{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,28]],"date-time":"2026-09-28T15:58:05Z","timestamp":1790611085060,"version":"4.1.0"},"reference-count":84,"publisher":"MDPI AG","issue":"9","license":[{"start":{"date-parts":[[2023,9,18]],"date-time":"2023-09-18T00:00:00Z","timestamp":1694995200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCDD"],"abstract":"<jats:p>Objective: bicuspid aortic valve (BAV) stands as the most prevalent congenital heart condition intricately linked to aortic pathologies encompassing aortic regurgitation (AR), aortic stenosis, aortic root dilation, and aortic dissection. The aetiology of BAV is notably intricate, involving a spectrum of genes and polymorphisms. Moreover, BAV lays the groundwork for an array of structural heart and aortic disorders, presenting varying degrees of severity. Establishing a tailored clinical approach amid this diverse range of BAV-related conditions is of utmost significance. In this comprehensive review, we delve into the epidemiology, aetiology, associated ailments, and clinical management of BAV, encompassing imaging to aortic surgery. Our exploration is guided by the perspectives of the aortic team, spanning six distinct guidelines. Methods: We conducted an exhaustive search across databases like PubMed, Ovid, Scopus, and Embase to extract relevant studies. Our review incorporates 84 references and integrates insights from six different guidelines to create a comprehensive clinical management section. Results: BAV presents complexities in its aetiology, with specific polymorphisms and gene disorders observed in groups with elevated BAV prevalence, contributing to increased susceptibility to other cardiovascular conditions. The altered hemodynamics inherent to BAV instigate adverse remodelling of the aorta and heart, thus fostering the development of epigenetically linked aortic and heart diseases. Employing TTE screening for first-degree relatives of BAV patients might be beneficial for disease tracking and enhancing clinical outcomes. While SAVR is the primary recommendation for indicated AVR in BAV, TAVR might be an option for certain patients endorsed by adept aortic teams. In addition, proficient teams can perform aortic valve repair for AR cases. Aortic surgery necessitates personalized evaluation, accounting for genetic makeup and risk factors. While the standard aortic replacement threshold stands at 55 mm, it may be tailored to 50 mm or even 45 mm based on patient-specific considerations. Conclusion: This review reiterates the significance of considering the multifactorial nature of BAV as well as the need for further research to be carried out in the field.<\/jats:p>","DOI":"10.3390\/jcdd10090398","type":"journal-article","created":{"date-parts":[[2023,9,18]],"date-time":"2023-09-18T02:17:30Z","timestamp":1695003450000},"page":"398","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["A Comprehensive Review of Management Strategies for Bicuspid Aortic Valve (BAV): Exploring Epidemiology, Aetiology, Aortopathy, and Interventions in Light of Recent Guidelines"],"prefix":"10.3390","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9076-8296","authenticated-orcid":false,"given":"Halil Ibrahim","family":"Bulut","sequence":"first","affiliation":[{"name":"Cerrahpasa School of Medicine, Istanbul University-Cerrahpasa, Istanbul 34098, Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4931-4049","authenticated-orcid":false,"given":"Arian","family":"Arjomandi Rad","sequence":"additional","affiliation":[{"name":"Medical Sciences Division, University of Oxford, Oxford OX1 3AZ, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0973-2083","authenticated-orcid":false,"given":"Angeliki-Aikaterini","family":"Syrengela","sequence":"additional","affiliation":[{"name":"School of Medicine of Crete, 71500 Heraklion, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5857-5841","authenticated-orcid":false,"given":"Iakovos","family":"Ttofi","sequence":"additional","affiliation":[{"name":"Department of Cardiothoracic Surgery, Oxford University Hospital NHS Foundation Trust, Oxford OX3 9DU, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jasmina","family":"Djordjevic","sequence":"additional","affiliation":[{"name":"Department of Cardiothoracic Surgery, Oxford University Hospital NHS Foundation Trust, Oxford OX3 9DU, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ramanjit","family":"Kaur","sequence":"additional","affiliation":[{"name":"Department of Cardiothoracic Surgery, Oxford University Hospital NHS Foundation Trust, Oxford OX3 9DU, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amar","family":"Keiralla","sequence":"additional","affiliation":[{"name":"Department of Cardiothoracic Surgery, Oxford University Hospital NHS Foundation Trust, Oxford OX3 9DU, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9334-9604","authenticated-orcid":false,"given":"George","family":"Krasopoulos","sequence":"additional","affiliation":[{"name":"Department of Cardiothoracic Surgery, Oxford University Hospital NHS Foundation Trust, Oxford OX3 9DU, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,9,18]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"126","DOI":"10.1136\/heartjnl-2013-304920","article-title":"Aortic dilatation patterns and rates in adults with bicuspid aortic valves: A comparative study with Marfan syndrome and degenerative aortopathy","volume":"100","author":"Detaint","year":"2014","journal-title":"Heart"},{"key":"ref_2","doi-asserted-by":"crossref","unstructured":"Aluru, J.S., Barsouk, A., Saginala, K., Rawla, P., and Barsouk, A. (2022). Valvular Heart Disease Epidemiology. Med. Sci., 10.","DOI":"10.3390\/medsci10020032"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"478","DOI":"10.3934\/molsci.2017.4.478","article-title":"Molecular insights into bicuspid aortic valve development and the associated aortopathy","volume":"4","author":"Bolar","year":"2017","journal-title":"AIMS Mol. Sci."},{"key":"ref_4","unstructured":"Kodolitsch, Y.V., and Kaemmerer, H. (2017). Aortopathy, Springer."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"2691","DOI":"10.1161\/CIRCULATIONAHA.113.007851","article-title":"Bicuspid aortic valve: Identifying knowledge gaps and rising to the challenge from the International Bicuspid Aortic Valve Consortium (BAVCon)","volume":"129","author":"Michelena","year":"2014","journal-title":"Circulation"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"2363","DOI":"10.1016\/j.jacc.2015.03.575","article-title":"Long-Term Risk for Aortic Complications After Aortic Valve Replacement in Patients With Bicuspid Aortic Valve Versus Marfan Syndrome","volume":"65","author":"Itagaki","year":"2015","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_7","doi-asserted-by":"crossref","unstructured":"Junco-Vicente, A., Del R\u00edo-Garc\u00eda, \u00c1., Mart\u00edn, M., and Rodr\u00edguez, I. (2021). Update in Biomolecular and Genetic Bases of Bicuspid Aortopathy. Int. J. Mol. Sci., 22.","DOI":"10.3390\/ijms22115694"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"355","DOI":"10.1093\/icvts\/ivt518","article-title":"Risk of proximal aortic dissection in patients with bicuspid aortic valve: How to address this controversy?","volume":"18","author":"Girdauskas","year":"2014","journal-title":"Interact. Cardiovasc. Thorac. Surg."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"1321","DOI":"10.1016\/j.jcmg.2013.08.009","article-title":"Aortic surgery for ascending aortic aneurysms under 5.0 cm in diameter in the presence of bicuspid aortic valve","volume":"6","author":"Kallenbach","year":"2013","journal-title":"JACC Cardiovasc. Imaging"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"S454","DOI":"10.21037\/jtd.2017.05.11","article-title":"Bicuspid aortic valve syndrome: A multidisciplinary approach for a complex entity","volume":"9","author":"Lorca","year":"2017","journal-title":"J. Thorac. Dis."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"561","DOI":"10.1001\/jama.2020.27205","article-title":"Prevalence of bicuspid aortic valve and associated aortopathy in newborns in Copenhagen, Denmark","volume":"325","author":"Sillesen","year":"2021","journal-title":"JAMA"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"1226","DOI":"10.1016\/j.jtcvs.2007.01.039","article-title":"A classification system for the bicuspid aortic valve from 304 surgical specimens","volume":"133","author":"Sievers","year":"2007","journal-title":"J. Thorac. Cardiovasc. Surg."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"1921","DOI":"10.3389\/fphys.2018.01921","article-title":"Bicuspid aortic valve: An update in morphology, genetics, biomarker, complications, imaging diagnosis and treatment","volume":"9","author":"Liu","year":"2019","journal-title":"Front. Physiol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"180297","DOI":"10.1155\/2012\/180297","article-title":"Genetic insights into bicuspid aortic valve formation","volume":"2012","author":"Laforest","year":"2012","journal-title":"Cardiol. Res. Pract."},{"key":"ref_15","doi-asserted-by":"crossref","unstructured":"Bernard, C., Morgant, M.C., Guillier, D., Cheynel, N., and Bouchot, O. (2022). Point on the Aortic Bicuspid Valve. Life, 12.","DOI":"10.3390\/life12040518"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"398","DOI":"10.1016\/j.pcad.2020.06.005","article-title":"Genetics in bicuspid aortic valve disease: Where are we?","volume":"63","author":"Prakash","year":"2020","journal-title":"Prog. Cardiovasc. Dis."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"91","DOI":"10.1016\/j.ijcard.2022.04.022","article-title":"Heritability of aortic valve stenosis and bicuspid enrichment in families with aortic valve stenosis","volume":"359","author":"Boureau","year":"2022","journal-title":"Int. J. Cardiol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"447","DOI":"10.1093\/ehjqcco\/qcab047","article-title":"Cost-effectiveness analysis of screening for first-degree relatives of patients with bicuspid aortic valve","volume":"7","author":"Tessler","year":"2021","journal-title":"Eur. Heart J. Qual. Care Clin. Outcomes"},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"186","DOI":"10.1007\/s00246-013-0804-3","article-title":"Bicuspid aortic valve: A neglected feature of Shone\u2019s complex?","volume":"35","author":"Michelena","year":"2014","journal-title":"Pediatr. Cardiol."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"e41","DOI":"10.1016\/j.jtcvs.2018.02.115","article-title":"The American Association for Thoracic Surgery consensus guidelines on bicuspid aortic valve\u2013related aortopathy: Full online-only version","volume":"156","author":"Borger","year":"2018","journal-title":"J. Thorac. Cardiovasc. Surg."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"517","DOI":"10.1530\/EJE-12-0434","article-title":"Cardiovascular findings and management in Turner syndrome: Insights from a French cohort","volume":"167","author":"Donadille","year":"2012","journal-title":"Eur. J. Endocrinol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"2912","DOI":"10.1002\/ajmg.a.38417","article-title":"Congenital heart defects in molecularly proven Kabuki syndrome patients","volume":"173","author":"Digilio","year":"2017","journal-title":"Am. J. Med. Genet. Part A"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"80","DOI":"10.1111\/j.1399-0004.1995.tb03928.x","article-title":"Congenital Cardiac Malformations in Adams-Oliver Syndrome","volume":"47","author":"Zapata","year":"2008","journal-title":"Clin. Genet."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"1590","DOI":"10.1016\/j.jacc.2007.07.021","article-title":"Hypoplastic left heart syndrome is heritable","volume":"50","author":"Hinton","year":"2007","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"e011943","DOI":"10.1161\/JAHA.119.011943","article-title":"Thoracic aortic dissection in tetralogy of Fallot: A review of the National Inpatient Sample Database","volume":"8","author":"Egbe","year":"2019","journal-title":"J. Am. Heart Assoc."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"406","DOI":"10.1016\/j.jtcvs.2016.10.088","article-title":"Cardiovascular operations for Loeys-Dietz syndrome: Intermediate-term results","volume":"153","author":"Patel","year":"2017","journal-title":"J. Thorac. Cardiovasc. Surg."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"612","DOI":"10.3389\/fphys.2017.00612","article-title":"Genetic bases of bicuspid aortic valve: The contribution of traditional and high-throughput sequencing approaches on research and diagnosis","volume":"8","author":"Giusti","year":"2017","journal-title":"Front. Physiol."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"939","DOI":"10.1002\/ajmg.a.32770","article-title":"Aortic root dilation in patients with 22q11.2 deletion syndrome","volume":"149A","author":"John","year":"2009","journal-title":"Am. J. Med. Genet. A"},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"e006906","DOI":"10.1161\/JAHA.117.006906","article-title":"Advances in the understanding of the genetic determinants of congenital heart disease and their impact on clinical outcomes","volume":"7","author":"Russell","year":"2018","journal-title":"J. Am. Heart Assoc."},{"key":"ref_30","doi-asserted-by":"crossref","unstructured":"Williams, K., Carson, J., and Lo, C. (2019). Genetics of Congenital Heart Disease. Biomolecules, 9.","DOI":"10.3390\/biom9120879"},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"101949","DOI":"10.1016\/j.gde.2022.101949","article-title":"Molecular Genetic Mechanisms of Congenital Heart Disease","volume":"75","author":"Choudhury","year":"2022","journal-title":"Curr. Opin. Genet. Dev."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"42","DOI":"10.1038\/s41588-018-0265-y","article-title":"ROBO4 variants predispose individuals to bicuspid aortic valve and thoracic aortic aneurysm","volume":"51","author":"Gould","year":"2019","journal-title":"Nat. Genet."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"211","DOI":"10.1038\/pr.2014.67","article-title":"Rare GATA5 sequence variants identified in individuals with bicuspid aortic valve","volume":"76","author":"Bonachea","year":"2014","journal-title":"Pediatr. Res."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"277","DOI":"10.1016\/j.yjmcc.2012.05.009","article-title":"Rare non-synonymous variations in the transcriptional activation domains of GATA5 in bicuspid aortic valve disease","volume":"53","author":"Padang","year":"2012","journal-title":"J. Mol. Cell. Cardiol."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"1219","DOI":"10.3892\/ijmm.2014.1700","article-title":"GATA5 loss-of-function mutations associated with congenital bicuspid aortic valve","volume":"33","author":"Shi","year":"2014","journal-title":"Int. J. Mol. Med."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"115","DOI":"10.1016\/j.gene.2018.04.018","article-title":"GATA6 loss-of-function mutation contributes to congenital bicuspid aortic valve","volume":"663","author":"Xu","year":"2018","journal-title":"Gene"},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"469","DOI":"10.1016\/j.amjcard.2017.11.012","article-title":"Gata4 loss-of-function mutation and the congenitally bicuspid aortic valve","volume":"121","author":"Li","year":"2018","journal-title":"Am J Cardiol."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"139","DOI":"10.1055\/s-0041-1730294","article-title":"Bicuspid aortic valve: Genetic and clinical insights","volume":"9","author":"Tessler","year":"2021","journal-title":"Aorta"},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"e019006","DOI":"10.1161\/JAHA.120.019006","article-title":"Genetic Etiology of Left-Sided Obstructive Heart Lesions: A Story in Development","volume":"10","author":"Parker","year":"2021","journal-title":"J. Am. Heart Assoc."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"256","DOI":"10.1093\/qjmed\/hcab317","article-title":"Identification of a novel GATA binding protein 5 variant (c. 830C > T\/p. P277L) damaging the nuclear translocation and causing tetralogy of Fallot","volume":"115","author":"Jin","year":"2022","journal-title":"QJM Int. J. Med."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"515","DOI":"10.1038\/s10038-022-01036-x","article-title":"Identification of non-synonymous variations in ROBO1 and GATA5 genes in a family with bicuspid aortic valve disease","volume":"67","author":"Jaouadi","year":"2022","journal-title":"J. Hum. Genet."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"1114","DOI":"10.1136\/heartjnl-2021-320428","article-title":"Contribution of NOTCH1 genetic variants to bicuspid aortic valve and other congenital lesions","volume":"108","author":"Debiec","year":"2022","journal-title":"Heart"},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"1","DOI":"10.3892\/etm.2022.11240","article-title":"A novel KLF13 mutation underlying congenital patent ductus arteriosus and ventricular septal defect, as well as bicuspid aortic valve","volume":"23","author":"Abhinav","year":"2022","journal-title":"Exp. Ther. Med."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"e0035","DOI":"10.1161\/CIRCGEN.121.003523","article-title":"CELSR1 risk alleles in familial bicuspid aortic valve and hypoplastic left heart syndrome","volume":"15","author":"Theis","year":"2022","journal-title":"Circ. Genom. Precis. Med."},{"key":"ref_45","doi-asserted-by":"crossref","unstructured":"Bonachea, E.M., Zender, G., White, P., Corsmeier, D., Newsom, D., Fitzgerald-Butt, S., Garg, V., and McBride, K.L. (2014). Use of a Target-ed, Combinatorial next-Generation Sequencing Approach for the Study of Bicuspid Aortic Valve. BMC Med. Genom.","DOI":"10.1186\/1755-8794-7-56"},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"7827","DOI":"10.1002\/jcp.29436","article-title":"Epigenetic response of endothelial cells to different wall shear stress magnitudes: A report of new mechano-miRNAs","volume":"235","author":"Rashad","year":"2020","journal-title":"J. Cell. Physiol."},{"key":"ref_47","unstructured":"Cao, K., and Sucosky, P. (2014). Predictions of Wall-shear Stress on the Bicuspid Aortic Valves Using Three-dimensional Fluid-structure Interaction Models. Circulation, 130."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"1760","DOI":"10.1007\/s10439-012-0527-2","article-title":"In vitro characterization of bicuspid aortic valve hemodynamics using particle image velocimetry","volume":"40","author":"Saikrishnan","year":"2012","journal-title":"Ann. Biomed. Eng."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"e128","DOI":"10.1093\/ejcts\/ezt666","article-title":"The fate of the bicuspid valve aortopathy after aortic valve replacement","volume":"45","author":"Charitos","year":"2014","journal-title":"Eur. J. Cardio-Thorac. Surg."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"1521","DOI":"10.1016\/j.athoracsur.2021.05.078","article-title":"Outcomes of bicuspid aortic valve thoracic aorta (4.0\u20134.5 cm) after aortic valve replacement","volume":"113","author":"Longi","year":"2022","journal-title":"Ann. Thorac. Surg."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"177","DOI":"10.1016\/j.carpath.2010.12.001","article-title":"The aortic valve microenvironment and its role in calcific aortic valve disease","volume":"20","author":"Yip","year":"2011","journal-title":"Cardiovasc. Pathol."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"340","DOI":"10.1016\/j.atherosclerosis.2012.07.023","article-title":"Detection of hydroxyapatite in calcified cardiovascular tissues","volume":"224","author":"Lee","year":"2012","journal-title":"Atherosclerosis"},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"832","DOI":"10.1161\/01.CIR.0000157137.59691.0B","article-title":"The bicuspid aortic valve: Adverse outcomes from infancy to old age","volume":"111","author":"Lewin","year":"2005","journal-title":"Circulation"},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"566","DOI":"10.1136\/heartjnl-2017-311560","article-title":"Anatomical and clinical predictors of valve dysfunction and aortic dilation in bicuspid aortic valve disease","volume":"104","author":"Evangelista","year":"2018","journal-title":"Heart"},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"65","DOI":"10.1007\/s11886-019-1144-6","article-title":"Aortic regurgitation","volume":"21","author":"Flint","year":"2019","journal-title":"Curr. Cardiol. Rep."},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"1323","DOI":"10.1136\/heartjnl-2016-309916","article-title":"Contemporary natural history of bicuspid aortic valve disease: A systematic review","volume":"103","author":"Masri","year":"2017","journal-title":"Heart"},{"key":"ref_57","unstructured":"Zhu, Y., Imbrie-Moore, A.M., Paulsen, M.J., Priromprintr, B., Wang, H., Lucian, H.J., Farry, J.M., and Woo, Y.J. (2020). Novel bicuspid aortic valve model with aortic regurgitation for hemodynamic status analysis using an ex vivo simulator. J. Thorac. Cardiovasc. Surg."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"278","DOI":"10.1016\/j.echo.2021.11.012","article-title":"Aortic Root Anatomy Is Related to the Bicuspid Aortic Valve Phenotype","volume":"35","author":"Milleron","year":"2022","journal-title":"J. Am. Soc. Echocardiogr."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"687","DOI":"10.3389\/fphys.2017.00687","article-title":"Pathogenic mechanisms of bicuspid aortic valve aortopathy","volume":"8","author":"Yassine","year":"2017","journal-title":"Front. Physiol."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"1227","DOI":"10.4330\/wjc.v6.i12.1227","article-title":"Etiology of bicuspid aortic valve disease: Focus on hemodynamics","volume":"6","author":"Atkins","year":"2014","journal-title":"World J. Cardiol."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"1338","DOI":"10.1016\/j.athoracsur.2006.10.074","article-title":"Natural history of ascending aortic aneurysms in the setting of an unreplaced bicuspid aortic valve","volume":"83","author":"Davies","year":"2007","journal-title":"Ann. Thorac. Surg."},{"key":"ref_62","first-page":"36368","article-title":"Incidence of bicuspid valve related aortic dissection: A systematic review and meta-analysis","volume":"11","author":"Eranki","year":"2022","journal-title":"Ann. Cardiothorac. Surg."},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"1104","DOI":"10.1001\/jama.2011.1286","article-title":"Incidence of aortic complications in patients with bicuspid aortic valves","volume":"306","author":"Michelena","year":"2011","journal-title":"JAMA"},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"1666","DOI":"10.1016\/j.athoracsur.2015.04.126","article-title":"Aortic dissection in patients with bicuspid aortic valve\u2013associated aneurysms","volume":"100","author":"Wojnarski","year":"2015","journal-title":"Ann. Thorac. Surg."},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"1668","DOI":"10.1136\/heartjnl-2013-304606","article-title":"Type A aortic dissection in patients with bicuspid aortic valves: Clinical and pathological comparison with tricuspid aortic valves","volume":"99","author":"Eleid","year":"2013","journal-title":"Heart"},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"2873","DOI":"10.1093\/eurheartj\/ehu281","article-title":"2014 ESC Guidelines on the diagnosis and treatment of aortic diseases: Document covering acute and chronic aortic diseases of the thoracic and abdominal aorta of the adult The Task Force for the Diagnosis and Treatment of Aortic Diseases of the European Society of Cardiology (ESC)","volume":"35","author":"Erbel","year":"2014","journal-title":"Eur. Heart J."},{"key":"ref_67","doi-asserted-by":"crossref","unstructured":"Isselbacher, E.M., Preventza, O., Hamilton Black, J., Augoustides, J.G., Beck, A.W., Bolen, M.A., Braverman, A.C., Bray, B.E., Brown-Zimmerman, M.M., and Chen, E.P. (2022). 2022 ACC\/AHA Guideline for the Diagnosis and Management of Aortic Disease: A Report of the American Heart Association\/American College of Cardiology Joint Committee on Clinical Practice Guide-lines. Circulation, 146.","DOI":"10.1161\/CIR.0000000000001106"},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"e25","DOI":"10.1016\/j.jacc.2020.11.018","article-title":"2020 ACC\/AHA Guideline for the Management of Patients with Valvular Heart Disease","volume":"77","author":"Otto","year":"2021","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"561","DOI":"10.1093\/eurheartj\/ehab395","article-title":"2021 ESC\/EACTS Guidelines for the management of valvular heart disease","volume":"43","author":"Vahanian","year":"2021","journal-title":"Eur. Heart J."},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1056\/NEJMoa1912846","article-title":"Early surgery or conservative care for asymptomatic aortic stenosis","volume":"382","author":"Kang","year":"2020","journal-title":"N. Engl. J. Med."},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"118","DOI":"10.1093\/ejcts\/ezx018","article-title":"Early surgery versus conventional treatment for asymptomatic severe aortic regurgitation with normal ejection fraction and left ventricular dilatation","volume":"52","author":"Wang","year":"2017","journal-title":"Eur. J. Cardio-Thorac. Surg."},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"377","DOI":"10.1111\/jocs.14032","article-title":"Aortic valve repair or replacement in patients with aortic regurgitation: A systematic review and meta-analysis","volume":"34","author":"Wong","year":"2019","journal-title":"J. Card. Surg."},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"e000502","DOI":"10.1136\/openhrt-2016-000502","article-title":"Bicuspid aortic valve disease: Systematic review and meta-analysis of surgical aortic valve repair","volume":"3","author":"Salcher","year":"2016","journal-title":"Open Heart"},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"1043","DOI":"10.1161\/CIRCULATIONAHA.120.048048","article-title":"Transcatheter Aortic Valve Replacement in Bicuspid Aortic Valve Stenosis","volume":"143","author":"Vincent","year":"2021","journal-title":"Circulation"},{"key":"ref_75","doi-asserted-by":"crossref","unstructured":"Saad, M., Seoudy, H., and Frank, D. (2021). Challenging Anatomies for TAVR\u2014Bicuspid and Beyond. Front. Cardiovasc. Med., 8.","DOI":"10.3389\/fcvm.2021.654554"},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"1497","DOI":"10.1016\/j.jcin.2022.06.001","article-title":"Rationale, Definitions, Techniques, and Outcomes of Commissural Alignment in TAVR","volume":"15","author":"Tang","year":"2022","journal-title":"JACC Cardiovasc. Interv."},{"key":"ref_77","doi-asserted-by":"crossref","unstructured":"Merdler, I., Case, B., Bhogal, S., Reddy, P.K., Sawant, V., Zhang, C., Ali, S., Ben-Dor, I., Satler, L.F., and Rogers, T. (2023). Early experience with the Evolut FX self-expanding valve vs. Evolut PRO+ for patients with aortic stenosis undergoing TAVR. Cardiovasc. Revascularization Med., epub ahead of print.","DOI":"10.1016\/j.carrev.2023.06.003"},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"2144","DOI":"10.1016\/j.jcin.2021.07.052","article-title":"Outcomes After Transcatheter Aortic Valve Replacement in Bicuspid Versus Tricuspid Anatomy","volume":"14","author":"Montalto","year":"2021","journal-title":"JACC Cardiovasc. Interv."},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"351","DOI":"10.21037\/acs-2022-bav-22","article-title":"Procedural and clinical outcomes of transcatheter aortic valve replacement in bicuspid aortic valve patients: A systematic review and meta-analysis","volume":"11","author":"Chen","year":"2022","journal-title":"Ann. Cardiothorac. Surg."},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"1652","DOI":"10.1016\/j.jcin.2022.06.036","article-title":"Sex Difference in Outcomes Following Transcatheter Aortic Valve Replacement in Bicuspid Aortic Stenosis","volume":"15","author":"He","year":"2022","journal-title":"JACC Cardiovasc. Interv."},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"298","DOI":"10.21037\/acs-2020-surd-33","article-title":"Sutureless and rapid deployment implantation in bicuspid aortic valve: Results from the sutureless and rapid-deployment aortic valve replacement international registry","volume":"9","author":"Miceli","year":"2020","journal-title":"Ann. Cardiothorac. Surg."},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"3355","DOI":"10.1111\/jocs.16795","article-title":"The use of sutureless and rapid-deployment aortic valve prosthesis in patients with bicuspid aortic valve: A focused review","volume":"37","author":"King","year":"2022","journal-title":"J. Card. Surg."},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"68","DOI":"10.21037\/jovs.2018.03.08","article-title":"How can genetic diagnosis inform the decision of when to operate?","volume":"4","author":"Ouzounian","year":"2018","journal-title":"J. Vis. Surg."},{"key":"ref_84","doi-asserted-by":"crossref","unstructured":"Syrengela, A.-A., Shahjahan, S., Bulut, H.I., Ttofi, I., Djordjevic, J., and Krasopoulos, G. (2023). How Important are Genetics in the Management of Thoracic Aortic Aneurysms and Acute Aortic Syndromes? What do we Know Today?. J. Surg., 8, epub ahead of print.","DOI":"10.29011\/2575-9760.001818"}],"container-title":["Journal of Cardiovascular Development and Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2308-3425\/10\/9\/398\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T20:52:44Z","timestamp":1760129564000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2308-3425\/10\/9\/398"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,9,18]]},"references-count":84,"journal-issue":{"issue":"9","published-online":{"date-parts":[[2023,9]]}},"alternative-id":["jcdd10090398"],"URL":"https:\/\/doi.org\/10.3390\/jcdd10090398","relation":{},"ISSN":["2308-3425"],"issn-type":[{"value":"2308-3425","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,9,18]]}}}