Bicuspid Aortic Valve: Unlocking the Morphogenetic Puzzle

Longobardo, Luca; Jain, Renuka; Carerj, Scipione; Zito, Concetta; Khandheria, Bijoy K.

doi:10.1016/j.amjmed.2016.03.009

Cited by 27 publications

(30 citation statements)

References 56 publications

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“…However, recently, it has been described that regardless of whether or not there is dysfunctional BAV by echocardiogram (23) both the hemodynamics and aortic wall stress are affected. This supports the hemodynamic etiopathogenic mechanism, but also substantiates that even an apparently normal BAV in echocardiogram is, in fact, intrinsically dysfunctional (2).…”

Section: Aortic Dilatation: Intrinsic or Induced Development?mentioning

confidence: 51%

“…Anyway the most common BAV phenotype and typical pattern is the fusion of the right and left cusps, resulting in an anteroposterior leaflet orientation, followed by the fusion of the right and non-coronary cusps (2,10). Otherwise, pattern of aortic dilatation is also diverse.…”

Section: Different Morphotypes In Bav Disease: From Valve Morphology mentioning

confidence: 99%

“…The main classifications of BAV morphology have been recently resumed by Longobardo et al (2). Since Roberts, that based its classification in a study of autopsy cases, to Kang using multidetector computed tomography, different systems have been proposed, like the one by Brandenburg et al based on commissures and raphe position according to 2-dimensional echocardiography (3,6,7).…”

Section: Different Morphotypes In Bav Disease: From Valve Morphology mentioning

confidence: 99%

“…Thus, the risk of segmental aneurysm formation may be determined depending on the direction of the abnormal produced flow jet (2). Moreover, this abnormal flow may be determined by the fusion BAV phenotype, being right-left cusp fusion associated with root dilatation and right-non cusp fusion with tubular ascending and arch dilatation (2,24).…”

Section: Aortic Dilatation: Intrinsic or Induced Development?mentioning

confidence: 99%

“…Different phenotypes related to valve morphology have been described, also distinct genetics alterations are widely accepted and the pathogenesis of associated aortic dilatation is not completely clear (2). Echocardiography, mainly transthoracic is the main diagnostic tool for BAV but transesophageal and 3-dimensional echocardiography are sometimes necessary to confirm diagnosis.…”

Section: Introductionmentioning

confidence: 99%

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Bicuspid aortic valve syndrome: a multidisciplinary approach for a complex entity

et al. 2017

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Bicuspid aortic valve (BAV) or bicuspid aortopathy is the most common congenital heart disease.It can be clinically silent and it is often identified as an incidental finding in otherwise healthy, asymptomatic patients. However, it can be dysfunctioning at birth, even requiring neonatal intervention, or, in time, lead to aortic stenosis, aortic insufficiency, and endocarditis, and also be associated with aortic aneurysm and aortic dissection. Given its prevalence and significant complications, it is estimated that BAV is responsible for more deaths and morbidity than the combined effects of all the other congenital heart defects. Pathology of BAV is still not well known and many questions are unresolved. In this manuscript we review some aspects on bicuspid aortopathy, a heterogeneous and frequent disease in which like some authors have previously described, complex gene environment are present. Further investigations and, what is more, multidisciplinary teams are needed to improve our knowledge on this really fascinating disease.

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Section: Aortic Dilatation: Intrinsic or Induced Development?mentioning

confidence: 51%

Section: Different Morphotypes In Bav Disease: From Valve Morphology mentioning

confidence: 99%

Section: Different Morphotypes In Bav Disease: From Valve Morphology mentioning

confidence: 99%

Section: Aortic Dilatation: Intrinsic or Induced Development?mentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Bicuspid aortic valve syndrome: a multidisciplinary approach for a complex entity

et al. 2017

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Bicuspid Aortopathy

2017

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Purpose: Our study aimed to investigate the potential pathogenetic theories of different phenotype prevalence in bicuspid aortopathy. Methods: A total of 407 bicuspid aortic valve (BAV) patients with aortic dilation were retrospectively reviewed. Association was determined between aortic valve lesion types and aortic configurations to confirm the homogeneous BAV subsets, and then, dominance analysis was used to evaluate the relative importance of two components of aortic valve lesion (BAV phenotype and valvular dysfunction) that associated with aortic configurations in each subgroup. Results: Dominance analysis showed that Type-1 LR was the dominant contributor (79.0% and 79.6%) associated with the higher prevalence of the dilation of aortic root (AoR) and ascending aorta (AAo) in BAV patients with Type-1 LR and aortic regurgitation (AR) or aortic stenosis (AS) + AR. However, AS was the main contributor (60.0%) associated with the raised incidence of the dilation of AAo and proximal aortic arch (PArc) in Type-0 LAT and AS. Conclusions: Different dominant pathogenetic theory determined the phenotype of BAV aortopathy. In patients of Type-1 LR with AR, inherent disposition is mainly responsible for the higher frequency of AoR dilation. Valve-related hemodynamics determined greater prevalence of the dilation of AAo and PArc in patients of Type-0 LAT with AS.

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The rare combination of pathologies: Bicuspid aortic valve combined with left atrial myxoma

Wang,

Yu,

2024

Sonography

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Bicuspid Aortic Valve: Unlocking the Morphogenetic Puzzle

Cited by 27 publications

References 56 publications

Bicuspid aortic valve syndrome: a multidisciplinary approach for a complex entity

Bicuspid aortic valve syndrome: a multidisciplinary approach for a complex entity

Bicuspid Aortopathy

The rare combination of pathologies: Bicuspid aortic valve combined with left atrial myxoma

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