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Review

Bicuspid aortic valve disease: recent insights in pathophysiology and treatment

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Pages 295-308 | Published online: 10 Jan 2014

Keep up to date with the latest research on this topic with citation updates for this article.

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Tamir Friedman, Arya Mani & John A Elefteriades. (2008) Bicuspid aortic valve: clinical approach and scientific review of a common clinical entity. Expert Review of Cardiovascular Therapy 6:2, pages 235-248.
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Sing-Chien Yap, Johanna JM Takkenberg, Maarten Witsenburg, Folkert J Meijboom & Jolien W Roos-Hesselink. (2005) Aortic stenosis at young adult age. Expert Review of Cardiovascular Therapy 3:6, pages 1087-1098.
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Articles from other publishers (52)

Pasquale Totaro, Simone Morganti, Ferdinando Auricchio & Stefano Pelenghi. (2023) Aortic wall thickness in dilated ascending aorta: Comparison between tricuspid and bicuspid aortic valve. Archives of Cardiovascular Diseases 116:11, pages 498-505.
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Nimrat Grewal, Evaldas Girdauskas, Mohammed Idhrees, Bashi Velayudhan, Robert Klautz, Antoine Driessen & Robert E. Poelmann. (2023) Structural abnormalities in the non-dilated ascending aortic wall of bicuspid aortic valve patients. Cardiovascular Pathology 62, pages 107478.
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Faraz Longi, Felix Orelaru, Jeffrey ClemenceJr.Jr., Aroma Naeem, Xiaoting Wu & Bo Yang. (2022) Outcomes of Bicuspid Aortic Valve Thoracic Aorta (4.0-4.5 cm) After Aortic Valve Replacement. The Annals of Thoracic Surgery 113:5, pages 1521-1528.
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Raj Verma, Gideon Cohen, Jillian Colbert & Paul W.M. Fedak. (2022) Bicuspid aortic valve associated aortopathy: 2022 guideline update. Current Opinion in Cardiology Publish Ahead of Print.
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Mi Chen, Wangli Xu, Yan Ding, Honglei Zhao, Pei Wang, Bo Yang, Huanyu Qiao, Wei Zhang, Chenyang Zhou, Junnan Jia, Tao Bai, Jinrong Xue, Junming Zhu, Yongmin Liu, Weimin Li & Lizhong Sun. (2021) Integrated Aortic-Valve-And-Ascending-Aortic Replacement vs. Partial Replacement in Bicuspid Aortic Valve-Related Aortopathy. Frontiers in Cardiovascular Medicine 8.
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Petra Büttner, Lukas Feistner, Philipp Lurz, Holger Thiele, Joshua D. Hutcheson & Florian Schlotter. (2021) Dissecting Calcific Aortic Valve Disease—The Role, Etiology, and Drivers of Valvular Fibrosis. Frontiers in Cardiovascular Medicine 8.
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Guang-Yin Peng, Yang Lin, Jing-Jing Li, Ying Wang, Hao-Yue Huang & Zhen-Ya Shen. (2019) The Application of Induced Pluripotent Stem Cells in Pathogenesis Study and Gene Therapy for Vascular Disorders: Current Progress and Future Challenges. Stem Cells International 2019, pages 1-9.
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Iolanda Aquila, Giacomo Frati, Sebastiano Sciarretta, Santo Dellegrottaglie, Daniele Torella & Michele Torella. (2019) New imaging techniques project the cellular and molecular alterations underlying bicuspid aortic valve development. Journal of Molecular and Cellular Cardiology 129, pages 197-207.
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Junfeng Yan, Ann-Cathrin Lehsau, Benjamin Sauer, Barbara Pieper, Salah A. Mohamed, Bart L. Loeys, Harry C. Dietz, Lut Van Laer, Andrew S. McCallion, Per Eriksson, Anders Franco-Cereceda, Luc Mertens, Seema Mital, Salah A. Mohamed & Gregor Andelfinger. (2019) Comparison of biomechanical properties in ascending aortic aneurysms of patients with congenital bicuspid aortic valve and Marfan syndrome. International Journal of Cardiology 278, pages 65-69.
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Michael A. Borger, Paul W.M. Fedak, Elizabeth H. Stephens, Thomas G. Gleason, Evaldas Girdauskas, John S. Ikonomidis, Ali Khoynezhad, Samuel C. Siu, Subodh Verma, Michael D. Hope, Duke E. Cameron, Donald F. Hammer, Joseph S. Coselli, Marc R. Moon, Thoralf M. Sundt, Alex J. Barker, Michael Markl, Alessandro Della Corte, Hector I. Michelena & John A. Elefteriades. (2018) The American Association for Thoracic Surgery consensus guidelines on bicuspid aortic valve–related aortopathy: Full online-only version. The Journal of Thoracic and Cardiovascular Surgery 156:2, pages e41-e74.
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Mihyun Kim, Chi Young Shim, Seong-Chan You, In-Jeong Cho, Geu-Ru Hong, Jong-Won Ha & Namsik Chung. (2017) Characteristics of Carotid Artery Structure and Mechanical Function and Their Relationships with Aortopathy in Patients with Bicuspid Aortic Valves. Frontiers in Physiology 8.
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Ali Fatehi Hassanabad, Alex J. Barker, David Guzzardi, Michael Markl, Chris Malaisrie, Patrick M. McCarthy & Paul W. M. FedakFedakFedak. (2017) Evolution of Precision Medicine and Surgical Strategies for Bicuspid Aortic Valve-Associated Aortopathy. Frontiers in Physiology 8.
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Josephina Haunschild, Isabel N. Schellinger, Sandy von Salisch, Farhad Bakhtiary, Martin Misfeld, Friedrich Wilhelm Mohr, Uwe Raaz & Christian D. Etz. (2017) Granular Media Calcinosis in the Aortic Walls of Patients With Bicuspid and Tricuspid Aortic Valves. The Annals of Thoracic Surgery 103:4, pages 1178-1185.
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Jessica Aijia Liu & Martin Cheung. (2016) Neural crest stem cells and their potential therapeutic applications. Developmental Biology 419:2, pages 199-216.
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Jiao Jiao, Wei Xiong, Lunchang Wang, Jiong Yang, Ping Qiu, Hiroyuki Hirai, Lina Shao, Dianna Milewicz, Y. Eugene Chen & Bo Yang. (2016) Differentiation defect in neural crest-derived smooth muscle cells in patients with aortopathy associated with bicuspid aortic valves. eBioMedicine 10, pages 282-290.
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Yang Li, You-Bin Deng, Xiao-Jun Bi, Ya-Ni Liu, Jun Zhang & Li Li. (2016) Evaluation of myocardial strain and artery elasticity using speckle tracking echocardiography and high-resolution ultrasound in patients with bicuspid aortic valve. The International Journal of Cardiovascular Imaging 32:7, pages 1063-1069.
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Pamela Lazar-Karsten, Gazanfer Belge, Detlev Schult-Badusche, Tim Focken, Arlo Radtke, Junfeng Yan, Pramod Ranabhat & Salah Mohamed. (2016) Generation and Characterization of Vascular Smooth Muscle Cell Lines Derived from a Patient with a Bicuspid Aortic Valve. Cells 5:2, pages 19.
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Valentina PaloschiJesper R. GådinShaukat KhanHanna M. BjörckLei DuShohreh MalekiJoy RoyJan H.M. LindemanSalah A. MohamedTakeshi TsudaAnders Franco-CerecedaPer Eriksson. (2015) Aneurysm Development in Patients With a Bicuspid Aortic Valve Is Not Associated With Transforming Growth Factor-β Activation. Arteriosclerosis, Thrombosis, and Vascular Biology 35:4, pages 973-980.
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Nimrat Grewal, Adriana C. Gittenberger-de Groot, Robert E. Poelmann, Robert J.M. Klautz, Johannes H.N. Lindeman, Marie-José Goumans, Meindert Palmen, Salah A. Mohamed, Hans-Hinrich Sievers, Ad J.J.C. Bogers & Marco C. DeRuiter. (2014) Ascending aorta dilation in association with bicuspid aortic valve: A maturation defect of the aortic wall. The Journal of Thoracic and Cardiovascular Surgery 148:4, pages 1583-1590.
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Subodh Verma & Samuel C. Siu. (2014) Aortic Dilatation in Patients with Bicuspid Aortic Valve. New England Journal of Medicine 370:20, pages 1920-1929.
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Johan Petrini, Jonas Jenner, Anette Rickenlund, Per Eriksson, Anders Franco-Cereceda, Kenneth Caidahl & Maria J. Eriksson. (2014) Elastic Properties of the Descending Aorta in Patients with a Bicuspid or Tricuspid Aortic Valve and Aortic Valvular Disease. Journal of the American Society of Echocardiography 27:4, pages 393-404.
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Fei Qiong Huang & Ju Le Tan. (2014) Pattern of Aortic Dilatation in Different Bicuspid Aortic Valve Phenotypes and its Association with Aortic Valvular Dysfunction and Elasticity. Heart, Lung and Circulation 23:1, pages 32-38.
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Bulat A. Ziganshin & John A. Elefteriades. 2014. Evidence-Based Cardiology Consult. Evidence-Based Cardiology Consult 331 353 .
Ignacio Oulego-Erroz, Paula Alonso-Quintela, María Mora-Matilla, Sandra Gautreaux Minaya & Santiago Lapeña-López de Armentia. (2013) Ascending aorta elasticity in children with isolated bicuspid aortic valve. International Journal of Cardiology 168:2, pages 1143-1146.
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C. R. Balistreri, C. Pisano, G. Candore, E. Maresi, M. Codispoti & G. Ruvolo. (2012) Focus on the unique mechanisms involved in thoracic aortic aneurysm formation in bicuspid aortic valve versus tricuspid aortic valve patients: clinical implications of a pilot study. European Journal of Cardio-Thoracic Surgery 43:6, pages e180-e186.
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Dick Wågsäter, Valentina Paloschi, Roeland Hanemaaijer, Kjell Hultenby, Ruud A. Bank, Anders Franco‐Cereceda, Jan H. N. Lindeman & Per Eriksson. (2013) Impaired Collagen Biosynthesis and Cross‐linking in Aorta of Patients With Bicuspid Aortic Valve. Journal of the American Heart Association 2:1.
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Giuseppe Santarpia, Giancarlo Scognamiglio, Giovanni Di Salvo, Michele D'Alto, Berardo Sarubbi, Emanuele Romeo, Ciro Indolfi, Maurizio Cotrufo & Raffaele Calabrò. (2012) Aortic and left ventricular remodeling in patients with bicuspid aortic valve without significant valvular dysfunction: A prospective study. International Journal of Cardiology 158:3, pages 347-352.
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Christian Vergara, Francesca Viscardi, Luca Antiga & Giovanni Battista Luciani. (2011) Influence of Bicuspid Valve Geometry on Ascending Aortic Fluid Dynamics: A Parametric Study. Artificial Organs 36:4, pages 368-378.
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Christian D. Etz, Martin Misfeld, Michael A. Borger, Maximilian Luehr, Elfriede Strotdrees & Friedrich-Wilhelm Mohr. (2012) Current Indications for Surgical Repair in Patients with Bicuspid Aortic Valve and Ascending Aortic Ectasia. Cardiology Research and Practice 2012, pages 1-9.
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Christian D. Etz, Michael A. Borger, Martin Misfeld & Friedrich-Wilhelm Mohr. (2011) Patienten mit bikuspider Aortenklappe und moderat erweiterter Aorta ascendensPatients with bicuspid aortic valve and modestly dilated ascending aorta. Zeitschrift für Herz-,Thorax- und Gefäßchirurgie 25:2, pages 99-106.
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Michel J. Van Dyck, Christine Watremez, Munir Boodhwani, Jean-Louis Vanoverschelde & Gebrine El Khoury. (2010) Transesophageal Echocardiographic Evaluation During Aortic Valve Repair Surgery. Anesthesia & Analgesia 111:1, pages 59-70.
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Ori Wald, Amit Korach & Oz M. Shapira. (2010) Should Aortas in Patients with Bicuspid Aortic Valve Really be Resected at an Earlier Stage than Tricuspid? PRO. Cardiology Clinics 28:2, pages 289-298.
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Jean-Loïc Rose, Alain Lalande, Olivier Bouchot, El-Bey Bourennane, Paul M. Walker, Patricia Ugolini, Chantal Revol-Muller, Raymond Cartier & François Brunotte. (2010) Influence of age and sex on aortic distensibility assessed by MRI in healthy subjects. Magnetic Resonance Imaging 28:2, pages 255-263.
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Nikolaos Tzemos, Erik Lyseggen, Candice Silversides, Michal Jamorski, Jeffrey H. Tong, Paula Harvey, John Floras & Samuel Siu. (2010) Endothelial Function, Carotid–Femoral Stiffness, and Plasma Matrix Metalloproteinase-2 in Men With Bicuspid Aortic Valve and Dilated Aorta. Journal of the American College of Cardiology 55:7, pages 660-668.
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Shi-Min Yuan & Hua Jing. (2010) The bicuspid aortic valve and related disorders. Sao Paulo Medical Journal 128:5, pages 296-301.
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J. F. Matthias Bechtel, M. Misfeld, C. Schmidtke & Hans-H. Sievers. 2010. Aortic Root Surgery. Aortic Root Surgery 89 101 .
Massimo Lombardi. (2009) Do we need a new syndrome within the heterogeneity of bicuspid aortic valve patients?. Journal of Cardiovascular Medicine 10:6, pages 443-445.
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Daniela Prandstraller, Laura Mazzanti, Alessandro Giardini, Luigi Lovato, Federica Tamburrino, Emanuela Scarano, Alessandro Cicognani, Rossella Fattori & Fernando M Picchio. (2009) Correlations of phenotype and genotype in relation to morphologic remodelling of the aortic root in patients with Turner’s syndrome. Cardiology in the Young 19:03, pages 264.
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L. LuAnn Minich, Lloyd Y. Tani, Richard V. Williams & Michael D. Puchalski. 2009. Valvular Heart Disease: A Companion to Braunwald's Heart Disease. Valvular Heart Disease: A Companion to Braunwald's Heart Disease 399 423 .
Alan C. Braverman & Michael A. Beardslee. 2009. Valvular Heart Disease: A Companion to Braunwald's Heart Disease. Valvular Heart Disease: A Companion to Braunwald's Heart Disease 169 186 .
Frederick J. Schoen. (2008) Evolving Concepts of Cardiac Valve Dynamics. Circulation 118:18, pages 1864-1880.
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John A. Elefteriades. (2008) Thoracic Aortic Aneurysm: Reading the Enemy's Playbook. Current Problems in Cardiology 33:5, pages 203-277.
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M J Collins, J Butany, M A Borger, B H Strauss & T E David. (2008) Implications of a congenitally abnormal valve: a study of 1025 consecutively excised aortic valves. Journal of Clinical Pathology 61:4, pages 530-536.
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M J Collins, V Dev, B H Strauss, P W M Fedak & J Butany. (2008) Variation in the histopathological features of patients with ascending aortic aneurysms: a study of 111 surgically excised cases. Journal of Clinical Pathology 61:4, pages 519-523.
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Peter Matt, Zongming Fu, Thierry Carrel, David L. Huso, Stefan Dirnhofer, Ivan Lefkovits, Hans-Reinhard Zerkowski & Jennifer E. Van Eyk. (2007) Proteomic alterations in heat shock protein 27 and identification of phosphoproteins in ascending aortic aneurysm associated with bicuspid and tricuspid aortic valve. Journal of Molecular and Cellular Cardiology 43:6, pages 792-801.
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Shafie S Fazel & Tirone E David. (2007) Aortic valve-sparing operations for aortic root and ascending aortic aneurysms. Current Opinion in Cardiology 22:6, pages 497-503.
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Peter Matt, Anne von Orelli, Franziska Bernet, Thomas Grussenmeyer, Ivan Lefkovits & Hans-Reinhard Zerkowski. (2016) Proteomics of Ascending Aortic Aneurysm with Bicuspid or Tricuspid Aortic Valve. Asian Cardiovascular and Thoracic Annals 15:3, pages 185-190.
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Ramanath Majumdar, Marineh Yagubyan, Gobinda Sarkar, Mark E. Bolander & Thoralf M. SundtIIIIII. (2006) Bicuspid aortic valve and ascending aortic aneurysm are not associated with germline or somatic homeobox NKX2-5 gene polymorphism in 19 patients. The Journal of Thoracic and Cardiovascular Surgery 131:6, pages 1301-1305.
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