scholarly journals Differences in Aortopathy in Patients with a Bicuspid Aortic Valve with or without Aortic Coarctation

2020 ◽  
Vol 9 (2) ◽  
pp. 290
Author(s):  
Anthonie Duijnhouwer ◽  
Allard van den Hoven ◽  
Remy Merkx ◽  
Michiel Schokking ◽  
Roland van Kimmenade ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Aortic Dissection ◽  
Bicuspid Aortic Valve ◽  
Aortic Coarctation ◽  
Care Center ◽  
Aortic Surgery ◽  
Tertiary Care ◽  
Aortic Diameter ◽  
Aortic Dilatation ◽  
Significant Difference

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.

scholarly journals Variability in surveillance practice for patients with diagnosis of bicuspid aortic valve syndrome

2021 ◽  
Author(s):  
Arianna Kahler-Quesada ◽  
Ishani Vallabhajosyula ◽  
Sameh Yousef ◽  
Makoto Mori ◽  
Roland Assi ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Care Center ◽  
Aortic Surgery ◽  
Tertiary Care ◽  
Tertiary Care Center ◽  
Aortic Pathology ◽  
Increased Risk ◽  
Surveillance Practice

Background/Aim: In patients with bicuspid aortic valves, guidelines call for regular follow-up to monitor disease progression and guide timely intervention. We aimed to evaluate how closely these recommendations are followed at a tertiary care center. Methods: This was retrospective cohort study at a tertiary care center. Among 48,504 patients who received echocardiograms between 2013-2018, 245 patients were identified to have bicuspid aortic valve. Bivariate analyses compared patient and echocardiographic characteristics between patients who did and did not receive follow-up by a cardiovascular specialist. Results: The mean age of the cohort was 55.2  15.6 years and 30.2% were female. During a median follow-up of 3.5  2.2 years, 72.7% of patients had at least one visit with a cardiovascular specialist after diagnosis of bicuspid aortic valve by echocardiogram. Patients followed by specialists had a higher proportion of follow-up surveillance by echocardiogram (78.7% vs. 34.3%, p< .0001), or by CT or MRI (41.0% vs. 3.0%, p < .0001), and were more likely to undergo valve or aortic surgery compared with patients not followed by specialists. Patients with moderate to severe valvular or aortic pathology (aortic stenosis/regurgitation, dilated ascending aorta) were not more likely to be followed by a cardiovascular specialist or receive follow-up echocardiograms. Conclusions: Follow-up care for patients with bicuspid aortic valve was highly variable, and surveillance imaging was performed sparsely despite guidelines. There is an urgent need for surveillance and clinical follow-up mechanisms to monitor this patient population with increased risk of progressive valvulopathy and aortopathy.

scholarly journals Ascending aortic dissection in a pregnant patient with neonatally repaired coarctation of aorta and bicuspid aortic valve

2019 ◽  
Vol 12 (12) ◽  
pp. e233367
Author(s):  
Mark Philip Cassar ◽  
Asad Shabbir ◽  
Elizabeth Orchard ◽  
James Stirrup
Keyword(s):  
Aortic Valve ◽  
Aortic Dissection ◽  
Bicuspid Aortic Valve ◽  
Aortic Surgery ◽  
Pregnant Patient ◽  
Haemodynamic Instability ◽  
Coarctation Of Aorta ◽  
Blurred Vision ◽  
Life Threatening ◽  
History Of

Aortic dissection is characterised by a tear in the intimal and medial layers of the endovascular aortic wall which propagates distally. Here, we discuss the case of a 35-year-old woman who was 37 weeks pregnant and presented with dizziness and blurred vision. She had a history of a neonatal end-to-end repair of a coarctation of aorta, a known bicuspid aortic valve and a dilated ascending aorta under surveillance. A transthoracic echocardiogram revealed an ascending aortic dissection. An emergency CT aortogram was performed which confirmed the diagnosis. The patient underwent emergency caesarean section and aortic surgery, with a good outcome for mother and baby. The case highlights the atypical nature of presentation and the absence of haemodynamic instability. Atypical and unexplained symptoms on a background of congenital heart disease should trigger a referral to cardiology with thorough investigation, often with echocardiography, to exclude rare and life-threatening complications.

Abstract 2069: Aortic Valve Replacement In Patients With Bicuspid Aortic Valve Disease Is Not Followed By Progressive Aortic Dilatation: A 15-year Study

Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Ayyaz Ali ◽  
Amit Patel ◽  
Darren Freed ◽  
Yasir Abu-Omar ◽  
Ahmad Y Sheikh ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Aortic Valve Disease ◽  
Valve Disease ◽  
Aortic Diameter ◽  
Implantation Technique ◽  
Aortic Dilatation ◽  
Aortic Pathology ◽  
Ascending Aortic Diameter

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.

scholarly journals Bicuspid Aortic Valve: Role of Multiple Gene Variants in Influencing the Clinical Phenotype

2018 ◽  
Vol 2018 ◽  
pp. 1-9 ◽  
Author(s):  
Elena Sticchi ◽  
Rosina De Cario ◽  
Alberto Magi ◽  
Sabrina Giglio ◽  
Aldesia Provenzano ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Aortic Root ◽  
Rare Variants ◽  
Aortic Surgery ◽  
Genetic Diagnosis ◽  
Clinical Manifestations ◽  
Aortic Dilatation ◽  
Reduced Penetrance ◽  
Next Generation Sequencing Ngs

Background. Bicuspid aortic valve (BAV) is a common congenital heart defect with increased prevalence of aortic dilatation and dissection. BAV has an autosomal dominant pattern of inheritance with reduced penetrance and variable expressivity. BAV has been described as an isolated trait or associated with other clinical manifestations in syndromic conditions. Identification of a syndromic condition in a BAV patient is clinically relevant in order to personalize indication to aortic surgery. We aimed to point out how genetic diagnosis by next-generation sequencing (NGS) can improve management of a patient with complex BAV clinical picture. Methods and Results. We describe a 45-year-old Caucasian male with BAV, thoracic aortic root and ascending aorta dilatation, and connective features evocative but inconclusive for clinical diagnosis of Marfan syndrome (MFS). Targeted (91 genes) NGS was used. Proband genetic variants were investigated in first-degree relatives. Proband carried 5 rare variants in 4 genes: FBN1(p.Asn542Ser and p.Lys2460Arg), NOTCH1(p.Val1739Met), LTBP1(p.Arg1330Gln), and TGFBR3(p.Arg423Trp). The two FBN1 variants were inherited in cis by the mother, showing systemic features evocative of MFS, but with a milder phenotype than that observed in the proband. Careful clinical observation along with the presence of the FBN1 variants allowed diagnosis of MFS in the proband and in his mother. NOTCH1 variant was found in mother and brother, not exhibiting BAV, thus not definitely supporting/excluding association with BAV. Interestingly, the proband, his brother and father, all showing root dilatation, and his sister, with upper range aortic root dimension, were carriers of a TGFBR3 variant. LTBP1 might also modulate the vascular phenotype. Conclusions. Our results underline the usefulness of NGS together with family evaluation in diagnosis of patients with monogenic traits and overlapping clinical manifestations due to contribution of the same genes and/or presence of comorbidities determined by different genes.

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 ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Surgical Intervention ◽  
Current Knowledge ◽  
Aortic Diameter ◽  
Aortic Dilatation ◽  
Frequent Monitoring ◽  
Increased Risk ◽  
Clinically Significant ◽  
Medical Burden

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.

scholarly journals Hemodynamic Characteristics of Dilated Ascending Aorta in Patients with Bicuspid Aortic Valve

2021 ◽  
Author(s):  
Tie Zheng ◽  
Shijie Lu ◽  
Shuai Zhu ◽  
Jiafu Ou ◽  
Jun-Ming Zhu
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Numerical Models ◽  
Outer Part ◽  
Flow Patterns ◽  
Ascending Aorta ◽  
Aortic Diameter ◽  
Aortic Dilatation ◽  
Distribution Features ◽  
Aortic Size

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 .

scholarly journals Contributing Factor of Proximal Arch Dilation in Patients with Bicuspid Aortic Valve—Wall Shear Stress or Upward Extension of Ascending Aorta Dilation?

2020 ◽  
Vol 23 (4) ◽  
pp. E435-E440
Author(s):  
Fei Li ◽  
Qi Gao ◽  
En Qiao ◽  
Gang Yin ◽  
Ru-Jiao Zhang ◽  
...  
Keyword(s):  
Shear Stress ◽  
Aortic Valve ◽  
Wall Shear Stress ◽  
Bicuspid Aortic Valve ◽  
Ascending Aorta ◽  
Aortic Diameter ◽  
Selective Approach ◽  
Significant Difference ◽  
Wall Shear ◽  
The One

Purpose: The dilation of proximal arch (PArc) was suspected to develop as a result of valve-related hemodynamics or in consequence to the upward extension of the ascending aorta (AAo) dilation. We aimed to investigate the one that could be the possible contributing factor in patients with bicuspid aortic valve (BAV). Methods: All enrolled BAV patients underwent four-dimensional flow magnetic resonance imaging. Contour-averaged circumferential wall shear stress (WSScirc,avg) and the diameter of the middle of AAo (mid-AAo) were compared between the patients with and without PArc dilation. Additionally, we analyzed the correlation between WSScirc,avg and aortic diameter at PArc section, as well as the correlation between the diameter of mid-AAo and that of PArc. Results: No significant difference was observed in WSScirc,avg at PArc section between the patients with and without PArc dilation (P = .621). However, the diameter of mid-AAo in the patients who suffered PArc dilation was higher than those without it (P = .007). In addition, the aortic diameter did not correlate with the WSScirc,avg at PArc level (R = -0.068, P = .701). The correlation was observed between PArc diameter and mid-AAo diameter (R = 0.521, P = .002). Conclusion: Hemodynamics may not contribute to the development of PArc dilation. PArc diameter correlated with mid-AAo diameter, indicating PArc dilation may be secondary to the upward extension of AAo dilation. The influence of AAo dilation extending upward could be treated by AAo replacement; hence, a selective approach to transverse PArc replacement might be appropriate.

Genetic diseases of the aorta: aortic diseases related to Marfan syndrome and other genetic abnormalities

ESC CardioMed ◽  
2018 ◽  
pp. 2611-2613
Author(s):  
Bernard Iung
Keyword(s):  
Aortic Valve ◽  
Aortic Dissection ◽  
Bicuspid Aortic Valve ◽  
Aortic Surgery ◽  
Genetic Diseases ◽  
Rapid Progression ◽  
Aortic Diseases ◽  
Genetic Abnormalities ◽  
History Of ◽  
Associated Risk Factors

Bicuspid aortic valve affects 1–2% of babies at birth and is frequently associated with thoracic aortic aneurysm but the risk of aortic dissection is low. Indications for aortic surgery are based on studies on natural history. Aortic surgery is recommended when the maximum aortic diameter is greater than 55 mm in patients with a bicuspid aortic valve. Intervention is indicated at an earlier stage when there are associated risk factors, in particular familial history of aortic dissection or rapid progression, or if there is an indication for aortic valve replacement. The choice between partial or total replacement of the aorta should be based on anatomy, but also take into account the patient’s age and operative risk.

Genetic diseases of the aorta: aortic diseases related to Marfan syndrome and other genetic abnormalities

ESC CardioMed ◽  
2018 ◽  
pp. 2611-2613
Author(s):  
Bernard Iung
Keyword(s):  
Aortic Valve ◽  
Aortic Dissection ◽  
Bicuspid Aortic Valve ◽  
Aortic Surgery ◽  
Genetic Diseases ◽  
Rapid Progression ◽  
Aortic Diseases ◽  
Genetic Abnormalities ◽  
History Of ◽  
Associated Risk Factors

Bicuspid aortic valve affects 1–2% of babies at birth and is frequently associated with thoracic aortic aneurysm but the risk of aortic dissection is low. Indications for aortic surgery are based on studies on natural history. Aortic surgery is recommended when the maximum aortic diameter is greater than 55 mm in patients with a bicuspid aortic valve. Intervention is indicated at an earlier stage when there are associated risk factors, in particular familial history of aortic dissection or rapid progression, or if there is an indication for aortic valve replacement. The choice between partial or total replacement of the aorta should be based on anatomy, but also take into account the patient’s age and operative risk.

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