Elsevier

American Heart Journal

Volume 157, Issue 3, March 2009, Pages 467-473.e1
American Heart Journal

Clinical Investigation
Valvular and Congenital Heart Disease
National time trends in congenital heart defects, Denmark, 1977-2005

https://doi.org/10.1016/j.ahj.2008.10.017Get rights and content

Background

Time trends in congenital heart defects (CHD) by specific phenotype and with long follow-up time are rarely available for an entire population. We present trends in national CHD prevalences over the past 3 decades.

Methods

We linked information from the National Patient Register, the Causes of Death Register, and the Danish Cytogenetic Central Register for all persons born in Denmark, 1977 to 2005, and registered in the Civil Registration System, yielding a cohort of 1,763,591 persons—18,207 with CHD. Individuals with CHDs were classified by phenotype (heterotaxia, conotruncal defect, atrioventricular septal defect, anomalous pulmonary venous return, left and right ventricular outflow tract obstructions, septal defects, complex defects, associations, patent ductus arteriosus, unspecified, and other specified) by combining International Classification of Diseases codes using a hierarchical approach.

Results

From 1977 to 2005, the overall CHD birth prevalence increased from 73 to 113 per 10,000 live births. Generally, prevalence increased for defects diagnosed in infancy, until 1996–1997, and then stabilized. For each 5-year interval, isolated septal defects and severe defects increased by 22% (95% CI, 20%-25%) and 5% (95% CI, 4%-7%), respectively. Among the severe defects, conotruncal defects and atrioventricular septal defect showed the largest prevalence increases. Women had a lower prevalence of severe defects during the 1980s. The CHD prevalence increase was unchanged when persons with extracardiac defects or chromosomal aberrations were excluded.

Conclusions

CHD birth prevalence increased from the beginning of the 1980s but stabilized in the late 1990s.

Section snippets

Data sources

Since April 1, 1968, the Danish Civil Registration System has registered demographic, residence, vital status, and kinship information on all persons residing in Denmark, aided by the unique personal identification number assigned to each Danish resident. The personal identification number permits accurate linkage of individual-level information from Denmark's nationwide population-based registers, including the National Patient Register (NPR), the Medical Birth Register, the Causes of Death

Results

Of 1,763,591 persons born in Denmark in the period 1977 to 2005, 18,207 had ≥1 CHDs, yielding an overall CHD prevalence of 103 per 10,000 live births (Table I). Isolated septal defects were the most prevalent defects, comprising nearly one third of all CHDs. Conotruncal defect prevalence was 11.5 per 10,000 live births. Atrioventricular septal defect had a prevalence of 4.46 per 10,000 live births, and the left and right heart obstructive defects had prevalences of 6.35 and 3.61 per 10,000 live

Discussion

We identified and categorized congenital CHDs in >18,000 persons born in Denmark during a 29-year period, for an overall prevalence of 103 per 10,000 live births. The prevalence of CHDs increased from 73 to 113 per 10,000 live births during the study period—1977 to 2005. Among singletons, the prevalence of severe CHDs increased overall by 25%, whereas VSD prevalence doubled and ASD prevalence tripled. Increasing numbers of CHDs diagnosed in infancy contributed most to the increases in

Disclosures

The authors are solely responsible for the design and conduct of this study, all study analyses, and the drafting and editing of the manuscript and its final contents.

Acknowledgements

We thank Dr Lorenzo Botto for sharing his classification scheme with us, and Jan Hansen for providing the data from the Danish Cytogenetic Central Register.

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