Svetlana V Glinianaia1, Judith Rankin1, Anna Pierini2,3, Alessio Coi2, Michele Santoro2, Joachim Tan4, Abigail Reid4, Ester Garne5, Maria Loane6, Joanne Given6, Clara Cavero-Carbonell7, Hermien E K de Walle8, Miriam Gatt9, Mika Gissler10, Anna Heino10, Babak Khoshnood11, Kari Klungsøyr12,13, Nathalie Lelong11, Amanda J Neville14, Daniel S Thayer15, David Tucker16, Stine K Urhøj5, Diana Wellesley17, Oscar Zurriaga7, Joan K Morris4. 1. Faculty of Medical Sciences, Population Health Sciences Institute, Newcastle University, Newcastle upon Tyne, United Kingdom. 2. Unit of Epidemiology of Rare diseases and Congenital Anomalies, Institute of Clinical Physiology, National Research Council, Pisa, Italy. 3. Fondazione Toscana Gabriele Monasterio, Pisa, Italy. 4. Population Health Research Institute, St George's, University of London, London, United Kingdom. 5. Pediatric Department, Hospital Lillebaelt, Kolding, Denmark. 6. Faculty of Life & Health Sciences, Ulster University, Northern Ireland, United Kingdom. 7. Rare Diseases Research Unit, Foundation for the Promotion of Health and Biomedical Research in the Valencian Region, Valencia, Spain. 8. Department of Genetics, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands. 9. Malta Congenital Anomalies Registry, Directorate for Health Information and Research, Tal-Pietà, Malta. 10. Information Services Department, THL Finnish Institute for Health and Welfare. Helsinki, Finland. 11. Obstetrical, Perinatal and Pediatric Epidemiology Research Team (EPOPé), INSERM-INRA, Université de Paris, Center of Research in Epidemiology and Statistics (CRESS), Paris, France. 12. Department of Global Public Health and Primary Care, University of Bergen, Bergen, Norway. 13. Division of Mental and Physical Health, Norwegian Institute of Public Health, Bergen, Norway. 14. Center for Clinical and Epidemiological Research, University of Ferrara, Ferrara, Italy. 15. Faculty of Health and Life Science, Swansea University, Swansea, United Kingdom. 16. Public Health Wales, Swansea, United Kingdom. 17. Wessex Clinical Genetics Service, Princess Anne Hospital, Southampton, United Kingdom.
Abstract
OBJECTIVES: To investigate the survival up to age 10 for children born alive with a major congenital anomaly (CA). METHODS: This population-based linked cohort study (EUROlinkCAT) linked data on live births from 2005 to 2014 from 13 European CA registries with mortality data. Pooled Kaplan-Meier survival estimates up to age 10 were calculated for these children (77 054 children with isolated structural anomalies and 4011 children with Down syndrome). RESULTS: The highest mortality of children with isolated structural CAs was within infancy, with survival of 97.3% (95% confidence interval [CI]: 96.6%-98.1%) and 96.9% (95% CI: 96.0%-97.7%) at age 1 and 10, respectively. The 10-year survival exceeded 90% for the majority of specific CAs (27 of 32), with considerable variations between CAs of different severity. Survival of children with a specific isolated anomaly was higher than in all children with the same anomaly when those with associated anomalies were included. For children with Down syndrome, the 10-year survival was significantly higher for those without associated cardiac or digestive system anomalies (97.6%; 95% CI: 96.5%-98.7%) compared with children with Down syndrome associated with a cardiac anomaly (92.3%; 95% CI: 89.4%-95.3%), digestive system anomaly (92.8%; 95% CI: 87.7%-98.2%), or both (88.6%; 95% CI: 83.2%-94.3%). CONCLUSIONS: Ten-year survival of children born with congenital anomalies in Western Europe from 2005 to 2014 was relatively high. Reliable information on long-term survival of children born with specific CAs is of major importance for parents of these children and for the health care professionals involved in their care.
OBJECTIVES: To investigate the survival up to age 10 for children born alive with a major congenital anomaly (CA). METHODS: This population-based linked cohort study (EUROlinkCAT) linked data on live births from 2005 to 2014 from 13 European CA registries with mortality data. Pooled Kaplan-Meier survival estimates up to age 10 were calculated for these children (77 054 children with isolated structural anomalies and 4011 children with Down syndrome). RESULTS: The highest mortality of children with isolated structural CAs was within infancy, with survival of 97.3% (95% confidence interval [CI]: 96.6%-98.1%) and 96.9% (95% CI: 96.0%-97.7%) at age 1 and 10, respectively. The 10-year survival exceeded 90% for the majority of specific CAs (27 of 32), with considerable variations between CAs of different severity. Survival of children with a specific isolated anomaly was higher than in all children with the same anomaly when those with associated anomalies were included. For children with Down syndrome, the 10-year survival was significantly higher for those without associated cardiac or digestive system anomalies (97.6%; 95% CI: 96.5%-98.7%) compared with children with Down syndrome associated with a cardiac anomaly (92.3%; 95% CI: 89.4%-95.3%), digestive system anomaly (92.8%; 95% CI: 87.7%-98.2%), or both (88.6%; 95% CI: 83.2%-94.3%). CONCLUSIONS: Ten-year survival of children born with congenital anomalies in Western Europe from 2005 to 2014 was relatively high. Reliable information on long-term survival of children born with specific CAs is of major importance for parents of these children and for the health care professionals involved in their care.
Authors: Alessio Coi; Michele Santoro; Anna Pierini; Judith Rankin; Svetlana V Glinianaia; Joachim Tan; Abigail-Kate Reid; Ester Garne; Maria Loane; Joanne Given; Elisa Ballardini; Clara Cavero-Carbonell; Hermien E K de Walle; Miriam Gatt; Laura García-Villodre; Mika Gissler; Sue Jordan; Sonja Kiuru-Kuhlefelt; Stine Kjaer Urhoj; Kari Klungsøyr; Nathalie Lelong; L Renée Lutke; Amanda J Neville; Makan Rahshenas; Ieuan Scanlon; Diana Wellesley; Joan K Morris Journal: Orphanet J Rare Dis Date: 2022-03-29 Impact factor: 4.123