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. 2022 Nov;36(6):792-803.
doi: 10.1111/ppe.12884. Epub 2022 Jun 8.

Temporal and geographical variations in survival of children born with congenital anomalies in Europe: A multi-registry cohort study

Michele Santoro  1 , Alessio Coi  1 , Anna Pierini  1   2 , Judith Rankin  3 , Svetlana V Glinianaia  3 , Joachim Tan  4 , Abigail Reid  4 , Ester Garne  5 , Maria Loane  6 , Joanne Given  6 , Amaia Aizpurua  7 , Gianni Astolfi  8 , Ingeborg Barisic  9 , Clara Cavero-Carbonell  10 , Hermien E K de Walle  11 , Elly Den Hond  12 , Laura García-Villodre  10 , Miriam Gatt  13 , Mika Gissler  14 , Sue Jordan  15 , Babak Khoshnood  16 , Sonja Kiuru-Kuhlefelt  14 , Kari Klungsøyr  17   18 , Nathalie Lelong  16 , Renée Lutke  11 , Olatz Mokoroa  7 , Vera Nelen  12 , Amanda J Neville  19 , Ljubica Odak  9 , Anke Rissmann  20 , Ieuan Scanlon  15 , Stine Kjaer Urhoj  5 , Diana Wellesley  21 , Wladimir Wertelecki  22 , Lyubov Yevtushok  22 , Joan K Morris  4
Affiliations

Temporal and geographical variations in survival of children born with congenital anomalies in Europe: A multi-registry cohort study

Michele Santoro et al. Paediatr Perinat Epidemiol. 2022 Nov.

Abstract

Background: Congenital anomalies are a major cause of perinatal, neonatal and infant mortality.

Objectives: The aim was to investigate temporal changes and geographical variation in survival of children with major congenital anomalies (CA) in different European areas.

Methods: In this population-based linkage cohort study, 17 CA registries members of EUROCAT, the European network for the surveillance of CAs, successfully linked data on 115,219 live births with CAs to mortality records. Registries estimated Kaplan-Meier survival at 28 days and 5 years of age and fitted Cox's proportional hazards models comparing mortality at 1 year and 1-9 years of age for children born during 2005-2014 with those born during 1995-2004. The hazard ratios (HR) from each registry were combined centrally using a random-effects model. The 5-year survival conditional on having survived to 28 days of age was calculated.

Results: The overall risk of death by 1 year of age for children born with any major CA in 2005-2014 decreased compared to 1995-2004 (HR 0.68, 95% confidence interval [CI] 0.53, 0.89). Survival at 5 years of age ranged between registries from 97.6% to 87.0%. The lowest survival was observed for the registry of OMNI-Net (Ukraine) (87.0%, 95% CI 86.1, 87.9).

Conclusions: Survival of children with CAs improved for births in 2005-2014 compared with 1995-2004. The use of CA registry data linked to mortality data enables investigation of survival of children with CAs. Factors such as defining major CAs, proportion of terminations of pregnancy for foetal anomaly, source of mortality data and linkage methods are important to consider in the design of future studies and in the interpretation of the results on survival of children with CAs.

Keywords: cohort study; congenital anomalies; registry; survival.

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Conflict of interest statement

None declared.

Figures

FIGURE 1
FIGURE 1
Five‐year survival compared with 5‐year survival conditional on having survived to 28 days in children with a major congenital anomaly (CA) (birth year period: 2005–2014). (A) ‘All anomalies’, (B) ‘Severe congenital heart defects’ U = Ukraine: Omni‐Net; M = Malta; F = Finland. Data of ‘Severe congenital heart defects’ for Denmark: Funen could not be displayed due to small numbers
FIGURE 2
FIGURE 2
Five‐year survival in children with a major congenital anomaly compared with the prevalence of children with a major congenital anomaly (CA) (birth year period: 2005–2014). (A) ‘All anomalies’, (B) ‘Severe congenital heart defects’ U = Ukraine: Omni‐Net; M = Malta; F = Finland
See this image and copyright information in PMC

References

    1. Lozano R, Naghavi M, Foreman K, et al. Global and regional mortality from 235 causes of death for 20 age groups in 1990 and 2010: a systematic analysis for the global burden of disease study 2010. Lancet. 2012;380:2095‐2128. - PMC - PubMed
    1. Murray CJ, Ezzati M, Flaxman AD, et al. GBD 2010: a multi‐investigator collaboration for global comparative descriptive epidemiology. Lancet. 2012;380:2055‐2058. - PubMed
    1. Hug L, Alexander M, You D, Alkema L. UN inter‐agency Group for Child Mortality Estimation. National, regional, and global levels and trends in neonatal mortality between 1990 and 2017, with scenario‐based projections to 2030: a systematic analysis. Lancet Glob Health. 2019. Jun;7(6):e710‐e720. doi: 10.1016/S2214-109X(19)30163-9 - DOI - PMC - PubMed
    1. Rosano A, Botto LD, Botting B, Mastroiacovo P. Infant mortality and congenital anomalies from 1950 to 1994: an international perspective. J Epidemiol Community Health. 2000;54(9):660‐666. - PMC - PubMed
    1. Liu L, Johnson HL, Cousens S, et al. Global, regional, and national causes of child mortality: an updated systematic analysis for 2010 with time trends since 2000. Lancet. 2012;379:2151‐2161. - PubMed

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