Causes of death in children with congenital anomalies up to age 10 in 8 European countries

Anke Rissmann, Joachim Tan, SV Glinianaia, J Rankin, Anna Pierini, Michele Santoro, Alessio Coi, Garne Ester, Maria Loane, Joanne Given, Abigail Reid, Amaia Aizpurua, Diana Akhmedzhanova, Elisa Ballardini,, Ingeborg Barisic, Clara Cavero-Carbonell, Hermien de Walle, Miriam Gatt, Mika Gissler, Anna HeinoSue Jordan, Stine Kjær Urhøj, Kari Klungsøyr, Renée Lutke, Olatz Mokoroa, Amanda Neville, Daniel S Thayer, Diana Wellesley, Lyubov Yevtushok, Oscar Zurriaga, Joan Morris

Research output: Contribution to journalArticlepeer-review


Background Congenital anomalies (CAs) increase the risk of death during infancy and childhood. This study aimed to evaluate the accuracy of using death certificates to estimate the burden of CAs on mortality for children under 10 years old. Methods Children born alive with a major CA between 1st January 1995 and 31st December 2014, from 13 population-based European CA registries were linked to mortality records up to their 10th birthday or 31st December 2015, whichever was earlier. Results In total 4,199 neonatal, 2,100 post-neonatal and 1,087 deaths in children aged 1 to 9 years were reported. The underlying cause of death was a CA in 71% (95% CI: 64%-78%) of neonatal and 68% (95% CI: 61%-74%) of post-neonatal infant deaths. For neonatal deaths the proportions varied by registry from 45% to 89% and by anomaly from 53% for Down syndrome to 94% for Tetralogy of Fallot. In children aged 1-9, 49% (95% CI: 42%-57%) were attributed to a CA. Comparing mortality in children with anomalies to population mortality predicts that over 90% of all deaths at all ages are attributable to the anomalies. The exact ICD9/ICD10 CA code was often not reported for any cause of death, even for lethal anomalies such as Trisomy 13 (20% with incorrect codes). Conclusions Data on the underlying cause of death from death certificates alone are not sufficient to evaluate the burden of CAs on infant and childhood mortality across countries and over time. Linked data from CA registries and death certificates are necessary for obtaining accurate estimates.
Original languageEnglish
JournalBMJ Open
Publication statusAccepted/In press - 20 Aug 2022

Bibliographical note

This project has received funding from the European Union’s Horizon 2020 research and innovation programme under grant agreement No. 733001.
The views presented here are those of the authors only, and the European Commission is not responsible for any use that may be made of the information presented here.


  • Epidemiology
  • Mortality


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