Cited by 169Congenital Heart Disease StudiesCongenital Anomalies and Fetal SurgeryPrenatal Screening and Diagnostics

Live-Born Major Congenital Heart Disease in Denmark: Incidence, Detection Rate, and Termination of Pregnancy Rate From 1996 to 2013

Open access
Lytzen, Rebekka, Vejlstrup, Niels, Bjerre, Jesper, Petersen, Olav Bjørn, Leenskjold, Stine, Dodd, James Keith, Jørgensen, Finn Stener, Søndergaard, Lars·2018·JAMA Cardiology

Abstract

Importance: The occurrence of major congenital heart disease (CHD) is affected by several variables. Determining the development of the true incidence is critical to the establishment of proper treatment of these patients. Objective: To evaluate time trends in incidence, detection rate, and termination of pregnancy (TOP) rate of major CHD in fetuses in Denmark and assess the influence of the introduction of general prenatal screening in 2004. Design, Setting, and Participants: Nationwide, population-based, retrospective observational study in Denmark from 1996 to 2013 that included a consecutive sample of 14 688 live-born children and terminated fetuses diagnosed as having CHD. Patient records on TOP and children with major CHD were reviewed to validate the diagnoses. Major CHD included univentricular heart, transposition of the great arteries, congenitally corrected transposition of the great arteries, truncus arteriosus, interrupted aortic arch, atrioventricular septal defects, double outlet right ventricle, coarctatio of the aorta, Ebstein anomaly, pulmonary atresia with ventricular septal defect, pulmonary atresia with intact ventricular septum, and tetralogy of Fallot. Data were analyzed between January 2017 and March 2018. Main Outcomes and Measures: Temporal changes in incidence, detection rate, and TOP of major CHD. Results: Of 14 688 children and fetuses diagnosed with CHD, 2695 (18.4%; 95% CI, 17.8-19.1) had major CHD. A total of 7131 boys (1304 with major CHD) and 6926 girls (920 with major CHD) were included, with a median age of 11 years (interquartile range, 6-15 years). During the study period, the live-birth incidence of CHD was constant at 1.22% (95% CI, 1.18-1.26), whereas it decreased for major CHD. When including TOP, the incidence of major CHD did not change over time. The detection rate of major CHD increased from 4.5% (95% CI, 1.2-7.8) to 71.0% (95% CI, 63.3-78.7) (P < .001). At the end of the study, all cases of double outlet right ventricle, Ebstein anomaly, congenitally corrected transposition of the great arteries, and pulmonary atresia with ventricular septal defect were detected prenatally, whereas coarctation of the aorta had the lowest detection rate (21.7%; 95% CI, 3.5-40.0). The TOP rate increased from 0.6% (95% CI, -0.6 to 1.9) to 39.1% (95% CI, 30.9-47.4) (P < .001) among all major CHD. For prenatally diagnosed major CHD, 57.8% of cases were terminated and the proportion did not change significantly throughout the study. Diagnoses leading to TOP included all major CHD diagnoses. Conclusions and Relevance: Detection rates of major CHD improved during the study. This has led to increased TOP rates, with a subsequent 39% decrease in the live-birth incidence of major CHD.

The claims and statistics of this paper haven’t been checked yet.

Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.

A few free checks a day · papers already verified open instantly · results are public

Integrity signals

Registry

Crossref · 10.1001/jamacardio.2018.2009

Cites

35 of 35 references verified0 citation errors · 0 not checkable

Statistics

Claims the paper makes

The claims and statistics of this paper haven’t been checked yet.

Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.

A few free checks a day · papers already verified open instantly · results are public

Claims other papers make about this one

Each is checked against this paper’s own text: does it say what the citing paper says it does?

Self-check

Percentages, sums and reported differences re-computed from the paper’s own numbers.

The self-check needs the paper’s full text.

Cites

Flagged first, then by citations

Cited by

Sorted by citations

Authors
Lytzen, R., Vejlstrup, N., Bjerre, J., Petersen, O. B., Leenskjold, S., Dodd, J. K., Jørgensen, F. S., & Søndergaard, L.
Affiliations
  • Rigshospitalet (DK)
  • Aarhus University Hospital (DK)
  • Aalborg University Hospital (DK)
  • Hvidovre Hospital (DK)
Corresponding author
Lytzen, Rebekka
Venue
JAMA Cardiology
Published
2018-09-01
Volume
3
Issue
9
Pages
829
ISSN
2380-6583 (ISSN-L)
OpenAlex
W2884472067
Cited by
169
References
35
Citations by year
2026: 16 · 2025: 26 · 2024: 31 · 2023: 22 · 2022: 18 · 2021: 24 · 2020: 17 · 2019: 14 · 2018: 1
FWCI
10.91
Type
journal article
Language
en
Open access
Licence
cc-by
Locations
  • JAMA Cardiology (publishedVersion, cc-by)
  • Europe PMC (PubMed Central) (publishedVersion)
  • PubMed Central (publishedVersion)
  • PubMed (publishedVersion)
  • VBN Forskningsportal (Aalborg Universitet) (submittedVersion, other-oa)
  • Scopus (Elsevier) (submittedVersion, other-oa)
  • Research at the University of Copenhagen (University of Copenhagen) (submittedVersion)
Funders
Rigshospitalet
Topics
  • Health Sciences › Medicine › Epidemiology › Congenital Heart Disease Studies
  • Health Sciences › Medicine › Surgery › Congenital Anomalies and Fetal Surgery
  • Health Sciences › Medicine › Pediatrics, Perinatology and Child Health › Prenatal Screening and Diagnostics
Keywords
major congenital heart disease, incidence, prenatal screening, abortion, fetal diagnosis, detection rate, temporal trends, single ventricle, transposition of the great arteries, interrupted aortic arch, atrioventricular septal defect, tetralogy of Fallot, Denmark, live birth, fetal death, sex differences, prenatal diagnosis of congenital heart disease
MeSH terms
Abortion, Induced, Adolescent, Child, Denmark, Female, Heart Defects, Congenital, Humans, Pregnancy, Prenatal Diagnosis, Retrospective Studies, Incidence, Early Diagnosis

About these checks

Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.

Statistics and arithmetic are recomputed, not judged.

Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.

What counts as a claim ›How we measure confidence ›How we check this ›

Are you an author, or do you see a problem with this page? Request a review