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Review
. 2022 Jul 20;12(7):1751.
doi: 10.3390/diagnostics12071751.

Bicuspid Aortic Valve in Children and Adolescents: A Comprehensive Review

Gaia Spaziani  1 , Francesca Girolami  1 , Luigi Arcieri  2 , Giovanni Battista Calabri  1 , Giulio Porcedda  1 , Chiara Di Filippo  1 , Francesca Chiara Surace  2 , Marco Pozzi  2 , Silvia Favilli  1
Affiliations
Review

Bicuspid Aortic Valve in Children and Adolescents: A Comprehensive Review

Gaia Spaziani et al. Diagnostics (Basel). .

Abstract

Bicuspid aortic valve (BAV) is the most common congenital heart defect. Prevalence of isolated BAV in the general pediatric population is about 0.8%, but it has been reported to be as high as 85% in patients with aortic coarctation. A genetic basis has been recognized, with great heterogeneity. Standard BAV terminology, recently proposed on the basis of morpho-functional assessment by transthoracic echocardiography, may be applied also to the pediatric population. Apart from neonatal stenotic BAV, progression of valve dysfunction and/or of the associated aortic dilation seems to be slow during pediatric age and complications are reported to be much rarer in comparison with adults. When required, because of severe BAV dysfunction, surgery is most often the therapeutic choice; however, the ideal initial approach to treat severe aortic stenosis in children or adolescents is not completely defined yet, and a percutaneous approach may be considered in selected cases as a palliative option in order to postpone surgery. A comprehensive and tailored evaluation is needed to define the right intervals for cardiologic evaluation, indications for sport activity and the right timing for intervention.

Keywords: bicuspid aortic valve; children; diagnostic imaging; echocardiography; natural history.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
2D Echocardiography parasternal short axis view (systolic frame) at the level of aortic valve shows a bicuspid aortic valve in an 8-year-old patient.
Figure 2
Figure 2
2D Echocardiography parasternal short axis view (systolic frame) at the level of aortic valve shows an unicuspid aortic valve in a 12-year-old patient.
Figure 3
Figure 3
Cardiac magnetic resonance imaging of the left ventricular outflow tract shows a dilation of the ascending aorta in a 16-year-old patient with bicuspid aortic valve.
See this image and copyright information in PMC

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