[Congenital heart defects in the county of Fyn. Epidemiology and mortality 1986-1995].
BACKGROUND
The aim was to describe the prevalence of congenital heart diseases (CHD), mortality during the first year of life, and the impact of associated malformations on survival.


METHODS
All children born in the County of Fyn in 1986-1995, who were diagnosed with CHD and recorded in the Eurocat Registry of Congenital Malformation were entered. The registry is population-based and the information is derived from multiple sources. It covers live births, fetal deaths with a GA (gestational age) > or = 20 weeks, and induced abortions after prenatal diagnosis of congenital malformations. Only cases diagnosed by echocardiography, catheterisation, surgery, or post mortem examination were included.


RESULTS
Four hundred and forty-six infants born during the study period were diagnosed with CHD before 1998, giving a prevalence of 8.0 per 1,000 live births. The prevalence of ASD + VSD had increased in the period of 1991-1995 compared to 1986-1990 although not significantly. Ten per cent had karyotype anomalies and nine per cent had associated malformations. Only 3% were diagnosed prenatally, 49% were diagnosed before the age of one week, and ten per cent were diagnosed after the first year of life. CHD mortality during the first year of life was 18% (80/446) and was significantly increased for infants with associated malformations (p < 0.01) or karyotype anomalies other than Down syndrome (p < 0.01).


CONCLUSIONS
The prevalence of CHD in the County of Fyn corresponds to the data in the literature. At the time of introduction of colour Doppler echocardiography there was an increase in the proportion of infants diagnosed with ASD and VSD, although not significantly. Mortality was high, but 20% of deaths were infants with a hypoplastic left heart. In regions with prenatal ultrasound screening for malformations, this was often diagnosed prenatally followed by termination of pregnancy. For infants with CHD, it is important to verify, whether there are associated malformations or karyotype anomalies, as they have a significant impact on the prognosis.
D003718:Denmark / Q000453:epidemiology; D005260:Female; D005313:Fetal Death / Q000453:epidemiology; D006330:Heart Defects, Congenital / Q000000981:diagnostic imaging / Q000453:epidemiology* / Q000401:mortality; D006801:Humans; D007223:Infant; D007226:Infant Mortality; D007231:Infant, Newborn; D008297:Male; D011247:Pregnancy; D015995:Prevalence; D012042:Registries; D016216:Ultrasonography, Prenatal
Medfødte hjertesygdomme i Fyns Amt. Epidemiologi og mortalitet 1986-1995.