scholarly journals Undiagnosed Bicuspid Aortic Valve: A Silent Danger

2020 ◽  
Vol 11 (2) ◽  
Author(s):  
Ngow HA ◽  
Wan Khairina WMN
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Congenital Abnormality ◽  
Severe Disease ◽  
Cardiac Risk ◽  
Late Presentation ◽  
Early Embryogenesis ◽  
Aortic Cusp ◽  
Valvular Stenosis ◽  
Aortic Incompetence

The normal aortic valve has three leaflets, which are almost equal in size. A bicuspid aortic valve developed as a result of abnormal aortic cusp formation during early embryogenesis. Complications such as valvular stenosis or incompetence are well known. Delayed in identifying the congenital abnormality or the complications may cause morbidity and mortality. We report two cases of congestive cardiac failure as the results of undiagnosed bicuspid aortic valve with severe aortic incompetence. Both young men in their productive age without cardiac risk factors, were unfortunate to present too late. Although one of them was lucky to survive the ordeal, most late presentation ends up gravely. Aortic valve replacement can be offered if early detection is made. Those with severe disease at diagnosis may require a heart transplant.

scholarly journals Bicuspid Aortic Valve Disease and Ascending Aortic Aneurysms: Gaps in Knowledge

2012 ◽  
Vol 2012 ◽  
pp. 1-16 ◽  
Author(s):  
Katie L. Losenno ◽  
Robert L. Goodman ◽  
Michael W. A. Chu
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Aortic Valve Disease ◽  
Aortic Aneurysms ◽  
Valve Disease ◽  
Cardiac Anomaly ◽  
Aortic Dilatation ◽  
Congenital Cardiac Anomaly ◽  
Valvular Stenosis ◽  
Ongoing Surveillance

The bicuspid aortic valve is the most common congenital cardiac anomaly in developed nations. The abnormal bicuspid morphology of the aortic valve results in valvular dysfunction and subsequent hemodynamic derangements. However, the clinical presentation of bicuspid aortic valve disease remains quite heterogeneous with patients presenting from infancy to late adulthood with variable degrees of valvular stenosis and insufficiency and associated abnormalities including aortic coarctation, hypoplastic left heart structures, and ascending aortic dilatation. Emerging evidence suggests that the heterogeneous presentation of bicuspid aortic valve phenotypes may be a more complex matter related to congenital, genetic, and/or connective tissue abnormalities. Optimal management of patients with BAV disease and associated ascending aortic aneurysms often requires a thoughtful approach, carefully assessing various risk factors of the aortic valve and the aorta and discerning individual indications for ongoing surveillance, medical management, and operative intervention. We review current concepts of anatomic classification, pathophysiology, natural history, and clinical management of bicuspid aortic valve disease with associated ascending aortic aneurysms.

Bicuspid aortic valve disease and competitive sports: key considerations and challenges

2019 ◽  
pp. 233-238
Author(s):  
Benjamin S. Wessler ◽  
Natesa G. Pandian
Keyword(s):  
Aortic Valve ◽  
Aortic Stenosis ◽  
Aortic Regurgitation ◽  
Bicuspid Aortic Valve ◽  
Aortic Dilation ◽  
Valvular Stenosis ◽  
Valvular Function ◽  
A Minor ◽  
Valve Lesion

Bicuspid aortic valve (BAV) is a common congenital disorder. It could simply be a minor anatomic abnormality or be associated with progressive aortic stenosis, aortic regurgitation, and aortic dilation. If an athlete is recognized to have a BAV, questions arise with regard to whether they can pursue their selected sports, particularly elite athletic activity, and what type of follow-up examinations are necessary and how often should be done. Valvular disorders such as the degree of aortic stenosis and aortic regurgitation, aortic size, and coexisting disorders are also influencing factors. The absence of robust controlled studies, which are difficult to perform, make decision-making difficult, although recommendations by expert panels provide some guidance. The general consensus is that athletes with BAV with normal valvular function and no aortic dilation can participate in all athletic activities. Those with mild aortic dilation should undergo annual screening, some more frequently than others. Those with moderate or severe valvular stenosis or regurgitation should be managed based on the haemodynamic impact of the valve lesion. Athletes with coexisting lesions or syndromes should be evaluated comprehensively. The overall recommendation to an individual athlete should incorporate many factors and employ a multidisciplinary approach.

Dynamically-Loaded 3-D Model to Study the ECM Organization of Aortic Smooth Muscle Cells in Aneurysmal Patients With Bicuspid Aortic Valve

2011 ◽  
Author(s):  
Wei He ◽  
Julie Phillippi ◽  
Christopher E. Miller ◽  
David A. Vorp ◽  
Thomas G. Gleason
Keyword(s):  
Smooth Muscle ◽  
Aortic Valve ◽  
Smooth Muscle Cells ◽  
Bicuspid Aortic Valve ◽  
Elective Surgery ◽  
Muscle Cells ◽  
The United States ◽  
Aortic Aneurysms ◽  
Aortic Replacement ◽  
Valvular Stenosis

Rupture of aortic aneurysms and dissections are the fifteenth leading cause of a death in the United States [1]. Over 40% of patients undergoing elective surgery for ascending aortic replacement due to thoracic aortic aneurysm (TAA) have a congenital defect in the aortic valve know as bicuspid aortic valve (BAV) [2]. BAV patients have uniformly larger diameter aortic roots and ascending aortas compared to age- and sex-matched controls [3] and abnormal elasticity even in the absence of valvular stenosis or aneurysm [4] and this greatly increases the risk of aortic dissection and sudden death [5]. The cause of TAA is uncertain, but recent studies suggest that oxidative stress may play a role in the pathogenesis of TAAs by degrading the extracellular matrix (ECM). We identified that BAV smooth muscle cells (SMCs) lack sufficient resistance to reactive oxygen species to maintain ECM homeostasis [6, 7].

scholarly journals Perforation of Bicuspid Aortic Valve mimicking Trileaflet Aortic Valve on Transesophageal Echocardiography

2014 ◽  
Vol 2 (2) ◽  
pp. 77-79
Author(s):  
Soumendu Pal
Keyword(s):  
Differential Diagnosis ◽  
Aortic Valve ◽  
Transesophageal Echocardiography ◽  
Aortic Regurgitation ◽  
Bicuspid Aortic Valve ◽  
Surgical Repair ◽  
Aortic Incompetence

ABSTRACT Endocarditis of bicuspid aortic valve (BAV) can lead to valve perforation and result in severe aortic incompetence. Perforation of valve cusps should be considered in the differential diagnosis of BAV patients presenting with AR, and the condition should not be mistaken as a regurgitant trileaflet AV on transesophageal echocardiography (TEE). Accurate preoperative characterization of the mechanism of aortic regurgitation by TEE is important for determining the type of surgical repair. How to cite this article Pal S. Perforation of Bicuspid Aortic Valve mimicking Trileaflet Aortic Valve on Transesophageal Echocardiography. J Perioper Echocardiogr 2014;2(2):77-79.

Increased rotational flow in the proximal aortic arch is associated with its dilation in bicuspid aortic valve disease

2019 ◽  
Vol 20 (12) ◽  
pp. 1407-1417 ◽  
Author(s):  
Lydia Dux-Santoy ◽  
Andrea Guala ◽  
Gisela Teixidó-Turà ◽  
Aroa Ruiz-Muñoz ◽  
Giuliana Maldonado ◽  
...  
Keyword(s):  
Magnetic Resonance ◽  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Flow Dynamics ◽  
Rotational Flow ◽  
4D Flow ◽  
Flow Parameters ◽  
Valvular Stenosis ◽  
Male Sex ◽  
The Right

Abstract Aims Aortic dilation in bicuspid aortic valve (BAV) might extend to the proximal arch. Arch flow dynamics and their relationship with this segment dilation are still unexplored. Using 4D-flow cardiovascular magnetic resonance, we analysed flow dynamics in the arch for each BAV morphotype and their association with this segment dilation. Methods and results One hundred and eleven BAV patients (aortic diameters ≤55 mm, non-severe valvular disease), 21 age-matched tricuspid aortic valve (TAV) patients with dilated arch and 24 healthy volunteers (HV) underwent 4D-flow. BAV were classified per fusion morphotype: 75% right-left (RL-BAV), and per arch dilation: 57% dilated, mainly affecting the right-noncoronary (RN) BAV (86% dilated vs. 47% in RL-BAV). Peak velocity, jet angle, normalized displacement, in-plane rotational flow (IRF), wall shear stress, and systolic flow reversal ratio (SFRR) were calculated along the thoracic aorta. ANCOVA and multivariate linear regression analyses were used to identify correlates of arch dilation. BAV had higher rotational flow and eccentricity than TAV in the proximal arch. Dilated compared with non-dilated BAV had higher IRF being more pronounced in the RN-morphotype. RN-BAV, IRF, and SFRR were independently associated with arch dilation. Aortic stenosis and male sex were independently associated with arch dilation in RL-BAV. Flow parameters associated with dilation converged to the values found in HV in the distal arch. Conclusion Increased rotational flow could explain dilation of the proximal arch in RN-BAV and in RL-BAV patients of male sex and with valvular stenosis. These patients may benefit from a closer follow-up with cardiac magnetic resonance or computed tomography.

scholarly journals Double Orifice Mitral Valve and Bicuspid Aortic Valve: Pieces of the Same Single Puzzle?

2015 ◽  
Vol 2015 ◽  
pp. 1-3
Author(s):  
Faysal Şaylık ◽  
Ferit Onur Mutluer ◽  
Aydın Tosu ◽  
Murat Selçuk
Keyword(s):  
Mitral Valve ◽  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Congenital Abnormality ◽  
Congenital Abnormalities ◽  
Pathophysiological Mechanism ◽  
Review Of The Literature ◽  
Congenital Lesions ◽  
Rare Congenital Abnormality ◽  
Double Orifice

Double orifice mitral valve is a very rare congenital abnormality. Well known associations of this pathology with other congenital lesions point to a complex and central pathophysiological mechanism leading to a sequence of pathologies. These associations have long been realized and arbitrarily defined as Shone complex. We would like to present a 21-year-old patient with double orifice mitral valve associated with bicuspid aortic valve, with a brief review of the literature on possible central mechanisms leading to different subsets of congenital abnormalities involving these two.

scholarly journals Coarctation Stenting in a Rare Case with Congenitally Corrected Transposition of the Great Arteries and the Bicuspid Aortic Valve

2020 ◽  
Author(s):  
Zahra Khajali ◽  
Majid Maleki ◽  
Bahram Mohebbi ◽  
Maryam Aliramezany
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Congenital Abnormality ◽  
Heart Defects ◽  
Coarctation Of The Aorta ◽  
Live Births ◽  
Rare Congenital Abnormality ◽  
Congenitally Corrected Transposition ◽  
Corrected Transposition

Congenitally corrected transposition of the great arteries (cc-TGA) is a rare congenital abnormality that occurs in 1 per 33000 live births. This abnormality comprises nearly 0.05% of all congenital heart defects, with at least 90% of cc-TGA patients having associated cardiac defects; some of these associated defects are, however, very rare. In this case report, we describe a 22-year-old man who referred to our hospital for the evaluation of hypertension and cardiac murmurs. Via echocardiography and catheterization, the patient was finally diagnosed with cc-TGA, bicuspid aortic valve, and coarctation of the aorta. He underwent successful percutaneous transarterial coarctoplasty without any complications at early and 6 months’ follow-up visits.

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