Tetralogy of Fallot. A population-based study of epidemiology, associated malformations and survival in western Denmark 1984-1992

Scand Cardiovasc J. 1999;33(1):45-8. doi: 10.1080/14017439950142037.


The study describes the epidemiology and mortality of tetralogy of Fallot (TOF) in a population-based study in Western Denmark. Ninety-two infants with TOF were born during 1984-1992. Prevalence was 3.01 per 10,000 livebirths. Karyotype anomalies were present in 12 (13%) and extracardiac malformations in 16 (17%) of the infants. Down syndrome, cleft palate, cleft lip and palate and combined skeletal, gastrointestinal and renal lesions (VACTERL association) were prevalent. Twenty-four infants died (26% of total), 13 (54%) of the deaths occurring during the first year of life. Mortality was significantly increased in infants with extracardiac malformations (50% vs 19%, p < 0.05). Eighteen deaths (75% of total deaths) occurred before corrective surgery and 7 of these deaths were sudden. Extracardiac malformation(s) in infants with TOF is a significant risk factor for death. The study stresses the importance of population-based studies for the assessment of mortality from congenital heart malformations. Overall mortality may be very different from mortality related to cardiac surgery.

MeSH terms

  • Chi-Square Distribution
  • Child
  • Child, Preschool
  • Chromosome Aberrations
  • Congenital Abnormalities / epidemiology*
  • Denmark / epidemiology
  • Female
  • Humans
  • Infant
  • Infant, Newborn
  • Male
  • Mortality / trends
  • Prevalence
  • Survival Analysis
  • Tetralogy of Fallot / epidemiology*
  • Tetralogy of Fallot / surgery
  • Treatment Outcome