Survival in Children With Congenital Heart Disease: Have We Reached a Peak at 97%?
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Published on: July 18, 2014
Zacharias Mandalenakis1, Kok Wai Giang1, Peter Eriksson1
1Department of Molecular and Clinical Medicine Institute of Medicine Sahlgrenska Academy University of Gothenburg Sweden.
Insights
Survival for children with congenital heart disease (CHD) has significantly improved since the 1980s. However, survival rates have plateaued this century, with over 97% now reaching adulthood, necessitating lifelong management.
Area of Science:
- Pediatric Cardiology
- Public Health
- Medical Registries
Background:
- Advances in pediatric healthcare have occurred over decades.
- Uncertainty remains regarding continued survival increases in children with congenital heart disease (CHD).
Purpose of the Study:
- To examine survival trends in children diagnosed with congenital heart disease (CHD).
- To compare mortality risk in pediatric CHD patients versus the general population.
Main Methods:
- Utilized nationwide Swedish health registries from 1980 to 2017.
- Included 64,396 patients with CHD and 639,012 matched controls.
- Employed Cox proportional regression and Kaplan-Meier survival analysis.
Main Results:
- Mortality risk was 17.7 times higher in children with CHD compared to controls.
- Highest mortality occurred within the first four years of life.
- Survival rates increased substantially by birth decade, reaching 97% for those born 2010-2017, with no improvement post-2000.
Conclusions:
- Survival in children with congenital heart disease (CHD) has significantly increased since the 1980s.
- No significant survival improvement observed in the 21st century.
- Over 97% of children with CHD now reach adulthood, emphasizing the need for lifelong care.
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.
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