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>Journals >Cirugía y Cirujanos >Year 2010, Issue 3


Martínez-Quintana E, Rodríguez-González F, Medina-Gil JM, Ágredo-Muñoz J, Nieto-Lago V
Evolución clínica en pacientes con síndrome de Down y cardiopatía congénita
Cir Cir 2010; 78 (3)

Language: Español
References: 24
Page: 245-250
PDF: 264.97 Kb.

[Full text - PDF]

ABSTRACT

Background: Long-term complications of Down syndrome patients with congenital heart disease are poorly known.
Methods: We carried out a retrospective study of Down syndrome patients with congenital heart disease and patients with atrioventricular septal defect with and without Down syndrome.
Results: Between 2004 and 2008, 317 patients with congenital heart disease were followed-up in the Adult Congenital Heart Disease Unit. Of these patients, 19 (6%) with a mean age of 26.8 ± 8.1 years had Down syndrome. Atrioventricular septal defect was the most frequent congenital heart disease (63%) followed by ventricular septal defect (26%). Ten patients (53%) were operated on during childhood. Three of these patients required reoperation during adulthood (two patients due to left ventricle outflow tract obstruction and one patient due to left atrioventricular valve insufficiency). Four patients (21%) had Eisenmenger syndrome with improvement of functional class in those treated with bo sentan, two patients (10.5%) had bacterial endocarditis and two patients (10.5%) died. No significant differences were seen in left atrioventricular valve insufficiency between atrioventricular septal defect in patients with and without Down syndrome (1.5 ± 0.9 vs. 1.7 ± 0.8, p = 0.689).
Conclusions: Left atrioventricular valve insufficiency and left ventricle outflow tract obstruction were the most frequent long-term complications requiring surgical reinterventionin patients with atrioventricular septal defect.


Key words: Congenital heart disease, Down syndrome, pulmonary hypertension.


REFERENCES

  1. Rodríguez LH, Reyes JN. Cardiopatías congénitas en el síndrome de Down. Bol Med Hosp Infant Mex 1984;41:622-625.

  2. de Rubens-Figueroa J, del Pozzo-Magaña B, Pablos-Hach JL, Calderón- Jiménez C, Castrejón-Urbina R. Heart malformations in children with Down syndrome. Rev Esp Cardiol 2003;56:894-899.

  3. Hayes C, Johnson Z, Thornton L, Fogarty J, Lyons R, O’Connor M, et al. Ten-year survival of Down syndrome births. Int J Epidemiol 1997;26:822-829.

  4. El-Najdawi EK, Driscoll DJ, Puga FJ, Dearani JA, Spotts BE, Mahoney DW, et al. Operation for partial atrioventricular septal defect: a forty-year review. J Thorac Cardiovasc Surg 2000;119:880-889.

  5. di Carlo DC, Marino B. Atrioventricular canal with Down syndrome or normal chromosomes: distinct prognosis with surgical management? J Thorac Cardiovasc Surg 1994;107:1368-1370.

  6. Lange R, Guenther T, Busch R, Hess J, Schreiber C. The presence of Down syndrome is not a risk factor in complete atrioventricular septal defect repair. J Thorac Cardiovasc Surg 2007;134:304-310.

  7. Dunlop KA, Mulholland HC, Casey FA, Craig B, Gladstone DJ. A ten year review of atrioventricular septal defects. Cardiol Young 2004;14:15-23.

  8. Redmond JM, Silove ED, De Giovanni JV, Wright JG, Sreeram N, Brawn WJ, et al. Complete atrioventricular septal defects: the influence of associated cardiac anomalies on surgical management and outcome. Eur J Cardiothorac Surg 1996;10:991-995.

  9. Marino B, Vairo U, Corno A, Nava S, Guccione P, Calabrò R, et al. Atrioventricular canal in Down syndrome. Prevalence of associated cardiac malformations compared with patients without Down syndrome.Am J Dis Child 1990;144:1120-1122.

  10. Frid C, Drott P, Lundell B, Rasmussen F, Annerén G. Mortality in Down’s syndrome in relation to congenital malformations. J Intellect Disabil Res 1999;43:234-241.

  11. Leonard S, Bower C, Petterson B, Leonard H. Survival of infants born with Down’s syndrome: 1980-96. Paediatr Perinat Epidemiol2000;14:163-171.

  12. Glasson EJ, Sullivan SG, Hussain R, Petterson BA, Montgomery PD, Bittles AH. The changing survival profile of people with Down’s syndrome: implications for genetic counselling. Clin Genet 2002;62:390-393.

  13. Quiñones MA, Otto CM, Stoddard M, Waggoner A, Zoghbi WA. Doppler Quantification Task Force of the Nomenclature and Standards Committee of the American Society of Echocardiography. Recommendations 0for quantification of Doppler echocardiography: a report from the Doppler Quantification Task Force of the Nomenclature and Standards Committee of the American Society of Echocardiography. J Am Soc Echocardiogr 2002;15:167-184

  14. Dragulescu A, Ghez O, Fraisse A, Gaudart J, Amedro P, Kreitmann B, et al. Long-term results of complete atrio-ventricular canal correction. Arch Mal Coeur Vaiss 2007;100:416-421.

  15. Formigari R, Di Donato RM, Gargiulo G, Di Carlo D, Feltri C, Picchio FM, et al. Better surgical prognosis for patients with complete atrioventricular septal defect and Down’s syndrome. Ann Thorac Surg 2004;78:666-672.

  16. Van Arsdell GS, Williams WG, Boutin C, Trusler GA, Coles JG, Rebeyka IM, et al. Subaortic stenosis in the spectrum of atrioventricular septal defects. Solutions may be complex and palliative. J Thorac Cardiovasc Surg 1995;110:1534-1541.

  17. Daliento L, Somerville J, Presbitero P, Menti L, Brach-Prever S, Rizzoli G, et al. Eisenmenger syndrome: factors relating to deterioration and death. Eur Heart J 1998;19:1845-1855.

  18. Diller GP, Gatzoulis MA. Pulmonary vascular disease in adults with congenital heart disease. Circulation 2007;115:1039-1050.

  19. Kabbani MS, Giridhar S, Elbarbary M, Elgamal MA, Najm H, Godman M. Postoperative cardiac intensive care outcome for Down syndrome children. Saudi Med J 2005;26:943-946.

  20. Stos B, Dembour G, Ovaert C, Barrea C, Arape A, Stijns M, et al. Risks and benefits of cardiac surgery in Down’s syndrome with congenital heart disease. Arch Pediatr 2004;11:1197-1201.

  21. Duffels MG, Vis JC, van Loon RL, Nieuwkerk PT, van Dijk AP, Hoendermis ES, et al. Effect of bosentan on exercise capacity and quality of life in adults with pulmonary arterial hypertension associated with congenital heart disease with and without Down’s syndrome. Am J Cardiol 2009;103:1309-1315.

  22. Duffels MG, Vis JC, van Loon RL, Berger RM, Hoendermis ES, van Dijk AP, et al. Down patients with Eisenmenger syndrome: is bosentan treatment an option? Int J Cardiol 2009;134:378-383.

  23. Goldhaber SZ, Brown WD, Robertson N, Rubin IL, Sutton MG. Aortic regurgitation and mitral valve prolapse with Down’s syndrome: a casecontrol study. J Ment Defic Res 1988;32:333-336.

  24. Hamada T, Gejyo F, Koshino Y, Murata T, Omori M, Nishio M, et al. Echocardiographic evaluation of cardiac valvular abnormalities in adults with Down’s syndrome. Tohoku J Exp Med 1998;185:31-35.






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