National time trends in congenital heart defects, Denmark, 1977-2005

Department of Epidemiology Research, Statens Serum Institut, Copenhagen, Denmark.
American heart journal (Impact Factor: 4.56). 04/2009; 157(3):467-473.e1. DOI: 10.1016/j.ahj.2008.10.017
Source: PubMed

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

  • [Show abstract] [Hide abstract]
    ABSTRACT: To conduct a population-based study examining the occurrence of congenital heart defects (CHDs) in relation to maternal smoking during the first trimester of pregnancy. This retrospective case-control study used Washington State birth certificates from 1989 to 2011 and linked hospital discharge International Classification of Diseases, 9th revision, codes to identify singleton nonsyndromic CHD cases and determine maternal prenatal smoking status. We calculated ORs from multivariate logistic regression models to compare maternal first-trimester smoking status (any and daily number of cigarettes) among 14 128 cases, both overall and by phenotype, and 60 938 randomly selected controls frequency matched on birth year. Offspring of mothers reporting cigarette use in the first trimester of pregnancy were more likely to be born with a CHD (aOR 1.16 [1.08-1.24]) independent of demographic characteristics and other prenatal risk factors for CHDs. Maternal smoking was most strongly associated with pulmonary valve anomalies (aOR 1.48 [95% CI: 1.15-1.90]), pulmonary artery anomalies (aOR 1.71 [1.40-2.09]), and isolated atrial septal defects (aOR 1.22 [1.08-1.38]). The association between maternal smoking and CHDs was stronger with increasing number of daily cigarettes and among older (35+ years) mothers compared with younger mothers. We provide evidence that maternal smoking during pregnancy is a risk factor for select CHD phenotypes. Maternal smoking may account for 1.4% of all CHDs. New findings include a strong dose-dependence of the association and augmented risk in older mothers. Copyright © 2015 Elsevier Inc. All rights reserved.
    Journal of Pediatrics 01/2015; DOI:10.1016/j.jpeds.2014.11.042 · 3.74 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Cardiovascular malformations are a singularly important class of birth defects and, due to dramatic improvements in medical and surgical care, there are now large numbers of adult survivors. The etiologies are complex, but there is strong evidence that genetic factors play a crucial role. Over the last 15 years there has been enormous progress in the discovery of causative genes for syndromic heart malformations and in rare families with Mendelian forms. The rapid characterization of genomic disorders as major contributors to congenital heart defects is also notable. The genes identified encode many transcription factors, chromatin regulators, growth factors and signal transduction pathways- all unified by their required roles in normal cardiac development. Genome-wide sequencing of the coding regions promises to elucidate genetic causation in several disorders affecting cardiac development. Such comprehensive studies evaluating both common and rare variants would be essential in characterizing gene-gene interactions, as well as in understanding the gene-environment interactions that increase the susceptibility to congenital heart defects.
    European journal of medical genetics 04/2014; DOI:10.1016/j.ejmg.2014.04.010 · 1.49 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Background The reasons for decreasing birth prevalence of congenital heart defects (CHD) in several European countries and Canada are not fully understood. We present CHD prevalence among live births, stillbirths, and terminated pregnancies in an entire nation over a period of 16 years. Methods Information on all births in the Medical Birth Registry of Norway, 1994-2009, was updated with information on CHD from the hospitals´ Patient Administrative Systems, the National Hospital´s clinical database for children with heart disease, and the Cause of Death Registry. Individuals with heart defects were assigned specific cardiac phenotypes. Results Among 954,413 births, 13,081 received a diagnosis of CHD (137.1 per 10,000 births, 133.2 per 10,000 live births). The prevalence per 10,000 births was: heterotaxia 1.6; conotruncal defects 11.6; atrioventricular septal defects 5.6; anomalous pulmonary venous return 1.1; left outflow obstructions 8.7; right outflow obstructions 5.6; septal defects 65.5, isolated ductus arteriosus (PDA) 24.6; and other specified or unspecified CHD 12.7. Excluding preterm PDA, the CHD prevalence was 123.4 per 10,000; per year, the prevalence increased with 3.5% (95% confidence interval 2.5, 4.4) in 1994-2005, and declined with 9.8% (-16.7, -2.4) from 2005 onwards. Severe CHD prevalence was 30.7 per 10,000; per year increase was 2.3% (1.1, 3.5) in 1994-2004, and per year decrease 3.4% (-6.6, -0.0) in 2004-2009. Numbers included severe CHD in stillbirths and terminated pregnancies. Conclusions The birth prevalence of CHD declined from around 2005. Specifically, the prevalence of severe CHD was reduced by 3.4% per year from 2004 through 2009.
    American Heart Journal 08/2014; DOI:10.1016/j.ahj.2014.07.030 · 4.56 Impact Factor