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Congenital cardiac disease in children with Down's syndrome in Guatemala

Published online by Cambridge University Press:  03 May 2005

Vladimiro L. Vida
Affiliation:
Unidad de Cirugía Cardiovascular de Guatemala (Pediatria), Gautemala
Joaquín Barnoya
Affiliation:
Unidad de Cirugía Cardiovascular de Guatemala (Pediatria), Gautemala
Luis A. Larrazabal
Affiliation:
Unidad de Cirugía Cardiovascular de Guatemala (Pediatria), Gautemala
Guillermo Gaitan
Affiliation:
Unidad de Cirugía Cardiovascular de Guatemala (Pediatria), Gautemala
Flor de Maria Garcia
Affiliation:
Unidad de Cirugía Cardiovascular de Guatemala (Pediatria), Gautemala
Aldo R. Castañeda
Affiliation:
Unidad de Cirugía Cardiovascular de Guatemala (Pediatria), Gautemala

Abstract

Background: Congenital cardiac disease is the greatest cause of death in patients with Down's syndrome during the first two years of life, with from two-fifths to two-thirds of those with Down's syndrome also having congenital cardiac malformations. The lesions within the heart can be single or multiple. Our objective was to evaluate the frequency and type of such congenital cardiac malformations in patients born with Down's in Guatemala, and to provide baseline information for further research. Methods: We reviewed all patients with Down's syndrome who underwent a cardiologic screening examination between January, 1997, and December, 2003, in the only department dealing with Paediatric Cardiology in Guatemala. Results: Of the 349 patients reviewed, 189 (54.1 per cent) also had an associated congenital cardiac malformation. The median age at diagnosis was 6 months, with a range from 2 to 13 months. In 152 patients (80.4 per cent), the cardiac lesion was isolated, while 37 patients (19.6 per cent) had multiple defects. The most common single defect was patency of the arterial duct, found in 54 of the 189 patients (28.6 per cent), followed by ventricular septal defect in 27.5 per cent, atrial septal defect in 12.7 per cent, and atrioventricular septal defect with common atrioventricular junction in 9.5 per cent. The most frequent concomitant malformation found co-existing with other congenital cardiac lesions was patency of the arterial duct, found in 17.5 per cent. Conclusions: As far as we are aware, ours is the first epidemiologic study concerning the frequency and type of congenital cardiac disease found in Guatemalan children with Down's syndrome. The high frequency of patency of the arterial duct, and the differential distribution of the cardiac malformations associated with Down's syndrome among Guatemalan children, differ from what has been reported in the United States of America, Europe, and Asia. This difference warrants further research.

Type
Original Article
Copyright
© 2005 Cambridge University Press

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References

Moss A. Heart disease in infants, children and adolescents. The Year Book Publisher, Vol. 49, Chicago, 1970.
Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol 2002; 39: 18901900.Google Scholar
Hoffman JI. Incidence, mortality and natural history. Paediatric Cardiology, 2nd edn, Vol. 1. Churchill Livingstone, Philadelphia, 2002, Chapter 6, pp 111131.
Greenwood RD, Nadas AS. The clinical course of cardiac disease in Down's syndrome. Pediatrics 1976; 58: 893897.Google Scholar
Grech V. Epidemiology, and diagnostic and surgical trends in atrioventricular septal defect in Malta. Eur J Epidemiol 1999; 15: 403405.Google Scholar
Figueroa JdR, Magana BdP, Hach J, Jimenez C, Urbina R. Heart malformations in children with Down's syndrome. Rev Esp Cardiol 2003; 56: 894895.Google Scholar
Jacobs EG, Leung MP, Karlberg J. Distribution of symptomatic congenital heart disease in Hong Kong. Pediatr Cardiol 2000; 21: 148157.Google Scholar
Busacca P, Pozzolini A, Minutiello L. Association between parachute mitral valve and Down's syndrome. Report of a case. G Ital Cardiol 1998; 28: 11441148.Google Scholar
Hoe TS, Chan KC, Boo NY. Cardiovascular malformations in Malaysian neonates with Down's syndrome. Singapore Med J 1990; 31: 474476.Google Scholar
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 Scholar
Down's J. Observation on an ethnic classification of idiots. Clinical Lecture Reports. London Hosp 1866; 3: 259.Google Scholar
Hamerton JL, Briggs SM, Giannelli F, Carter CO. Chromosome studies in detection of parents with high risk of second child with Down's syndrome. Lancet 1961; 2: 788791.Google Scholar
Castilla EE, Rittler M, Dutra MG, et al. Survival of children with Down's syndrome in South America. ECLAMC-Down'ssurv Group. Latin American Collaborative Study of Congenital Malformations. Am J Med Genet 1998; 79: 108111.Google Scholar
Frid C, Drott P, Lundell B, Rasmussen F, Anneren G. Mortality in Down's syndrome in relation to congenital malformations. J Intellect Disabil Res 1999; 43 (Pt 3): 234241.Google Scholar
Stoll C, Alembik Y, Dott B, Roth MP. Study of Down's syndrome in 238,942 consecutive births. Ann Genet 1998; 41: 4451.Google Scholar
Laursen HB. Congenital heart disease in Down's syndrome. Br Heart J 1976; 38: 3238.Google Scholar
Tubman TR, Shields MD, Craig BG, Mulholland HC, Nevin NC. Congenital heart disease in Down's syndrome: two year prospective early screening study. BMJ 1991; 302: 14251427.Google Scholar
Chin AJ, Keane JF, Norwood WI, Castaneda AR. Repair of complete common atrioventricular canal in infancy. J Thorac Cardiovasc Surg 1982; 84: 437445.Google Scholar
Hanley FL, Fenton KN, Jonas RA, et al. Surgical repair of complete atrioventricular canal defects in infancy. Twenty-year trends. J Thorac Cardiovasc Surg 1993; 106: 387394; discussion 394397.Google Scholar
Malec E, Mroczek T, Pajak J, Januszewska K, Zdebska E. Results of surgical treatment of congenital heart defects in children with Down's syndrome. Pediatr Cardiol 1999; 20: 351354.Google Scholar
Hayes C, Johnson Z, Thornton L, et al. Ten-year survival of Down's syndrome births. Int J Epidemiol 1997; 26: 822829.Google Scholar
Khoury MJ, Erickson JD. Improved ascertainment of cardiovascular malformations in infants with Down's syndrome, Atlanta, 1968 through 1989. Implications for the interpretation of increasing rates of cardiovascular malformations in surveillance systems. Am J Epidemiol 1992; 136: 14571464.Google Scholar
Mathew P, Moodie D, Sterba R, Murphy D, Rosenkranz E, Homa A. Long-term follow-up of children with Down's syndrome with cardiac lesions. Clin Pediatr (Phila) 1990; 29: 569574.Google Scholar
Velasquez T, Martinez C, Pezzia W, Gallardo N. Ventilatory effects of oxygen in high altitude natives. Respir Physiol 1968; 5: 211220.Google Scholar
Diaz GF, Velez J. Ductus arteriosus. In: Diaz GF, Sandoval N, Velez JF, Carrillo G (eds). Cardiologia Pediatrica. McGraw-Hill, Bogota, 2003, pp 296313.