International Consensus Statement on Nomenclature and Classification of the Congenital Bicuspid Aortic Valve and Its Aortopathy, for Clinical, Surgical, Interventional and Research Purposes.
Michelena, Hector I ; Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN, USA
Corte, Alessandro Della ; Department of Translational Medical Sciences, University of Campania "L. Vanvitelli", Naples, Italy
Evangelista, Arturo; Department of Cardiology, Hospital Vall d'Hebron, Vall d'Hebron Research Institute (VHIR) Ciber-CV, Barcelona, Spain
Maleszewski, Joseph J ; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA
Edwards, William D ; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA
Roman, Mary J; Division of Cardiology, Weill Cornell Medicine, New York, NY, USA
Devereux, Richard B ; Division of Cardiology, Weill Cornell Medicine, New York, NY, USA
Fernández, Borja ; Departamento de Biologia Animal, Facultad de Ciencias, Instituto de Investigación Biomédica de Málaga, Universidad de Málaga, Ciber-CV, Málaga, Spain
Asch, Federico M ; MedStar Health Research Institute, Washington, DC, USA
Barker, Alex J ; Department of Radiology, Children's Hospital Colorado, University of Colorado, Anschutz Medical Campus, Aurora, CO, USA
Sierra-Galan, Lilia M ; Cardiovascular Division, American British Cowdray Medical Center, Mexico City, Mexico
De Kerchove, Laurent; Cardiovascular Division, American British Cowdray Medical Center, Mexico City, Mexico
Fernandes, Susan M ; Division of Pediatric Cardiology, Department of Pediatrics, Stanford University, Palo Alto, CA, USA ; Division of Cardiovascular Medicine, Department of Medicine, Stanford University, Palo Alto, CA, USA
Fedak, Paul W M ; Department of Cardiac Sciences, Libin Cardiovascular Institute, Cumming School of Medicine, University of Calgary, Calgary, Canada
Girdauskas, Evaldas; Department of Cardiovascular Surgery, University Heart and Vascular Center Hamburg, Hamburg, Germany
Delgado, Victoria; Department of Cardiology, Leiden University Medical Center, Leiden, Netherlands
Abbara, Suhny ; Cardiothoracic Imaging Division, Department of Radiology, UT Southwestern Medical Center, Dallas, TX, USA
Lansac, Emmanuel; Department of Cardiac Surgery, Institute Mutualiste Montsouris, Paris, France
Prakash, Siddharth K; Department of Internal Medicine, McGovern Medical School, The University of Texas Health Science Center at Houston, Houston, TX, USA
Bissell, Malenka M ; Department of Biomedical Imaging Science, Leeds Institute to Cardiovascular and Metabolic Medicine, University of Leeds, Leeds, UK
Popescu, Bogdan A ; Department of Cardiology, University of Medicine and Pharmacy "Carol Davila"-Euroecolab, Emergency Institute for Cardiovascular Diseases "Prof. Dr. C. C. Iliescu", Bucharest, Romania
Hope, Michael D ; Department of Radiology and Biomedical Imaging, University of California San Francisco, San Francisco, CA, USA
Sitges, Marta; Cardiovascular Institute, Hospital Clinic, Universitat de Barcelona, IDIBAPS, CIBERCV, ISCIII (CB16/11/00354), CERCA Programme, Barcelona, Spain
Thourani, Vinod H; Department of Cardiovascular Surgery, Marcus Valve Center, Piedmont Heart Institute, Atlanta, GA, USA
Pibarot, Phillippe ; Department of Cardiology, Québec Heart & Lung Institute, Laval University Québec, Québec, Canada
Chandrasekaran, Krishnaswamy ; Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN, USA
Lancellotti, Patrizio ; Centre Hospitalier Universitaire de Liège - CHU > > Service de cardiologie ; Gruppo Villa Maria Care and Research, Maria Cecilia Hospital, Cotignola, and Anthea Hospital, Bari, Italy
Borger, Michael A; University Clinic of Cardiac Surgery, Leipzig Heart Center, Leipzig, Germany
Forrest, John K ; Yale University School of Medicine & Yale New Haven Hospital, New Haven, CT, USA
Webb, John; St Paul's Hospital, University of British Columbia, Vancouver, Canada
Milewicz, Dianna M; Department of Internal Medicine, McGovern Medical School, The University of Texas Health Science Center at Houston, Houston, TX, USA
Makkaar, Raj; Cedars Sinai Heart Institute, Los Angeles, CA, USA
Leon, Martin B; Department of Internal Medicine, McGovern Medical School, The University of Texas Health Science Center at Houston, Houston, TX, USA
Sanders, Stephen P; Cardiac Registry, Departments of Cardiology, Pathology and Cardiac Surgery, Boston Children's Hospital, Boston, MA, USA ; Department of Pediatrics, Harvard Medical School, Boston, MA, USA
Markl, Michael ; Yale University School of Medicine & Yale New Haven Hospital, New Haven, CT, USA
Ferrari, Victor A ; Cardiovascular Medicine Division, University of Pennsylvania Medical Center and Penn Cardiovascular Institute, Philadelphia, PA, USA
Roberts, William C; Baylor Heart and Vascular Institute, Baylor University Medical Center, Texas A&M School of Medicine, Dallas Campus, Dallas, TX, USA
Song, Jae-Kwan; University of Ulsan College of Medicine, Asan Medical Center, Seoul, South Korea
Blanke, Philipp; Department of Radiology, St. Paul's Hospital, Vancouver, BC, Canada
White, Charles S ; Department of Radiology, University of Maryland School of Medicine, Baltimore, MD, USA
Siu, Samuel; Schulich School of Medicine and Dentistry, London, ON, Canada
Svensson, Lars G; Heart, Vascular and Thoracic Institute, Cleveland Clinic, Cleveland, OH, USA
Braverman, Alan C ; Cardiovascular Division, Department of Medicine, Washington University School of Medicine, St. Louis, MO, USA
Bavaria, Joseph; Division of Cardiac Surgery, University of Pennsylvania, Philadelphia, PA, USA
Sundt, Thoralf M; Division of Cardiac Surgery, Massachusetts General Hospital, Boston, MA, USA
El Khoury, Gebrine; Cardiovascular Division, American British Cowdray Medical Center, Mexico City, Mexico
De Paulis, Ruggero ; Department of Cardiac Surgery, European Hospital and Unicamillus University Rome, Rome, Italy
Enriquez-Sarano, Maurice; Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN, USA
Bax, Jeroen J; Department of Cardiology, Leiden University Medical Center, Leiden, Netherlands
Otto, Catherine M; Division of Cardiology, University of Washington, Seattle, WA, USA
Schäfers, Hans-Joachim; Department of Thoracic and Cardiovascular Surgery, Saarland University Medical Center, Homburg/Saar, Germany
International Consensus Statement on Nomenclature and Classification of the Congenital Bicuspid Aortic Valve and Its Aortopathy, for Clinical, Surgical, Interventional and Research Purposes.
Publication date :
August 2021
Journal title :
Radiology. Cardiothoracic Imaging
eISSN :
2638-6135
Publisher :
Radiological Society of North America Inc., United States
Abbott Vascular, Edwards Lifesciences, GE Healthcare, MSD, Medtronic, and Novartis. Emmanuel Lansac: patent Extra Aortic ring annuloplasty device with Coroneo Inc. Phillippe Pibarot: funding from Edwards Lifesciences and Medtronic for echocardiography core laboratory services with no personal compensation. Michael A. Borger: discloses a financial relationship with Edwards Lifesciences, Medtronic, Abbott, and CryoLife. John K. Forrest: grant support and consultant to: Edwards Lifesciences, Medtronic Inc. John Webb: consultant to: Edwards Lifesciences, Abbott, Boston Scientific. Martin B. Leon: institutional clinical research grants from Abbott, BSC, Edwards and Medtronic. Michael Markl: research support—Siemens Healthineers, Research Grant—Circle Cardiovascular Imaging, Consulting—Circle Cardiovascular Imaging, Research Grant—Cryolife Inc. Victor A. Ferrari: Senior Advisory Board, Journal of Cardiovascular Magnetic Resonance. Philipp Blanke: consultant for Edwards Life-sciences and Circle Cardiovascular Imaging; and provides CT core lab services for Edwards Lifesciences, Medtronic, Neovasc, and Tendyne Holdings, for which he receives no direct compensation. Ruggero De Paulis: patent on aortic root graft with Terumo Aortic. Consultant for Edwards Lifesciences, Medtronic, and Terumo Aortic. Maurice Enriquez-Sarano: consulting fees from Edwards LLC, Cryolife, and ChemImage, Inc. The Department of Cardiology of the LUMC received unrestricted research grants from Abbott Vascular, Bayer, Biotronik, Bio-ventrix, Boston Scientific, Edwards Lifesciences, GE Healthcare, and Medtronic. The other authors report no conflict of interest.
Michelena HI, Della Corte A, Prakash SK, Milewicz DM, Evangelista A, Enriquez-Sarano M. Bicuspid aortic valve aortopathy in adults: Incidence, etiology, and clinical significance. Int J Cardiol 2015;201(400):7.
Michelena HI, Prakash SK, Della Corte A, Bissell MM, Anavekar N, Mathieu P et al. Bicuspid aortic valve: Identifying knowledge gaps and rising to the challenge from the International Bicuspid Aortic Valve Consortium (BAVCon). Circulation 2014;129(2691):704.
Michelena HI, Suri RM, Katan O, Eleid MF, Clavel MA, Maurer MJ et al. Sex differences and survival in adults with bicuspid aortic valves: Verification in 3 contemporary echocardiographic cohorts. J Am Heart Assoc 2016;5e004211.
Roberts WC. The congenitally bicuspid aortic valve. A study of 85 autopsy cases. Am J Cardiol 1970;26(72):83.
Michelena HI, Vallabhajosyula S, Prakash SK. Nosology spectrum of the bicuspid aortic valve condition: Complex-presentation valvulo-aortopathy. Circulation 2020;142(294):9.
Michelena HI, Chandrasekaran K, Topilsky Y, Messika-Zeitoun D, Della Corte A, Evangelista A et al. The bicuspid aortic valve condition: The critical role of echocardiography and the case for a standard nomenclature consensus. Prog Cardiovasc Dis 2018;61:404-15.
Niaz T, Poterucha JT, Johnson JN, Craviari C, Nienaber T, Palfreeman J et al. Incidence, morphology, and progression of bicuspid aortic valve in pediatric and young adult subjects with coexisting congenital heart defects. Congenit Heart Dis 2017;12(261):9.
Niaz T, Poterucha JT, Olson TM, Johnson JN, Craviari C, Nienaber T et al. Characteristic morphologies of the bicuspid aortic valve in patients with genetic syndromes. J Am Soc Echocardiogr 2018;31(194):200.
Michelena HI, Khanna AD, Mahoney D, Margaryan E, Topilsky Y, Suri RM et al. Incidence of aortic complications in patients with bicuspid aortic valves. JAMA 2011;306(1104):12.
Michelena HI, Della Corte A, Evangelista A, Maleszewski JJ, Enriquez-Sarano M, Bax JJ et al. Speaking a common language: Introduction to a standard terminology for the bicuspid aortic valve and its aortopathy. Prog Cardiovasc Dis 2020;63:419-24.
Masri A, Svensson LG, Griffin BP, Desai MY. Contemporary natural history of bicuspid aortic valve disease: A systematic review. Heart 2017;103:1323–30.
Brown ML, Burkhart HM, Connolly HM, Dearani JA, Cetta F, Li Z et al. Coarctation of the aorta: Lifelong surveillance is mandatory following surgical repair. J Am Coll Cardiol 2013;62(1020):5.
Fuchs MM, Attenhofer JC, Babovic-Vuksanovic D, Connolly HM, Egbe A. Long-term outcomes in patients with turner syndrome: A 68-year follow-up. J Am Heart Assoc 2019;8e011501.
Michelena HI, Katan O, Suri RM, Baddour LM, Enriquez-Sarano M. Incidence of infective endocarditis in patients with bicuspid aortic valves in the community. Mayo Clin Proc 2016;91(122):3.
Goldstein SA, Evangelista A, Abbara S, Arai A, Asch FM, Badano LP et al. Multimodality imaging of diseases of the thoracic aorta in adults: From the American Society of Echocardiography and the European Association of Cardiovascular Imaging: Endorsed by the Society of Cardiovascular Computed Tomography and Society for Cardiovascular Magnetic Resonance. J Am Soc Echocardiogr 2015;28(119):82.
Saric M, Armour AC, Arnaout MS, Chaudhry FA, Grimm RA, Kronzon I et al. Guidelines for the use of echocardiography in the evaluation of a cardiac source of embolism. J Am Soc Echocardiogr 2016;29(1):42.
Tanaka R, Yoshioka K, Niinuma H, Ohsawa S, Okabayashi H, Ehara S. Diagnostic value of cardiac CT in the evaluation of bicuspid aortic stenosis: Comparison with echocardiography and operative findings. AJR Am J Roentgenol 2010;195(895):9.
Gleeson TG, Mwangi I, Horgan SJ, Cradock A, Fitzpatrick P, Murray JG. Steady-state free-precession (SSFP) cine MRI in distinguishing normal and bicuspid aortic valves. J Magn Reson Imaging 2008;28(873):8.
Borger MA, Fedak PWM, Stephens EH, Gleason TG, Girdauskas E, Ikonomidis JS et al. The American Association for Thoracic Surgery consensus guidelines on bicuspid aortic valve-related aortopathy: Executive summary. J Thorac Cardiovasc Surg 2018;156(473):80.
Roberts WC, Ko JM. Frequency by decades of unicuspid, bicuspid, and tricuspid aortic valves in adults having isolated aortic valve replacement for aortic stenosis, with or without associated aortic regurgitation. Circulation 2005;111(920):5.
Yoon SH, Bleiziffer S, De Backer O, Delgado V, Arai T, Ziegelmueller J et al. Outcomes in transcatheter aortic valve replacement for bicuspid versus tricuspid aortic valve stenosis. J Am Coll Cardiol 2017;69(2579):89.
Perlman GY, Blanke P, Dvir D, Pache G, Modine T, Barbanti M et al. Bicuspid aortic valve stenosis: Favorable early outcomes with a next- generation transcatheter heart valve in a multicenter study. JACC Cardiovasc Interv 2016;9:817-24.
Roberts WC, Janning KG, Ko JM, Filardo G, Matter GJ. Frequency of congenitally bicuspid aortic valves in patients ≥80 years of age undergoing aortic valve replacement for aortic stenosis (with or without aortic regurgitation) and implications for transcatheter aortic valve implantation. Am J Cardiol 2012;109(1632):6.
Hagendorff A, Evangelista A, Fehske W, Schafers HJ. Improvement in the assessment of aortic valve and aortic aneurysm repair by 3-dimensional echocardiography. JACC Cardiovasc Imaging 2019;12(2225):44.
Schneider U, Feldner SK, Hofmann C, Schope J, Wagenpfeil S, Giebels C et al. Two decades of experience with root remodeling and valve repair for bicuspid aortic valves. J Thorac Cardiovasc Surg 2017;153(S65):S71.
Eleid MF, Forde I, Edwards WD, Maleszewski JJ, Suri RM, Schaff HV et al. Type A aortic dissection in patients with bicuspid aortic valves: Clinical and pathological comparison with tricuspid aortic valves. Heart 2013;99:1668–74.
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. Am J Cardiol 2009;104(1001):6.
Padang R, Enriquez-Sarano M, Pislaru SV, Maalouf JF, Nkomo VT, Mankad SV et al. Coexistent bicuspid aortic valve and mitral valve prolapse: Epidemiology, phenotypic spectrum, and clinical implications. Eur Heart J Cardiovasc Imaging 2019;20(677):86.
Tzemos N, Therrien J, Yip J, Thanassoulis G, Tremblay S, Jamorski MT et al. Outcomes in adults with bicuspid aortic valves. JAMA 2008;300:1317–25.
Kang JW, Song HG, Yang DH, Baek S, Kim DH, Song JM et al. Association between bicuspid aortic valve phenotype and patterns of valvular dysfunction and bicuspid aortopathy: Comprehensive evaluation using MDCT and echocardiography. JACC Cardiovasc Imaging 2013;6(150):61.
Angelini A, Ho SY, Anderson RH, Devine WA, Zuberbuhler JR, Becker AE et al. The morphology of the normal aortic valve as compared with the aortic valve having two leaflets. J Thorac Cardiovasc Surg 1989;98(362):7.
Sabet HY, Edwards WD, Tazelaar HD, Daly RC. Congenitally bicuspid aortic valves: A surgical pathology study of 542 cases (1991 through 1996) and a literature review of 2,715 additional cases. Mayo Clin Proc 1999;74(14):26.
Schaefer BM, Lewin MB, Stout KK, Gill E, Prueitt A, Byers PH et al. The bicuspid aortic valve: An integrated phenotypic classification of leaflet morphology and aortic root shape. Heart 2008;94(1634):8.
Sievers HH, Schmidtke C. A classification system for the bicuspid aortic valve from 304 surgical specimens. J Thorac Cardiovasc Surg 2007;133:1226–33.
Jilaihawi H, Chen M, Webb J, Himbert D, Ruiz CE, Rodes-Cabau J et al. A bicuspid aortic valve imaging classification for the TAVR era. JACC Cardiovasc Imaging 2016;9(1145):58.
Sun BJ, Lee S, Jang JY, Kwon O, Bae JS, Lee JH et al. Performance of a simplified dichotomous phenotypic classification of bicuspid aortic valve to predict type of valvulopathy and combined aortopathy. J Am Soc Echocardiogr 2017;30(1152):61.
Brandenburg RO Jr, Tajik AJ, Edwards WD, Reeder GS, Shub C, Seward JB. Accuracy of 2-dimensional echocardiographic diagnosis of congenitally bicuspid aortic valve: Echocardiographic-anatomic correlation in 115 patients. Am J Cardiol 1983;51(1469):73.
Murphy IG, Collins J, Powell A, Markl M, McCarthy P, Malaisrie SC et al. Comprehensive 4-stage categorization of bicuspid aortic valve leaflet morphology by cardiac MRI in 386 patients. Int J Cardiovasc Imaging 2017;33(1213):21.
Fernáindez B, Durán AC, Martire A, López D, Sans-Coma V. New embryological evidence for the formation of quadricuspid aortic valves in the Syrian hamster (Mesocricetus auratus). J Comp Pathol 1999;121:89–94.
Lopez-Garcia A, Carmen Fernandez M, Duran AC, Sans-Coma V, Fernandez B. Quadricuspid aortic valves in Syrian hamsters and their formation according to current knowledge on valvulogenesis. Jpn J Vet Res 2015;63(37):43.
Slostad BD, Witt CM, O’Leary PW, Maleszewski JJ, Scott CG, Dearani JA et al. Unicuspid aortic valve: Demographics, comorbidities, echocardio-graphic features, and long-term outcomes. Circulation 2019;140(1853):5.
Tsang MY, Abudiab MM, Ammash NM, Naqvi TZ, Edwards WD, Nkomo VT et al. Quadricuspid aortic valve: Characteristics, associated structural cardiovascular abnormalities, and clinical outcomes. Circulation 2016;133:312–9.
de Kerchove L, Mastrobuoni S, Froede L, Tamer S, Boodhwani M, van Dyck M et al. Variability of repairable bicuspid aortic valve phenotypes: Towards an anatomical and repair-oriented classification. Eur J Cardiothorac Surg 2019;56(351):9.
Barker AJ, Robinson JD, Markl M. Bicuspid aortic valve phenotype and aortopathy: Nomenclature and role of aortic hemodynamics. JACC Cardiovasc Imaging 2013;6921.
Michelena HI, Desjardins VA, Avierinos JF, 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(2776):84.
Roberts WC, Vowels TJ, Ko JM. Comparison of interpretations of valve structure between cardiac surgeon and cardiac pathologist among adults having isolated aortic valve replacement for aortic valve stenosis (+/- aortic regurgitation). Am J Cardiol 2009;103(1139):45.
Sievers HH, Hemmer W, Beyersdorf F, Moritz A, Moosdorf R, Lichtenberg A et al.;. on behalf of the Working Group for Aortic Valve Surgery of the German Society of Thoracic and Cardiovascular Surgery. The everyday used nomenclature of the aortic root components: The tower of Babel? Eur J Cardiothorac Surg 2012;41:478-82.
de Kerchove L, Jashari R, Boodhwani M, Duy KT, Lengele B, Gianello P et al. Surgical anatomy of the aortic root: Implication for valve-sparing reimplantation and aortic valve annuloplasty. J Thorac Cardiovasc Surg 2015;149(425):33.
Khelil N, Sleilaty G, Palladino M, Fouda M, Escande R, Debauchez M et al. Surgical anatomy of the aortic annulus: Landmarks for external annuloplasty in aortic valve repair. Ann Thorac Surg 2015;99(1220):6.
El KG, Glineur D, Rubay J, Verhelst R, d’Acoz Y, Poncelet A et al. Functional classification of aortic root/valve abnormalities and their correlation with etiologies and surgical procedures. Curr Opin Cardiol 2005;20:115–21.
Yang LT, Pellikka PA, Enriquez-Sarano M, Maalouf JF, Scott CG, Michelena HI. Stage B aortic regurgitation in bicuspid aortic valve: New observations on progression rate and predictors. JACC Cardiovasc Imaging 2020;13(1442):5.
Schneider U, Hofmann C, Aicher D, Takahashi H, Miura Y, Schafers HJ. Suture annuloplasty significantly improves the durability of bicuspid aortic valve repair. Ann Thorac Surg 2017;103(504):10.
Stefek HA, Lin KH, Rigsby CK, Michelena HI, Aouad P, Barker AJ et al. Eccentric enlargement of the aortic sinuses in pediatric and adult patients with bicuspid aortic valves: A cardiac MRI study. Pediatr Cardiol 2020;41(350):60.
Kong WK, Delgado V, Poh KK, Regeer MV, Ng AC, McCormack L et al. Prognostic implications of raphe in bicuspid aortic valve anatomy. JAMA Cardiol 2017;2(285):92.
Evangelista A, Gallego P, Calvo-Iglesias F, Bermejo J, Robledo-Carmona J, Sanchez V et al. Anatomical and clinical predictors of valve dysfunction and aortic dilation in bicuspid aortic valve disease. Heart 2018;104:566–73.
Yang LT, Enriquez-Sarano M, Michelena HI. The bicuspid aortic valve raphe: An evolving structure. Eur Heart J Cardiovasc Imaging 2020;21:590.
Kong WKF, Regeer MV, Poh KK, Yip JW, van Rosendael PJ, Yeo TC et al. Inter-ethnic differences in valve morphology, valvular dysfunction, and aortopathy between Asian and European patients with bicuspid aortic valve. Eur Heart J 2018;39(1308):13.
Fernandes S, Khairy P, Graham DA, Colan SD, Galvin TC, Sanders SP et al. Bicuspid aortic valve and associated aortic dilation in the young. Heart 2012;98(1014):9.
Della Corte A, Bancone C, Quarto C, Dialetto G, Covino FE, Scardone M et al. Predictors of ascending aortic dilatation with bicuspid aortic valve: A wide spectrum of disease expression. Eur J Cardiothorac Surg 2007;31:397–404; discussion 404-5.
Detaint D, Michelena HI, Nkomo VT, Vahanian A, Jondeau G, Sarano ME. Aortic dilatation patterns and rates in adults with bicuspid aortic valves: A comparative study with Marfan syndrome and degenerative aortopathy. Heart 2014;100(126):34.
Fernandes SM, Sanders SP, Khairy P, Jenkins KJ, Gauvreau K, Lang P et al. Morphology of bicuspid aortic valve in children and adolescents. J Am Coll Cardiol 2004;44(1648):51.
Fernandes SM, Khairy P, Sanders SP, Colan SD. Bicuspid aortic valve morphology and interventions in the young. J Am Coll Cardiol 2007;49:2211–4.
Sun BJ, Jin X, Song JK, Lee S, Lee JH, Park JB et al. Clinical characteristics of Korean patients with bicuspid aortic valve who underwent aortic valve surgery. Korean Circ J 2018;48(48):58.
Chandra S, Lang RM, Nicolarsen J, Gayat E, Spencer KT, Mor-Avi V et al. Bicuspid aortic valve: Inter-racial difference in frequency and aortic dimensions. JACC Cardiovasc Imaging 2012;5(981):9.
Fernandez B, Duran AC, Fernandez-Gallego T, Fernandez MC, Such M, Arque JM et al. Bicuspid aortic valves with different spatial orientations of the leaflets are distinct etiological entities. J Am Coll Cardiol 2009;54:2312–8.
Sans-Coma V, Fernandez B, Duran AC, Thiene G, Arque JM, Munoz-Chapuli R et al. Fusion of valve cushions as a key factor in the formation of congenital bicuspid aortic valves in Syrian hamsters. Anat Rec 1996;244:490–8.
Fernández B, Soto-Navarrete MT, López-Garía A, López-Unzu MÁ, Durán AC, Fernández MC. Bicuspid aortic valve in 2 model species and review of the literature. Vet Pathol 2020;57(321):31.
Phillips HM, Mahendran P, Singh E, Anderson RH, Chaudhry B, Henderson DJ. Neural crest cells are required for correct positioning of the developing outflow cushions and pattern the arterial valve leaflets. Cardiovasc Res 2013;99(452):60.
Soto-Navarrete MT, Lopez-Unzu MA, Duran AC, Fernandez B. Embryonic development of bicuspid aortic valves. Prog Cardiovasc Dis 2020;63(407):418.
Aicher D, Kunihara T, Abou Issa O, Brittner B, Graber S, Schafers H-J. Valve configuration determines long-term results after repair of the bicuspid aortic valve. Circulation 2011;123(178):85.
Sperling JS, Lubat E. Forme fruste or ‘Incomplete’ bicuspid aortic valves with very small raphes: The prevalence of bicuspid valve and its significance may be underestimated. Int J Cardiol 2015;184(1):5.
Douglas PS, Garcia MJ, Haines DE, Lai WW, Manning WJ, Patel AR. ACCF/ASE/AHA/ASNC/HFSA/HRS/SCAI/SCCM/SCCT/SCMR 2011 appropriate use criteria for echocardiography. a report of the American College of Cardiology Foundation Appropriate Use Criteria Task Force, American Society of Echocardiography, American Heart Association, American Society of Nuclear Cardiology, Heart Failure Society of America, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, Society of Critical Care Medicine, Society of Cardiovascular Computed Tomography,. Society for Cardiovascular Magnetic Resonance American College of Chest Physicians. J Am Soc Echocardiogr 2011;24(229):67.
Guala A, Rodriguez-Palomares J, Galian-Gay L, Teixido-Tura G, Johnson KM, Wieben O et al. Partial aortic valve leaflet fusion is related to deleterious alteration of proximal aorta hemodynamics. Circulation 2019;139:2707–9.
Michelena HI, Yang LT, Enriquez-Sarano M, Pochettino A. The elusive ‘forme fruste’ bicuspid aortic valve: 3D transoesophageal echocardiography to the rescue. Eur Heart J Cardiovasc Imaging 2020;21(1169):1169.
Wang Y, Wu B, Farrar E, Lui W, Lu P, Zhang D et al. Notch-Tnf signalling is required for development and homeostasis of arterial valves. Eur Heart J 2017;38(675):86.
Odelin G, Faure E, Coulpier F, Di Bonito M, Bajolle F, Studer M et al. Krox20 defines a subpopulation of cardiac neural crest cells contributing to arterial valves and bicuspid aortic valve. Development 2018;145:dev151944.
Hiratzka LF, Bakris GL, Beckman JA, Bersin RM, Carr VF, Casey DE Jr et al. 2010ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with Thoracic Aortic Disease: A report of the American College of Cardiology Foundation/ American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine. Circulation 2010;121: E266–369.
Campens L, Demulier L, De Groote K, Vandekerckhove K, De Wolf D, Roman MJ et al. Reference values for echocardiographic assessment of the diameter of the aortic root and ascending aorta spanning all age categories. Am J Cardiol 2014;114(914):20.
Devereux RB, de Simone G, Arnett DK, Best LG, Boerwinkle E, Howard BV et al. Normal limits in relation to age, body size and gender of two-dimensional echocardiographic aortic root dimensions in persons >15 years of age. Am J Cardiol 2012;110(1189):94.
Roman MJ, Devereux RB, Kramer-Fox R, O’Loughlin J. Two-dimensional echocardiographic aortic root dimensions in normal children and adults. Am J Cardiol 1989;64(507):12.
Sluysmans T, Colan SD. Theoretical and empirical derivation of cardiovascular allometric relationships in children. J Appl Physiol (1985) 2005;99:445–57.
Garcier JM, Petitcolin V, Filaire M, Mofid R, Azarnouch K, Ravel A et al. Normal diameter of the thoracic aorta in adults: A magnetic resonance imaging study. Surg Radiol Anat 2003;25(322):9.
Vriz O, Aboyans V, D’Andrea A, Ferrara F, Acri E, Limongelli G et al. Normal values of aortic root dimensions in healthy adults. Am J Cardiol 2014;114(921):7.
Wolak A, Gransar H, Thomson LE, Friedman JD, Hachamovitch R, Gutstein A et al. Aortic size assessment by noncontrast cardiac computed tomography: Normal limits by age, gender, and body surface area. JACC Cardiovasc Imaging 2008;1(200):9.
Park JY, Foley TA, Bonnichsen CR, Maurer MJ, Goergen KM, Nkomo VT et al. Transthoracic echocardiography versus computed tomography for ascending aortic measurements in patients with bicuspid aortic valve. J Am Soc Echocardiogr 2017;30(625):35.
Colan SD. The why and how of Z scores. J Am Soc Echocardiogr 2013;26:38–40.
Voges I, Jerosch-Herold M, Hedderich J, Pardun E, Hart C, Gabbert DD et al. Normal values of aortic dimensions, distensibility, and pulse wave velocity in children and young adults: A cross-sectional study. J Cardiovasc Magn Reson 2012;1477.
Jondeau G, Ropers J, Regalado E, Braverman A, Evangelista A, Teixedo G et al. International registry of patients carrying TGFBR1 or TGFBR2 mutations: Results of the MAC. (Montalcino Aortic Consortium). Circ Cardiovasc Genet 2016;9:548-58.
Silberbach M, Roos-Hesselink JW, Andersen NH, Braverman AC, Brown N, Collins RT et al.;. On behalf of the American Heart Association Council on Cardiovascular Disease in the Young; Council on Genomic and Precision Medicine; and Council on Peripheral Vascular Disease. Cardiovascular Health in Turner Syndrome: A scientific statement from the American Heart Association. Circ Genom Precis Med 2018;11: E000048.
Wojnarski CM, Svensson LG, Roselli EE, Idrees JJ, Lowry AM, Ehrlinger J et al. Aortic dissection in patients with bicuspid aortic valve-associated aneurysms. Ann Thorac Surg 2015;100(1666):73.discussion 1673-4.
Masri A, Kalahasti V, Svensson LG, Alashi A, Schoenhagen P, Roselli EE et al. Aortic cross-sectional area/height ratio and outcomes in patients with bicuspid aortic valve and a dilated ascending aorta. Circ Cardiovasc Imaging 2017;10e006249.
Della Corte A, Michelena HI, Citarella A, Votta E, Piatti F, Lo Presti F et al. Risk stratification in bicuspid aortic valve aortopathy: Emerging evidence and future perspectives. Curr Probl Cardiol 2021;46100428.
Della Corte A, Bancone C, Buonocore M, Dialetto G, Covino FE, Manduca S et al. Pattern of ascending aortic dimensions predicts the growth rate of the aorta in patients with bicuspid aortic valve. JACC Cardiovasc Imaging 2013;6(1301):10.
Della Corte A, Bancone C, Dialetto G, Covino FE, Manduca S, Montibello MV et al. The ascending aorta with bicuspid aortic valve: A phenotypic classification with potential prognostic significance. Eur J Cardiothorac Surg 2014;46(240):7.discussion 247.
Wojnarski CM, Roselli EE, Zhu Y, Carnes TA, Lowry AM et al. Machine-learning phenotypic classification of bicuspid aortopathy. J Thorac Cardiovasc Surg 2018;155:461-9.e4.
Adamo L, Braverman AC. Surgical threshold for bicuspid aortic valve aneurysm: A case for individual decision-making. Heart 2015;101(1361):7.
Avadhani SA, Martin-Doyle W, Shaikh AY, Pape LA. Predictors of ascending aortic dilation in bicuspid aortic valve disease: A five-year prospective study. Am J Med 2015;128(647):52.
Thanassoulis G, Yip JW, Filion K, Jamorski M, Webb G, Siu SC et al. Retrospective study to identify predictors of the presence and rapid progression of aortic dilatation in patients with bicuspid aortic valves. Nat Rev Cardiol 2008;5(821):8.
Girdauskas E, Geist L, Disha K, Kazakbaev I, Gro8 T, Schulz S et al. Genetic abnormalities in bicuspid aortic valve root phenotype: Preliminary results. Eur J Cardiothorac Surg 2017;52(156):62.
Pepe G, Nistri S, Giusti B, Sticchi E, Attanasio M, Porciani C et al. Identification of fibrillin 1 gene mutations in patients with bicuspid aortic valve (BAV) without Marfan syndrome. BMC Med Genet 2014;1523.
Barker AJ, Markl M, Burk J, Lorenz R, Bock J, Bauer S et al. Bicuspid aortic valve is associated with altered wall shear stress in the ascending aorta. Circ Cardiovasc Imaging 2012;5(457):66.
Rodriguez-Palomares JF, Dux-Santoy L, Guala A, Kale R, Maldonado G, Teixido-Tura G et al. Aortic flow patterns and wall shear stress maps by 4D-flow cardiovascular magnetic resonance in the assessment of aortic dilatation in bicuspid aortic valve disease. J Cardiovasc Magn Reson 2018;2028.
van Ooij P, Markl M, Collins JD, Carr JC, Rigsby C, Bonow RO et al. Aortic valve stenosis alters expression of regional aortic wall shear stress: New insights from a 4-dimensional flow magnetic resonance imaging study of 571 subjects. J Am Heart Assoc 2017;6e005959.
Fazel SS, Mallidi HR, Lee RS, Sheehan MP, Liang D, Fleischman D et al. The aortopathy of bicuspid aortic valve disease has distinctive patterns and usually involves the transverse aortic arch. J Thorac Cardiovasc Surg 2008;135:901-7, 907.e1-2
Girdauskas E, Disha K, Rouman M, Espinoza A, Borger MA, Kuntze T. Aortic events after isolated aortic valve replacement for bicuspid aortic valve root phenotype: Echocardiographic follow-up study. Eur J Cardiothorac Surg 2015;48(e71):6.
Forte A, Yin X, Fava M, Bancone C, Cipollaro M, De Feo M et al. Locally different proteome in aortas from patients with stenotic tricuspid and bicuspid aortic valvesdagger. EurJ Cardiothorac Surg 2019;56(458):69.
Guzzardi DG, Barker AJ, van Ooij P, Malaisrie SC, Puthumana JJ, Belke DD et al. Valve-related hemodynamics mediate human bicuspid aortopathy: Insights from wall shear stress mapping. J Am Coll Cardiol 2015;66:892–900.
Zeeshan A, Johnston DR, Rajeswaran J, Roselli EE, Soltesz EG et al. Durability of aortic valve cusp repair with and without annular support. Ann Thorac Surg 2018;105(739):48.
Lansac E, de Kerchove L. Aortic valve repair techniques: State of the art. Eur J Cardiothorac Surg 2018;53(1101):7.
Lindsay ME, Schepers D, Bolar NA, Doyle JJ, Gallo E, Fert-Bober J et al. Loss-of-function mutations in TGFB2 cause a syndromic presentation of thoracic aortic aneurysm. Nat Genet 2012;44(922):7.
Braverman AC, Roman MJ. Bicuspid aortic valve in Marfan syndrome. Circ Cardiovasc Imaging 2019;12e008860.
Yassine NM, Shahram JT, Body SC. Pathogenic mechanisms of bicuspid aortic valve aortopathy. Front Physiol 2017;8687.
Milewicz DM, Regalado ES. Use of genetics for personalized management of heritable thoracic aortic disease: How do we get there?. J Thorac Cardiovasc Surg 2015;149(S3):5.
Gillis E, Kumar AA, Luyckx I, Preuss C, Cannaerts E, van de Beek G et al. Candidate gene resequencing in a large bicuspid aortic valve-associated thoracic aortic aneurysm cohort: SMAD6 as an important contributor. Front Physiol 2017;8400.
Everett RJ, Tastet L, Clavel MA, Chin CWL, Capoulade R, Vassiliou VS et al. Progression of hypertrophy and myocardial fibrosis in aortic stenosis: A multicenter cardiac magnetic resonance study. Circ Cardiovasc Imaging 2018;11e007451.
Garcia J, Barker AJ, Murphy I, Jarvis K, Schnell S, Collins JD et al. Four-dimensional flow magnetic resonance imaging-based characterization of aortic morphometry and haemodynamics: Impact of age, aortic diameter, and valve morphology. Eur Heart J Cardiovasc Imaging 2016;17:877–84.
Blanke P, Weir-McCall JR, Achenbach S, Delgado V, Hausleiter J, Jilaihawi H et al. Computed tomography imaging in the context of transcatheter aortic valve implantation (TAVI)/transcatheter aortic valve replacement (TAVR): An expert consensus document of the Society of Cardiovascular Computed Tomography. J Cardiovasc Comput Tomogr 2019;13(1):20.
Francone M, Budde RPJ, Bremerich J, Dacher JN, Loewe C, Wolf F et al. CT and MR imaging prior to transcatheter aortic valve implantation: Standardisation of scanning protocols, measurements and reporting-a consensus document by the European Society of Cardiovascular Radiology (ESCR). Eur Radiol 2020;30(2627):50.
Amoretti F, Cerillo AG, Mariani M, Stefano P. A simple method to visualize the bicuspid aortic valve pathology by cardiac computed tomography. J Cardiovasc Comput Tomogr 2020;14(195):8.
Mack MJ, Leon MB, Thourani VH, Makkar R, Kodali SK, Russo M et al. Transcatheter aortic-valve replacement with a balloon-expandable valve in low-risk patients. N Engl J Med 2019;380:1695-705.
Popma JJ, Deeb GM, Yakubov SJ, Mumtaz M, Gada H, O’Hair D et al. Transcatheter aortic-valve replacement with a self-expanding valve in low-risk patients. N Engl J Med 2019;380:1706-15.
Smith CR, Leon MB, Mack MJ, Miller DC, Moses JW, Svensson LG et al. Transcatheter versus surgical aortic-valve replacement in high-risk patients. N Engl J Med 2011;364:2187-98.
Mylotte D, Lefevre T, Sondergaard L, Watanabe Y, Modine T, Dvir D et al. Transcatheter aortic valve replacement in bicuspid aortic valve disease. J Am Coll Cardiol 2014;64(2330):9.
Halim SA, Edwards FH, Dai D, Li Z, Mack MJ, Holmes DR et al. Outcomes of transcatheter aortic valve replacement in patients with bicuspid aortic valve disease: A report from the Society of Thoracic Surgeons/American College of Cardiology Transcatheter Valve Therapy Registry. Circulation 2020;141(1071):9.
Makkar RR, Yoon SH, Leon MB, Chakravarty T, Rinaldi M, Shah PB et al. Association between transcatheter aortic valve replacement for bicuspid vs tricuspid aortic stenosis and mortality or stroke. JAMA 2019;321:2193–202.
Forrest JK, Kaple RK, Ramlawi B, Gleason TG, Meduri CU, Yakubov SJ et al. Transcatheter aortic valve replacement in bicuspid versus tricuspid aortic valves from the STS/ACC TVT Registry. JACC Cardiovasc Interv 2020;13(1749):1759.
Yoon SH, Kim WK, Dhoble A, Milhorini Pio S, Babaliaros V, Jilaihawi H et al. Bicuspid aortic valve morphology and outcomes after transcatheter aortic valve replacement. J Am Coll Cardiol 2020;76(1018):30.
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