scholarly journals Progression and Outcomes of Non-dysfunctional Bicuspid Aortic Valve: Longitudinal Data From a Large Korean Bicuspid Aortic Valve Registry

2021 ◽  
Vol 7 ◽  
Author(s):  
Shinjeong Song ◽  
Jiwon Seo ◽  
Iksung Cho ◽  
Geu-Ru Hong ◽  
Jong-Won Ha ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Ascending Aorta ◽  
Limited Data ◽  
Tertiary Center ◽  
Valve Operation ◽  
And Function ◽  
Aorta Dilatation ◽  
Aorta Diameter

Background: Using echocardiographic surveillance, many patients are diagnosed with bicuspid aortic valve (BAV) without significant valve dysfunction. Limited data are available regarding the progression and outcomes of non-dysfunctional BAV.Methods and Results: We investigated 1,307 BAV patients (984 male, mean age 56 years) diagnosed from Jan 2003 through Dec 2018 in a single tertiary center. Seven hundred sixty-one patients underwent follow-up echocardiography at ≥1 year post-diagnosis. Non-dysfunctional BAV was defined as BAV without moderate aortic stenosis (AS) or aortic regurgitation (AR). The presence of aortopathy was defined as an ascending aorta diameter >37mm. Progression to significant BAV dysfunction, progression to severe aortopathy (ascending aorta diameter ≥45mm), and incidence of valve or aorta operation were analyzed. One hundred eighty-seven (25%) patients showed non-dysfunctional BAV. Among them, 104 (56%) had mild AS or AR, and 81 (43%) had aortopathy at indexed echocardiography. At 6.0 ± 3.8 years post-diagnosis, 56 (29%) progressed to dysfunctional BAV, 28 (15%) progressed to severe aortopathy, 22 (12%) underwent valve operation, and 19 (10%) experienced aorta operation. Eighty-nine percent of patients with normal BAV function and 61% of patients with mild AS or AR maintained non-dysfunctional BAV. More patients with aortopathy progressed to severe aortopathy (35 vs. 0% without aortopathy, p < 0.001), with a higher incidence of aorta operation (21 vs. 2%, p < 0.001).Conclusions: In patients with non-dysfunctional BAV, initial BAV function and degree of aorta dilatation might be important for progression and outcomes. Patients without any dysfunction or aortopathy tend to maintain good structure and function for 6 years.

scholarly journals Fate of mild-to-moderate bicuspid aortic valve disease untreated during ascending aorta replacement

2020 ◽  
Author(s):  
Alessandro Verzini ◽  
Marta Bargagna ◽  
Guido Ascione ◽  
Alessandra Sala ◽  
Davide Carino ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Ascending Aorta ◽  
Term Survival ◽  
Kaplan Meier ◽  
Aortic Root Surgery ◽  
Increased Risk ◽  
Aorta Replacement ◽  
Ascending Aorta Replacement

Background: Bicuspid aortic valve (BAV) is the most common congenital heart defect and it is responsible for an increased risk of developing aortic valve and ascending aorta complications. In case of mild to moderate BAV disease in patients undergoing supracoronary ascending aorta replacement, it is unclear whether a concomitant aortic valve replacement should be performed. Methods: From June 2002 to January 2020, 75 patients with mild-to-moderate BAV regurgitation (± mild-to-moderate stenosis) who underwent isolated supracoronary ascending aorta replacement were retrospectively analyze. Clinical and echocardiographic follow-up was 100% complete (mean: 7.4±3.9 years, max 16.4). Kaplan Meier estimates were employed to analyze long-term survival. Cumulative incidence function for time to re-operation, recurrence of aortic regurgitation (AR)≥3+ and aortic stenosis (AS) greater than moderate, with death as competing risk, were computed. Results: There was no hospital mortality and no cardiac death occurred. Overall survival at 12 years was 97.4±2.5%, 95% CI [83.16-99.63]. At follow-up there were no cases of aortic root surgery whereas 3 patients underwent AV replacement. At 12 years the CIF of reoperation was 2.6±2.5%, 95% CI [0.20-11.53]. At follow up, AR 3+/4+ was present in 1 pt and AS greater than moderate in 3. At 12 years the CIF of AR>2+/4+ was 5.1±4.98% and of AS>moderate 6.9±3.8%. Conclusions: In our study mild to moderate regurgitation of a BAV did not significantly worse at least up to 10 years after isolated supracoronary ascending aorta replacement.

scholarly journals THE DIFFERENT CENTRAL HEMODYNAMICS IN PATIENTS WITH BICUSPID AORTIC VALVE AND ITS ASSOCIATION WITH ASCENDING AORTA DILATATION

2010 ◽  
Vol 55 (10) ◽  
pp. A163.E1524
Author(s):  
In Jeong Cho ◽  
Chi Young Shim ◽  
Jeonggeun Moon ◽  
Sungha Park ◽  
Jong-Won Ha ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Ascending Aorta ◽  
Central Hemodynamics ◽  
Aorta Dilatation

Echocardiographic anatomy of ascending aorta dilatation: Correlations with aortic valve morphology and function

2006 ◽  
Vol 113 (3) ◽  
pp. 320-326 ◽  
Author(s):  
Alessandro Della Corte ◽  
Gianpaolo Romano ◽  
Francesco Tizzano ◽  
Cristiano Amarelli ◽  
Luca S. De Santo ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Ascending Aorta ◽  
Valve Morphology ◽  
And Function ◽  
Aorta Dilatation

scholarly journals Developmental changes in bicuspid aortic valve and associated aortopathy in small children

2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
J Boesgaard Norsk ◽  
A S Sillesen ◽  
A Axelsson Raja ◽  
M Munk Paerregaard ◽  
C Pihl ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Flow Velocity ◽  
Bicuspid Aortic Valve ◽  
Ascending Aorta ◽  
Early Years ◽  
Z Score ◽  
Valve Regurgitation ◽  
Aortic Valve Regurgitation ◽  
Valve Annulus

Abstract Background The prevalence of bicuspid aortic valve (BAV) in newborns is 0.8%. BAV is associated with an increased risk of aortic valve dysfunction and aortopathy. Aortopathy with increased aortic diameters has recently been reported in newborns with BAV. As most patients with BAV are diagnosed in adulthood the development of BAV and associated aortopathy during early years of life is not well described. Purpose The purpose of the study was to assess changes in aortic valve function and aortic dimensions at two to four years of age in children diagnosed with BAV neonatally. Methods Children with BAV were included from a population-based cohort study, in which newborns (n≈25,000) underwent standardized transthoracic echocardiography (TTE). Follow-up TTE was performed and analyzed according to established guidelines. Neonatal (baseline) and follow-up data were compared. Diameters were indexed to body surface area (BSA). Z-scores were calculated using formulas from the Pediatric Heart Network Echocardiogram Database. Results At follow-up 101 newborns with BAV (mean age 2 years 5 months, SD 11 months), 75% male) were examined from May 2019 to April 2021. From baseline to follow-up there was an increase in the BSA-adjusted diameter of the aortic valve annulus (z-score −0.19 at baseline vs 0.95 at follow up, p<0.001), and of the sino-tubular junction (z-score 0.16 at baseline vs 0.43 at follow up, p<0.05). Conversely, there was a decrease in the Z-score for the diameter measured 1 cm from the valve annulus (z-score 1.31 at baseline vs 0.68 at follow up, p<0.001) and at the widest point of the visualized ascending aorta (z-score 2.44 at baseline vs 1.45 at follow up, p<0.001). There was no significant change in the diameter of the sinus of Valsalva (z-score 0.83 at baseline vs 0.80 at follow up, p=0.92). The number of children with at least one aortic z-score >3 was 31 (30.6%) at baseline and 17 (17.8%) at follow up. At baseline mild aortic valve regurgitation was observed in 18 children (17.8%) and in 23 children at follow up (23%). Mean maximum systolic velocities across the aortic valve were 1.03 m/s at baseline (SD 0.24) and 1.11 m/s at follow up (SD 0.27), p<0.05. Aortic stenosis, defined as flow velocity >2.5 m/s was seen in one child both at baseline and at follow-up. Conclusion In children diagnosed with BAV neonatally, re-examination at the age of 2.5 years showed significant increases in the diameter of the aortic valve annulus and the sino-tubular junction, but a significant decrease in the diameter of the ascending aorta. The maximum blood flow velocity across the aortic valve increased and more children had developed mild aortic valve regurgitation. Thus, the bicuspid aortic valve and the associated aortopathy seem to undergo remodeling during early childhood. FUNDunding Acknowledgement Type of funding sources: Foundation. Main funding source(s): The Danish Children's Heart Foundation, Boernehjertefonden.

scholarly journals Predictors of Ascending Aorta Enlargement and Valvular Dysfunction Progression in Patients with Bicuspid Aortic Valve

2021 ◽  
Vol 10 (22) ◽  
pp. 5264
Author(s):  
Angela Lopez ◽  
Ilaria Dentamaro ◽  
Laura Galian ◽  
Francisco Calvo ◽  
Josep M. Alegret ◽  
...  
Keyword(s):  
Risk Factors ◽  
Growth Rate ◽  
Cardiovascular Risk ◽  
Aortic Valve ◽  
Cardiovascular Risk Factors ◽  
Bicuspid Aortic Valve ◽  
Aortic Root ◽  
Ascending Aorta ◽  
Valve Dysfunction

Bicuspid aortic valve (BAV) patients are at high risk of developing progressive aortic valve dysfunction and ascending aorta dilation. However, the progression of the disease is not well defined. We aimed to assess mid-long-term aorta dilation and valve dysfunction progression and their predictors. Patients were referred from cardiac outpatient clinics to the echocardiographic laboratories of 10 tertiary hospitals and followed clinically and by echocardiography for >5 years. Seven hundred and eighteen patients with BAV (median age 47.8 years [IQR 33–62], 69.2% male) were recruited. BAV without raphe was observed in 11.3%. After a median follow-up of 7.2 years [IQR5–8], mean aortic root growth rate was 0.23 ± 0.15 mm/year. On multivariate analysis, rapid aortic root dilation (>0.35 mm/year) was associated with male sex, hypertension, presence of raphe and aortic regurgitation. Annual ascending aorta growth rate was 0.43 ± 0.32 mm/year. Rapid ascending aorta dilation was related only to hypertension. Variables associated with aortic stenosis and regurgitation progression, adjusted by follow-up time, were presence of raphe, hypertension and dyslipidemia and basal valvular dysfunction, respectively. Intrinsic BAV characteristics and cardiovascular risk factors were associated with aorta dilation and valvular dysfunction progression, taking into account the inherent limitations of our study-design. Strict and early control of cardiovascular risk factors is mandatory in BAV patients.

P1824Aortic wall shear stress in bicuspid aortic valve disease - 10-year follow-up

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
M Hanigk ◽  
E Burgstaller ◽  
H Latus ◽  
N Shehu ◽  
J Zimmermann ◽  
...  
Keyword(s):  
Shear Stress ◽  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Aortic Valve Disease ◽  
Helical Flow ◽  
Ascending Aorta ◽  
Valve Disease ◽  
4D Flow

Abstract Introduction Bicuspid aortic valve (BAV) disease leads to deviant complex helical flow patterns in the aorta - especially in the mid-ascending (AoA) part. In association with congenital BAV, aortic wall alterations such as aortic dilatation and dissection may occur. Among others, wall shear stress (WSS) could be one parameter to contribute to the prediction of the long-term outcome of patients with BAV. 4D-flow in cardiovascular magnetic resonance has been established as a valid method to estimate WSS. Purpose The aim of this study is to reevaluate WSS and comparing it to values generated in the same patient cohort with bicuspid aortic valve disease in 2008. No one of the above had aortic dilation in 2008 but proven helical flow pattern. The long term follow-up study might show changes in WSS over the period of ten years. Methods Ten complete 4D flow datasets of patients (age at follow-up: median 34.5 years; range 19–41 years) with bicuspid aortic valve disease without enlargement of the aorta were obtained in 2008 and reevaluated in 2018/2019 in the same patient collective. Mean WSS values were calculated with identical specific software tools. All data were analyzed by two experienced investigators. Results Aortic diameters at the level of the mid AoA did not change significantly in the 10-year period. The WSS values were lower in 2018 at all levels of the ascending aorta (Table 1). Indexed aortic diameters at the level of the mid ascending aorta did not change, median difference 0.06 cm/m2 (range −0.1 cm/m2 to 0.2 cm/m2; p=0.28), absolute values of indexed AoA diameters in 2018/2019 ranged from 1.27 cm/m2 to 2.2 cm/m2 (median 1.76 cm/m2). Table 1 WSS magnitudinal [N/m2] 2008 (n=10) 2018 (n=10) Median difference p-value median range median range Level aortic bulb 0.95 0.80–1.46 0.42 0.34–0.82 −0.53 <0.01 Level mid ascending aorta 0.72 0.40–0.98 0.39 0.34–0.59 −0.33 <0.01 Level brachiocephalic trunc (BCT) 0.71 0.38–1.03 0.40 0.37–0.61 −0.31 <0.01 Conclusions Indexed AoA diameters in BAV disease did not change significantly over a 10-year period. WSS of AoA was less compared to values generated in 2008. This might be explained by a slight alteration in hemodynamic flow patterns by the aging aorta, but not by changes of the aortic diameters. Possibly a drop of WSS in BAV could serve as a marker for a benign long term course.

Ascending aorta dilatation in patients with bicuspid aortic valve stenosis: a prospective CMR study

2012 ◽  
Vol 23 (3) ◽  
pp. 642-649 ◽  
Author(s):  
Alexia Rossi ◽  
Denise van der Linde ◽  
Sing Chien Yap ◽  
Thomas Lapinskas ◽  
Sharon Kirschbaum ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Aortic Valve Stenosis ◽  
Ascending Aorta ◽  
Aorta Dilatation

Abstract 2131: The Association of Coarctation of Aorta Diminishes the Occurrence of Ascending Aorta Dilation in Children and Young Adults with Bicommissural Aortic Valves.

Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Thieu Nguyen ◽  
Andrea Z Beaton ◽  
Wyman W Lai ◽  
Prema Ramaswamy ◽  
Ira A Parness ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Ascending Aorta ◽  
Aortic Annulus ◽  
Sinotubular Junction ◽  
Z Scores ◽  
Group A ◽  
Group B ◽  
Aortic Dimensions

Objectives: To explore the difference in ascending aortic dilatation between subgroups of bicuspid aortic valve (BAV) patients with and without coarctation of the aorta (CoA). Methods: Our echocardiographic database (1993–2006) was searched for BAV patients with CoA (Group A) and without CoA (Group B). Measurements at the aortic annulus, root, sinotubular junction, and ascending aorta were obtained for each patient, and body surface area-adjusted Z-score values were compared. Exclusion criteria included more than mild aortic stenosis or regurgitation, previous balloon aortic valvuloplasty, or complex left heart disease; plus Turner, Noonan, Williams, and Marfan Syndromes. Results: The median age in Group A (n=53) was 11.3 yrs (range 0 to 30) with median follow-up of 7 yrs (0 to 12.7); median age in Group B (n=145) was 8.7 yrs (0 to 29) with median follow-up of 4 yrs (0 to 13.1). Group B patients had significantly greater aortic annulus, sinotubular junction, and ascending aortic dimensions (ascending aorta Z-scores shown in Figure , p<0.0001). Group A ascending aortic dimensions did not differ significantly from the normal population. The rate of growth of the ascending aorta in Group B was higher in the first 10 years of life. Conclusion: The ascending aorta in patients with bicuspid aortic valve and coarctation does not dilate to the same degree as patients with isolated bicuspid aortic valve. This may reflect an inherent difference in aortic wall properties between the two groups. Comparison of Ascending Aorta Z Scores

scholarly journals Circumferential wall shear stress predicts co-localized progressive dilation in bicuspid aortic valve patients

2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
A Guala ◽  
L Dux-Santoy ◽  
G Teixido-Tura ◽  
A Ruiz-Munoz ◽  
K M Johnson ◽  
...  
Keyword(s):  
Growth Rate ◽  
Shear Stress ◽  
Aortic Valve ◽  
Wall Shear Stress ◽  
Bicuspid Aortic Valve ◽  
Outer Region ◽  
Ascending Aorta ◽  
Axial Component ◽  
Wall Shear

Abstract Background Bicuspid aortic valve (BAV), a congenital heart defect, is associated with ascending aorta (AAo) dilation. Whether the high prevalence of dilation in BAV patients is related to alteration of aortic blood flow and thus in wall shear stress (WSS) [1,2], which have been associated with aortic wall degeneration [3], or intrinsic abnormalities of the aortic wall, such as altered aortic stiffness [4], has not been established. Recently, a technique for the semi-automatic quantification of progressive aortic dilation maps via image registration has been introduced [5]. Purpose To test whether ascending aorta WSS predicts co-localized progressive dilation in BAV patients. Methods Forty BAV patients free from moderate and severe aortic valve regurgitation (regurgitant fraction &lt;16%) and stenosis (maximum velocity at the aortic valve &lt;3m/s), with no previous aortic or aortic valve surgery or replacement and included in a double-blind clinical trial (BICATOR, NCT02679261) were enrolled. All patients underwent a baseline 4D flow CMR study to assess aortic hemodynamics, followed by two contrast-enhanced computed tomography angiographies to quantify progressive dilation. WSS was computed at 64 pre-specified standardized ascending aortic regions, automatically obtained dividing the ascending aorta into 8 equidistant longitudinal sections which were further divided along the circumference into 8 equal regions (I = inner, L = left, O = outer and R = right) [2]. WSS was also projected into axial and circumferential directions, as previously described [1,2]. Progressive dilation was assessed in terms of growth rate (GR), i.e. increase in diameter divided by follow-up duration [mm/year], following a previously described methodology [5], at the same 64 pre-specified ascending aortic locations. A two-tailed p-value &lt;0.05 was considered statistically significant. Results Demographic and clinical characteristics of the patients are shown in Table 1. WSS and growth rate maps are shown in Figure 1. Follow-up duration was 44.8±2.6 months. Growth rate (Figure 1A) was heterogeneously distributed, being highest (up to 0.26 mm/year) in the outer region of the mid AAo and in the inner region of the proximal-mid AAo. Circumferential WSS showed highest values in the outer region of the mid AAo (Figure 1C) while WSS (magnitude) and its axial component (Figure 1B and D) presented maximum values in the right region of the mid AAo. Maps of statistically significant association between GR and WSS values showed circumferential WSS to be correlated with GR in regions where progressive dilation was fastest, while WSS magnitude and its axial component resulted in limited associations with GR maps. Conclusions Circumferential wall shear stress predicts location-matched progressive dilation in bicuspid aortic valve patients. FUNDunding Acknowledgement Type of funding sources: Public grant(s) – National budget only. Main funding source(s): This study has received funding from the Instituto de Salud Carlos III (PI17/00381). Guala A. has received funding from Spanish Ministry of Science, Innovation and Universities (IJC2018-037349-I). Table 1. Demographics Figure 1. GR and WSS maps and correlations

scholarly journals Monocyte-to-HDL-cholesterol ratio is associated with Ascending Aorta Dilatation in Patients with Bicuspid Aortic Valve

2021 ◽  
Vol 21 (1) ◽  
pp. 96-104
Author(s):  
Burak Acar ◽  
Cagrı Yayla ◽  
Murat Gul ◽  
Mustafa Karanfil ◽  
Sefa Unal ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Vascular Diseases ◽  
Hdl Cholesterol ◽  
Ascending Aorta ◽  
Aortic Dilatation ◽  
Monocyte Count ◽  
Aorta Aneurysm ◽  
Cholesterol Ratio ◽  
Aorta Diameter

Background: The importance of monocyte count-to-HDL-cholesterol ratio (MHR) in cardio- vascular diseases has been shown in various studies. Ascending aortic dilatation (AAD) is a common complication in the patients with bicuspid aortic valve. In this study, we aimed to investigate the relationship between MHR and the presence of aortic dilatation in the pa- tients with bicuspid aortic valve. Methods: The study population included totally 347 patients with bicuspid aortic valve.169 patients with aortic dilatation (ascending aorta diameter ≥ 4.0 cm) and 178 patients with no aortic dilatation. Echocardiographic and laboratory measure- ment was done and compared between groups. Results: The mean age of the participants was 44.7 ± 15.4 years and average ascending aorta diameter was 3.2 ± 0.3 cm in dilatation negative group and 4.4 ± 0.4 cm in positive group. MHR was significantly increased in in patients with aortic dilatation. MHR and uric acid level was independently associated with the presence of aortic dilatation in the patients with bicuspid aortic valve. Conclusion: We found a significant relationship between MHR and aortic dilatation in the patients with bicuspid aortic valve. Keywords: Bicuspid aorta; aorta aneurysm; monocyte HDL ratio; inflammation.

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