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. 2020 Nov 17;9(22):e017704.
doi: 10.1161/JAHA.120.017704. Epub 2020 Nov 6.

Survival in Children With Congenital Heart Disease: Have We Reached a Peak at 97%?

Zacharias Mandalenakis  1 , Kok Wai Giang  1 , Peter Eriksson  1 , Hans Liden  2 , Mats Synnergren  2 , Håkan Wåhlander  2 , Maria Fedchenko  1 , Annika Rosengren  1 , Mikael Dellborg  1
Affiliations

Survival in Children With Congenital Heart Disease: Have We Reached a Peak at 97%?

Zacharias Mandalenakis et al. J Am Heart Assoc. .

Abstract

Background Despite advances in pediatric health care over recent decades, it is not clear whether survival in children with congenital heart disease (CHD) is still increasing. Methods and Results We identified all patients with CHD using nationwide Swedish health registries for 1980 to 2017. We examined the survival trends in children with CHD; we investigated the mortality risk in patients with CHD compared with matched controls without CHD from the general population using Cox proportional regression models and Kaplan-Meier survival analysis. Among 64 396 patients with CHD and 639 012 matched controls without CHD, 3845 (6.0%) and 2235 (0.3%) died, respectively. The mean study follow-up (SD) was 11.4 (6.3) years in patients with CHD. The mortality risk was 17.7 (95% CI, 16.8-18.6) times higher in children with CHD compared with controls. The highest mortality risk was found during the first 4 years of life in patients with CHD (hazard ratio [HR], 19.6; 95% CI, 18.5-20.7). When stratified by lesion group, patients with non-conotruncal defects had the highest risk (HR, 97.2; 95% CI, 80.4-117.4). Survival increased substantially according to birth decades, but with no improvement after the turn of the century where survivorship reached 97% in children with CHD born in 2010 to 2017. Conclusions Survival in children with CHD has increased substantially since the 1980s; however, no significant improvement has been observed this century. Currently, >97% of children with CHD can be expected to reach adulthood highlighting the need of life-time management.

Keywords: congenital heart disease; nationwide; pediatric; registry study; survivorship.

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Conflict of interest statement

None.

Figures

Figure 1
Figure 1. Kaplan–Meier survival curves of patients with congenital heart disease and matched controls according to birth period.
CHD indicates congenital heart disease.
Figure 2
Figure 2. Kaplan–Meier survival curves of patients with congenital heart disease with or without a cardiac intervention according to birth period.
See this image and copyright information in PMC

References

    1. Liu Y, Chen S, Zuhlke L, Black GC, Choy MK, Li N, Keavney BD. Global birth prevalence of congenital heart defects 1970–2017: updated systematic review and meta‐analysis of 260 studies. Int J Epidemiol. 2019;48:455–463. - PMC - PubMed
    1. Marelli AJ, Ionescu‐Ittu R, Mackie AS, Guo L, Dendukuri N, Kaouache M. Lifetime prevalence of congenital heart disease in the general population from 2000 to 2010. Circulation. 2014;130:749–756. - PubMed
    1. Mandalenakis Z, Rosengren A, Skoglund K, Lappas G, Eriksson P, Dellborg M. Survivorship in children and young adults with congenital heart disease in Sweden. JAMA Intern Med. 2017;177:224–230. - PubMed
    1. Moons P, Bovijn L, Budts W, Belmans A, Gewillig M. Temporal trends in survival to adulthood among patients born with congenital heart disease from 1970 to 1992 in Belgium. Circulation. 2010;122:2264–2272. - PubMed
    1. Morris CD, Menashe VD. 25‐year mortality after surgical repair of congenital heart defect in childhood. A population‐based cohort study. JAMA. 1991;266:3447–3452. - PubMed

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