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Ebook ASE''s Comprehensive echocardiography textbook (2nd edition): Part 2

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Ebook ASE''s Comprehensive echocardiography textbook (2nd edition): Part 2

SectionXV Aortic Stenosis94Aortic Stenosis MorphologySteven A. Goldstein, MDCONGENITAL AORTIC STENOSISBicuspid Aortic ValveCongenital aortic valve mal

Ebook ASE''s Comprehensive echocardiography textbook (2nd edition): Part 2lformation reflects a phenotypic continuum of unicuspid valve (severe form). bicuspid valve (moderate form I. tricuspid valve (normal, but may be abno

rmal}, and the rare quadncuspid forms Bicuspid aortic valves (BAY’S) are the result of abnormal cusp formation during the complex developmental proces Ebook ASE''s Comprehensive echocardiography textbook (2nd edition): Part 2

s In most cases, adjacent cusps fail to separate, resulting in one larger conjoined cusp and a smaller one Therefore. BAV (or bicomnussural aortic val

Ebook ASE''s Comprehensive echocardiography textbook (2nd edition): Part 2

ve) has partial or complete fiision of two of die aortic valve leaflets, with or without a central raphe, resulting in partial or complete absence of

SectionXV Aortic Stenosis94Aortic Stenosis MorphologySteven A. Goldstein, MDCONGENITAL AORTIC STENOSISBicuspid Aortic ValveCongenital aortic valve mal

Ebook ASE''s Comprehensive echocardiography textbook (2nd edition): Part 2 common congenital heart defect. Information on the prevalence of BAV comes primarily from pathology centers.1- Valvular aortic stenosis (AS), a chron

ic progressive disease, usually develops over decades Box 94 1 lists the most common etiologies of valvular AS. as illustrated in Figure 94 1 The majo Ebook ASE''s Comprehensive echocardiography textbook (2nd edition): Part 2

rity of cases of AS are acquired and result from degenerative (calcific) changes in an anatomically normal trileaflet aortic valve that becomes gradua

Ebook ASE''s Comprehensive echocardiography textbook (2nd edition): Part 2

lly dysfunctional over time. Congenitally abnormal valves may be stenotic at birth but usually become dysfunctional during early adolescence or early

SectionXV Aortic Stenosis94Aortic Stenosis MorphologySteven A. Goldstein, MDCONGENITAL AORTIC STENOSISBicuspid Aortic ValveCongenital aortic valve mal

Ebook ASE''s Comprehensive echocardiography textbook (2nd edition): Part 2 less common than in prior decades and is virtually always accompanied by mitral valve disease. Other forms of nonvalvular left ventricular outflow ob

suuction (e.g.. discrete subvalve AS. hypertiophic cardiomyopathy, and supravalve AS) are discussed in other chapteis.The most reliable estimate of BA Ebook ASE''s Comprehensive echocardiography textbook (2nd edition): Part 2

V prevalence is often considered to be die 1.37% reported by Larson and Edwards? Tile authors have a special expeitise in aortic valve disease and ama

Ebook ASE''s Comprehensive echocardiography textbook (2nd edition): Part 2

ssed 21.417 consecutive autopsies with 293 BAVs. An echocardiographic survey of primary school children demonstrated a BAV in 0.5% of males aiid 0.2%

SectionXV Aortic Stenosis94Aortic Stenosis MorphologySteven A. Goldstein, MDCONGENITAL AORTIC STENOSISBicuspid Aortic ValveCongenital aortic valve mal

Ebook ASE''s Comprehensive echocardiography textbook (2nd edition): Part 294.1 summarizes data on the prevalence of bicuspid valves. Bicuspid aortic valve is seen predominantly in males, with a 2:1 male-to-female ratio.’0-*2

Although BaV may occur in isolation, it may also be associated with other congenital cardiovascular malformations, including coarctation, patent duct Ebook ASE''s Comprehensive echocardiography textbook (2nd edition): Part 2

us arteriosus, supravalve AS. atrial septal defect, ventncular septal defect, sinus of Valsalva aneurysm, and coronary artery anomalies There are also

Ebook ASE''s Comprehensive echocardiography textbook (2nd edition): Part 2

several syndromes in which BAV is a part of left-sided obstructive lesions of left ventricular inflow and outflow obstruction. including Shone syndro

SectionXV Aortic Stenosis94Aortic Stenosis MorphologySteven A. Goldstein, MDCONGENITAL AORTIC STENOSISBicuspid Aortic ValveCongenital aortic valve mal

Ebook ASE''s Comprehensive echocardiography textbook (2nd edition): Part 2ral History of Bicuspid Aortic ValvesAlthough a few patients with BAV may go undetected or without clinical consequences for a lifetime, most will dev

elop complications The most important clinical consequences of BAV are valve stenosis, valve regurgitation, infective endocarditis, and aortic complic Ebook ASE''s Comprehensive echocardiography textbook (2nd edition): Part 2

ations such as dilatation, dissection, and rapture (Box 94.2). Estimates of the prevalence of these complications and outcomes have varied depending o

Ebook ASE''s Comprehensive echocardiography textbook (2nd edition): Part 2

n the era of the study, the cohort selected, and the method used to diagnose BAV (clinical exam vs. cardiac catheterization vs. echocardiography). Sev

SectionXV Aortic Stenosis94Aortic Stenosis MorphologySteven A. Goldstein, MDCONGENITAL AORTIC STENOSISBicuspid Aortic ValveCongenital aortic valve mal

Ebook ASE''s Comprehensive echocardiography textbook (2nd edition): Part 2tion of BAV, occurring in approximately 85% of all BAV cases.1 •18-21 Bicuspid aortic valve accounts for the majority of patients aged 15 to 65 years

with significant AS. The progression of the congenitally deformed valve to AS presumably reflects its propensity for premature fibrosis, stiffening, a Ebook ASE''s Comprehensive echocardiography textbook (2nd edition): Part 2

nd calcium deposition in these structurally abnormal valves.Aortic regurgitation, present in approximately 15% of patients with B AV.1 is usually due

Ebook ASE''s Comprehensive echocardiography textbook (2nd edition): Part 2

to dilation of tire sinotubular junction of the aortic loot, preventing cusp coaptation. It may also be caused bycusp piolapse. fibrotic reu action of

SectionXV Aortic Stenosis94Aortic Stenosis MorphologySteven A. Goldstein, MDCONGENITAL AORTIC STENOSISBicuspid Aortic ValveCongenital aortic valve mal

Ebook ASE''s Comprehensive echocardiography textbook (2nd edition): Part 2tients with a BaV develop stenosis and others regurgitation is not cleai. As mentioned, rarely, patients may not develop hemodynamics consequences. Ro

berts and colleagues reported three congenital BA Vs in nonagenarians who underwent surgery for AS.21 Why some patients with a congenital BAV do not b Ebook ASE''s Comprehensive echocardiography textbook (2nd edition): Part 2

ecome symptomatic until they are in their 90s and why others become symptomatic in early life is also unclear.Echocardiographic Features of Bicuspid A

Ebook ASE''s Comprehensive echocardiography textbook (2nd edition): Part 2

ortic ValvesThe roles of echocardiography in the detection and evaluation are listed in Box 94.3. Tire diagnosis of a BAV can usually be made by trans

SectionXV Aortic Stenosis94Aortic Stenosis MorphologySteven A. Goldstein, MDCONGENITAL AORTIC STENOSISBicuspid Aortic ValveCongenital aortic valve mal

Ebook ASE''s Comprehensive echocardiography textbook (2nd edition): Part 2rted for detecting BAV.22 -24 Tile most reliable and useful views are tile parasternal short-axis and long-axis views. The echocardiographic features

and then respective views are summarized in Box 94.3. Tire parasternal short-axis view (SAX) is extremely useful to examine tire number and position o Ebook ASE''s Comprehensive echocardiography textbook (2nd edition): Part 2

f the commissures, the opening pattern. the presence of a raphe, and the leaflet mobility. In contrast to the normal tricuspid aortic valve (TAV), whi

Ebook ASE''s Comprehensive echocardiography textbook (2nd edition): Part 2

ch opens m a triangular fashion with straightening of the leaflets (see Fig. 94.1; Fig 94.2. A), the BAV opens in an elliptical ("fish-mouth" or "foot

SectionXV Aortic Stenosis94Aortic Stenosis MorphologySteven A. Goldstein, MDCONGENITAL AORTIC STENOSISBicuspid Aortic ValveCongenital aortic valve mal

Ebook ASE''s Comprehensive echocardiography textbook (2nd edition): Part 2re the cusps failed to separate :0‘ The raphe is usually distinct and generally extends from389390SECTION XV Acrtc StenoseBox 94.1 Aortic Stenosis: Et

iology1.Congenital (unicuspid. bicuspid. Qjadnoicpidl2.Degenerative (sclerosis of previously normal valve)3.Rheumaticthe free m.irguv$ to the base of Ebook ASE''s Comprehensive echocardiography textbook (2nd edition): Part 2

the leaflet. Calcification commonly occurs first along this raphe, ultimately hindering the motion of the conjoined cusp Rarely, the leaflets are symm

Ebook ASE''s Comprehensive echocardiography textbook (2nd edition): Part 2

etric and there is no raphe—a "pure" bicuspid valve. Note that a false-negative diagnosis may occur when the raphe gives the appearance of a third coa

SectionXV Aortic Stenosis94Aortic Stenosis MorphologySteven A. Goldstein, MDCONGENITAL AORTIC STENOSISBicuspid Aortic ValveCongenital aortic valve mal

Ebook ASE''s Comprehensive echocardiography textbook (2nd edition): Part 2clock (see Figs 94 1 and 94 .2,8). When the commissures are deviated from those clock-face position, one should suspect a BAV and evaluate carefully.

An additional short-axis feature is a vanable degree of leaflet redundancy. In patients with very little redundancy of the leaflet margins, the develo Ebook ASE''s Comprehensive echocardiography textbook (2nd edition): Part 2

pment of stenosis is likely, whereas a significantly redundant leaflet with associated prolapse is more likely to lead to regurgitationThe morphologic

Ebook ASE''s Comprehensive echocardiography textbook (2nd edition): Part 2

patterns of BAV vary according to which commissures have fused, and a number of classifications have been devised that pertain to the orientation of

SectionXV Aortic Stenosis94Aortic Stenosis MorphologySteven A. Goldstein, MDCONGENITAL AORTIC STENOSISBicuspid Aortic ValveCongenital aortic valve mal

Ebook ASE''s Comprehensive echocardiography textbook (2nd edition): Part 2randenburg and colleagues.-- the posterior commissure was located at 4 or 5 o'clock and the anterior commissure was located at 9 or 10 o'clock when th

e valve is viewed in a parasternal slioit-axis view. The second most frequent type, fusion of tire right and noncoronaiy cusps, has been linked to aor Ebook ASE''s Comprehensive echocardiography textbook (2nd edition): Part 2

tic arch involvement50-55 and may also be related to an increased risk of AS and regurgitation compared with the other anatomic types.;' The least com

Ebook ASE''s Comprehensive echocardiography textbook (2nd edition): Part 2

mon type is fusion of the left and noDCOionary cusps? ‘ Miclreleua and colleagues similarly classified BA Vs as typical (right-left coronary cusp fusi

SectionXV Aortic Stenosis94Aortic Stenosis MorphologySteven A. Goldstein, MDCONGENITAL AORTIC STENOSISBicuspid Aortic ValveCongenital aortic valve mal

Ebook ASE''s Comprehensive echocardiography textbook (2nd edition): Part 2usion) if tire commissures were at 1 and 7 o'clock or 12 and 6 o'clock.19TABLE 94.1 Prevacoce of Bicuspid Aortic Vafces (8AV)AuthorYearBAV PrevalenceM

ethodReferenceWauchope192899960.5Autopsy2Gross193750000.56Autopsy3Larsen aid1964214171.37Autopsy4EdwardsDana et al196888030.59Autcpsy5Pai-peru19992000 Ebook ASE''s Comprehensive echocardiography textbook (2nd edition): Part 2

0.65Autopsy6etalBasso el á2W4»170.52O-echo8Mstnetal2005209460.82O-ecbo9

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