Source:

Sillesen
A-S
,
Vøgg
O
,
Pihl
C
, et al
.
Prevalence of bicuspid aortic valve and associated aortopathy in newborns in Copenhagen, Denmark
.
JAMA
.
2021
;
325
(
6
):
561
-
567
. doi:
https://doi.org/10.1001/jama.2020.27205

Investigators from Copenhagen, Denmark, conducted a cross-sectional study to assess the prevalence of bicuspid aortic valve (BAV) among newborns. Pregnant mothers in the catchment area of the 3 largest maternity wards in Copenhagen were enrolled prenatally from 2016–2018. Maternal pregnancy, delivery, and socio-demographic data were obtained throughout the study. All newborns received transthoracic echocardiography (TTE) within 60 days of birth.

The primary outcome was BAV as determined by TTE. BAV was defined as an aortic valve with a partial or complete obliteration of the commissure between 2 adjacent cusps, with or without a raphe. BAV was further characterized by subtype to reflect the number and spatial orientation of the raphes, with types 0, 1, and 2 indicating the number of raphes. Secondary outcomes included other measurements of structural and functional parameters of the left ventricle, main pulmonary artery, aortic valve, and aorta, including dimensions for the sinus of Valsalva and the tubular ascending aorta (using z scores).

The investigators used descriptive statistics to assess the characteristics of newborns with BAV and the prevalence of BAV. Investigators also matched all newborns with BAV with newborns who had a tricuspid aortic valve (non-BAV group) in order to compare outcomes between the 2 groups. Newborns with BAV were matched 1:2 with non-BAV newborns on gender, singleton pregnancy, gestational age, weight, and age at time of TTE.

There were 25,556 newborns included in analysis. BAV was identified in 196 newborns for a prevalence of 0.77% (95% CI, 0.67%, 0.88%). The most frequent BAV subtype identified was type 1, with the raphe positioned between the right and left coronary sinus. Newborns with BAV were more likely to be male than those without BAV (67.9% and 51.5%, respectively).

Compared to the matched non-BAV group, a higher proportion of newborns with BAV had aortic regurgitation (14.7% vs 1.3%; P <0.001), coarctation of the aorta (1.5% vs 0.0%; P = 0.01), and larger main pulmonary artery dimensions. In addition, newborns with (vs without) BAV had significantly larger sinuses of Valsalva and tubular ascending aortic dimensions, with 31.6% of newborns with BAV having significant (z scores of ≥3) aortic dilatation. There were no differences in left ventricular end-systolic and end-diastolic ejection fractions between the BAV and non-BAV groups.

The investigators conclude that aortopathy is common among newborns diagnosed with BAV.

Dr Spar has disclosed no financial relationship relevant to this commentary. This commentary does not contain a discussion of an unapproved/investigative use of a commercial product/device.

BAV is the most common congenital heart disease, affecting approximately 0.5% to 2.0% of the population, with a 3:1 male predominance. Normally, the aortic valve is trileaflet, with right, left, and non-coronary cusps. BAV describes malformations including the presence of 2 aortic valve leaflets and when there are 3...

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