Frequency of aortic root dilation in children with a bicuspid aortic valve

Bicuspid aortic valve (BAV) is associated with aortic root dilation and dissection in adults, but the age and conditions when dilation begins are unknown. Using echocardiographic data, we compared the aortic root dimensions and valve hemodynamics of 76 children with BAV with valves derived from 41 normal children. The children with BAV had significantly larger aortic roots (p <0.001) regardless of the presence of aortic stenosis or regurgitation.     

Association of aortic dilation with regurgitant, stenotic and functionally normal bicuspid aortic valves.

To determine whether aortic root dilation associated with a bicuspid aortic valve occurs independently of valvular hemodynamic abnormality, aortic root dimensions were measured by two-dimensional echocardiography in 83 adults with a functionally normal (n = 19), mildly regurgitant (n = 26), severely regurgitant (n = 27) or stenotic (n = 11) bicuspid aortic valve and compared with findings in normal subjects matched for age and gender. Aortic root measurements were made at four levels: anulus, sinuses of Valsalva, supraaortic ridge and proximal ascending aorta. Seventy-one percent of patients with a bicuspid aortic valve were men. When compared with control subjects, all hemodynamic subgroups showed a significantly larger aortic root size at three levels: sinuses of Valsalva, supraaortic ridge and proximal ascending aorta (p less than 0.05 to p less than 0.001). The prevalence of aortic root enlargement among all hemodynamic subgroups ranged from 9% to 59% at the level of the anulus, 36% to 78% at the sinuses, 47% to 79% at the supraaortic ridge and 50% to 64% in the ascending aorta. Thus, there is a high prevalence of aortic root enlargement in patients with a bicuspid aortic valve that occurs irrespective of altered hemodynamics or age. These findings support the hypothesis that bicuspid aortic valve and aortic root dilation may reflect a common developmental defect.     

Progressive dilation of the ascending aorta in children with isolated bicuspid aortic valve

Although patients with bicuspid aortic valves (BAVs) are predisposed to ascending aortic (AA) dilation, stenosis, and dissection, the development of aortic disease in children with BAVs is poorly described. The purposes of this study were to determine the rate of change of AA diameter in children with BAVs and to identify risk factors for the development of aortic dilation. The echocardiograms of 276 children aged<19 years (mean 8.5+/-5.3) with isolated BAVs were reviewed. Aortic measurements were normalized to z scores on the basis of body surface area. In a subset of 112 patients with serial examinations, aortic growth rates were calculated and risk factors for more rapid aortic growth determined. At presentation, 33 patients (12%) demonstrated marked AA dilation (z>4), and 70 (25%) were moderately abnormal (z between 2 and 4). The mean+/-SD AA diameter increased more than expected, at a rate of 0.18+/-0.30 z score per year (p<0.0001). In 61 patients with normal AA diameters on initial study, 22 (36%) had abnormal diameters, with z scores>2, at follow-up. Univariate analysis demonstrated right-noncoronary commissural fusion (p<0.02) and aortic valve gradient on initial examination (p<0.02) as significant predictors of AA growth. In multivariate analysis, however, the significance of gradient and valve morphology was diminished (p = 0.06 for both). In conclusion, the progression of AA diameter in patients with normal z scores on initial examination suggests that serial echocardiograms are required to screen for the development of significant aortic dilation.     

Comparison of the pattern of aortic dilation in children with the Marfan's syndrome versus children with a bicuspid aortic valve

Marfan's syndrome (MS) and bicuspid aortic valves (BAVs) are associated with aortic dilation. Despite their histologic similarities, the 2 diseases differ with regard to the location of maximal aortic dilation. Echocardiographic analysis of aortic dimensions was performed in children with MS, children who had aortic dilation in the setting of nonstenotic BAVs (peak gradient <16 mm Hg), and healthy controls. Data on 25 patients with MS, 31 with BAVs, and 65 controls were analyzed. Subjects with BAVs had greater dimensions at all levels of the aortic root compared with normal controls. Patients with BAVs also had greater dilation than those with MS at the level of the annulus and the ascending aorta, with dilation often extending cranially beyond the region of measurement. Conversely, patients with MS had more focal dilation at the sinuses of Valsalva compared with controls and patients with BAVs. In conclusion, despite similar histologic abnormalities, the anatomic pattern of aortic dilation differs in children with MS and BAVs.     

Dislocation of a transapically implanted aortic valve prosthesis with a functionally bicuspid aortic valve and ascending aortic aneurysm

In recent years, catheter-based aortic valve interventions have become established procedures for the treatment of high-risk and advanced age patients with aortic valve pathologies. One of the limitations of the widespread applicability of this procedure is the annulus size. Until recently, no prosthesis was available to treat patients with a large annulus. We report on a patient with high-grade aortic stenosis (AS) and a 27-mm annulus, who underwent transapical implantation (TAP) of an Edwards SAPIEN? 29-mm prosthesis (Edwards LifeScience, Irvine, CA, USA). Due to insufficient dilation of his heavily calcified, functionally bicuspid aortic valve leaflets during balloon aortic valvuloplasty (BAV), the TAP prosthesis did not anchor adequately. This was determined during follow-up as he developed progressive aortic insufficiency and orthopnea, and an echocardiography revealed that the valve had been displaced into the LVOT. A conventional aortic valve replacement and ascending aorta replacement were performed, at which time the TAP prosthesis was removed. The patient recovered uneventfully, and was discharged with a well-functioning aortic bioprosthetic valve and in good general condition.     

Progressive dilation of the diameter of the aortic root in adults with a bicuspid aortic valve

BACKGROUND: To determine the rate of progression of dilation of the aortic root in adults with a bicuspid aortic valve. METHODS: We reviewed retrospectively the transthoracic echocardiograms of 50 adults with a bicuspid aortic valve. Each patient had had at least two examinations made 12 months apart. Measurements were taken at four levels: at the basal attachment of the leaflets of the valve within the left ventricular outflow tract, at the widest point of the sinuses of Valsalva, at the sinutubular junction, and in the ascending aorta 1 cm beyond the sinutubular junction. RESULTS: Progressive dilation occurred at all levels, ranging from 0.3 mm/yr at the basal attachment within the left ventricular outflow tract to 1.0 mm/yr, 1 cm beyond sinutubular junction. These rates of dilation were greater than the reported rate of 0.8 mm per decade in the normal population. The rate of dilation found in the ascending aorta 1 cm beyond the sinutubular junction was significantly greater than at the other sites (p = 0.005). The 21 patients with baseline measurements greater than 34 mm had a significantly higher rate of progression (p = 0.007). Sex, age, and the degree of valvar obstruction or regurgitation did not significantly influence the rate of progression of dilation. CONCLUSION: There is a significantly higher rate of dilation of the aortic root in adults with a bicuspid aortic valve when compared to the normal population. Periodic evaluation of the ascending aorta is essential in these patients, even after replacement of the aortic valve. Other imaging modalities should be considered if the region beyond the sinutubular junction is not well visualized by transthoracic echocardiography.     

Progression rate of ascending aortic dilation in patients with normally functioning bicuspid and tricuspid aortic valves

Dilation of the ascending aorta (AA), which is disproportionate to associated valvular lesions, is a relatively well-recognized phenomenon in patients with a bicuspid aortic valve (BAV). The aim of this study was to evaluate the rate of changes in the AA dimensions and the outcome in patients with AA dilation and BAVs compared with patients with AA dilation and tricuspid aortic valves (TAVs). Serial transesophageal echocardiograms (>12 months apart) were performed in 113 consecutive patients (BAV, n=27 and TAV, n=86) with AA diameters of >or=40 and 相似文献   

Transfemoral implantation of CoreValve Evolut R in patient with functionally bicuspid aortic valve

Catheter based aortic valve replacement became largely adopted technique to treat patients with severe aortic stenosis in the setting of prohibitive risk and in high risk operation patients. Based on the positive clinical data from the Nordic Aortic Valve Intervention (NOTION) Trial [1] and from a subset analysis from the CoreValve U.S. High Risk Pivotal Trial Medtronic plc. announced CE mark for the self-expanding CoreValve™ Evolut™ R to obtain an expanded indication to treat aortic stenosis in patients who are at intermediate risk for open-heart surgery. Bicuspid aortic valve was deemed contraindicated for CoreValve implantation and nowadays is considered to be “off label” use even if multiple publications showed successful CoreValve implantation in patients with stenotic bicuspid aortic valve [2], [3]. Current case report tackles preprocedural evaluation of stenotic aortic valve anatomy and together with the relevant pictures illustrates recapurability and repositionability of abovementioned TAVI system in patient with funcionally bicuspid severely stenotic aortic valve.     

Balloon dilation of the aortic valve: studies in normal lambs and in children with aortic stenosis

To evaluate the risks of and optimal method for valve dilation in aortic stenosis, balloons of different sizes were used to dilate the normal aortic root in 16 lambs and then stenotic valves in 15 children. In the lambs, inflated balloon to aortic anulus diameter ratios ranged from 0.9 to 1.5. These hearts were examined immediately after the procedure. Ratios of 0.9 to 1.1 did not produce significant damage to the left ventricular outflow tract, whereas those of 1.2 to 1.5 produced tears or hematomas, or both, of the aortic valve leaflets (n = 3), mitral valve leaflets (n = 4) and interventricular septum (n = 4). The 15 patients, aged 10 days to 15 years, underwent 16 balloon aortic valvotomy procedures. The balloon-aortic anulus ratio ranged from 0.67 to 1.1 (mean 0.90). The average pressure gradient decreased 69% and, overall, the peak systolic gradient decreased from 86 +/- 21 to 28 +/- 14 mm Hg (p less than 0.01) and the aortic valve area increased from 0.44 +/- 0.11 to 0.73 +/- 0.22 cm2/m2 (p less than 0.01). Immediately after the procedure an increase in aortic regurgitation was noted in 8 (57%) of 14 patients, but was never greater than 3+ and has been well tolerated. Other early complications encountered consisted of transient left bundle branch block in two patients, temporary femoral artery occlusion in three and femoral artery rupture requiring operative management in one infant. Balloon valvotomy can reduce the transvalvular gradient in most patients with valvular aortic stenosis when a balloon less than 1.1 times the aortic root diameter is used.(ABSTRACT TRUNCATED AT 250 WORDS)     

Ascending aorta elasticity in children with isolated bicuspid aortic valve

Background

Aortic dilation is common in children with bicuspid aortic valve (BAV) but aortic complications are infrequent. The aim of this study was to investigate elastic properties of the ascending aorta (AAo) and its relation to AAo size in children with isolated BAV without significant valve dysfunction.

Methods

24 children with isolated BAV and 24 healthy controls with tricuspid aortic valve (TAV) matched by gender, age and body surface area (BSA) were studied. Aortic strain (AS), aortic distensibility (DIS) and aortic stiffness index (SI) were derived from M-mode echocardiography at the AAo together with cuff blood pressure recordings. BAV children with dilated AAo (z score ≥ 2) and non dilated (z score < 2) were compared.

Results

BAV children had larger aortas than controls at the sinuses of Valsalva, sinotubular junction and AAo (p < 0.05). AS was lower in BAV than in controls (10.15 ± 4.93 vs 16.93 ± 5.17 p = 0.000), DIS was lower in BAV than in controls (8.51 ± 3.90 vs 14.37 ± 4.20 p = 0.000) and SI was higher in BAV than in controls (7.19 ± 4.45 vs 4.05 ± 2.33 p = 0.04). There were no significant differences in AS, DIS and SI between children with dilated and non-dilated AAo. AS, DIS and SI were not related to BSA, age or AAo size.

Conclusions

AAo elasticity assessed by transthoracic echocardiography is impaired in BAV children without significant valve dysfunction compared to TAV children. Impaired elasticity seems to be independent from aortic dilation. Measuring aortic elasticity may help to identify children at greater risk for complications as adults.     

Absence of TGFBR1 and TGFBR2 mutations in patients with bicuspid aortic valve and aortic dilation

Mutations in the genes encoding transforming growth factor-beta receptor types I and II (TGFBR1 and TGFBR2, respectively) are commonly identified in patients with Loeys-Dietz syndrome, as well as some patients with Marfan's syndrome or familial thoracic aortic aneurysms and dissections. This suggests that there is considerable phenotypic heterogeneity associated with mutations in these genes. Because bicuspid aortic valve (BAV) is a congenital heart defect in patients with Loeys-Dietz syndrome, this study was conducted to investigate whether variants in TGFBR1 or TGFBR2 are responsible for sporadic BAV. Analysis of these genes in 35 patients with BAVs identified only known single-nucleotide polymorphisms or novel synonymous or intronic substitutions. In conclusion, mutations in TGFBR1 and TGFBR2 rarely cause sporadic BAV.     

Morphology of bicuspid aortic valve in children and adolescents

OBJECTIVES: The aim of this study was to determine the relationship between aortic valve morphology and valve dysfunction. BACKGROUND: The morphology of the bicuspid or bicommissural aortic valve (BAV) may predict the severity of valve dysfunction. Therefore, we assessed the relationship between BAV, aortic coarctation, and the degree of valve pathology in children. METHODS: A retrospective review of 1,135 patients with BAV who were identified between 1986 and 1999 was performed. Patients younger than 18 years of age with BAV that was identifiable via echocardiography were included. The most recent or last study of each patient before intervention or endocarditis was reviewed. Mild stenosis was defined as a valve gradient > or =2 m/s, moderate or greater aortic stenosis as > or =3.5 m/s. Aortic regurgitation was quantified using standard criteria. RESULTS: Median age was 3 years (range, 1 day to 17.9 years), and 67% of the patients were male. Right-coronary and left-coronary leaflet fusion were the most common types of BAV (70%). Aortic stenosis that was moderate or greater was observed most often in patients with right-coronary and non-coronary leaflet fusion (odds ratio 2.4, 95% confidence interval 1.6 to 3.6; p < or = 0.001). Similarly, right-coronary and non-coronary leaflet fusion was more often associated with moderate aortic regurgitation or greater (odds ratio 2.4, 95% confidence interval 1.2 to 4.7; p = 0.01). The majority of patients with aortic coarctation had fusion of the right-coronary and left-coronary leaflets (89%), and aortic coarctation was associated with lesser degrees of valve stenosis or regurgitation. CONCLUSIONS: Analysis of BAV morphology is of clinical and prognostic relevance. Fusion of the right-coronary and non-coronary leaflets was associated with more significant valve pathology, whereas fusion of the right-coronary and left-coronary leaflets was associated overwhelmingly with aortic coarctation and less aortic valve pathology.