Tetralogy of fallot - A population-based study of epidemiology, associatedmalformations and survival in western Denmark 1984-1992

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