Hostname: page-component-78c5997874-s2hrs Total loading time: 0 Render date: 2024-11-18T07:19:30.205Z Has data issue: false hasContentIssue false

A population-based study of extra-cardiac anomalies in children with congenital cardiac malformations

Published online by Cambridge University Press:  21 January 2005

Leif Eskedal
Affiliation:
Department of Pediatrics, Soerlandet Regional Hospital, Kristiansand, Norway
Petter Hagemo
Affiliation:
Section Pediatric Cardiology, Rikshospitalet University Hospital, Oslo, Norway
Anne Eskild
Affiliation:
Department of Obstetrics and Gynaecology, Ulleval University Hospital, and Division of Epidemiology, National Institute of Public Health, Kristiansand, Norway
Geir Aamodt
Affiliation:
Department of Biostatistics, Rikshospitalet University Hospital, Oslo, Norway
Karry Stephen Seiler
Affiliation:
Faculty of Health and Sport, Agder University College, Kristiansand, Norway
Erik Thaulow
Affiliation:
Section Pediatric Cardiology, Rikshospitalet University Hospital, Oslo, Norway

Abstract

We describe the prevalence of extra-cardiac anomalies in children with congenital cardiac malformations, and their impact on survival, compared to the outcome in children with the congenital cardiac lesions as the only recognised anomaly.

Our population comprises the 3527 children born with congenital cardiac anomalies between 1990 and 1999, and registered at the largest tertiary centre for Paediatric Cardiology in Norway. Extra-cardiac anomalies were found in one-fifth of the population, with Down's syndrome accounting for nearly one-third. Survival improved for children born between 1995 and 1999 compared with those born in the period from 1990 to 1994 for all groups, except for children with additional extra-cardiac anomalies in the absence of Down's syndrome. The results were the same for children undergoing surgical treatment of their cardiac malformation. The survival in children with Down's syndrome improved in comparable fashion to those without extra-cardiac anomalies. Children with extra-cardiac anomalies in the absence of Down's syndrome represent a heterogeneous group, with varying patterns of survival. Survival did not improve in these latter patients during the period of our study.

Type
Original Article
Copyright
© 2004 Cambridge University Press

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

Reller MD, Morris CD. Is Down's syndrome a risk factor for poor outcome after repair of congenital heart defects? J Pediatr 1998; 132: 738741.Google Scholar
Van Mierop LHS. Diagnostic code for congenital heart disease. Pediatr Cardiol 1984; 5: 331362.Google Scholar
Van Mierop LHS. Diagnostic code for congenital heart disease, supplement. Pediatr Cardiol 1986; 7: 3134.Google Scholar
Pradat P. Noncardiac malformations at major congenital heart defects. Pediatr Cardiol 1997; 18: 1118.Google Scholar
Goldmuntz E. The epidemiology and genetics of congenital heart disease. Clin Perinatol 2001; 28: 19.Google Scholar
Tennstedt C, Chaui R, Korner H, Dietel M. Spectrum of congenital heart defects and extracardiac malformations associated with chromosomal abnormalities: results of a seven year necropsy study. Heart 1999; 82: 3439.Google Scholar
Lurie IW, Kappetein AP, Loffredo CA, Ferencz C. Non-cardiac malformations in individuals with outflow tract defects of the heart: the Baltimore-Washington Infant Study (1981–1989). Am J Med Genet 1995; 59: 7689.Google Scholar
Harris JA, Francannet C, Pradat P, Robert E. The epidemiology of cardiovascular defects, Part 2: A study based on data from three large registries of congenital malformations. Pediatr Cardiol 2003; 24: 222235.Google Scholar
Grech V, Gatt M. Syndromes and malformations associated with congenital heart disease in a population-based study. Int J Cardiol 1999; 28: 151156.Google Scholar
Gaynor JW, Mahle WT, Cohen MI, et al. Risk factors for mortality after the Norwood procedure. Eur J Cardiothorac Surg 2002; 22: 8289.Google Scholar
Abu-Harb M, Hey E, Wren C. Death in infancy from unrecognised congenital heart disease. Arch Dis Child 1994; 71: 37.Google Scholar
Sharland GK, Lockhart SM, Chita SK, Allan LD. Factors influencing the outcome of congenital heart disease detected prenatally. Arch Dis Child 1991; 66: 284287.Google Scholar
Garne E, Stoll C, Clementi M. Evaluation of prenatal diagnosis of congenital heart diseases by ultrasound: experience from 20 European registries. Ultasound Obstet Gynecol 2001; 17: 386391.Google Scholar
Eskild A, Nesheim B, Berglund T, Totlandsdal JK, Andresen JF. Induced abortion attributed to fetal maldevelopment in Norway in 1996 and 1997. Tidsskr Nor Laegeforen 2000; 9: 10001003.Google Scholar
Rose V, Izukawa T, Moes AF. Syndromes of asplenia and polysplenia. A review of cardiac and non-cardiac malformations in 60 cases with special reference to diagnosis and prognosis. Br Heart J 1975; 37: 840852.Google Scholar
Wolfe MW, Vaeck JL, Kinard RE, Bailey CG. Prolonged and functional survival with the asplenia syndrome. Am J Med 1986; 81: 10891091.Google Scholar
Lin AE, Ticho BS, Houde K, Westgate MN, Holmes LB. Heterotaxy: associated conditions and hospital-based prevalence in newborns. Genet Med 2000; 2: 157172.Google Scholar
Gibbs JL, Monro JL, Cunningham D, Rickards A. Survival after surgery or therapeutic catheterisation for congenital heart disease in children in the United Kingdom: analysis of the central cardiac audit database for 2000–1. BMJ 2004; 328: 611615.Google Scholar
Al-Hay AA, MacNeill SJ, Yacoub M, Shore DF, Shinebourne EA. Complete atrioventricular septal defect, Down's syndrome, and surgical outcome: risk factors. Ann Thorac Surg 2003; 75: 412421.Google Scholar
Moller JH, Powel CB, Joransen JA, Borbas C. The pediatric care consortium – revisited. Jt Comm J Qual Improv 1994; 20: 661668.Google Scholar
Jenkins KJ, Gauvreau K. Center-specific differences in mortality: preliminary analyses using the risk adjustment in congenital heart surgery (RACHS-1) method. J Thorac Cardiovasc Surg 2001; 124: 97104.Google Scholar
Halnon NJ, Wetzel GT. Implications of improving survival in the management of life-threatening congenital heart disease: new challenges in pediatric cardiology. Curr Opin Cardiol 2002; 17: 271273.Google Scholar
Robinson WM, Newburger JW. Ethical issues concerning cardiac surgery in patients with syndromic abnormalities. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2003; 6: 147151.Google Scholar