Hostname: page-component-cd9895bd7-7cvxr Total loading time: 0 Render date: 2024-12-18T22:50:17.940Z Has data issue: false hasContentIssue false
  • English
  • Français

Long-term survival in children with atrioventricular septal defect and common atrioventricular valvar orifice in Sweden

Published online by Cambridge University Press:  20 January 2005

Christina Frid ,
Gudrun Björkhem ,
Anders Jonzon ,
Jan Sunnegårdh ,
Göran Annerén  and
Bo Lundell
Christina Frid
Affiliation:
Unit of Paediatrics, Department of Genetics and Pathology, University Hospital, Uppsala, Sweden
Gudrun Björkhem
Affiliation:
Paediatric Cardiology, Department of Paediatrics, University Hospital, Lund, Sweden
Anders Jonzon
Affiliation:
Unit of Paediatrics, Department of Genetics and Pathology, University Hospital, Uppsala, Sweden
Jan Sunnegårdh
Affiliation:
Paediatric Cardiology, Department of Paediatrics, The Queen Silvia Children's Hospital, Sahlgrenska University Hospital, Göteborg, Sweden
Göran Annerén
Affiliation:
Department of Women's and Children's Health and Clinical Genetics, Department of Genetics and Pathology, University Hospital, Uppsala, Sweden
Bo Lundell
Affiliation:
Paediatric Cardiology, Department of Paediatrics, Karolinska Institute, Astrid Lindgren Children's Hospital, Stockholm, Sweden
Get access Rights & Permissions [Opens in a new window]

Abstract

Background: The survival for patients with atrioventricular septal defect has improved markedly over the last decades and, during the same period, the survival of children with Down's syndrome has also increased. The aim of our study was to investigate long-term survival in patients having atrioventricular septal defect with common valvar orifice, but without associated significant congenital heart defects, in the setting of Down's syndrome, comparing the findings to those in chromosomally normal children with the same malformation. Methods and results: In a population-based retrospective study, we scrutinised the medical records from 801 liveborn children with atrioventricular septal defect born in Sweden during the period 1973 through 1997. Data on gender, presence or absence of Down's syndrome, associated congenital heart defects, date of birth, operation and death were recorded and followed up until 2001. An isolated atrioventricular septal defect with common atrioventricular valvar orifice was present in 502 children, of whom 86% had Down's syndrome. We found a significant reduction over time in age at operation, and in postoperative mortality at 30 days, from 28 to 1%. Using a multiple logistic regression model, we found no significant differences in mortality between genders, nor between those with or without Down's syndrome. Early corrective surgery could not be identified as a significant independent factor for survival. The 5-year postoperative survival in patients with Down's syndrome increased from 65% over the period from 1973 through 1977, to about 90% in the period 1993 through 1997, and the same trend was observed in chromosomally normal patients. Conclusions: Survival in uncomplicated atrioventricular septal defect with common atrioventricular valvar orifice has greatly increased, and surgical correction is now equally successful in patients with Down's syndrome and chromosomally normal patients, and for both genders. Death in connection with surgery is no longer the major threat, and focus must now be on long-term follow-up.

Keywords

Atrioventricular septal defectcongenital heart defectsDown's syndromeoperationsexsurvival
Type
Original Article
Information
Cardiology in the Young , Volume 14 , Issue 1 , February 2004 , pp. 24 - 31
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

Keith JD. Prevalence, incidence and epidemiology. In: Keith JD, Rowe RD, Vlad P (eds). Heart disease in infancy and childhood. New York, Macmillan 1978, 3rd edn, pp 3–13.
Perry LW, Ferencz C, Rubin JD, Loffredo CA. Infants with congenital heart disease: the cases. In: Ferencz C, Rubin JD, Loffredo CA, Magee CA (eds). Epidemiology of congenital heart disease. The Baltimore Infant Study 1981–1989. Mount Kisco, New York, Futura Publishing Company, Inc. 1993, pp 33–62.
Marino B, Vario U, Corno A, et al. Atrioventricular canal in Down's syndrome. Prevalence of associated cardiac malformations compared with patients without Down's syndrome. Am J Dis Child 1990; 144: 1120–1122.Google Scholar
Carmi R, Boughman JA, Ferencz C. Endocardial cushion defect: further studies of “isolated” versus “syndromic” occurrence. Am J Med Genet 1992; 43: 569–575.Google Scholar
Lillehei CW, Cohen M, Warden HE, Varco RL. The direct vision intracardiac correction of congenital anomalies by controlled cross circulation. Surgery 1955; 38: 11–29.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): 234–241.Google Scholar
Frid C, Annerén G, Rasmussen F, Sundelin C, Drott P. Utilization of Medical Care among Children with Down's syndrome. J Intellect Disabil Res 2002; 46: 310–317.Google Scholar
Bjork VO, Thorén C. New ways of treating children with congenital heart disease. Cor Vasa 1981; 23: 49–55.Google Scholar
Najm HK, Coles JG, Endo M, et al. Complete atrioventricular septal defects: results of repair, risk factors, and freedom from reoperation. Circulation 1997; 96: II-311–II-315.Google Scholar
Crawford FA Jr, Stroud MR. Surgical repair of complete atrioventricular septal defect. Ann Thorac Surg 2001; 72: 1621–1628; discussion 162.Google Scholar
Freedom RM, Lock J, Bricker J. Pediatric cardiology and cardiovascular surgery: 1950–2000. Circulation 2000; 102: IV58–IV68.Google Scholar
Trusler GA. Discussion of Mills NL, Ochsner JL, King TD. Correction of type C complete atrioventricular canal: surgical considerations. J Thorac Cardiovasc Surg 1971; 71: 20–28.Google Scholar
Kaplan EL, Meier T. Nonparametric estimates from incomplete observations. J Am Stat Assoc 1958; 53: 457–481.Google Scholar
Van Praagh S, Antoniadis S, Otero-Coto E, Leidenfrost RD, Van Praagh R. Common atrioventricular canal with and without conotruncal malformations: an anatomic study of 251 postmortem cases. In: Nora JJ, Takao A (eds). Congenital Heart Disease: causes and processes. Mount Kisco, New York, Futura Publishing Co 1984, pp 599–639.
Baird PA, Sadovnick AD. Life expectancy in Down syndrome. J Pediatr 1987; 110: 849–854.Google Scholar
Pinto FF, Nunes L, Ferras F, Sampayo F. Down's syndrome: different distribution of congenital heart diseases between the sexes. Int J Cardiol 1990; 27: 175–178.Google Scholar
Ferencz C, Rubin JD, McCarter RJ, et al. Congenital heart disease: prevalence at livebirth. The Baltimore-Washington Infant Study. Am J Epidemiol 1985; 121: 31–36.Google Scholar
Bull C, Rigby ML, Shinebourne EA. Should management of complete atrioventricular canal defect be influenced by coexistent Down's syndrome? Lancet 1985; 1: 1147–1149.Google Scholar
Yamaki S, Yasui H, Kado H, et al. Pulmonary vascular disease and operative indications in complete atrioventricular canal defect in early infancy. J Thorac Cardiovasc Surg 1993; 106: 398–405.Google Scholar
Clapp S, Perry BL, Farooki ZQ, et al. Down's syndrome, complete atrioventricular canal, and pulmonary vascular obstructive disease. J Thorac Cardiovasc Surg 1990; 100: 115–121.Google Scholar
Masuda M, Kado H, Kajihara N, et al. Early and late results of total correction of congenital cardiac anomalies in infancy. Jpn J Thorac Cardiovasc Surg 2001; 49: 497–503.Google Scholar
Pueschel SM, Annerén G, Durlach R, Flores J, Sustroá M, Verma IC. Guidelines for optimal medical care of persons with Down's syndrome. International League of Societies for Persons with Mental Handicap (ILSMH). Acta Paediatr 1995; 84 (7): 823–827.Google Scholar