Congenital Heart Defects in Europe Prevalence and Perinatal Mortality, 2000 to 2005

Background--This study determines the prevalence of Congenital Heart Defects (CHD), diagnosed prenatally or in
infancy, and fetal and perinatal mortality associated with CHD in Europe.
Methods and Results--Data were extracted from the European Surveillance of Congenital Anomalies central database for
29 population-based congenital anomaly registries in 16 European countries covering 3.3 million births during the
period 2000 to 2005. CHD cases (n26 598) comprised live births, fetal deaths from 20 weeks gestation, and
terminations of pregnancy for fetal anomaly (TOPFA). The average total prevalence of CHD was 8.0 per 1000 births,
and live birth prevalence was 7.2 per 1000 births, varying between countries. The total prevalence of nonchromosomal
CHD was 7.0 per 1000 births, of which 3.6% were perinatal deaths, 20% prenatally diagnosed, and 5.6% TOPFA.
Severe nonchromosomal CHD (ie, excluding ventricular septal defects, atrial septal defects, and pulmonary valve
stenosis) occurred in 2.0 per 1000 births, of which 8.1% were perinatal deaths, 40% were prenatally diagnosed, and 14%
were TOPFA (TOPFA range between countries 0% to 32%). Live-born CHD associated with Down syndrome occurred
in 0.5 per 1000 births, with 4-fold variation between countries.
Conclusion--Annually in the European Union, we estimate 36 000 children are live born with CHD and 3000 who are
diagnosed with CHD die as a TOFPA, late fetal death, or early neonatal death. Investing in primary prevention and
pathogenetic research is essential to reduce this burden, as well as continuing to improve cardiac services from in utero
to adulthood.