scholarly journals Aortic Dilatation in Patients With Bicuspid Aortic Valve

2021 ◽  
Vol 12 ◽  
Author(s):  
Jing Wang ◽  
Wenhui Deng ◽  
Qing Lv ◽  
Yuman Li ◽  
Tianshu Liu ◽  
...  

Bicuspid aortic valve (BAV) is the most common congenital cardiac abnormality. BAV aortic dilatation is associated with an increased risk of adverse aortic events and represents a potentially lethal disease and hence a considerable medical burden. BAV with aortic dilatation warrants frequent monitoring, and elective surgical intervention is the only effective method to prevent dissection or rupture. The predictive value of the aortic diameter is known to be limited. The aortic diameter is presently still the main reference standard for surgical intervention owing to the lack of a comprehensive understanding of BAV aortopathy progression. This article provides a brief comprehensive review of the current knowledge on BAV aortopathy regarding clinical definitions, epidemiology, natural course, and pathophysiology, as well as hemodynamic and clinically significant aspects on the basis of the limited data available.

2020 ◽  
Vol 9 (2) ◽  
pp. 290
Author(s):  
Anthonie Duijnhouwer ◽  
Allard van den Hoven ◽  
Remy Merkx ◽  
Michiel Schokking ◽  
Roland van Kimmenade ◽  
...  

Objective: The combination of aortic coarctation (CoA) and bicuspid aortic valve (BAV) is assumed to be associated with a higher risk of ascending aortic dilatation and type A dissection, and current European Society of Cardiology (ESC) guidelines advise therefore to operate at a lower threshold in the presence of CoA. The aim of our study is to evaluate whether the coexistence of CoA in BAV patients is indeed associated with a higher risk of ascending aortic events (AAE). Methods: In a retrospective study, all adult BAV patients visiting the outpatient clinic of our tertiary care center between February 2003 and February 2019 were included. The primary end point was an ascending aortic event (AAE) defined as ascending aortic dissection/rupture or preventive surgery. The secondary end points were aortic dilatation and aortic growth. Results: In total, 499 BAV patients (43.7% female, age 40.3 ± 15.7 years) were included, of which 121 (24%) had a history of CoA (cBAV). An aortic event occurred in 38 (7.6%) patients at a mean age of 49.0 ± 13.6 years. In the isolated BAV group (iBAV), significantly more AAE occurred, but this was mainly driven by aortic valve dysfunction as indication for aortic surgery. There was no significant difference in the occurrence of dissection or severely dilated ascending aorta (>50 mm) between the iBAV and cBAV patients (p = 0.56). The aortic diameter was significantly smaller in the cBAV group (30.3 ± 6.9 mm versus 35.7 ± 7.6 mm; p < 0.001). The median aortic diameter increase was 0.23 (interquartile range (IQR): 0.0–0.67) mm/year and was not significantly different between both groups (p = 0.74). Conclusion: Coexistence of CoA in BAV patients was not associated with a higher risk of aortic dissection, preventive aortic surgery, aortic dilatation, or more rapid aorta growth. This study suggests that CoA is not a risk factor in BAV patients, and the advice to operate at lower diameter should be reevaluated.


Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Ayyaz Ali ◽  
Amit Patel ◽  
Darren Freed ◽  
Yasir Abu-Omar ◽  
Ahmad Y Sheikh ◽  
...  

Objectives A bicuspid aortic valve may be associated with an aortopathy, this may lead to progressive aortic dilatation over time. It is uncertain whether the ascending aorta should be replaced prophylactically during AVR in these patients. We analyzed change in ascending aortic diameter following AVR, to determine whether a clinically important aortic pathology exists in patients with bicuspid aortic valve disease. Methods Demographic, operative and clinical data were obtained retrospectively through casenote review. AVR was performed using a homograft or porcine stentless valve using the subcoronary implantation technique. Patients were grouped according to whether their native aortic valve was identified as tricuspid (TC) or bicuspid (BC) at operation. Serial transthoracic echocardiograms were analyzed to measure pre-operative and post-operative ascending aortic diameter. Results 217 patients underwent AVR between 1 st January 1991 and 1 st January 2001. Ninety patients had a bicuspid aortic valve, in the remaining 127 the valve was tricuspid. The bicuspid group was younger ( BC 62yr +/− 15, TC 71yr +/− 12 yrs; p < 0.001). Follow-up echocardiography was performed 6.0 +/− 4.3 years post-operatively. Pre-operative ascending aortic diameter was similar (BC 3.2 +/− 0.5, TC 3.2 +/− 0.5 cm; p = 0.56) There was no difference in the increase in ascending aortic diameter over follow-up (BC 0.1 +/− 0.5, TC 0.0 +/− 0.5 cm; p = 0.34) Conclusion The clinical importance of “bicuspid aortopathy” in an older age group appears to be minimal. Additional aortic procedures designed to protect against progressive aortic aneurysmal disease in this setting are not justified.


2006 ◽  
Vol 91 (5) ◽  
pp. 1785-1788 ◽  
Author(s):  
Carolyn A. Bondy ◽  
Phillip L. Van ◽  
Vladimir K. Bakalov ◽  
Vincent B. Ho

Background: In recent years many girls with Turner syndrome (TS) have been treated with supraphysiological doses of GH to increase adult height. In addition to promoting statural growth, GH may have direct effects on the cardiovascular system. Objective: We sought to determine whether GH treatment affects aortic diameter in girls with TS because there is an increased risk for aortic dilation and dissection in this syndrome. Methods: In a retrospective, cross-sectional study, we compared ascending and descending aortic diameters measured by magnetic resonance imaging in GH-treated (n = 53) vs. untreated (n = 48) patients with TS participating in a National Institutes of Health protocol between 2001 and 2004. Results: The average duration of GH treatment was 4.7 with se 0.4 yr (range 2–11 yr). The two groups were similar in age and weight, but GH-treated subjects were on average 8 cm taller (P = 0.002). The diameter of the ascending aorta was increased by 7.3% and descending aorta by 8.9% in the GH-treated group. However, after correction for age, height, weight, and presence of bicuspid aortic valve and coarctation, using a multiple regression, neither history of GH treatment nor the length of GH treatment had an effect on the aortic diameter. Weight (P = 0.02), height (P = 0.001), and presence of bicuspid aortic valve (P = 0.0001) were associated with larger ascending aortic diameter, whereas age (P = 0.008), height (P = 0.02), and history of coarctation (P = 0.006) were associated with larger descending aortic diameter. Conclusions: GH treatment of girls with TS does not seem to affect ascending or descending aortic diameter above the increase related to the larger body size.


2021 ◽  
Author(s):  
Tie Zheng ◽  
Shijie Lu ◽  
Shuai Zhu ◽  
Jiafu Ou ◽  
Jun-Ming Zhu

Abstract Objective: Aim of this study is to investigate the influence of aortic diameter on hemodynamic environment characteristics in patient with the bicuspid aortic valve (BAV) and dilated ascending aorta (AAo) .Methods: In this study, an MRI of one BAV patient with 4.5 cm AAo was collected and numerical model was constructed. Based on the images,the other three numerical models were constructed with different ascending aortic size with 4.0cm, 5.0cm and 5.5cm respectively while the size and the geometry of other parts were fixed. Then hemodynamics in these four models was simulated numerically and the flow patterns and loading distributions were investigated.Results: Hemodynamics environments in the AAo were simulated with different aortic size. As the aortic diameter increases, we find: 1. the blood flow becomes more disturbing;2.the wall pressure at ascending aortic is higher; 3. the wall shear stress at the ascending aortic decreases; 4.oscillatory shear index of the outer part on the proximal AAo increases;5. all these hemodynamic parameters described above are asymmetrically distributed in dilated AAo and more parts of aorta would be affected as the AAo dilatation progresses.Conclusions: The study revealed that the diameter of ascending aortic can significantly influence the magnitude and distribution of the dynamics. There are altered flow patterns, pressure difference, WSS and OSI distribution features in bicuspid aortic valve patients with vascular dilatation. As the extent of aortic dilatation increases especially exceed 5.5cm,this study support the recent guideline that aortic replacement should be considered .


2012 ◽  
Vol 2012 ◽  
pp. 1-8 ◽  
Author(s):  
Brigitte Laforest ◽  
Mona Nemer

Bicuspid aortic valve (BAV) is the most common congenital heart defect, affecting 1-2% of the population. It is generally diagnosed late in adulthood when deterioration of the abnormal leaflet becomes clinically evident. BAV patients have an increased risk of developing serious complications, including stenosis, regurgitation, endocarditis, dilation of the aorta, aortic dissection, and aneurysm. BAV is a heritable trait, but the genetic basis underlying this cardiac malformation remains poorly understood. In the last decade, thanks to studies in animal models as well as genetic and biochemical approaches, a large number of genes that play important roles in heart development have been identified. These discoveries provided valuable insight into cardiac morphogenesis and uncovered congenital-heart-disease-causing genes. This paper will summarize the current knowledge of valve morphogenesis as well as our current understanding of the genetic pathways involved in BAV formation. The impact of these advances on human health including diagnosis of BAV and prevention of cardiovascular complications in individuals with BAV or with a family history of BAV is also discussed.


2017 ◽  
Vol 25 (3) ◽  
pp. 192-198
Author(s):  
Pablo Straneo ◽  
Gabriel Parma ◽  
Natalia Lluberas ◽  
Alvaro Marichal ◽  
Gerardo Soca ◽  
...  

Background Bicuspid aortic valve patients have an increased risk of aortic dilatation. A deficit of nitric oxide synthase has been proposed as the causative factor. No correlation between flow-mediated dilation and aortic diameter has been performed in patients with bicuspid aortic valves and normal aortic diameters. Being a hereditary disease, we compared echocardiographic features and endothelial function in these patients and their first-degree relatives. Methods Comprehensive physical examinations, routine laboratory tests, transthoracic echocardiography, and measurements of endothelium-dependent and non-dependent flow-mediated vasodilatation were performed in 18 bicuspid aortic valve patients (14 type 1 and 4 type 2) and 19 of their first-degree relatives. Results The first-degree relatives were younger (36.7 ± 18.8 vs. 50.5 ± 13.9 years, p = 0.019) with higher ejection fractions (64.6% ± 1.7% vs. 58.4% ± 9.5%, p = 0.015). Aortic diameters indexed to body surface area were similar in both groups, the except the tubular aorta which was larger in bicuspid aortic valve patients (19.3 ± 2.7 vs. 17.4 ± 2.2 mm·m−2, p = 0.033). Flow-dependent vasodilation was similar in both groups. A significant inverse correlation was found between non-flow-dependent vasodilation and aortic root diameter in patients with bicuspid aortic valve ( R = −0.57, p = 0.05). Conclusions Bicuspid aortic valve patients without aortopathy have larger ascending aortic diameters than their first-degree relatives. Endothelial function is similar in both groups, and there is no correlation with ascending aorta diameter. Nonetheless, an inverse correlation exists between non-endothelial-dependent dilation and aortic root diameter in bicuspid aortic valve patients.


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
G Goudot ◽  
T Mirault ◽  
C Cheng ◽  
M Gruest ◽  
J Amoah ◽  
...  

Abstract Background Magnetic resonance imaging allows evaluation of aortic stiffness by the maximum rate of systolic distension (MRSD) a new prognosis factor of aortic dilatation in patients with bicuspid aortic valve (BAV). MRSD requires a continuous monitoring of the aortic diameter during the cardiac cycle, not accessible to conventional echocardiography contrary to ultrafast ultrasound imaging (UF). Purpose To develop specific aortic sequences in ultrafast ultrasound imaging (UF) to provide access to the aortic MRSD Methods Tissue Doppler allowed a precise estimation of the movement of each wall and the fine variation of the aortic diameter. To automatically track the anterior and posterior aortic walls during the cardiac cycle, we developed in the laboratory a specific interface (Figure). MRSD was the maximum of the derivative of the diameter chande over time. To assess this new technique, 24 patients (10 BAV patients and 14 controls, mean age 45.8 vs. 40.7 years, p=0.464, respectively) were consecutively included at a reference center for BAV. The ascending aorta was evaluated at the sinus of Valsalva, the tubular aorta and the aortic arch with a phased array probe (Supersonic Imagine) and dedicated sequences at 2000 frames/s. Results The lab-made interface allowed to track the aortic diameter and to calculate the MRSD from the UF acquisitions for each patient. We found lower MRSD at the sinus of Valsalva in case of BAV in accordance with previously demonstrated higher stiffness at this segment by our team (Table). Table 1. UF Aortic parameters for BAV patients and controls BAV patients Controls p (Mann Whitney) Sinus of Valsalva Diameter (mm) 26.2 [22.4–32.5] 27.09 [23.5–29.5] 0.796 MRSD (s–1) 1.05 [0.73–1.19] 1.51 [1.28–1.99] 0.023 Tubular ascending aorta Diameter 31.4 [29.4–32.2] 28.9 [22.6–31.5] 0.328 MRSD 0.94 [0.59–1.27] 1.09 [0.87–1.41] 0.353 Aortic arch Diameter 24.2 [23.7–24.8] 24.2 [18.9–24.5] 0.673 MRSD 0.57 [0.35–1.07] 0.85 [0.76–1.02] 0.257 Results are median [25th–75th percentile]. Figure 1 Conclusion UF allows evaluation of aortic stiffness by MRSD using dedicated sequence and interface. As echocardiography, UF is easily accessible and therefore deserves attention from cardiologists taking care of BAV patients to evaluate the segmental aortic remodeling associated with BAV.


Author(s):  
L. Cozijnsen ◽  
R. L. Braam ◽  
M. Bakker-de Boo ◽  
A. M. Otten ◽  
J. G. Post ◽  
...  

Abstract Aim To determine the prevalence of undiagnosed bicuspid aortic valve (BAV) and isolated aortic dilatation in first-degree relatives (FDRs) of patients with isolated BAV and to explore the recurrence risk of BAV in different subgroups of probands with BAV. Recent American College of Cardiology (ACC)/American Heart Association (AHA) Guidelines recommend family screening in patients with associated aortopathy only. Methods During follow-up visits, patients with isolated BAV received a printed invitation for their FDRs advising cardiac screening. Results From 2012–2019, 257 FDRs of 118 adult BAV patients were screened, among whom 63 (53%) index patients had undergone aortic valve surgery (AVS), including concomitant aortic replacement in 25 (21%). Of the non-operated index patients, 31 (26%) had aortic dilatation (> 40 mm). Mean age of the FDRs was 48 years (range 4–83) and 42% were male. The FDR group comprised 20 parents, 103 siblings and 134 offspring. Among these FDRs, 12 (4.7%) had a previously undiagnosed BAV and 23 (8.9%) had an isolated aortic dilatation. FDRs of the probands with previous AVS (n = 147) had a risk ratio for BAV of 2.25 (95% confidence interval (CI) 0.62–8.10). FDRs of the probands with BAV and repaired or unrepaired aortic dilatation (n = 127) had a risk ratio for BAV of 0.51 (95% CI 0.16–1.66). Conclusion Screening FDRs of patients with isolated BAV resulted in a reasonable yield of 14% new cases of BAV or isolated aortic dilatation. A trend towards an increased risk of BAV in FDRs was observed in the probands with previous AVS, whereas this risk seemed to be diminished in the probands with associated aortic dilatation. This latter finding does not support the restrictive ACC/AHA recommendation.


2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
A Babu ◽  
Z Meng ◽  
O Oji ◽  
TJ Bowker ◽  
HB Xiao

Abstract Funding Acknowledgements Type of funding sources: None. Introduction Aortic dilatation is most commonly associated with hypertension, bicuspid aortic valve and connective tissue diseases such as Marfan’s. It is a precursor to life threatening complications such as rupture or dissection of the aorta. The current ESC Guidelines recommend surgical intervention once the aortic diameter is &gt;50 mm in patients with Marfan syndrome (Ic) and &gt;55 mm in patients with a bicuspid or normal aortic valve morphology (IIb). There is currently a lack of literature on the prevalence of aortic dilatation in the general or even hospital population although there is historical data suggesting the incidence of thoracic aortic aneurysm to be 5.9 cases per 100,000. Purpose The aim is to investigate the prevalence of aortic dilatation by echocardiography in our hospital population which may help lay the foundation for population studies and identify prognostic factors which may determine the time of surgical intervention. Methods We carried out a retrospective survey using the digital echocardiogram archive and the electronic patient record system at our hospital. This survey covered the period between 1st October 2016 and 1st November 2018. For randomisation  purposes, all transthoracic echocardiograms (TTE) performed on every Thursday during this period were included. All patients with an echocardiographic report of aortic root or ascending aorta dilatation were enrolled. Other information including echocardiographic dimensions along with demographics and past medical history was collected. Results During this 24-month period, we analysed a total of 3019 TTEs. 209 patients (6.9%) were reported to have aortic dilatation. 137 (66%) were male and the median age was 67 years. The mean height and weight were 169cm and 80kg, respectively.  A bicuspid aortic valve was confirmed in 10 (4.8%) patients. 132 (63%) patients had a history of hypertension. On echocardiogram, 75 (36%) patients had septal hypertrophy and 26 (12.4%) had a dilated left ventricle.   Conclusion Our findings are unique and for the first time, to our knowledge, we report the echocardiographic prevalence of aortic dilatation in the hospital population (6.9%). It is a staggering 40-fold increase when compared to the the prevalence of aortic aneurysm, the most likely end point of aortic dilatation. Based on our figures, there would be at least 400 patients with a dilated aorta in a year in our hospital alone. The prevalence of bicuspid aortic valve in our cohort (4.8%) was nearly three times higher than the general population where it is quoted as 1-2%. Our study also emphasised the established link between hypertension and aortic dilatation with an increased frequency in our cohort (63%) compared to the global prevalence (31%). Given the devastating sequelae of aortic dilatation and its increased prevalence in our patient population, it will be very important to keep these patients under routine surveillance and particularly those with hypertension.


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
M C Carrero ◽  
L De Stefano ◽  
I Constantin ◽  
G Masson ◽  
M Mezzadra ◽  
...  

Abstract Background 2D transthoracic echocardiogram (TTE) is the technique of choice in the diagnosis of bicuspid aortic valve (BAV). Computed tomography (CT) is widely used in BAV to measure aortic diameters. However, in some cases CT or magnetic resonance (MRI) can add important information to TTE regarding valvular degeneration and morphotype. We designed the present prospective study to determine the agreement between TTE and gated CT in the assessment of aortic valve morphology, fibrosis, calcification and measurements of thoracic aorta. We also aimed to analyze the utility of CT in the evaluation of BAV patients, in addition to aortic measurements. Methods We included 30 consecutive patients with BAV (mean age 45 ± 15.7 years ; 73.3% men) who underwent both TTE and ECG-gated cardiac and aortic CT for valvular and aortic assessment in a follow-up protocol with a time interval between TTE and CT of 4 ± 2.6 months. We performed measurements of thoracic aorta at 6 levels (annulus, Valsalva sinus: VS, sinotubular junction, ascendent, arch and isthmus) with both techniques following guideline recommendations blinded to the results of the other technique. Several measurements of VS were performed in short-axis view (double-oblique method) (maximal diameter perpendicular to the valve opening, sinus to sinus, raphe to sinus, commissure to commissure) at systole and diastole with CT. Valve phenotype, presence of raphe, calcification scoring, aortic valve prolapse and fibrosis were also determined with both techniques and maximum aortic diameters were compared. An indexed aortic diameter &gt; 21 mm/m2 was considered as aortic dilation and assimetryc root was defined when differences between CT measurements were ≥5mm. Results In 7 patients (23.3%) aortic diameter differences at Sinus by TTE and CT were ≥3mm. Concerning ascending aorta measurements, there was better agreement and only 2 cases (6.6%) showed differences ≥ 3mm. In 2 patients with severe calcification valve morphology was identified only with CT. There was good agreement between TTE and CT in calcium quantification in patients with valvular calcium score over 2000 AU (n = 5) and in those without calcification. However, TTE failed in identification of valvular fibrosis in 5 patients. 10 patients (30%) had aortic dilatation with CT and 7 according to TTE measurements. The 3 patients that were not identified as dilated in TTE had dilatation at the distal tubular portion. CT led to identification of coronary anomalies in 5 patients (16.7%), most of them anomalous high origin above the sinotubular junction. Conclusions Although TTE is the gold-standard in the diagnosis and follow-up of patients with BAV, CT was useful to confirm aortic measurements and to identify valvular fibrosis, assimetry, coronary anomalies and dilatation at the tubular portion. CT can add important information to TTE regarding valvular morphotype and aortic measurements, although radiation and cost should be evaluated. Abstract P811 Figure. Calcification agreement TTE and CT


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