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Long-term survival in children with atrioventricular septal defect and common atrioventricular valvar orifice in Sweden
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health. (Övrig pediatrisk forskning/Nordvall)
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health. (Perinatal, neonatal och barnkardiologisk forskning/Sedin)
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2004 (English)In: Cardiology in the Young, ISSN 1047-9511, E-ISSN 1467-1107, Vol. 14, no 1, 24-31 p.Article in journal (Refereed) Published
Abstract [en]

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 reduc tion 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.

Place, publisher, year, edition, pages
2004. Vol. 14, no 1, 24-31 p.
National Category
Medical and Health Sciences
Identifiers
URN: urn:nbn:se:uu:diva-90075PubMedID: 15237667OAI: oai:DiVA.org:uu-90075DiVA: diva2:162233
Available from: 2002-12-17 Created: 2002-12-17 Last updated: 2015-09-03Bibliographically approved
In thesis
1. Children with Down syndrome -: an epidemiological study with special focus on congenital heart defects
Open this publication in new window or tab >>Children with Down syndrome -: an epidemiological study with special focus on congenital heart defects
2002 (Swedish)Doctoral thesis, comprehensive summary (Other academic)
Abstract [en]

To assess the impact of congenital malformations in Down syndrome (DS) on morbidity, mortality and outcome at birth, information on all children with DS born in the northern part of Sweden in 1973-80 (n=211) and 1995-98 (n=88) was collected. Most common were congenital heart defects (CHD), dominated by atrioventricular septal defects (AVSD). Up to age 10 years, morbidity and mortality were more than 10 times higher in DS children with CHD than in healthy DS children. The DS children seemed more vulnerable at birth than Swedish children in general: they had increased frequencies of Cesarean sections, premature birth, asphyxia, and low birthweight, and higher proportions of children small for gestational age, regardless of the presence of CHD. Infant mortality decreased from 14.2% to 2.3% between the two periods.

All children with AVSD with and without DS born in Sweden 1973-1997 (n=801) were followed up retrospectively to 2001. Children with isolated AVSD without complex additional CHDs were studied more closely (n=502). A reduction in age at operation and postoperative mortality (from 28 to 1%) was observed. No significant difference in 5-year postoperative mortality between genders or between DS and non-DS children was found. The 5-year postoperative mortality in DS decreased from 35% in 1973-77 to about 10% in 1993-97.

CHD had a major influence on morbidity, infectionrate and mortality in DS, but not on DS birth variables. The formerly high mortality in CHD is now reduced. In isolated AVSD measures seem equally successful in DS and non-DS children. Mortality is still 3 times higher in DS children with isolated AVSD than in healthy DS children.

Place, publisher, year, edition, pages
Uppsala: Acta Universitatis Upsaliensis, 2002. 62 p.
Series
Comprehensive Summaries of Uppsala Dissertations from the Faculty of Medicine, ISSN 0282-7476 ; 1218
Keyword
Pediatrics, Down syndrome, congenital malformations, mortality, morbidity, neonatal data, congenital heart defect, atrioventricular septal defect, operation, gender, Pediatrik
National Category
Pediatrics
Research subject
Pediatrics
Identifiers
urn:nbn:se:uu:diva-3142 (URN)91-554-5496-8 (ISBN)
Public defence
2003-01-31, Rudbeckssalen, Rudbecks Laboratoriet, Uppsala, 09:15
Opponent
Available from: 2002-12-17 Created: 2002-12-17Bibliographically approved

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