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 assesment of mortality from congenital heart malformations.
Overall mortality may be very different from mortality related to cardiac surgery.
Mots-clés Pascal : Tétralogie Fallot, Homme, Danemark, Europe, Epidémiologie, Mortalité, Pronostic, Appareil circulatoire pathologie, Cardiopathie, Maladie congénitale
Mots-clés Pascal anglais : Fallot tetralogy, Human, Denmark, Europe, Epidemiology, Mortality, Prognosis, Cardiovascular disease, Heart disease, Congenital disease
Notice produite par :
Inist-CNRS - Institut de l'Information Scientifique et Technique
Cote : 99-0156753
Code Inist : 002B12A08. Création : 16/11/1999.