Background and aims Severe congenital heart defects (CHDs) still represent one of the main causes of infant death. The risk factors associated with cardiac surgery and postoperative mortality are well known. We aimed to describe the rates, causes and time trends of mortality before surgery—including termination of pregnancies and palliative care—in fetuses and children below 2 years of age with severe CHDs.
Methods and results Data concerning all 789 345 pregnancies in Norway from 2004 to 2016 were retrieved from the Medical Birth Registry of Norway, the Oslo University Hospital’s Clinical Registry for Congenital Heart Defects, the Norwegian Cause of Death Registry, the National Registry, Statistics Norway, autopsy reports and medical records. When including termination of pregnancy and stillbirths, the number of fetuses and children with severe CHDs that did not reach the age of 2 years was 31%. Mortality among the 2359 live-born infants with severe CHDs was 10%, of whom 58% died before surgery. Of the preoperative deaths, 81% died in a palliative care setting, and comorbidity and univentricular CHDs were common among these infants. Together, palliative care and termination of pregnancy accounted for 86% of deaths in cases of severe CHDs, and this proportion increased during the study period (annual percent changes 1.3, 95% CI 0.4 to 2.1, p<0.001), mainly due to an increased termination rate.
Conclusions Termination of pregnancy accounted for the majority of the deaths in fetuses and children with severe CHDs. Among live-born children, most preoperative deaths occurred in a palliative care setting and were strongly related to comorbidities and/or univentricular hearts.
- palliative care
- congenital heart defect
- preoperative mortality
- termination of pregnancy
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Contributors GW contributed to the study concept and design, data collection and interpretation, literature review and revised and approved the manuscript. JJ contributed to the study concept and design, data interpretation, literature review and revised and approved the manuscript. VS contributed to the study concept, literature review and revised and approved the manuscript. GD and AER contributed to the study concept and revised and approved the manuscript. HH contributed to the study concept and design, data interpretation, literature review and revised and approved the manuscript.
Funding This work was supported by the Norwegian Association for Children with Congenital Heart Disease, the Oslo University Hospital and Sørlandet Hospital HF, Norway.
Disclaimer The funders have no role in the design and conduct of the study, in the collection, analysis and interpretation of the data, and in the preparation, review or approval of the manuscript.
Competing interests None declared.
Patient consent for publication Not required.
Ethics approval The study was approved by the Regional Committee for Medical and Health Research Ethics South East Norway (REK nr. 2010/392).
Provenance and peer review Not commissioned; externally peer reviewed.
Data availability statement Data are stored in 'eReg Versjon 2.4.0 OUS-HF' and are available upon reasonable request. They are not to be reused without approval from the Regional Committee for Medical and Health Research Ethics South East Norway.
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