Prevalence of Bicuspid Aortic Valve in Newborns

Quick Takes

  • The prevalence of bicuspid aortic valve (BAV) in this large, population-based cohort was 0.77%.
  • Aortopathy was common in neonates with BAV, suggesting that it also represents a fetal malformation.

Study Questions:

What is the prevalence of bicuspid aortic valve (BAV) and BAV subtypes and associated aortopathy in a large, population-based cohort of newborns?


The Copenhagen Baby Heart Study was a cross-sectional, population-based study open to all newborns born in Copenhagen, Denmark, 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.


A total of 25,556 newborns (51% male; mean age, 12 days) underwent echocardiography. BAV was diagnosed in 196 newborns (prevalence, 0.77%), with a male-female ratio of 2.1:1. The most common type was type 1 (1 raphe with the raphe positioned between the right and left coronary sinuses (75%), while the second most common type was also type 1, with the raphe between the right coronary and noncoronary sinuses (13.8%). Aortic regurgitation was more prevalent in newborns with BAV (n = 29 [14.7%]) than those without BAV (1.3%). Newborns with BAV had higher flow velocities across the valve, and larger aortic root and tubular ascending aortic diameters than those without BAV. Aortopathy was seen in 65 newborns (33.2%) with BAV (62 with aortic z score ≥3; 3 with coarctation).


The prevalence of BAV was 0.77%. Aortopathy was common in newborns with BAV, suggesting that it also represents a fetal malformation.


This large, population-based study demonstrated the prevalence of BAV to be 0.77%. Importantly, the study showed a high rate of aortopathy in neonates, which supports the theory of underlying disease of the aorta as an important etiology of aortic dilatation. As families of some infants with significant heart disease associated with BAV (severe aortic valve dysfunction, coarctation of the aorta, left and right ventricular size discrepancy) may have not chosen to participate in the study, the reported prevalence of BAV may be a slight underestimate.

Clinical Topics: Congenital Heart Disease and Pediatric Cardiology, Noninvasive Imaging, Valvular Heart Disease, Vascular Medicine, Congenital Heart Disease, CHD and Pediatrics and Imaging, Echocardiography/Ultrasound

Keywords: Aortic Diseases, Aortic Valve Insufficiency, Dilatation, Echocardiography, Gestational Age, Heart Defects, Congenital, Heart Valve Diseases, Infant, Newborn, Pediatrics, Pregnancy, Twin, Tricuspid Valve

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