Bicuspid Aortic Valve Leaflet Morphology in Patients With and Without Coarctation of the Aorta

preprint OA: closed
Full text JSON View at publisher

Abstract

Abstract Introduction : Bicuspid aortic valve (BAV) is the most common congenital heart defect. BAV is often seen with other left-sided structural abnormalities, such as coarctation of the aorta. Furthermore, diagnosing coarctation of the aorta can sometimes be challengingwith transthoracic echocardiography. This study aimed to assess if the presence of coarctation of the aorta is associated with a specific BAV morphology. Methods and materials : All patients referred to Ghaem Mashhad Hospital for echocardiography were included in the study if they had a BAV. The morphology of the aortic valve was classified based on the fusion of the cusps (anteroposterior opening and mediolateral opening), and the existence of coarctation of aorta was determined based on the findings in echocardiography or the CT angiography. Then, the data were compared in two groups. Results : Out of 134 patients (104 men and 30 women), 81 had anteroposterior morphology, and 53 had mediolateral morphology. Among the patients with anteroposterior morphology, 43 patients (69.4%) had coarctation of aorta, and 38 patients (52.8%) had no coarctation of aorta. Among patients with mediolateral morphology, 19 (30.6%) were diagnosed with coarctation of the aorta, while 34 (47.2%) showed no evidence of coarctation of the aorta (p = 0.049). Conclusion The present study showed that the morphology of the aortic valve contribute to predicting the presence of coarctation of the aorta.
Full text 60,152 characters · extracted from preprint-html · click to expand
Bicuspid Aortic Valve Leaflet Morphology in Patients With and Without Coarctation of the Aorta | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Research Article Bicuspid Aortic Valve Leaflet Morphology in Patients With and Without Coarctation of the Aorta Zahra Oskoueyan, Reza Hassanabadi, Faezeh Oskoueyan, Leila Bigdelu This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-7718515/v1 This work is licensed under a CC BY 4.0 License Status: Posted Version 1 posted You are reading this latest preprint version Abstract Introduction : Bicuspid aortic valve (BAV) is the most common congenital heart defect. BAV is often seen with other left-sided structural abnormalities, such as coarctation of the aorta. Furthermore, diagnosing coarctation of the aorta can sometimes be challenging with transthoracic echocardiography. This study aimed to assess if the presence of coarctation of the aorta is associated with a specific BAV morphology. Methods and materials : All patients referred to Ghaem Mashhad Hospital for echocardiography were included in the study if they had a BAV. The morphology of the aortic valve was classified based on the fusion of the cusps (anteroposterior opening and mediolateral opening), and the existence of coarctation of aorta was determined based on the findings in echocardiography or the CT angiography. Then, the data were compared in two groups. Results : Out of 134 patients (104 men and 30 women), 81 had anteroposterior morphology, and 53 had mediolateral morphology. Among the patients with anteroposterior morphology, 43 patients (69.4%) had coarctation of aorta, and 38 patients (52.8%) had no coarctation of aorta. Among patients with mediolateral morphology, 19 (30.6%) were diagnosed with coarctation of the aorta, while 34 (47.2%) showed no evidence of coarctation of the aorta (p = 0.049). Conclusion The present study showed that the morphology of the aortic valve contribute to predicting the presence of coarctation of the aorta. bicuspid aortic valve coarctation of aorta aortic stenosis aortic insufficiency Introduction Bicuspid aortic valve (BAV) is a congenital heart defect affecting around 1–2% of the population. This condition is linked to a higher likelihood of developing issues related to the aortic valve in the future ( 1 , 2 ). Approximately 95% of BAVs include a third rudimentary commissure, commonly known as a raphe, which can vary in height. Functionally, considerable aortic stenosis often occurs between the ages of 50 and 60 years, whereas regurgitation tends to develop around the age of 30 years ( 3 , 4 ). Crucially, BAV is not limited to a singular disease entity. Instead, it entails several disorders that differ in genetic and molecular aspects, resulting in a diverse range of alterations in the development of the heart and aorta ( 5 , 6 ). Coarctation of the aorta (CoA) is well-acknowledged to occur concurrently in many of these patients. Before the development of repair techniques, aortic dissection accounted for 19% of deaths among individuals with coarctation of the aorta. This risk was notably higher in those with a bicuspid aortic valve (BAV) ( 5 ). The prevalence of aortic coarctation of the aorta in patients with BAV varies between 22% and 36%, depending on the age groups investigated. Nevertheless, the incidence of aortopathy in patients with BAV who also have a coarctation of aorta (cBAV) is currently unknown ( 6 – 8 ). The present study aimed to compare the bicuspid aortic morphology in patients with and without the coarctation of the aorta. Because some patients have a poor view and diagnosis of the coarctation in inexperience people are so hard so we want to study if the morphology of the aorta was helpful to possible prediction of the presence of coarctation or not. Methods and materials Study design Patients over 17 years old who had been referred to Ghaem Hospital for echocardiography during two years and who had bicuspid aortic valve were included in the study. According to the latest guidelines, the echocardiographic studies were conducted with patients in the left lateral position using Siemens and Philips devices and a 2.5-4 MHz probe. Aortic morphology was observed during systole, and in the case of a fusion between left coronary and right coronary cusps, the patient was labeled as anteroposterior fusion, and in the case of fusion between right coronary and non-coronary cusps or left coronary and non-coronary cusps, the patient was labeled as mediolateral fusion. Before do the echocardiography whole physical exam was done for all patients including; synchronous pulses of radial and femoral artery and rediofemoral delay was assessed also systolic blood pressure of upper limb and lower limb was measured. For the purpose of assessing the coarctation of the aorta, the patients were examined from the suprasternal view. The presence of the coarctation of the aorta was defined as a peak aortic gradient greater than 20 mmHg; in case necessary, other modalities such as CT angiography were used. In cases that have typical finding of coarctation in echocardiography (such as peak gradient more than 20 mmHg and other typical findings) we confirm our data with CT angiography. Patients with a previous history of surgery due to coarctation of the aorta or stent implantation who had no aortic valve replacement were not excluded from the study since a surgery or stent implantation would not change the artic morphology. Statistical analysis The continuous variables exhibited a non-parametric distribution and were therefore presented as median and interquartile range (IQR), whereas categorical data were provided as frequency and percentage. The Mann-Whitney U test was used to compare groups based on continuous data, whereas Fischer's exact tests were used for categorical variables. Post hoc analyses were conducted using multiple Fischer's exact tests with Bonferroni correction. A significance level of p < 0.05 was deemed statistically significant. The statistical analyses were conducted using SPSS V.25.0. The data registry of the echocardiography department at Mashhad University of Medical Sciences was utilized for data collection Ethics The Ethics Committee of Mashhad University of Medical Sciences approved the present study (IR.MUMS.fm.REC.1395.262). Results In the examined samples, 104 patients (77.6%) were men, and 30 patients (22.4%) were women. Fifteen (11.2%) were smokers, and 7 (5.2%) were diabetic; fourteen (10.4%) had hyperlipidemia, and seven patients (5.2%) had ischemic heart disease. Comparing the two morphological groups regarding gender, 58.7% of men had an anteroposterior valve, and 41.3% had a mediolateral valve (p-value: 0.42). 66.7% of women had an anteroposterior valve, and 33.3% had a mediolateral valve. Also, the comparison of two groups with and without coarctation of the aorta in terms of sex showed that 44.2% of men had coarctation of the aorta Furthermore, 53.3% of women had coarctation of aorta (p-value: 0.378) (Table 1 ). Table 1 The association between sex and aortic morphology and presence of coarctation p-value Male Female Morphology Anteroposterior 61 (58.7) 20 (66.7) 0.429 Mediolateral 43 (41.3) 10 (33.3) Coarctation of aorta Yes 46 (44.2) 16 (53.3) 0.378 No 58 (55.8) 14 (46.7) Out of 81 patients with anteroposterior morphology (60.4% of the total), 43 patients (53.0%) had coarctation of aorta. In the mediolateral morphology group with 53 patients (39.6% of the total), 19 (35.8%) had coarctation of aorta. There was a significant difference between the two morphology groups regarding the coarctation of the aorta (p-value: 0.049) (Table 2 ). In the other word, of 62 patients with coarctation of the aorta, 69.4% had an anteroposterior valve, and 30.6% had a mediolateral valve. In the group of patients without coarctation of the aorta, 52.8% had an anteroposterior valve, and 47.2% had a mediolateral valve. (Table 2 ) Among patients with anteroposterior morphology, 12.5% had no aortic regurgitation, 37.5% had mild regurgitation, 32.5% had moderate regurgitation, and 17.5% had severe regurgitation. The corresponding proportions in the mediolateral morphology group were 7.5%, 35.8%, 35.8%, and 20.8%, respectively. There were no significant differences between the groups in the frequency of aortic regurgitation (p-value: 0.789). (Table 2 ) In the anteroposterior group, 38.6% had no aortic stenosis, 6.8% had mild aortic stenosis, 6.8% had moderate aortic stenosis, and 7.6% had severe aortic stenosis. In the group with mediolateral morphology, 22% had no aortic stenosis, 8.3% had mild aortic stenosis, 2.3% had moderate aortic stenosis, and 7.6% had severe aortic stenosis. No significant differences were observed in the frequency of aortic stenosis between the groups (p-value: 0.231). About 47.4% of patients with anteroposterior valve had dilated aorta, and 52.6% of them did not have dilated aorta. 46.2% of patients with mediolateral aortic valves had dilated aorta (p-value: 0.886) (Table 2 ). Table 2 The association between aortic morphology and aortic regurgitation, stenosis, and dilation Anteroposterior Mediolateral p-value Coarctation Yes 43 (53%) 19 (35.8%) 0.049 No 38 (46.91%) 34 (64.1%) Aortic regurgitation None 10 (12.3%) 4 (7.5) 0.789 Mild 31(38.2%) 19 (35.8) Moderate 26 (32%) 19 (35.8) Severe 14 (17.2%) 11 (20.7) Aortic stenosis None 51 (62.9%) 29 (54.7%) 0.231 Mild 11(13.5%) 11 (20.7%) Moderate 9 (11.1%) 3 (5.6%) Severe 10 (12.3%) 10 (18.8%) Aortic dilation Yes 39(48.1%) 25 (52.8) 0.886 No 42 (51.8%) 28 (52.8) Discussion Although the association between bicuspid aortic valve (BAV) and coarctation of the aorta (CoA) is well-established, prior CoA repair has generally had minimal impact on the management of BAV itself. The significance of the concurrent presence of these two conditions remains incompletely understood. In this study, we sought to investigate whether there is a meaningful relationship between the morphology of the bicuspid aortic valve and the presence of CoA, as understanding this association could have important implications for risk stratification, surveillance, and clinical management. Our aim was to determine if certain BAV morphologies are more frequently associated with CoA, which might explain differences in patient outcomes or the development of related complications. The results showed that the frequency of CoA differed significantly between BAV morphology groups; however, no significant differences were found regarding aortic regurgitation, stenosis, or dilation. Notably, increased blood pressure was more common among patients with CoA compared to those without, highlighting the hemodynamic implications of this coexisting condition. Our research has indicated that BAV is typically associated with other non-valvular cardiovascular anomalies, including CoA, coronary artery anomalies, sinus of Valsalva aneurysm, aortic aneurysm, aortic dissection, supravalvular aortic stenosis, patent ductus arteriosus, ventricular septal defect, Shone complex, familial aneurysm syndromes, thoracic aortic dissection, and Turner syndrome ( 9 ). Following this, the present study discovered that the most common complications that presented in BAV patients were CoA, aortic regurgitation, stenosis, and dilation. Previous studies have shown aortic stenosis to be more common among the anteroposterior (AP) BAV subtype ( 10 , 11 ). Although a higher number of aortic stenosis cases in our cohort had an AP BAV phenotype, the difference was not statistically significant. Recent data further indicate that abnormal blood flow patterns associated with valve morphology not only predispose AP-fused valves to earlier-onset aortic stenosis, but also to accelerated dilation of the ascending aorta—seen in up to 60–80% of BAV patients—through combined genetic and hemodynamic mechanisms ( 9 ). These findings support the need for close longitudinal surveillance of valve function and aortic dimensions, especially in AP morphological subtypes. Our research has indicated that BAV is typically associated with other non-valvular cardiovascular anomalies, including CoA, coronary artery anomalies, sinus of Valsalva aneurysm, aortic aneurysm, aortic dissection, supravalvular aortic stenosis, patent ductus arteriosus, ventricular septal defect, Shone complex, familial aneurysm syndromes, thoracic aortic dissection, and Turner syndrome ( 9 ). In line with this, the present study found that the most common complications observed in patients with BAV were CoA, aortic regurgitation, stenosis, and dilation. Previous studies have shown aortic stenosis to be more common among patients with the anteroposterior (AP) BAV subtype ( 10 , 11 ). Although a higher number of aortic stenosis cases in our cohort had an AP BAV phenotype, the difference was not statistically significant. Recent data further indicate that abnormal blood flow patterns associated with valve morphology not only predispose AP-fused valves to earlier-onset aortic stenosis, but also to accelerated dilation of the ascending aorta—seen in up to 60–80% of BAV patients—through combined genetic and hemodynamic mechanisms ( 9 , 12 – 14 ). These mechanisms involve both inherited factors and abnormal flow dynamics that create asymmetrical shear stress on the aortic wall, leading to distinct segmental dilation patterns depending on BAV phenotype. Understanding these underlying processes highlights the importance of close longitudinal surveillance of valve function and aortic dimensions, particularly in patients with the AP subtype. The present study showed that the anteroposterior (AP) and mediolateral (ML) phenotypes of BAV accounted for 60% and 40% of cases, respectively. Most previous studies have reported the predominance of the AP subtype, except for a few outliers ( 10 ). Additionally, our findings demonstrated that these morphological subtypes were not significantly associated with gender, which is consistent with prior research ( 11 ). A study by Tabrizi et al. in 2018, which evaluated 300 adults with BAV, similarly reported that the AP morphology was significantly more common than the ML subtype among patients with coarctation of the aorta, aligning with our results ( 14 ). In our study, the AP morphology was more frequent among patients with CoA (53%) compared to those without CoA (46.9%), while the ML subtype was less common in patients with CoA (35.8%) compared to those without (64.1%). This difference was statistically significant (p = 0.049), indicating that CoA tends to occur more frequently in patients with an AP BAV phenotype. These findings further underscore the importance of considering valve morphology when assessing the risk of associated cardiovascular anomalies in patients with BAV. The present study had some limitations. Given the rarity of coarctation of aorta, the present study found a limited number of events, necessitating the results to be interpreted cautiously. Furthermore, the size of the ascending aorta is dependent of age, which could impact the results in a small sample size. Also, the individuals included in our study were patients who were receiving medical attention at a specialized facility. As a result, the findings of our study may not apply to a broader community. In conclusion, the results of the present study showed that coarctation of the aorta was more frequently observed among those with AP BAV phenotype. Furthermore, increased blood pressure was more commonly observed among those with coarctation of aorta. Declarations Ethics approval and consent to participate This study was approved by the Ethics Committee of Mashhad University of Medical Sciences (approval code: IR.MUMS.fm.REC.1395.262). Written informed consent was obtained from all individual participants included in the study. Consent for publication Not applicable. Funding The authors received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors. Author Contribution L.B, Z.O and R.H wrote the main manuscript text and created tables and figures. All authors reviewed the manuscript References Braverman AC, Güven H, Beardslee MA, Makan M, Kates AM, Moon MR. The bicuspid aortic valve. Current problems in cardiology. 2005;30(9):470-522. Rinnström D, Engström KG, Johansson B. Subtypes of bicuspid aortic valves in coarctation of the aorta. Heart and vessels. 2014;29:354-63. Masri A, Kalahasti V, Alkharabsheh S, Svensson LG, Sabik JF, Roselli EE, et al. Characteristics and long-term outcomes of contemporary patients with bicuspid aortic valves. The Journal of thoracic and cardiovascular surgery. 2016;151(6):1650-9. e1. Michelena HI, Desjardins VA, Avierinos J-F, Russo A, Nkomo VT, Sundt TM, et al. Natural history of asymptomatic patients with normally functioning or minimally dysfunctional bicuspid aortic valve in the community. Circulation. 2008;117(21):2776-84. Lim MS, Bannon PG, Celermajer DS. Bicuspid aortic valve: different clinical profiles for subjects with versus without repaired aortic coarctation. Open Heart. 2020;7(2):e001429. Merkx R, Duijnhouwer AL, Vink E, Roos-Hesselink JW, Schokking M. Aortic diameter growth in children with a bicuspid aortic valve. The American journal of cardiology. 2017;120(1):131-6. Ciotti GR, Vlahos AP, Silverman NH. Morphology and function of the bicuspid aortic valve with and without coarctation of the aorta in the young. The American journal of cardiology. 2006;98(8):1096-102. Oliver JM, Alonso-Gonzalez R, Gonzalez AE, Gallego P, Sanchez-Recalde A, Cuesta E, et al. Risk of aortic root or ascending aorta complications in patients with bicuspid aortic valve with and without coarctation of the aorta. The American journal of cardiology. 2009;104(7):1001-6. Rodríguez-Palomares JF, Dux-Santoy L, Guala A, Galian-Gay L, Evangelista A. Mechanisms of aortic dilation in patients with bicuspid aortic valve: JACC state-of-the-art review. Journal of the American College of Cardiology. 2023;82(5):448-64. Kim YG, Sun BJ, Park GM, Han S, Kim DH, Song JM, et al. Aortopathy and bicuspid aortic valve: haemodynamic burden is main contributor to aortic dilatation. Heart. 2012;98(24):1822-7. Moaref A, Khavanin M, Shekarforoush S. Aortic distensibility in bicuspid aortic valve patients with normal aortic diameter. Therapeutic advances in cardiovascular disease. 2014;8(4):128-32. Girdauskas E, Schulz S, Borger MA, Mierzwa M, Kuntze T. Transforming growth factor-beta receptor type II mutation in a patient with bicuspid aortic valve disease and intraoperative aortic dissection. Ann Thorac Surg. 2011;91(5):e70-1. Prakash SK, Bossé Y, Muehlschlegel JD, Michelena HI, Limongelli G, Della Corte A, et al. A roadmap to investigate the genetic basis of bicuspid aortic valve and its complications: insights from the International BAVCon (Bicuspid Aortic Valve Consortium). J Am Coll Cardiol. 2014;64(8):832-9. Toufan Tabrizi M, Rahimi Asl R, Nazarnia S, Pourafkari L. Evaluation of relationship between bicuspid aortic valve phenotype with valve dysfunction and associated aortopathy. J Cardiovasc Thorac Res. 2018;10(4):236-42. Additional Declarations No competing interests reported. Cite Share Download PDF Status: Posted Version 1 posted You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. We do this by developing innovative software and high quality services for the global research community. Our growing team is made up of researchers and industry professionals working together to solve the most critical problems facing scientific publishing. Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-7718515","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"Research Article","associatedPublications":[],"authors":[{"id":528448084,"identity":"91c3a21a-961a-4b56-8650-8c3280bbf486","order_by":0,"name":"Zahra Oskoueyan","email":"","orcid":"","institution":"Mashhad University of Medical Sciences","correspondingAuthor":false,"prefix":"","firstName":"Zahra","middleName":"","lastName":"Oskoueyan","suffix":""},{"id":528448085,"identity":"955aa445-fb2b-4fc2-bbcc-2319013c77f3","order_by":1,"name":"Reza Hassanabadi","email":"","orcid":"","institution":"Mashhad University of Medical Sciences","correspondingAuthor":false,"prefix":"","firstName":"Reza","middleName":"","lastName":"Hassanabadi","suffix":""},{"id":528448086,"identity":"76c709c0-0129-4a83-b688-1e8ff0dc02fd","order_by":2,"name":"Faezeh Oskoueyan","email":"","orcid":"","institution":"Mashhad University of Medical Sciences","correspondingAuthor":false,"prefix":"","firstName":"Faezeh","middleName":"","lastName":"Oskoueyan","suffix":""},{"id":528448087,"identity":"d47b7007-7db6-44aa-8faa-affa713e292b","order_by":3,"name":"Leila Bigdelu","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAAA2ElEQVRIiWNgGAWjYBACe2YGBgmGAgYZfhAvoYAILYbNIC0GDDySDSAtBkRoMTgA1QJiALnE2NLOfvHGB4PDPMbnVyd+eGDAIM8vdgC/FntmnmLLGUAtZjfebpYAOsxw5uwEArY086RJ84C1nN0A0pJgcJuAFqDiNOk/IIfNOLv5B5Fa2I9JgzQa8PduI84WoMOYLXsM0nkkbvBus0gwkCDsF3v+4w9v/KiwluPvP7v55o8KG3l+aQJaGIAxAqElwColCCkHAfYHEJr/ADGqR8EoGAWjYCQCAPx3QYqiX2vRAAAAAElFTkSuQmCC","orcid":"","institution":"Mashhad University of Medical Sciences","correspondingAuthor":true,"prefix":"","firstName":"Leila","middleName":"","lastName":"Bigdelu","suffix":""}],"badges":[],"createdAt":"2025-09-26 06:53:31","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-7718515/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-7718515/v1","draftVersion":[],"editorialEvents":[],"editorialNote":"","failedWorkflow":false,"files":[{"id":93664461,"identity":"a47c405e-9ec4-48d2-ada8-fe18d23a79e9","added_by":"auto","created_at":"2025-10-16 08:51:37","extension":"docx","order_by":0,"title":"","display":"","copyAsset":false,"role":"acdc-reference","size":44971,"visible":true,"origin":"","legend":"","description":"","filename":"BAV.docx","url":"https://assets-eu.researchsquare.com/files/rs-7718515/v1/b2d32386c87c0e820bca7f97.docx"},{"id":93664463,"identity":"25f07033-8a35-4609-9cf3-66d133993c49","added_by":"auto","created_at":"2025-10-16 08:51:37","extension":"json","order_by":1,"title":"","display":"","copyAsset":false,"role":"acdc-reference","size":5586,"visible":true,"origin":"","legend":"","description":"","filename":"8ed85c3d6f1c403bace927628965d4ff.json","url":"https://assets-eu.researchsquare.com/files/rs-7718515/v1/3da64881cc2dac605f89f7c4.json"},{"id":93664464,"identity":"cf45d1b3-b9eb-48d1-a02e-4800162591b7","added_by":"auto","created_at":"2025-10-16 08:51:37","extension":"xml","order_by":2,"title":"","display":"","copyAsset":false,"role":"acdc-reference","size":50541,"visible":true,"origin":"","legend":"","description":"","filename":"8ed85c3d6f1c403bace927628965d4ff1enriched.xml","url":"https://assets-eu.researchsquare.com/files/rs-7718515/v1/fb57d29fed65b011bbd158d1.xml"},{"id":93664462,"identity":"10ae1aff-d959-43b8-a227-60cd9d971e65","added_by":"auto","created_at":"2025-10-16 08:51:37","extension":"xml","order_by":3,"title":"","display":"","copyAsset":false,"role":"acdc-reference","size":49233,"visible":true,"origin":"","legend":"","description":"","filename":"8ed85c3d6f1c403bace927628965d4ff1structuring.xml","url":"https://assets-eu.researchsquare.com/files/rs-7718515/v1/05e8a2ce8490678b49d16b6e.xml"},{"id":93664465,"identity":"bbdcb372-0159-42b3-bfd3-9f0495ae5c7d","added_by":"auto","created_at":"2025-10-16 08:51:37","extension":"html","order_by":4,"title":"","display":"","copyAsset":false,"role":"acdc-reference","size":55472,"visible":true,"origin":"","legend":"","description":"","filename":"earlyproof.html","url":"https://assets-eu.researchsquare.com/files/rs-7718515/v1/ed9938d3056c2bcc4f083310.html"},{"id":94474382,"identity":"1c92bc21-78e2-4f6f-bf55-e94a0c23927c","added_by":"auto","created_at":"2025-10-27 15:48:44","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":371537,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-7718515/v1/17316781-8087-4274-84e6-4f1866190ae1.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"Bicuspid Aortic Valve Leaflet Morphology in Patients With and Without Coarctation of the Aorta","fulltext":[{"header":"Introduction","content":"\u003cp\u003eBicuspid aortic valve (BAV) is a congenital heart defect affecting around 1\u0026ndash;2% of the population. This condition is linked to a higher likelihood of developing issues related to the aortic valve in the future (\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e, \u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e). Approximately 95% of BAVs include a third rudimentary commissure, commonly known as a raphe, which can vary in height. Functionally, considerable aortic stenosis often occurs between the ages of 50 and 60 years, whereas regurgitation tends to develop around the age of 30 years (\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e, \u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e). Crucially, BAV is not limited to a singular disease entity. Instead, it entails several disorders that differ in genetic and molecular aspects, resulting in a diverse range of alterations in the development of the heart and aorta (\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e, \u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e).\u003c/p\u003e\u003cp\u003eCoarctation of the aorta (CoA) is well-acknowledged to occur concurrently in many of these patients. Before the development of repair techniques, aortic dissection accounted for 19% of deaths among individuals with coarctation of the aorta. This risk was notably higher in those with a bicuspid aortic valve (BAV) (\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e). The prevalence of aortic coarctation of the aorta in patients with BAV varies between 22% and 36%, depending on the age groups investigated. Nevertheless, the incidence of aortopathy in patients with BAV who also have a coarctation of aorta (cBAV) is currently unknown (\u003cspan additionalcitationids=\"CR7\" citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e).\u003c/p\u003e\u003cp\u003eThe present study aimed to compare the bicuspid aortic morphology in patients with and without the coarctation of the aorta. Because some patients have a poor view and diagnosis of the coarctation in inexperience people are so hard so we want to study if the morphology of the aorta was helpful to possible prediction of the presence of coarctation or not.\u003c/p\u003e"},{"header":"Methods and materials","content":"\u003cdiv id=\"Sec3\" class=\"Section2\"\u003e\u003ch2\u003eStudy design\u003c/h2\u003e\u003cp\u003ePatients over 17 years old who had been referred to Ghaem Hospital for echocardiography during two years and who had bicuspid aortic valve were included in the study. According to the latest guidelines, the echocardiographic studies were conducted with patients in the left lateral position using Siemens and Philips devices and a 2.5-4 MHz probe. Aortic morphology was observed during systole, and in the case of a fusion between left coronary and right coronary cusps, the patient was labeled as anteroposterior fusion, and in the case of fusion between right coronary and non-coronary cusps or left coronary and non-coronary cusps, the patient was labeled as mediolateral fusion.\u003c/p\u003e\u003cp\u003eBefore do the echocardiography whole physical exam was done for all patients including; synchronous pulses of radial and femoral artery and rediofemoral delay was assessed also systolic blood pressure of upper limb and lower limb was measured. For the purpose of assessing the coarctation of the aorta, the patients were examined from the suprasternal view. The presence of the coarctation of the aorta was defined as a peak aortic gradient greater than 20 mmHg; in case necessary, other modalities such as CT angiography were used. In cases that have typical finding of coarctation in echocardiography (such as peak gradient more than 20 mmHg and other typical findings) we confirm our data with CT angiography. Patients with a previous history of surgery due to coarctation of the aorta or stent implantation who had no aortic valve replacement were not excluded from the study since a surgery or stent implantation would not change the artic morphology.\u003c/p\u003e\u003c/div\u003e\u003cdiv id=\"Sec4\" class=\"Section2\"\u003e\u003ch2\u003eStatistical analysis\u003c/h2\u003e\u003cp\u003eThe continuous variables exhibited a non-parametric distribution and were therefore presented as median and interquartile range (IQR), whereas categorical data were provided as frequency and percentage. The Mann-Whitney U test was used to compare groups based on continuous data, whereas Fischer's exact tests were used for categorical variables. Post hoc analyses were conducted using multiple Fischer's exact tests with Bonferroni correction. A significance level of p\u0026thinsp;\u0026lt;\u0026thinsp;0.05 was deemed statistically significant. The statistical analyses were conducted using SPSS V.25.0. The data registry of the echocardiography department at Mashhad University of Medical Sciences was utilized for data collection\u003c/p\u003e\u003c/div\u003e\n\u003ch3\u003eEthics\u003c/h3\u003e\n\u003cp\u003e The Ethics Committee of Mashhad University of Medical Sciences approved the present study (IR.MUMS.fm.REC.1395.262).\u003c/p\u003e"},{"header":"Results","content":"\u003cp\u003eIn the examined samples, 104 patients (77.6%) were men, and 30 patients (22.4%) were women. Fifteen (11.2%) were smokers, and 7 (5.2%) were diabetic; fourteen (10.4%) had hyperlipidemia, and seven patients (5.2%) had ischemic heart disease.\u003c/p\u003e\u003cp\u003eComparing the two morphological groups regarding gender, 58.7% of men had an anteroposterior valve, and 41.3% had a mediolateral valve (p-value: 0.42). 66.7% of women had an anteroposterior valve, and 33.3% had a mediolateral valve. Also, the comparison of two groups with and without coarctation of the aorta in terms of sex showed that 44.2% of men had coarctation of the aorta Furthermore, 53.3% of women had coarctation of aorta (p-value: 0.378) (Table\u0026nbsp;\u003cspan refid=\"Tab1\" class=\"InternalRef\"\u003e1\u003c/span\u003e).\u003c/p\u003e\u003cp\u003e\u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"Yes\" id=\"Tab1\" border=\"1\"\u003e\u003ccaption language=\"En\"\u003e\u003cdiv class=\"CaptionNumber\"\u003eTable 1\u003c/div\u003e\u003cdiv class=\"CaptionContent\"\u003e\u003cp\u003eThe association between sex and aortic morphology and presence of coarctation\u003c/p\u003e\u003c/div\u003e\u003c/caption\u003e\u003ccolgroup cols=\"5\"\u003e\u003cdiv align=\"left\" class=\"colspec\" colname=\"c1\" colnum=\"1\"\u003e\u003c/div\u003e\u003cdiv align=\"left\" class=\"colspec\" colname=\"c2\" colnum=\"2\"\u003e\u003c/div\u003e\u003cdiv align=\"left\" class=\"colspec\" colname=\"c3\" colnum=\"3\"\u003e\u003c/div\u003e\u003cdiv align=\"char\" char=\".\" class=\"colspec\" colname=\"c4\" colnum=\"4\"\u003e\u003c/div\u003e\u003cdiv align=\"char\" char=\".\" class=\"colspec\" colname=\"c5\" colnum=\"5\"\u003e\u003c/div\u003e\u003cthead\u003e\u003ctr\u003e\u003cth align=\"left\" colspan=\"4\" nameend=\"c4\" namest=\"c1\"\u003e\u0026nbsp;\u003c/th\u003e\u003cth align=\"left\" colname=\"c5\"\u003e\u003cp\u003ep-value\u003c/p\u003e\u003c/th\u003e\u003c/tr\u003e\u003c/thead\u003e\u003ctbody\u003e\u003ctr\u003e\u003ctd align=\"left\" colspan=\"2\" nameend=\"c2\" namest=\"c1\"\u003e\u0026nbsp;\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003eMale\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u003cp\u003eFemale\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\" morerows=\"1\" rowspan=\"2\"\u003e\u003cp\u003eMorphology\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003eAnteroposterior\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e61 (58.7)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e20 (66.7)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c5\" morerows=\"1\" rowspan=\"2\"\u003e\u003cp\u003e0.429\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003eMediolateral\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e43 (41.3)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e10 (33.3)\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\" morerows=\"1\" rowspan=\"2\"\u003e\u003cp\u003eCoarctation of aorta\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003eYes\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e46 (44.2)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e16 (53.3)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c5\" morerows=\"1\" rowspan=\"2\"\u003e\u003cp\u003e0.378\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003eNo\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e58 (55.8)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e14 (46.7)\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003c/tbody\u003e\u003c/colgroup\u003e\u003c/table\u003e\u003c/div\u003e\u003c/p\u003e\u003cp\u003eOut of 81 patients with anteroposterior morphology (60.4% of the total), 43 patients (53.0%) had coarctation of aorta. In the mediolateral morphology group with 53 patients (39.6% of the total), 19 (35.8%) had coarctation of aorta. There was a significant difference between the two morphology groups regarding the coarctation of the aorta (p-value: 0.049) (Table\u0026nbsp;\u003cspan refid=\"Tab2\" class=\"InternalRef\"\u003e2\u003c/span\u003e).\u003c/p\u003e\u003cp\u003eIn the other word, of 62 patients with coarctation of the aorta, 69.4% had an anteroposterior valve, and 30.6% had a mediolateral valve. In the group of patients without coarctation of the aorta, 52.8% had an anteroposterior valve, and 47.2% had a mediolateral valve. (Table\u0026nbsp;\u003cspan refid=\"Tab2\" class=\"InternalRef\"\u003e2\u003c/span\u003e)\u003c/p\u003e\u003cp\u003eAmong patients with anteroposterior morphology, 12.5% had no aortic regurgitation, 37.5% had mild regurgitation, 32.5% had moderate regurgitation, and 17.5% had severe regurgitation. The corresponding proportions in the mediolateral morphology group were 7.5%, 35.8%, 35.8%, and 20.8%, respectively. There were no significant differences between the groups in the frequency of aortic regurgitation (p-value: 0.789). (Table\u0026nbsp;\u003cspan refid=\"Tab2\" class=\"InternalRef\"\u003e2\u003c/span\u003e)\u003c/p\u003e\u003cp\u003eIn the anteroposterior group, 38.6% had no aortic stenosis, 6.8% had mild aortic stenosis, 6.8% had moderate aortic stenosis, and 7.6% had severe aortic stenosis. In the group with mediolateral morphology, 22% had no aortic stenosis, 8.3% had mild aortic stenosis, 2.3% had moderate aortic stenosis, and 7.6% had severe aortic stenosis. No significant differences were observed in the frequency of aortic stenosis between the groups (p-value: 0.231). About 47.4% of patients with anteroposterior valve had dilated aorta, and 52.6% of them did not have dilated aorta. 46.2% of patients with mediolateral aortic valves had dilated aorta (p-value: 0.886) (Table\u0026nbsp;\u003cspan refid=\"Tab2\" class=\"InternalRef\"\u003e2\u003c/span\u003e).\u003c/p\u003e\u003cp\u003e\u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"Yes\" id=\"Tab2\" border=\"1\"\u003e\u003ccaption language=\"En\"\u003e\u003cdiv class=\"CaptionNumber\"\u003eTable 2\u003c/div\u003e\u003cdiv class=\"CaptionContent\"\u003e\u003cp\u003eThe association between aortic morphology and aortic regurgitation, stenosis, and dilation\u003c/p\u003e\u003c/div\u003e\u003c/caption\u003e\u003ccolgroup cols=\"5\"\u003e\u003cdiv align=\"left\" class=\"colspec\" colname=\"c1\" colnum=\"1\"\u003e\u003c/div\u003e\u003cdiv align=\"left\" class=\"colspec\" colname=\"c2\" colnum=\"2\"\u003e\u003c/div\u003e\u003cdiv align=\"left\" class=\"colspec\" colname=\"c3\" colnum=\"3\"\u003e\u003c/div\u003e\u003cdiv align=\"char\" char=\".\" class=\"colspec\" colname=\"c4\" colnum=\"4\"\u003e\u003c/div\u003e\u003cdiv align=\"left\" class=\"colspec\" colname=\"c5\" colnum=\"5\"\u003e\u003c/div\u003e\u003cthead\u003e\u003ctr\u003e\u003cth align=\"left\" colspan=\"2\" nameend=\"c2\" namest=\"c1\"\u003e\u0026nbsp;\u003c/th\u003e\u003cth align=\"left\" colname=\"c3\"\u003e\u003cp\u003eAnteroposterior\u003c/p\u003e\u003c/th\u003e\u003cth align=\"left\" colname=\"c4\"\u003e\u003cp\u003eMediolateral\u003c/p\u003e\u003c/th\u003e\u003cth align=\"left\" colname=\"c5\"\u003e\u003cp\u003ep-value\u003c/p\u003e\u003c/th\u003e\u003c/tr\u003e\u003c/thead\u003e\u003ctbody\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\" morerows=\"1\" rowspan=\"2\"\u003e\u003cp\u003eCoarctation\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003eYes\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e43 (53%)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e19 (35.8%)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c5\" morerows=\"1\" rowspan=\"2\"\u003e\u003cp\u003e0.049\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003eNo\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e38 (46.91%)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e34 (64.1%)\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\" morerows=\"3\" rowspan=\"4\"\u003e\u003cp\u003eAortic regurgitation\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003eNone\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e10 (12.3%)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e4 (7.5)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c5\" morerows=\"3\" rowspan=\"4\"\u003e\u003cp\u003e0.789\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003eMild\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e31(38.2%)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e19 (35.8)\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003eModerate\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e26 (32%)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e19 (35.8)\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003eSevere\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e14 (17.2%)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e11 (20.7)\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\" morerows=\"3\" rowspan=\"4\"\u003e\u003cp\u003eAortic stenosis\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003eNone\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e51 (62.9%)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e29 (54.7%)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c5\" morerows=\"3\" rowspan=\"4\"\u003e\u003cp\u003e0.231\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003eMild\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e11(13.5%)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e11 (20.7%)\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003eModerate\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e9 (11.1%)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e3 (5.6%)\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003eSevere\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e10 (12.3%)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e10 (18.8%)\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\" morerows=\"1\" rowspan=\"2\"\u003e\u003cp\u003eAortic dilation\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003eYes\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e39(48.1%)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e25 (52.8)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c5\" morerows=\"1\" rowspan=\"2\"\u003e\u003cp\u003e0.886\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003eNo\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e42 (51.8%)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e28 (52.8)\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003c/tbody\u003e\u003c/colgroup\u003e\u003c/table\u003e\u003c/div\u003e\u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003eAlthough the association between bicuspid aortic valve (BAV) and coarctation of the aorta (CoA) is well-established, prior CoA repair has generally had minimal impact on the management of BAV itself. The significance of the concurrent presence of these two conditions remains incompletely understood. In this study, we sought to investigate whether there is a meaningful relationship between the morphology of the bicuspid aortic valve and the presence of CoA, as understanding this association could have important implications for risk stratification, surveillance, and clinical management. Our aim was to determine if certain BAV morphologies are more frequently associated with CoA, which might explain differences in patient outcomes or the development of related complications. The results showed that the frequency of CoA differed significantly between BAV morphology groups; however, no significant differences were found regarding aortic regurgitation, stenosis, or dilation. Notably, increased blood pressure was more common among patients with CoA compared to those without, highlighting the hemodynamic implications of this coexisting condition.\u003c/p\u003e\u003cp\u003eOur research has indicated that BAV is typically associated with other non-valvular cardiovascular anomalies, including CoA, coronary artery anomalies, sinus of Valsalva aneurysm, aortic aneurysm, aortic dissection, supravalvular aortic stenosis, patent ductus arteriosus, ventricular septal defect, Shone complex, familial aneurysm syndromes, thoracic aortic dissection, and Turner syndrome (\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e). Following this, the present study discovered that the most common complications that presented in BAV patients were CoA, aortic regurgitation, stenosis, and dilation. Previous studies have shown aortic stenosis to be more common among the anteroposterior (AP) BAV subtype (\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e, \u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e). Although a higher number of aortic stenosis cases in our cohort had an AP BAV phenotype, the difference was not statistically significant. Recent data further indicate that abnormal blood flow patterns associated with valve morphology not only predispose AP-fused valves to earlier-onset aortic stenosis, but also to accelerated dilation of the ascending aorta\u0026mdash;seen in up to 60\u0026ndash;80% of BAV patients\u0026mdash;through combined genetic and hemodynamic mechanisms (\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e). These findings support the need for close longitudinal surveillance of valve function and aortic dimensions, especially in AP morphological subtypes.\u003c/p\u003e\u003cp\u003eOur research has indicated that BAV is typically associated with other non-valvular cardiovascular anomalies, including CoA, coronary artery anomalies, sinus of Valsalva aneurysm, aortic aneurysm, aortic dissection, supravalvular aortic stenosis, patent ductus arteriosus, ventricular septal defect, Shone complex, familial aneurysm syndromes, thoracic aortic dissection, and Turner syndrome (\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e). In line with this, the present study found that the most common complications observed in patients with BAV were CoA, aortic regurgitation, stenosis, and dilation. Previous studies have shown aortic stenosis to be more common among patients with the anteroposterior (AP) BAV subtype (\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e, \u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e). Although a higher number of aortic stenosis cases in our cohort had an AP BAV phenotype, the difference was not statistically significant. Recent data further indicate that abnormal blood flow patterns associated with valve morphology not only predispose AP-fused valves to earlier-onset aortic stenosis, but also to accelerated dilation of the ascending aorta\u0026mdash;seen in up to 60\u0026ndash;80% of BAV patients\u0026mdash;through combined genetic and hemodynamic mechanisms (\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e, \u003cspan additionalcitationids=\"CR13\" citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e). These mechanisms involve both inherited factors and abnormal flow dynamics that create asymmetrical shear stress on the aortic wall, leading to distinct segmental dilation patterns depending on BAV phenotype. Understanding these underlying processes highlights the importance of close longitudinal surveillance of valve function and aortic dimensions, particularly in patients with the AP subtype.\u003c/p\u003e\u003cp\u003eThe present study showed that the anteroposterior (AP) and mediolateral (ML) phenotypes of BAV accounted for 60% and 40% of cases, respectively. Most previous studies have reported the predominance of the AP subtype, except for a few outliers (\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e). Additionally, our findings demonstrated that these morphological subtypes were not significantly associated with gender, which is consistent with prior research (\u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e). A study by Tabrizi et al. in 2018, which evaluated 300 adults with BAV, similarly reported that the AP morphology was significantly more common than the ML subtype among patients with coarctation of the aorta, aligning with our results (\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e). In our study, the AP morphology was more frequent among patients with CoA (53%) compared to those without CoA (46.9%), while the ML subtype was less common in patients with CoA (35.8%) compared to those without (64.1%). This difference was statistically significant (p\u0026thinsp;=\u0026thinsp;0.049), indicating that CoA tends to occur more frequently in patients with an AP BAV phenotype. These findings further underscore the importance of considering valve morphology when assessing the risk of associated cardiovascular anomalies in patients with BAV.\u003c/p\u003e\u003cp\u003eThe present study had some limitations. Given the rarity of coarctation of aorta, the present study found a limited number of events, necessitating the results to be interpreted cautiously. Furthermore, the size of the ascending aorta is dependent of age, which could impact the results in a small sample size. Also, the individuals included in our study were patients who were receiving medical attention at a specialized facility. As a result, the findings of our study may not apply to a broader community.\u003c/p\u003e\u003cp\u003eIn conclusion, the results of the present study showed that coarctation of the aorta was more frequently observed among those with AP BAV phenotype. Furthermore, increased blood pressure was more commonly observed among those with coarctation of aorta.\u003c/p\u003e"},{"header":"Declarations","content":"\u003ch2\u003eEthics approval and consent to participate\u003c/h2\u003e\n\u003cp\u003eThis study was approved by the Ethics Committee of Mashhad University of Medical Sciences (approval code: IR.MUMS.fm.REC.1395.262). Written informed consent was obtained from all individual participants included in the study.\u003c/p\u003e\n\u003ch2\u003e\u003cstrong\u003eConsent for publication\u003c/strong\u003e\u003c/h2\u003e\n\u003cp\u003eNot applicable.\u003c/p\u003e\n\u003ch2\u003eFunding\u003c/h2\u003e\n\u003cp\u003eThe authors received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.\u003c/p\u003e\n\u003ch2\u003eAuthor Contribution\u003c/h2\u003e\n\u003cp\u003eL.B, Z.O and R.H wrote the main manuscript text and created tables and figures. All authors reviewed the manuscript\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\n\u003cli\u003eBraverman AC, G\u0026uuml;ven H, Beardslee MA, Makan M, Kates AM, Moon MR. The bicuspid aortic valve. Current problems in cardiology. 2005;30(9):470-522.\u003c/li\u003e\n\u003cli\u003eRinnstr\u0026ouml;m D, Engstr\u0026ouml;m KG, Johansson B. Subtypes of bicuspid aortic valves in coarctation of the aorta. Heart and vessels. 2014;29:354-63.\u003c/li\u003e\n\u003cli\u003eMasri A, Kalahasti V, Alkharabsheh S, Svensson LG, Sabik JF, Roselli EE, et al. Characteristics and long-term outcomes of contemporary patients with bicuspid aortic valves. The Journal of thoracic and cardiovascular surgery. 2016;151(6):1650-9. e1.\u003c/li\u003e\n\u003cli\u003eMichelena HI, Desjardins VA, Avierinos J-F, Russo A, Nkomo VT, Sundt TM, et al. Natural history of asymptomatic patients with normally functioning or minimally dysfunctional bicuspid aortic valve in the community. Circulation. 2008;117(21):2776-84.\u003c/li\u003e\n\u003cli\u003eLim MS, Bannon PG, Celermajer DS. Bicuspid aortic valve: different clinical profiles for subjects with versus without repaired aortic coarctation. Open Heart. 2020;7(2):e001429.\u003c/li\u003e\n\u003cli\u003eMerkx R, Duijnhouwer AL, Vink E, Roos-Hesselink JW, Schokking M. Aortic diameter growth in children with a bicuspid aortic valve. The American journal of cardiology. 2017;120(1):131-6.\u003c/li\u003e\n\u003cli\u003eCiotti GR, Vlahos AP, Silverman NH. Morphology and function of the bicuspid aortic valve with and without coarctation of the aorta in the young. The American journal of cardiology. 2006;98(8):1096-102.\u003c/li\u003e\n\u003cli\u003eOliver JM, Alonso-Gonzalez R, Gonzalez AE, Gallego P, Sanchez-Recalde A, Cuesta E, et al. Risk of aortic root or ascending aorta complications in patients with bicuspid aortic valve with and without coarctation of the aorta. The American journal of cardiology. 2009;104(7):1001-6.\u003c/li\u003e\n\u003cli\u003eRodr\u0026iacute;guez-Palomares JF, Dux-Santoy L, Guala A, Galian-Gay L, Evangelista A. Mechanisms of aortic dilation in patients with bicuspid aortic valve: JACC state-of-the-art review. Journal of the American College of Cardiology. 2023;82(5):448-64.\u003c/li\u003e\n\u003cli\u003eKim YG, Sun BJ, Park GM, Han S, Kim DH, Song JM, et al. Aortopathy and bicuspid aortic valve: haemodynamic burden is main contributor to aortic dilatation. Heart. 2012;98(24):1822-7.\u003c/li\u003e\n\u003cli\u003eMoaref A, Khavanin M, Shekarforoush S. Aortic distensibility in bicuspid aortic valve patients with normal aortic diameter. Therapeutic advances in cardiovascular disease. 2014;8(4):128-32.\u003c/li\u003e\n\u003cli\u003eGirdauskas E, Schulz S, Borger MA, Mierzwa M, Kuntze T. Transforming growth factor-beta receptor type II mutation in a patient with bicuspid aortic valve disease and intraoperative aortic dissection. Ann Thorac Surg. 2011;91(5):e70-1.\u003c/li\u003e\n\u003cli\u003ePrakash SK, Boss\u0026eacute; Y, Muehlschlegel JD, Michelena HI, Limongelli G, Della Corte A, et al. A roadmap to investigate the genetic basis of bicuspid aortic valve and its complications: insights from the International BAVCon (Bicuspid Aortic Valve Consortium). J Am Coll Cardiol. 2014;64(8):832-9.\u003c/li\u003e\n\u003cli\u003eToufan Tabrizi M, Rahimi Asl R, Nazarnia S, Pourafkari L. Evaluation of relationship between bicuspid aortic valve phenotype with valve dysfunction and associated aortopathy. J Cardiovasc Thorac Res. 2018;10(4):236-42.\u003c/li\u003e\n\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":true,"highlight":"","institution":"","isAcceptedByJournal":false,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true},"keywords":"bicuspid aortic valve, coarctation of aorta, aortic stenosis, aortic insufficiency","lastPublishedDoi":"10.21203/rs.3.rs-7718515/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-7718515/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003e\u003cem\u003eIntroduction\u003c/em\u003e: Bicuspid aortic valve (BAV) is the most common congenital heart defect. BAV is often seen with other left-sided structural abnormalities, such as coarctation of the aorta. Furthermore, diagnosing coarctation of the aorta can sometimes be challenging\u003c/p\u003e\u003cp\u003ewith transthoracic echocardiography. This study aimed to assess if the presence of coarctation of the aorta is associated with a specific BAV morphology.\u003c/p\u003e\u003cp\u003e\u003cem\u003eMethods and materials\u003c/em\u003e: All patients referred to Ghaem Mashhad Hospital for echocardiography were included in the study if they had a BAV. The morphology of the aortic valve was classified based on the fusion of the cusps (anteroposterior opening and mediolateral opening), and the existence of coarctation of aorta was determined based on the findings in echocardiography or the CT angiography. Then, the data were compared in two groups.\u003c/p\u003e\u003cp\u003e\u003cem\u003eResults\u003c/em\u003e: Out of 134 patients (104 men and 30 women), 81 had anteroposterior morphology, and 53 had mediolateral morphology. Among the patients with anteroposterior morphology, 43 patients (69.4%) had coarctation of aorta, and 38 patients (52.8%) had no coarctation of aorta. Among patients with mediolateral morphology, 19 (30.6%) were diagnosed with coarctation of the aorta, while 34 (47.2%) showed no evidence of coarctation of the aorta (p\u0026thinsp;=\u0026thinsp;0.049).\u003c/p\u003e\u003cp\u003e\u003cem\u003eConclusion\u003c/em\u003e The present study showed that the morphology of the aortic valve contribute to predicting the presence of coarctation of the aorta.\u003c/p\u003e","manuscriptTitle":"Bicuspid Aortic Valve Leaflet Morphology in Patients With and Without Coarctation of the Aorta","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2025-10-16 08:51:32","doi":"10.21203/rs.3.rs-7718515/v1","editorialEvents":[{"type":"communityComments","content":0}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"c5233e9d-8206-465a-8d39-b5dd0f09b641","owner":[],"postedDate":"October 16th, 2025","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[],"tags":[],"updatedAt":"2025-10-27T14:33:12+00:00","versionOfRecord":[],"versionCreatedAt":"2025-10-16 08:51:32","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-7718515","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-7718515","identity":"rs-7718515","version":["v1"]},"buildId":"8U1c8b4HqxoKbykW_rLl7","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}

Text is read by the "Ask this paper" AI Q&A widget below. Extraction quality varies by source — PMC NXML preserves structure cleanly, OA-HTML may include some navigation residue, and OA-PDF can have broken hyphenation. The publisher copy (via DOI) is the canonical version.

My notes (saved in your browser only)

Ask this paper AI returns verbatim quotes from the full text · source: preprint-html

Answers must be backed by verbatim quotes from this paper's full text. Hallucinated quotes are dropped automatically; if no verbatim passage answers the question, we say so. How this works

Citation neighborhood (no data yet)

We don't have any in-corpus citations linked to this paper yet. This is a recent paper (2025) — citers typically take a year or two to land, and the OpenAlex reference graph may still be filling in.

Source provenance

europepmc
last seen: 2026-05-20T01:45:00.602351+00:00