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American Heart Association, Circulation: Arrhythmia and Electrophysiology, 6(11), 2018

DOI: 10.1161/circep.117.005757

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Nationwide Study of Sudden Cardiac Death in People With Congenital Heart Defects Aged 0 to 35 Years

This paper was not found in any repository, but could be made available legally by the author.
This paper was not found in any repository, but could be made available legally by the author.

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Abstract

Background: Congenital heart defects (CHD) are among the leading causes of sudden cardiac death (SCD) in the young. Nationwide incidence of SCD in people with CHD (SCD-CHD) has not been established in the young general population. The aims of this study were to investigate incidence of SCD-CHD and whether incidence of SCD-CHD in infants declined after implementation of nationwide fetal ultrasound screening in Denmark. Methods: All deaths (n=11 451) among people aged 0 to 35 years in Denmark in 2000 to 2009 (24.4 million person-years) were included. Danish death certificates, autopsy reports, records from hospitals and general practitioners, and data from nationwide Danish registries were used to identify SCD-CHD cases. Results: We identified 90 (11%) cases of SCD-CHD from 809 SCD. The incidence rate of SCD-CHD was 0.4 per 100 000 person-years among people aged 0 to 35 years. In total, 53 (59%) were diagnosed with CHD before death. Incidence of SCD was 9.6× higher among patients with CHD compared with people without CHD ( P <0.01). Annual incidence of physical activity–related SCD-CHD among patients aged 2 to 35 years diagnosed with CHD was 0.9 per 100 000. The annual incidence rate of SCD-CHD in infants declined after implementation of nationwide fetal ultrasound screening (incidence rate ratio, 3.8; P <0.01). Conclusions: The proportion of SCD-CHD in the young was 11%, which is higher than previously reported. Physical activity–related SCD-CHD was a rare event among patients with CHD. We observed an ≈4-fold lower incidence of SCD-CHD among infants born after implementation of nationwide screening.