The Bicuspid Aortic Valve

Lewin, Mark B.; Otto, Catherine M

doi:10.1161/01.cir.0000157137.59691.0b

Cited by 140 publications

(56 citation statements)

References 23 publications

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“…69 Although usually uncomplicated in early life, bicuspid AV frequently eventuates in aortic stenosis or regurgitation, infective endocarditis, and aortic dilation and/or dissection later in life. 70 Bicuspid AVs underlie Ͼ67% of aortic stenosis in children and Ϸ50% in adults. 71 Recent studies have confirmed previous reports of familial clustering of bicuspid AV, left ventricular outflow tract obstruction malformations, and other cardiovascular malformations, suggesting that these common valvular malformations are genetic defects leading to faulty valvulogenesis and/or cardiogenesis.…”

Section: Bicuspid Avmentioning

confidence: 99%

Evolving Concepts of Cardiac Valve Dynamics

2008

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Abstract-Considerable progress has been made in recent years toward elucidating a conceptual framework that integrates the dynamic functional structure, mechanical properties, and pathobiological behavior of the cardiac valves. This communication reviews the evolving paradigm of a continuum of heart valve structure, function, and pathobiology and explores its implications. Specifically, we discuss (1) the interactions of valve biology and biomechanics (eg, correlations of function with structure at the cell, tissue, and organ levels and mechanical considerations, development, endothelial cell and interstitial cell biology, extracellular matrix biology, homeostasis, and adaptation to environmental change); (2) Key Words: aortic valve Ⅲ mitral valve Ⅲ pathology Ⅲ prosthesis Ⅲ tissue engineering I mportant conceptual advances, new data, and evolution in our understanding and application of the principles underlying the dynamic functional, biological, and mechanical behavior of the cardiac valves have occurred in recent years. Research in heart valve biology and disease has been enabled by the availability of cultures of heart valve cells, computational methodology, the design and use of in vitro and in vivo experimental systems that model elements of valve biological and pathobiological activity, and targeted study of normally functioning and pathological native and substitute human valves. Collectively, these developments have facilitated a growing understanding of how shortand long-term biomechanical valve function at the organ level relates to tissue and cell structure and normal valve function, elucidated the pathological anatomy and mechanisms of valvular dysfunction, fostered improvements in tissue heart valve substitutes and surgical repairs, and informed innovative approaches to heart valve tissue engineering and regeneration. [1][2][3][4] In this communication, we highlight key recent insights into heart valve function and dysfunction and their implications for the prevention, diagnosis, and treatment of clinical heart valve disease, currently and in the future. The heart valves are tissue structures whose motions are driven by mechanical forces exerted by the surrounding blood and heart. The ability of the valves to permit unobstructed forward flow depends on the mobility, pliability, and structural integrity of their leaflets (in the TV and MV) and cusps (in the PV and AV).The individual AV cusps attach to the aortic wall in a crescentic (or semilunar) fashion, ascending to the commissures (where adjacent cusps come together at the aorta) and descending to the basal attachment of each cusp to the aortic wall. Behind the cusps are dilated pockets in the aortic root, called sinuses of Valsalva, which bulge with each ejection of blood. The AV cusps and their respective sinuses are named for their relationship to the coronary artery ostia that arise from them, normally a left, a right, and a noncoronary (cusp and associated sinus). In the middle of the free edge of each cusp on the ventricular surface is a ...

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Section: Bicuspid Avmentioning

confidence: 99%

Evolving Concepts of Cardiac Valve Dynamics

2008

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“…Performing such examinations prior to surgery may have been useful for our patient. Although UAV is rare at 0.02% (1,2) in the general population, it is observed in 4-5% of patients undergoing surgery for AS (17), and thus, it may not necessarily be rare in these patients. If TTE or TEE was performed upon the suspicion of UAV, then a preoperative diagnosis could have been possible.…”

Section: Discussionmentioning

confidence: 99%

“…Unicuspid aortic valve (UAV) is a very rare congenital disease, with an incidence of 0.02% (1,2). This condition is divided into acommissural and unicommissural types; the former exhibits severe valve dysfunction and requires treatment from the neonatal period through childhood (3,4).…”

Section: Introductionmentioning

confidence: 99%

An Adult Case of Unicommissural Unicuspid Aortic Valve Diagnosed Based on the Intraoperative Findings

et al. 2016

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We herein report an adult case of unicommissural unicuspid aortic valve (UAV). A 59-year-old man, who was noted to have a cardiac murmur at 31 years of age, was admitted to our hospital due to acute heart failure. Severe calcification in the aortic valve with severe low-flow/low-gradient aortic stenosis and moderate aortic regurgitation was observed and thought to be the cause of heart failure, however, the etiology of aortic valve dysfunction was not clear. Aortic valve replacement was subsequently performed, and unicommissural UAV was diagnosed according to the intraoperative findings. UAV is very rare congenital aortic valve disease which is rarely diagnosed preoperatively.

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“…While the more common bicuspid aortic valve has an incidence of 0.9-1.36% among the general population compared to unicuspid aortic valve which is about 50 fold less common with an incidence of 0.02% (Lewin, 2005;Mookadam, 2010a). Unicuspid aortic valve is more common in males with gender ratio of 4:1 in males:females (Mookadam, 2010a).…”

Section: Epidemiologymentioning

confidence: 99%