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

Frid, C; Björkhem, Gudrun LU ; Jonzon, A; Sunnegardh, J; Anneren, G and Lundell, B (2004) In Cardiology in the Young 14(1). p.24-31
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... (More)
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 806 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 65176 over the period from 1973 through 1977, to about 90176 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. (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
Down's, atrioventricular septal defect, congenital heart defects, syndrome, operation, sex, survival
in
Cardiology in the Young
volume
14
issue
1
pages
24 - 31
publisher
Cambridge University Press
external identifiers
  • pmid:15237667
  • wos:000220664000005
  • scopus:5444235836
ISSN
1467-1107
DOI
10.1017/S1047951104001052
language
English
LU publication?
yes
id
1f1f6d85-3fbf-41cf-9d40-05e0c31c3cea (old id 281899)
date added to LUP
2007-10-22 19:00:12
date last changed
2017-02-12 03:32:16
@article{1f1f6d85-3fbf-41cf-9d40-05e0c31c3cea,
  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 806 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 65176 over the period from 1973 through 1977, to about 90176 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.},
  author       = {Frid, C and Björkhem, Gudrun and Jonzon, A and Sunnegardh, J and Anneren, G and Lundell, B},
  issn         = {1467-1107},
  keyword      = {Down's,atrioventricular septal defect,congenital heart defects,syndrome,operation,sex,survival},
  language     = {eng},
  number       = {1},
  pages        = {24--31},
  publisher    = {Cambridge University Press},
  series       = {Cardiology in the Young},
  title        = {Long-term survival in children with atrioventricular septal defect and common atrioventricular valvar orifice in Sweden},
  url          = {http://dx.doi.org/10.1017/S1047951104001052},
  volume       = {14},
  year         = {2004},
}