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Prevalence of Bicuspid Aortic Valve and Associated Aortopathy in Newborns in Copenhagen, Denmark

Educational Objective
To learn about bicuspid aortic valve and aortopathy.
1 Credit CME
Key Points

Question  What is the prevalence of bicuspid aortic valve among newborns and what is the extent of associated disease of the aorta?

Findings  This cross-sectional study included 25 556 newborns in Denmark between 2016 and 2018 who underwent transthoracic echocardiography. The prevalence of bicuspid aortic valve was 0.77%; of these, aortopathy was present in 33%.

Meaning  Among newborns in Copenhagen, the prevalence of bicuspid aortic valve was 0.77% and associated aortic disease was common, suggesting that it was a fetal malformation.


Importance  The prevalence and characteristics of bicuspid aortic valve (BAV) are mainly reported from selected cohorts. BAV is associated with aortopathy, but it is unclear if it represents a fetal developmental defect or is secondary to abnormal valve dynamics.

Objective  To determine the prevalence of BAV and BAV subtypes and to describe the associated aortopathy in a large, population-based cohort of newborns.

Design, Setting, and Participants  The Copenhagen Baby Heart Study was a cross-sectional, population-based study open to all newborns born in Copenhagen between April 1, 2016, and October 31, 2018. Newborns with BAV were matched 1:2 to newborns with a tricuspid aortic valve (non-BAV group) on sex, singleton/twin pregnancy, gestational age, weight, and age at time of examination.

Exposures  Transthoracic echocardiography within 60 days after birth.

Main Outcomes and Measures  Primary outcome was BAV prevalence and types, ie, number of raphes and spatial orientation of raphes or cusps (no raphes), according to the classification system of Sievers and Schmidtke (classified as type 0, 1, or 2, with numbers indicating the number of raphes). Secondary outcome was valve function and BAV-associated aortopathy, defined as aortic diameter z score of 3 or greater or coarctation.

Results  In total, 25 556 newborns (51.7% male; mean age, 12 [SD, 8] days) underwent echocardiography. BAV was diagnosed in 196 newborns (prevalence, 0.77% [95% CI, 0.67%-0.88%]), with male-female ratio 2.1:1. BAV was classified as type 0 in 17 newborns (8.7% [95% CI, 5.5%-13.5%]), type 1 in 178 (90.8% [95% CI, 86.0%-94.1%]) (147 [75.0% {95% CI, 68.5%-80.5%}] right-left coronary raphe, 27 [13.8% {95% CI, 9.6%-19.3%}] right coronary–noncoronary raphe, 4 [2.0% {95% CI, 0.8%-5.1%}] left coronary–noncoronary raphe), and type 2 in 1 (0.5% [95% CI, 0.1%-2.8%]). Aortic regurgitation was more prevalent in newborns with BAV (n = 29 [14.7%]) than in those without BAV (1.3%) (absolute % difference, 13.4% [95% CI, 7.8%-18.9%]; P < .001). Newborns with BAV had higher flow velocities across the valve (0.67 [95% CI, 0.65-0.69] m/s vs 0.61 [95% CI, 0.60-0.62] m/s; mean difference, 0.06 m/s [95% CI, 0-0.1]) and larger aortic root and tubular ascending aortic diameters than those without BAV (10.7 [95% CI, 10.7-10.9] mm vs 10.3 [95% CI, 10.2-10.4] mm; mean difference, 0.43 mm [95% CI, 0.2-0.6 mm] and 9.8 [95% CI, 9.6-10.0] mm vs 9.4 [95% CI, 9.3-9.5] mm; mean difference, 0.46 mm [95% CI, 0.30-0.70], respectively) (P < .001 for all). Aortopathy was seen in 65 newborns (33.2%) with BAV (62 with aortic z score ≥3; 3 with coarctation).

Conclusions and Relevance  Among newborns in Copenhagen, the prevalence of BAV was 0.77%. Aortopathy was common in newborns with BAV, suggesting that it also represents a fetal malformation.

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Article Information

Corresponding Author: Anne-Sophie Sillesen, MD, PhD, Department of Cardiology, Copenhagen University Hospital Herlev, Borgmester Ib Juuls vej 1, 2730 Herlev, Denmark (

Accepted for Publication: January 7, 2021.

Author Contributions: Dr Sillesen had full access to all of the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis. Drs Iversen and Bundgaard shared last authorship.

Concept and design: Sillesen, Axelsson Raja, Sundberg, Zingenberg, Vejlstrup, Iversen, Bundgaard.

Acquisition, analysis, or interpretation of data: Sillesen, Vøgg, Pihl, Axelsson Raja, Vedel, Jørgensen, Vejlstrup, Iversen, Bundgaard.

Drafting of the manuscript: Sillesen, Vejlstrup, Bundgaard.

Critical revision of the manuscript for important intellectual content: Sillesen, Vøgg, Pihl, Axelsson Raja, Sundberg, Vedel, Zingenberg, Jørgensen, Iversen, Bundgaard.

Statistical analysis: Sillesen, Pihl, Iversen, Bundgaard.

Obtained funding: Sillesen, Pihl, Axelsson Raja, Iversen, Bundgaard.

Administrative, technical, or material support: Sillesen, Vøgg, Pihl, Axelsson Raja, Sundberg, Vedel, Vejlstrup, Iversen, Bundgaard.

Supervision: Axelsson Raja, Zingenberg, Jørgensen, Vejlstrup, Iversen, Bundgaard.

Conflict of Interest Disclosures: Dr Axelsson Raja reported receiving funding for research not relevant to the present work from Novo Nordisk, paid to her institution. No other disclosures were reported.

Funding/Support: This work was supported by the Danish Heart Association, the Danish Children’s Heart Foundation, Candy’s Foundation, the Toyota Foundation, and the Herlev-Gentofte Hospital Research Foundation.

Role of Funders/Sponsors: The funders had no role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication.

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