Aortic Valve Prolapse Echo

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Aortic Valve Prolapse Echo
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Aortic valve prolapse and regurgitation have been found in the Turkish cohort of Gürgün et al. [67] in 5% of patients versus none of unaffected controls, a difference that did not reach statistical significance, similar to other studies [66].
Keith Kleinman MD , in Harriet Lane Handbook , 2021
Clinical characteristics are summarized in Table 7.3 . 3
Grading of heart murmurs: Intensified by states of higher cardiac output (e.g., anemia, anxiety, fever, exercise). 3
Grade II: Murmur softer than heart sounds, but audible
Grade III: Murmur moderately loud, equally loud as heart sounds, not accompanied by a thrill
Grade IV: Murmur louder than heart sounds, associated with a thrill
Grade V: Audible with a stethoscope barely on the chest
Grade VI: Audible with a stethoscope off the chest
Caused by a disturbance of the laminar flow of blood; frequently produced as the diameter of the blood’s pathway decreases and velocity increases.
Present in >80% of children sometime during childhood, most commonly beginning at age 3 to 4 years.
Accentuated in high-output states, especially with fever and anemia.
Normal electrocardiogram (ECG) and radiographic findings.
NOTE: ECG and chest radiograph are not routinely used, nor are they cost-effective screening tools for distinguishing benign from pathologic murmurs.
A murmur is more likely to be pathologic when one or more of the following are present: Symptoms (e.g., chest pain, dyspnea with exertion, syncope with exertion); cyanosis; a systolic murmur that is loud (grade ≥3/6), harsh, pansystolic, or long in duration; diastolic murmur; abnormal heart sounds; presence of a click; abnormally strong or weak pulses. 3,4
Systolic and diastolic heart murmurs ( Box 7.2 ).
Aortic valve prolapse and regurgitation have been found in the Turkish cohort of Gürgün et al. [67] in 5% of patients versus none of unaffected controls, a difference that did not reach statistical significance, similar to other studies [66] . In some cases, aortic valves may have normal morphology, and regurgitation may develop progressively in the context of left ventricle enlargement.
On the other hand, when aortic insufficiency is acute, a marked thinning and redundancy of aortic leaflets, with or without mobile masses and/or free echo space within the annulus and/or ventricular septum assessed by TEE, may be considered pathognomonic of BD [72,73] . However, these findings may resemble infectious endocarditis and even meet the major Duke criteria. Specifically, aortic regurgitation due to BD can be misdiagnosed as an infective endocarditis when an echo free space mimics aortic root abscess, producing a peculiar image of “vegetation-like” mobile lesions. To semiquantify the severity of the lesions it may be useful to focus the echocardiographic study on aortic cusp and on adjacent segments of the ascending aorta and interventricular septum.
The majority of surgical cases for the treatment of severe aortic insufficiency showed a male predominance (93%, 79%, and 57% in [73–75] , respectively), confirming the higher propensity of BD in manifesting as a systemic disease in men [63] .
The aorta itself does appear to be abnormal in several patients, although aortic aneurysms are considered very rare in BD [63] . Aortic root dilatation, which is thought to precede aneurysm formation, is one of the findings commonly reported in echocardiographic studies in asymptomatic BD patients [59,60,92] . An aorta dilation >4 cm was found in 48% of BD patients randomly selected for a Turkish TEE study [67] , while in another Italian study 30% of subjects undergoing TTE had dilation of the ascending aorta [69] ( Fig. 21.2 ).
Figure 21.2 . CT angiography of a 54-year-old man affected with BD, with mucocutaneous and vascular involvement. Sagittal (A) and transverse (B) sections showing aneurysmatic dilatation of ascending aorta with parietal thickening ( white arrows ).
However, some authors did not find statistically significant enlargement of aortic root diameter in BD patients as compared to controls [70,76] . One of the reasons for the discrepancy of these data might be the different mean ages of the study patients and the different disease durations [63,70] .
In addition to aortic dilatation, echocardiographic studies have shown reduced aortic distensibility, which may help explain the propensity of aneurysm formation in the course of the disease as well as the increased incidence of aneurysmal dilatation of Valsalva sinus [67] . As previously stated, the underlying vasculitic process may indeed weaken the aortic wall and the structures near the aortic sinus, contributing to aneurysmal dilatation [77] .
Cases of cardiac BD complicated with rupture of Valsalva sinus aneurysm have been reported [78] ; aortic root dissection associated with perforation of the left valsalva sinus into the left ventricular outflow tract was described in one of them [77] . In the latter case, pathology of the aortic wall and valves revealed focal fibrinoid necrosis and myxoid degeneration with inflammatory cell infiltration [77] ( Fig. 21.3 ).
Figure 21.3 . A case of aortic root dissection associated with perforation of the left Valsalva sinus into the left ventricular outflow tract in a 49-year-old woman affected with BD [77] . (A) Parasternal short axis view at the aortic level shows the echolucent cavity (∗) between the aortic root and the left and right Valsalva sinus, and a perforation ( arrow ) of the left Valsalva sinus. (B) Short-axis view of the aortic root with color-flow imaging demonstrating blood flow streaming through the perforation of the left Valsalva sinus into the dissection between the aortic root and the Valsalva sinus.
The vasculitic process of the aortic wall determining adherence and fistula formation to the right atrium was the most probable underlying cause in another case of aorto-atrial fistula without concomitant aneurysm [79] .
Douglas P. Zipes MD , in Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine , 2019
Echocardiography is helpful in identifying the cause of AR ( Fig. 68.15 ) and may demonstrate a bicuspid valve, thickening of the valve cusps, other congenital abnormalities, prolapse of the valve, a flail leaflet, or vegetation ( see Chapter 14 ). In addition to leaflet anatomy and motion, the size and shape of the aortic root can be evaluated, although visualization of the ascending aorta is not always adequate, necessitating additional imaging tests in some cases. Transthoracic echocardiography (TTE) usually is satisfactory, but transesophageal echocardiography (TEE) often provides more detail, particularly of the aortic root. TTE is useful for the measurement of LV end-diastolic and end-systolic dimensions and volumes, EF, and mass 59,134 ( Videos 68.2A
High-frequency fluttering of the anterior leaflet of the mitral valve during diastole may be seen in acute and chronic AR. However, it does not develop when the mitral valve is rigid, as occurs with rheumatic involvement. This sign, unlike the Austin Flint murmur, is present even in mild AR and results from the movement imparted to the anterior leaflet of the mitral valve by the jet of blood regurgitating from the aorta.
Doppler echocardiography and color flow Doppler imaging are the most sensitive and accurate noninvasive techniques for the diagnosis and evaluation of AR. They readily detect mild degrees of AR that may be inaudible on physical examination. Both aortic regurgitant orifice size and aortic regurgitant flow can be estimated quantitatively 59,139 ( see Fig. 14.50 and Video 68.3A
CMR provides accurate measurements of regurgitant volumes and the regurgitant orifice in AR ( Fig. 68.16 ). It is the most accurate noninvasive technique for assessing LV end-systolic volume, diastolic volume, and mass ( see Chapter 17 ). CMR accurately quantifies the severity of AR on the basis of the antegrade and retrograde flow volumes in the ascending aorta and is recommended when echocardiographic evaluation of regurgitation is suboptimal. 73,141-143
For angiographic assessment of AR, contrast material should be injected rapidly (i.e., at 25 to 35 mL/sec) into the aortic root, and filming should be carried out in the right and left anterior oblique projections ( see Chapter 19 ). Opacification may be improved by filming during a Valsalva maneuver.
Morbidity and mortality are directly related to the degree of connective tissue abnormality in involved organ systems. Cardiovascular abnormalities of dilation of the ascending aorta and mitral valve prolapse, subluxation of the lens of the eye, chest cavity deformities and scoliosis, and the potential for pneumothorax are serious prognostic indicators.
Treatment of patients with Marfan's syndrome consists of annual medical examination with a cardiovascular emphasis, frequent ophthalmologic examination, scoliosis screening, and echocardiography. Physical activity is often restricted and redirected in an attempt to protect the aorta.
Antibiotic prophylaxis has been recommended for infective endocarditis, regardless of clinical evidence of valvular disease. Beta-blockers such as propranolol are often used to reduce aortic stress and have been shown to significantly reduce both the rate of aortic dilation and the risk of serious complications. Mortality has been drastically reduced with the use of composite grafts to replace the aortic valve and the region containing the aortic aneurysm. The prognosis for untreated aneurysms of the ascending aorta is extremely poor.
Nicholas T. Kouchoukos MD , in Kirklin/Barratt-Boyes Cardiac Surgery , 2013
Important organic mitral regurgitation may coexist with severe aortic valve disease, and repair or replacement of both valves is the proper surgical procedure (see Chapter 13 ). However, mitral regurgitation of grade 1 or 2 (based on grades 1 to 4) may occur in the presence of aortic valve disease even when the mitral leaflets are normal, presumably because of anular dilatation. The mitral regurgitation is usually abolished or considerably reduced by aortic valve replacement. A19
When encountering this situation, the surgeon should evaluate the mitral valve by intraoperative TEE. If it seems structurally normal and regurgitation is no more than grade 2, the valve should not be repaired or replaced. In borderline cases, it is best to discontinue CPB after repair or replacement of the aortic valve and to reassess mitral valve function using TEE with the heart beating and ejecting. If mitral regurgitation continues to be important, CPB is recommenced, and the mitral valve is repaired with an anuloplasty band or ring.
JOHN F. KEANE , DONALD C. FYLER , in Nadas' Pediatric Cardiology (Second Edition) , 2006
Spontaneous decrease in defect size and even closure has been long recognized. The reported frequencies of such events have varied considerably, related to age, defect location, follow-up duration, and especially methods of detection, particularly echocardiography. Inlet (endocardial cushion), typical malalignment, and subpulmonary defects are usually large and remain so. Although shunting in the former types remains substantial, in the latter, it often decreases as the right coronary cusp of the aortic valve prolapses into the defect, thus functionally reducing the orifice size. Membranous and muscular defects often decrease or close, this often involving the septal leaflet of the tricuspid valve in the membranous variety (often referred to as an “aneurysm of the membranous septum”). In a recent echocardiographic study, although of short duration, 15% of membranous defects closed spontaneously, as did some 57% of muscular defects (often small and multiple). 36 It is of interest that a few large apical defects close by enlargement of muscle bundles near the moderator band such that the right ventricular apex remains in continuity only with the left ventricular cavity 53 (see Figs. 30-12 and 30-15 ). Other recent spontaneous closure rates include 6% 50 and 15%, the latter over more than 20 years of follow-up. 52
Namsik Chung MD, PhD, FASE , in Dynamic Echocardiography , 2011
The primary target organs of Behçet's disease are the mucous membranes of the mouth, genitalia, and eye. However, the cardiovascular manifestations of Behçet's disease have attracted much attention in recent years. Cardiovascular manifestations have been reported in 7% to 46% of patients, and death occurs in up to 20% of those with marked vascular involvement. 1 There have been sporadic reports of endocarditis, myocarditis, pericarditis, acute myocardial infarction, aortic aneurysm, ventricular thrombosis, congestive cardiomyopathy, and valve dysfunction. 2 , 3 Previous studies have reported a 6% to 50% prevalence of mitral and aortic valve prolapse , whereas other authors have observed 25% mitral valve prolapse prevalence. 4-6 Investigations regarding the natural history of Behçet's disease have reported that cardiovascular complications related to aortic regurgitation (AR) and sinus of Valsalva aneurysm were the leading cause of death in patients with Behçet's disease. 7 The contributing factors for AR in Behçet's disease have been suggested as the dilation of the aortic annulus caused by atypical inflammation, destruction of the valve itself by the diffuse aortitis of the ascending aorta, and aneurysm of sinus of Valsalva. 8 , 9 Because involvement of the ascending thoracic aorta in Behçet's disease frequently results in severe AR, surgical treatment is mandatory in these patients. However, after aortic valve surgery for AR associated with Behçet's disease, valve detachment and pseudoaneurysm can occur postoperatively, requiring repeat operations in some patients. 10 , 11 Although the pathogenesis for the high rate of tissue destruction at the operation site remains unknown, it has been suggested that the reason is “arterial wall aphthae” resulting from pathergy phenomenon, indicating that the aneurysmal wall of the artery has severe medial necrosis resembling the epidermal and dermal junction necrosis seen in the mucocutaneous lesions. 12
There are currently six clinical situations in which BAV is useful.
Predilation during transcatheter aortic valve implantation procedures. This method will be discussed in Chapter 17 .
Cardiogenic shock. Patients who present with aortic stenosis and cardiogenic shock may be stabilized for the short term.
Congestive heart failure preceding aortic valve replacement. Among patients with severe left ventricular dysfunction or shock in whom aortic valve replacement is planned, balloon dilatation may be performed to allow improvement in left ventricular performance before surgery. Prerenal azotemia associated with their medical therapy may improve after aortic valve prolapse .
Preparation for major noncardiac surgery. Patients found to have aortic stenosis during the evaluation for major noncardiac surgery may undergo valvuloplasty. This is especially useful for patients with malignancies.
Preparation for hospice transfer. Hospital-bound patients with severe aortic stenosis who are not candidates for valve replacement surgery may undergo balloon dilatation with successful short-term improvement. This is useful for patients who are dependent on intravenous pressors and in an intensive care unit. Although valvuloplasty does not improve their long-term prognosis, it may allow them to be transferred to a regular floor or discharged from the hospital so that they may have a better quality of life, at least in the short term.
Diagnostic test in low gradient, low output aortic stenosis. There is a group of patients in whom balloon valvuloplasty may be performed as a diagnostic test. This is useful when the valve area is between 0.8 and 1.0 cm 2 with low cardiac output and a low transvalvular pressure gradient. In this group of patients, the severity of valvular stenosis is especially difficult to ascertain. Poor ventricular function has made therapy in this group difficult. In the past, valve replacement could be performed and, if the patient had improvement in left ventricular function, then survival was good. Unfortunately, for those patients who did not show improvement in left ventricular performance, perioperative mortality was very high. Balloon dilatation may be performed and serial echocardiography used to monitor changes in left ventricular function. If symptoms and left ventricular performance improve with opening of the aortic valve using valvuloplasty, later valve replacement surgery can be undertaken with a high expectation of long-term success.
In vitro evaluation of balloon dilatation for stenotic bioprosthetic valves has been disappointing. Prosthetic tissue is often friable and is frequently not severely calcified, but the potential for leaflet perforation or avulsion is significant in this group of patients. Thus, balloon dilation of bioprosthetic aortic valves alone is infrequently performed.
Brian D. Soriano , David S. Owens , in Echocardiography in Congenital Heart Disease , 2012
Typically fusion of the intercoronary commissure is present.
The entire length of the commissure does not necessarily have to be fused; partial fusion can be present.
Less common anatomic variants include unicuspid ( Fig. 12-1 ) and quadricuspid AVs ( Fig. 12-2 ).
In truncus arteriosus, individuals are born with a single semilunar valve, which can be quadricuspid.
Rheumatic aortic stenosis (AS) and regurgitation.
Usually associated with ventricular septal defects (VSDs), especially with subpulmonary (supracristal) defects.
Leaflet thickness, position, and mobility are best evaluated in the parasternal long axis and short axis. M-mode cursors can evaluate motion with good temporal resolution. With modern-day ultrasound carts, however, temporal resolution of the 2D images is almost as fine.
In adults, estimations of AV area can determined by using the continuity equation or, less frequently, by 2D planimetry.
Valve areas are less frequently measured in pediatric patients due to the relative lack of precision in smaller patients as well as the low frequency of low-output AS for which the AV area would be diagnostically useful.
Three-dimensional imaging of the AV can assist in evaluating both morphology and depth of the cusps.
PW Doppler and continuous wave (CW) Doppler of the subaortic and supravalvular regions should be obtained to determine whether any additional obstruction exists ( Fig. 12-3 ).
Peak velocity and mean gradients can be obtained from the apical windows. The mean gradient from this view best correlates with peak-to-peak estimations by catheterization.
Overestimation of pressure gradients can occur when velocities are obtained in the suprasternal notch (SSN) or high parasternal views. Pressure recovery phenomena may account for these discrepancies.
Severity of AS can be underestimated when there is left ventricular systolic dysfunction.
The degree of aortic regurgitation (AR) can be determined using both color Doppler and spectral Doppler.
PW Doppler of the ascending and descending aorta should be obtained to evaluate the volume of regurgitation.
Guidelines for assessing valve stenosis and regurgitation are outlined in Table 12-2 .
Congenital AV disease is usually due to abnormalities in the number of valv
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