Congenital heart defects in Europe: prevalence and perinatal mortality, 2000 to 2005

Helen Dolk, Maria Loane, Ester Garne, European Surveillance of Congenital Anomalies (EUROCAT) Working Group, Marie-Claude Addor, Marian Bakker, Ingeborg Barisic, Sebastiano Bianca, Patricia Boyd, Elisa Calzolari, Berenice Doray, Miriam Gatt, Martin Haeusler, Babak Khoshnood, Kari Klungsoyr Melve, Anna Latos-Bielenska, Bob McDonnell, Carmel Mullaney, Vera Nelen, Mary O'Mahony, Anna Pierini, Simone Poetzsch, Annette Queisser-Luft, Hanitra Randrianaivo, Judith Rankin, Joaquin Salvador, David Tucker, Christine Verellen-Dumoulin, Diana Wellesley, Wladimir Wertelecki, Helen Dolk, Maria Loane, Ester Garne, European Surveillance of Congenital Anomalies (EUROCAT) Working Group, Marie-Claude Addor, Marian Bakker, Ingeborg Barisic, Sebastiano Bianca, Patricia Boyd, Elisa Calzolari, Berenice Doray, Miriam Gatt, Martin Haeusler, Babak Khoshnood, Kari Klungsoyr Melve, Anna Latos-Bielenska, Bob McDonnell, Carmel Mullaney, Vera Nelen, Mary O'Mahony, Anna Pierini, Simone Poetzsch, Annette Queisser-Luft, Hanitra Randrianaivo, Judith Rankin, Joaquin Salvador, David Tucker, Christine Verellen-Dumoulin, Diana Wellesley, Wladimir Wertelecki

Abstract

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 (n=26 598) 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.

Source: PubMed

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