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

Abstract

IMPORTANCEThe 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. RESULTSIn 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

Prenatal cardiac biometry and flow assessment in fetuses with bicuspid aortic valve at 20 weeks' gestation: multicenter cohort study

“…In addition, we found that more than three times as many mothers of fetuses with BAV smoked prior to pregnancy compared with mothers of fetuses with a normal aortic valve, which was also similar to findings of the primary work of CBHS 1 . From oncological research, it has been proposed that smoking alters the Notch signaling pathway, which is essential in embryogenesis and which regulates cell life and cardiovascular development processes 20 .…”

“…As illustrated in Figure 2, the diameters of the aorta and pulmonary artery were generally larger in fetuses with BAV, but all within the general distribution of the values in controls. Although there was a significant difference in the aorta and pulmonary artery diameters between fetuses with BAV and controls, in line with the neonatal findings in this cohort 1 , the statistical significance of the difference was lost following Bonferroni correction of the P-values, in contrast to the neonatal findings. Hence, the Bonferroni correction may have imposed a Type-II error in the present study, which had a smaller sample size than the neonatal study.…”

A Rare Case of Bicuspid Aortic Valve With Recurrent Endocarditis Complicated by an Aortic Root Pseudoaneurysm

“…Bicuspid aortic valve has been described as one of the common cardiac congenital defects. Its prevalence is estimated to be between 0.5% and 2% in the general population [4]. Bicuspid aortic valve is associated with the risk of developing many cardiac complications such as aortic stenosis, aortic dissection, and endocarditis [4].…”

Temporal Trends in Diagnosis, Treatments, and Outcomes in Patients With Bicuspid Aortic Valve

“…Bicuspid aortic valve (BAV) is the most common congenital heart valve disease, and the burden of BAV disease is greater than for other congenital anomalies (1)(2)(3). BAV can present in diverse spectrum and affect valve function from non-dysfunctional to severe aortic stenosis (AS) or severe aortic regurgitation (AR) (4)(5)(6).…”