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

Circ Arrhythm Electrophysiol. 2018 Jun;11(6):e005757. doi: 10.1161/CIRCEP.117.005757.

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.

Keywords: death, sudden, cardiac; exercise; heart defects, congenital; incidence; sports.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Age Distribution
  • Child
  • Child, Preschool
  • Death, Sudden, Cardiac / epidemiology*
  • Denmark / epidemiology
  • Echocardiography
  • Female
  • Heart Defects, Congenital / diagnostic imaging
  • Heart Defects, Congenital / mortality*
  • Humans
  • Incidence
  • Infant
  • Infant, Newborn
  • Male
  • Prognosis
  • Registries
  • Risk Assessment
  • Risk Factors
  • Time Factors
  • Ultrasonography, Prenatal / methods
  • Young Adult