Statistical Report 2004 (unpublished).Google Scholar
14. Parvathy, U, Balakrishnan, KR, Ranjith, MS, Saldanha, R, Sai, S, Vakamudi, M. Surgical experience with congenital heart disease in Down’s syndrome. Indian Heart J 2000; 52: 438441.Google Scholar
15. Jaiyesimi, O, Baichoo, V. Cardiovascular malformations in Omani Arab children with Down’s syndrome. Cardiol Young 2007; 17: 166171.Google Scholar