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.     

Management of the ascending aorta in patients with bicuspid aortic valve disease

Bicuspid aortic valve (BAV) disease is the most common form of congenital heart disease, affecting 1-2% of the population. Only 20% of patients will maintain normal valve function throughout their life and more than 30% of patients will develop serious morbidity. It is a highly heritable condition, with transmission likely to be autosomal dominant. Patients with BAV have a 10-fold risk of aortic dissection when compared to the normal population. Management of BAV associated aortopathy represents a significant clinical challenge.     

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.     

Approach to the patient with bicuspid aortic valve and ascending aorta aneurysm

Opinion statement Bicuspid aortic valve (BAV) disease is a common congenital heart valve abnormality accounting for a large number of valve replacements in the United States. Although still incompletely understood, the natural history of BAV disease is severe aortic stenosis and associated ascending aortic dilatation. In addition to the increased risk of endocarditis, aortic dissection and severe aortic valve dysfunction are responsible for most fatal complications. Thus, early and precise recognition of this condition is mandatory. The new American College of Cardiology/American Heart Association recommendations highlight the role of MRI and CT as complimentary tools to echocardiography for the diagnosis and surveillance of the morphology of the aortic valve and ascending aorta. Moreover, better understanding of the cellular mechanisms, including inflammation, bone formation, atherosclerotic-like processes, and aortic wall abnormalities, as well as the heritability and genetic predisposition for the disease, will define the potential for targeted medical therapies in the future. Currently, the treatment of this condition is primarily surgical. Although combined valve and ascending aorta replacement has been the most common surgical approach in the past, the increased cumulative risk of thrombotic and embolic events among these young patients has led to more conservative approaches. Several valve-sparing approaches with comparable mid-term results compared with the classic procedures have recently been reported. However, longer follow-up studies will be helpful to better define the advantages of these new surgical options. After a quick overview of the natural history of the BAV, this article provides an updated approximation of the current knowledge of the pathophysiology as well as the recommendations for the management and treatment of this disease.     

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.     

Aortic valve repair of congenital bicuspid aortic valve associated with aneurysm of the ascending aorta

Two unusual cases of congenital bicuspid aortic valve associated with aneurysm of the ascending aorta are reported. One patient with a 7-cm ascending aortic dilatation and aortic regurgitation (AR) (II/IV), and another with a 6-cm ascending aorta and AR (III/IV), presented for treatment. Replacement of the ascending aorta and aortic valve repair were performed in both cases. Aortic valve repair included resection of the raphe, leaflet plication and subcommissural annuloplasty. Both patients had satisfactory results in the early postoperative period. Despite the promising outcomes after surgery in these patients, long-term changes in valve function and durability remain unknown. Additional close observation and monitoring are required before the procedure can be recommended as the standard of care.     

Progression of aortic dilation in children with a functionally normal bicuspid aortic valve

Adults with bicuspid aortic valves (BAVs) are at risk for progressive aortic dilation independent of valve function. The evolution of aortic dilation in children with functionally normal BAVs has not been studied. In this study, ascending aortic diameters were assessed in a group of children with functionally normal BAVs (peak gradient < or =16 mm Hg) to determine whether progressive dilation occurs during childhood. A cohort of 101 patients and 97 controls were studied with echocardiography. There were no differences in mean age (9.0 +/- 4.8 vs 8.7 +/- 6.1 years, p = 0.7) or body surface area (1.1 +/- 0.4 vs 1.1 +/- 0.5 m2, p = 0.9). Patients had significantly greater aortic dimensions than controls in all measured regions of the aorta, with the most striking discrepancy in the ascending aorta (2.3 +/- 0.6 vs 1.8 +/- 0.5 cm, p <0.0001). The size discrepancy was present from birth and persisted throughout childhood. Repeated measurements were performed in a subgroup of 28 patients and 25 controls. Patients had significantly greater increases in ascending aortic dimension than controls per year of follow-up (1.2 +/- 0.08 vs 0.6 +/- 0.08 mm/year, p <0.0001). In conclusion, BAV is complicated by progressive aortic dilation beginning in childhood.     

Relation of aortic wall alterations and ascending aorta diameter in patients with bicuspid aortic valve

Patients with a bicuspid aortic valve tend to develop local enlargement in the convexity of the ascending aorta. There is controversial discussion as to whether this is a dilatation caused by abnormal hemodynamic stress or an aneurysm due to a common developmental defect of the aortic valve and the aortic media. Systematic studies of aortic wall specimens from patients with different diameters of the ascending aorta have not been available until now. We investigated histologically (HE, E.v.G., alpha-Actin, Alcian blue/van Gieson) aortic wall tissue obtained from 107 patients (31 female, 76 male, mean age 60.9+/-12.8 years) with a bicuspid aortic valve. According to the preoperative measurement of the ascending aorta diameter, which was done by CT scan, angiography or echocardiography, the patients were divided into three groups [Group 1: ascending aorta diameter <3.8 cm; Group 2: diameter >/=3.8-4.9 cm and Group 3: diameter >/=5.0 cm]. We looked for histological signs of dilatation or more severe structural changes. All patients of group 1 showed normal findings in histological examination. In 23 of 43 patients in group 2 (65.1%), we found histological signs of dilatation. Histological signs of dilatation were present in all patients in group 3. We conclude that in patients with a bicuspid aortic valve histological signs of dilatation were more frequently found with increasing diameter of the ascending aorta. More severe histological changes, such as cystic media necrosis, were not present.     

Comparison of aortic valve dysfunction and ascending aorta dimension between patients with different bicuspid aortic valve morphology

正Objective To compare the characteristics of aortic valve dysfunction and ascending aorta dimension in patients with different bicuspid aortic valve(BAV)morphology.Methods A total of 197 patients who underwent aortic valve replacement between April 2014 and March2015 and were diagnosed with BAV by patholgy were included,and their clinical data were retrospectively ana-     

Usefulness of bicuspid aortic valve phenotype to predict elastic properties of the ascending aorta

Bicuspid aortic valve (BAV) affects about 0.5% to 2% of the population and predisposes patients to aortic dilation and dissection. We hypothesized that aortic size and elastic properties are related to BAV phenotype. In a retrospective study of 158 consecutive patients with BAV referred for echocardiography, the phenotype was defined as anterior-posterior (A-P) leaflet orientation or right-left (R-L) leaflet orientation. The 29 subjects with R-L BAV were matched 1:1 for age, gender, and grade of aortic valve dysfunction with 29 subjects with A-P BAV. Aortic dimensions were measured at the sinuses of Valsalva, ascending aorta, and aortic arch. Distensibility and stiffness index were calculated using cuff blood pressure. Mean age was 41.5 years (range 21 to 67), and 59% were men. Aortic diameter was larger with A-P BAV than R-L at the sinuses (mean +/- 1 SD 3.48 +/- 0.49 vs 3.06 +/- 0.59, p <0 .01) and smaller at the arch (2.34 +/- 0.40 vs 2.83 +/- 0.45, p <0.001). At the sinuses, A-P BAV had a higher stiffness index (median 12.98, range 2.78 to 42.07 vs 6.41, range 2.75 to 59.72, p <0.01) and lower distensibility. Stiffness index in the ascending aorta and arch (but not at the sinus) increased with age. In conclusion, A-P BAV is associated with a larger stiffer sinus of Valsalva and smaller arch diameter. The potential impact of BAV phenotype and aortic elasticity on clinical outcomes merits further study.     

Changes in size of ascending aorta and aortic valve function with time in patients with congenitally bicuspid aortic valves

Bicuspid aortic valve (BAV) is associated with premature valve dysfunction and abnormalities of the ascending aorta. Limited data exist regarding serial changes of aortic dilation in patients with BAV. We studied paired transthoracic echocardiograms of 68 patients with BAV (mean age 44 years) and with at least 2 examinations >12 months apart (mean follow-up 47 months) to characterize the progression of aortic dilation and the natural history of valve function. We measured aortic root and ascending aortic diameters at baseline and follow-up. We measured aortic gradients and severity of aortic regurgitation (AR). During follow-up, aortic diameters increased at the sinuses of Valsalva by 1.9 mm (95% confidence interval [CI] 1.3 to 2.5), at the sinotubular junction by 1.6 mm (95% CI 0.8 to 2.3), and at the proximal ascending aorta by 2.7 mm (95% CI 1.9 to 3.6). Mean rate of diameter progression was 0.5 mm/year at the sinuses of Valsalva (95% CI 0.3 to 0.7), 0.5 mm/year at the sinotubular junction (95% CI 0.3 to 0.7), and 0.9 mm/year at the proximal ascending aorta (95% CI 0.6 to 1.2). Progression was observed regardless of hemodynamic function at baseline. Mean aortic valve gradient increased significantly from baseline to follow-up (17.6 mm Hg vs 25.7 mm Hg, p <0.001). The degree of AR increased during follow-up in 17 patients (25%). In addition, progression of aortic diameter dilation occurred irrespective of baseline valve function in adult patients with BAV. We also observed considerable progression of aortic gradients and AR over time.     

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.     

Configuration of the ascending aorta in patients with bicuspid and tricuspid aortic valve disease undergoing aortic valve replacement with or without reduction aortoplasty

BACKGROUND AND AIM OF THE STUDY: Patients with bicuspid aortic valves (BAV) tend to develop dilatation of the ascending aorta. The study aim was to analyze differences in aortic root diameter and configuration in patients with bicuspid and tricuspid aortic valve disease. METHODS: A retrospective analysis was conducted of the angiographies of 461 patients allocated to four groups with: (i) BAV disease with (n = 179) and (ii) without (n = 78) dilatation of the ascending aorta; (iii) tricuspid aortic valve disease (TAV) and dilatation of the ascending aorta (n = 154); and (iv) coronary artery disease (CAD), TAV and normal diameter of the ascending aorta (n=50). Diameters and distances in the aortic root region were measured, and the ascending aorta configuration analyzed. RESULTS: The diameter of the ascending aorta in patients with BAV and dilatation was significantly larger than in those with TAV and dilatation (26.6 +/- 5.22 versus 24.4 +/- 3.74 mm/m2, p = 0.002). Distances between aortic valve level and point of maximum diameter of the ascending aorta at the outer and inner curve of the vessel in patients with BAV without dilatation were greater than those of the CAD group (31.1 +/- 5.27 versus 28.0 +/- 4.86 mm/m2, p = 0.002 and for the indexed values 21.6 +/- 4.05 versus 20.0 +/- 2.71 mm/m2, p = 0.011). All patients with BAV and enlargement of the ascending aorta showed asymmetric dilatation of the vessel. CONCLUSION: All patients with BAV had an abnormal configuration of the ascending aorta. In cases with enlargement of the ascending aorta exclusively, asymmetric dilatation at the convexity of the vessel occurred. Patients with BAV and normal ascending aorta diameter showed an elongation of this vessel segment.     

Proteomics of ascending aortic aneurysm with bicuspid or tricuspid aortic valve

Bicuspid aortic valve is often associated with lesions of the ascending aorta, which differ histologically from those in tricuspid valve patients. We undertook proteomic analyses to assess differences at the proteome level. Aortic samples were collected from 20 patients undergoing aortic valve and/or ascending aortic replacement; 9 had a bicuspid valve: 5 with aortic aneurysm (diameter > 50 mm) and 4 without dilation; 11 had a tricuspid valve: 6 with aortic aneurysm and 5 without dilation. Patients with histologically proven connective tissue disorders were excluded. Samples were dissected, solubilized, and subjected to 2-dimensional gel electrophoresis. Gel patterns showed an average of 580 protein spots in samples from bicuspid valve patients, and 564 spots in those with tricuspid valves. Comparative analysis revealed a correlation coefficient of 0.93 for protein expression in the bicuspid valve group compared to the tricuspid group. Three protein spots were significantly over-expressed and 4 were significantly down-regulated in the bicuspid group compared to the tricuspid group. The lowest correlation in protein expression was between non-dilated aortic tissues. These differences between aortic tissues of bicuspid and tricuspid valve patients suggest that mechanisms of aortic dilation might differ, at least in part, between such patients.     

Dilatation of the ascending aorta and serum alpha 1-antitrypsin level in patients with bicuspid aortic valve

Predictors of aortic dilatation are not well described in patients with bicuspid aortic valves (BAV). This study sought to examine the relationship between proximal aortic dilatation and matrix metalloproteinase-9 (MMP-9) and alpha 1-antitrypsin (α1AT) levels in patients with BAV. All patients underwent echocardiography using a standard protocol, and aortic measurements were taken in end-diastole. We studied 82 patients with BAV and categorized them into two groups according to aortic dimensions corrected for body surface area and age. The plasma level of α1AT was routinely determined using a BN ProSpec analyzer (Siemens Healthcare Diagnostics, Marburg, Germany), and that of MMP-9 were determined by ELISA (RayBiotech Inc. Norcross, GA, USA). Statistical analysis was performed using the Statistical Package for Social Sciences (SPSS; SPSS Inc., Chicago, IL, USA) software for Windows version 12. This study included patients with BAV with no or mild valvular impairment. There were no significant differences between groups in terms of gender, body surface area, associated hypertension, diabetes mellitus, hyperlipidemia, or smoking. The mean ascending aortic diameter was 4.38?±?0.5?mm in group 1 and 3.34?±?0.35?mm in group 2 (p?相似文献