International consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional and research purposes

doi:10.1093/ejcts/ezab038

. 2021 Sep 11;60(3):448-476.

doi: 10.1093/ejcts/ezab038.

International consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional and research purposes

Hector I Michelena¹, Alessandro Della Corte², Arturo Evangelista³, Joseph J Maleszewski⁴, William D Edwards⁴, Mary J Roman⁵, Richard B Devereux⁵, Borja Fernández⁶, Federico M Asch⁷, Alex J Barker⁸, Lilia M Sierra-Galan⁹, Laurent De Kerchove¹⁰, Susan M Fernandes^{11

12}, Paul W M Fedak¹³, Evaldas Girdauskas¹⁴, Victoria Delgado¹⁵, Suhny Abbara¹⁶, Emmanuel Lansac¹⁷, Siddharth K Prakash¹⁸, Malenka M Bissell¹⁹, Bogdan A Popescu²⁰, Michael D Hope²¹, Marta Sitges²², Vinod H Thourani²³, Phillippe Pibarot²⁴, Krishnaswamy Chandrasekaran¹, Patrizio Lancellotti^{25

26}, Michael A Borger²⁷, John K Forrest²⁸, John Webb²⁹, Dianna M Milewicz¹⁸, Raj Makkar³⁰, Martin B Leon³¹, Stephen P Sanders^{32

33}, Michael Markl³⁴, Victor A Ferrari³⁵, William C Roberts³⁶, Jae-Kwan Song³⁷, Philipp Blanke³⁸, Charles S White³⁹, Samuel Siu⁴⁰, Lars G Svensson⁴¹, Alan C Braverman⁴², Joseph Bavaria⁴³, Thoralf M Sundt⁴⁴, Gebrine El Khoury¹⁰, Ruggero De Paulis⁴⁵, Maurice Enriquez-Sarano¹, Jeroen J Bax¹⁵, Catherine M Otto⁴⁶, Hans-Joachim Schäfers⁴⁷; Endorsed by the Heart Valve Society (HVS), European Association of Cardiovascular Imaging (EACVI), Society of Thoracic Surgeons (STS), American Association for Thoracic Surgery (AATS), Society for Cardiovascular Magnetic Resonance (SCMR), Society of Cardiovascular Computed Tomography (SCCT), North American Society for Cardiovascular Imaging (NASCI) and the International Bicuspid Aortic Valve Consortium (BAVCon)

Affiliations

¹ Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN, USA.
² Department of Translational Medical Sciences, University of Campania "L. Vanvitelli", Naples, Italy.
³ Department of Cardiology, Hospital Vall d'Hebron, Vall d'Hebron Research Institute (VHIR) Ciber-CV, Barcelona, Spain.
⁴ Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA.
⁵ Division of Cardiology, Weill Cornell Medicine, New York, NY, USA.
⁶ Departamento de Biología Animal, Facultad de Ciencias, Instituto de Investigación Biomédica de Málaga, Universidad de Málaga, Ciber-CV, Málaga, Spain.
⁷ MedStar Health Research Institute, Washington, DC, USA.
⁸ Department of Radiology, Children's Hospital Colorado, University of Colorado, Anschutz Medical Campus, Aurora, CO, USA.
⁹ Cardiovascular Division, American British Cowdray Medical Center, Mexico City, Mexico.
¹⁰ Division of Cardiothoracic and Vascular Surgery, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium.
¹¹ Division of Pediatric Cardiology, Department of Pediatrics, Stanford University, Palo Alto, CA, USA.
¹² Division of Cardiovascular Medicine, Department of Medicine, Stanford University, Palo Alto, CA, USA.
¹³ Department of Cardiac Sciences, Libin Cardiovascular Institute, Cumming School of Medicine, University of Calgary, Calgary, Canada.
¹⁴ Department of Cardiovascular Surgery, University Heart and Vascular Center Hamburg, Hamburg, Germany.
¹⁵ Department of Cardiology, Leiden University Medical Center, Leiden, Netherlands.
¹⁶ Cardiothoracic Imaging Division, Department of Radiology, UT Southwestern Medical Center, Dallas, TX, USA.
¹⁷ Department of Cardiac Surgery, Institute Mutualiste Montsouris, Paris, France.
¹⁸ Department of Internal Medicine, McGovern Medical School, The University of Texas Health Science Center at Houston, Houston, TX, USA.
¹⁹ Department of Biomedical Imaging Science, Leeds Institute to Cardiovascular and Metabolic Medicine, University of Leeds, Leeds, UK.
²⁰ Department of Cardiology, University of Medicine and Pharmacy "Carol Davila"-Euroecolab, Emergency Institute for Cardiovascular Diseases "Prof. Dr. C. C. Iliescu", Bucharest, Romania.
²¹ Department of Radiology and Biomedical Imaging, University of California San Francisco, San Francisco, CA, USA.
²² Cardiovascular Institute, Hospital Clínic, Universitat de Barcelona, IDIBAPS, CIBERCV, ISCIII (CB16/11/00354), CERCA Programme, Barcelona, Spain.
²³ Department of Cardiovascular Surgery, Marcus Valve Center, Piedmont Heart Institute, Atlanta, GA, USA.
²⁴ Department of Cardiology, Québec Heart & Lung Institute, Laval University Québec, Québec, Canada.
²⁵ Department of Cardiology, University of Liège Hospital, GIGA Cardiovascular Sciences, CHU Sart Tilman,Liège, Belgium.
²⁶ Gruppo Villa Maria Care and Research, Maria Cecilia Hospital, Cotignola, and Anthea Hospital, Bari, Italy.
²⁷ University Clinic of Cardiac Surgery, Leipzig Heart Center, Leipzig, Germany.
²⁸ Yale University School of Medicine & Yale New Haven Hospital, New Haven, CT, USA.
²⁹ St Paul's Hospital, University of British Columbia, Vancouver, Canada.
³⁰ Cedars Sinai Heart Institute, Los Angeles, CA, USA.
³¹ Division of Cardiology, Columbia University Irving Medical Center/NY Presbyterian Hospital, New York, NY, USA.
³² Cardiac Registry, Departments of Cardiology, Pathology and Cardiac Surgery, Boston Children's Hospital, Boston, MA, USA.
³³ Department of Pediatrics, Harvard Medical School, Boston, MA, USA.
³⁴ Department of Radiology, Feinberg School of Medicine, Northwestern University, Chicago, IL, USA.
³⁵ Cardiovascular Medicine Division, University of Pennsylvania Medical Center and Penn Cardiovascular Institute, Philadelphia, PA, USA.
³⁶ Baylor Heart and Vascular Institute, Baylor University Medical Center, Texas A & M School of Medicine, Dallas Campus, Dallas, TX, USA.
³⁷ University of Ulsan College of Medicine, Asan Medical Center, Seoul, South Korea.
³⁸ Department of Radiology, St. Paul's Hospital, Vancouver, BC, Canada.
³⁹ Department of Radiology, University of Maryland School of Medicine, Baltimore, MD, USA.
⁴⁰ Schulich School of Medicine and Dentistry, London, ON, Canada.
⁴¹ Heart, Vascular and Thoracic Institute, Cleveland Clinic, Cleveland, OH, USA.
⁴² Cardiovascular Division, Department of Medicine, Washington University School of Medicine, St. Louis, MO, USA.
⁴³ Division of Cardiac Surgery, University of Pennsylvania, Philadelphia, PA, USA.
⁴⁴ Division of Cardiac Surgery, Massachusetts General Hospital, Boston, MA, USA.
⁴⁵ Department of Cardiac Surgery, European Hospital and Unicamillus University Rome, Rome, Italy.
⁴⁶ Division of Cardiology, University of Washington, Seattle, WA, USA.
⁴⁷ Department of Thoracic and Cardiovascular Surgery, Saarland University Medical Center, Homburg/Saar, Germany.

PMID: 34293102
DOI: 10.1093/ejcts/ezab038

Free article

International consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional and research purposes

Hector I Michelena et al. Eur J Cardiothorac Surg. 2021.

Free article

. 2021 Sep 11;60(3):448-476.

doi: 10.1093/ejcts/ezab038.

Authors

Affiliations

¹ Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN, USA.
² Department of Translational Medical Sciences, University of Campania "L. Vanvitelli", Naples, Italy.
³ Department of Cardiology, Hospital Vall d'Hebron, Vall d'Hebron Research Institute (VHIR) Ciber-CV, Barcelona, Spain.
⁴ Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA.
⁵ Division of Cardiology, Weill Cornell Medicine, New York, NY, USA.
⁶ Departamento de Biología Animal, Facultad de Ciencias, Instituto de Investigación Biomédica de Málaga, Universidad de Málaga, Ciber-CV, Málaga, Spain.
⁷ MedStar Health Research Institute, Washington, DC, USA.
⁸ Department of Radiology, Children's Hospital Colorado, University of Colorado, Anschutz Medical Campus, Aurora, CO, USA.
⁹ Cardiovascular Division, American British Cowdray Medical Center, Mexico City, Mexico.
¹⁰ Division of Cardiothoracic and Vascular Surgery, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium.
¹¹ Division of Pediatric Cardiology, Department of Pediatrics, Stanford University, Palo Alto, CA, USA.
¹² Division of Cardiovascular Medicine, Department of Medicine, Stanford University, Palo Alto, CA, USA.
¹³ Department of Cardiac Sciences, Libin Cardiovascular Institute, Cumming School of Medicine, University of Calgary, Calgary, Canada.
¹⁴ Department of Cardiovascular Surgery, University Heart and Vascular Center Hamburg, Hamburg, Germany.
¹⁵ Department of Cardiology, Leiden University Medical Center, Leiden, Netherlands.
¹⁶ Cardiothoracic Imaging Division, Department of Radiology, UT Southwestern Medical Center, Dallas, TX, USA.
¹⁷ Department of Cardiac Surgery, Institute Mutualiste Montsouris, Paris, France.
¹⁸ Department of Internal Medicine, McGovern Medical School, The University of Texas Health Science Center at Houston, Houston, TX, USA.
¹⁹ Department of Biomedical Imaging Science, Leeds Institute to Cardiovascular and Metabolic Medicine, University of Leeds, Leeds, UK.
²⁰ Department of Cardiology, University of Medicine and Pharmacy "Carol Davila"-Euroecolab, Emergency Institute for Cardiovascular Diseases "Prof. Dr. C. C. Iliescu", Bucharest, Romania.
²¹ Department of Radiology and Biomedical Imaging, University of California San Francisco, San Francisco, CA, USA.
²² Cardiovascular Institute, Hospital Clínic, Universitat de Barcelona, IDIBAPS, CIBERCV, ISCIII (CB16/11/00354), CERCA Programme, Barcelona, Spain.
²³ Department of Cardiovascular Surgery, Marcus Valve Center, Piedmont Heart Institute, Atlanta, GA, USA.
²⁴ Department of Cardiology, Québec Heart & Lung Institute, Laval University Québec, Québec, Canada.
²⁵ Department of Cardiology, University of Liège Hospital, GIGA Cardiovascular Sciences, CHU Sart Tilman,Liège, Belgium.
²⁶ Gruppo Villa Maria Care and Research, Maria Cecilia Hospital, Cotignola, and Anthea Hospital, Bari, Italy.
²⁷ University Clinic of Cardiac Surgery, Leipzig Heart Center, Leipzig, Germany.
²⁸ Yale University School of Medicine & Yale New Haven Hospital, New Haven, CT, USA.
²⁹ St Paul's Hospital, University of British Columbia, Vancouver, Canada.
³⁰ Cedars Sinai Heart Institute, Los Angeles, CA, USA.
³¹ Division of Cardiology, Columbia University Irving Medical Center/NY Presbyterian Hospital, New York, NY, USA.
³² Cardiac Registry, Departments of Cardiology, Pathology and Cardiac Surgery, Boston Children's Hospital, Boston, MA, USA.
³³ Department of Pediatrics, Harvard Medical School, Boston, MA, USA.
³⁴ Department of Radiology, Feinberg School of Medicine, Northwestern University, Chicago, IL, USA.
³⁵ Cardiovascular Medicine Division, University of Pennsylvania Medical Center and Penn Cardiovascular Institute, Philadelphia, PA, USA.
³⁶ Baylor Heart and Vascular Institute, Baylor University Medical Center, Texas A & M School of Medicine, Dallas Campus, Dallas, TX, USA.
³⁷ University of Ulsan College of Medicine, Asan Medical Center, Seoul, South Korea.
³⁸ Department of Radiology, St. Paul's Hospital, Vancouver, BC, Canada.
³⁹ Department of Radiology, University of Maryland School of Medicine, Baltimore, MD, USA.
⁴⁰ Schulich School of Medicine and Dentistry, London, ON, Canada.
⁴¹ Heart, Vascular and Thoracic Institute, Cleveland Clinic, Cleveland, OH, USA.
⁴² Cardiovascular Division, Department of Medicine, Washington University School of Medicine, St. Louis, MO, USA.
⁴³ Division of Cardiac Surgery, University of Pennsylvania, Philadelphia, PA, USA.
⁴⁴ Division of Cardiac Surgery, Massachusetts General Hospital, Boston, MA, USA.
⁴⁵ Department of Cardiac Surgery, European Hospital and Unicamillus University Rome, Rome, Italy.
⁴⁶ Division of Cardiology, University of Washington, Seattle, WA, USA.
⁴⁷ Department of Thoracic and Cardiovascular Surgery, Saarland University Medical Center, Homburg/Saar, Germany.

PMID: 34293102
DOI: 10.1093/ejcts/ezab038

Abstract

This International Consensus Classification and Nomenclature for the congenital bicuspid aortic valve condition recognizes 3 types of bicuspid valves: 1. The fused type (right-left cusp fusion, right-non-coronary cusp fusion and left-non-coronary cusp fusion phenotypes); 2. The 2-sinus type (latero-lateral and antero-posterior phenotypes); and 3. The partial-fusion (forme fruste) type. The presence of raphe and the symmetry of the fused type phenotypes are critical aspects to describe. The International Consensus also recognizes 3 types of bicuspid valve-associated aortopathy: 1. The ascending phenotype; 2. The root phenotype; and 3. Extended phenotypes.

Keywords: Aortopathy; Bicuspid aortic valve; Classification; Nomenclature.

This article has been co-published with permission in the European Journal of Cardio-Thoracic Surgery, The Annals of Thoracic Surgery, the Journal of Thoracic and Cardiovascular Surgery, and in Radiology: Cardiothoracic Imaging. All rights reserved. © 2021 Jointly between the European Association for Cardio-Thoracic Surgery, The Society of Thoracic Surgeons, the American Association for Thoracic Surgery, and the Radiological Society of North America. The articles are identical except for minor stylistic and spelling differences in keeping with each journal's style.

PubMed Disclaimer

Comment in

What is in a name for bicuspid aortic valve aortopathy?
Sádaba JR, Álvarez-Asiain V. Sádaba JR, et al. Eur J Cardiothorac Surg. 2021 Sep 11;60(3):477-478. doi: 10.1093/ejcts/ezab033. Eur J Cardiothorac Surg. 2021. PMID: 34292333 No abstract available.
The bicuspid aortic valve complex: still a mystery.
Sievers HH. Sievers HH. Eur J Cardiothorac Surg. 2021 Sep 11;60(3):479-480. doi: 10.1093/ejcts/ezab073. Eur J Cardiothorac Surg. 2021. PMID: 34292340 No abstract available.

Cited by

Bicuspid Morphology and Rapid Deployment Valve Replacement: Is This Still a Contraindication?
von der Linden J, Herrmann F, Belyaev S, Juchem G, Peterss S, Hagl C, Dashkevich A. von der Linden J, et al. J Clin Med. 2023 Nov 29;12(23):7390. doi: 10.3390/jcm12237390. J Clin Med. 2023. PMID: 38068441 Free PMC article.
Aortic Regurgitation in Bicuspid Aortic Valve: The Role of Multimodality Imaging.
La Mura L, Lembo M, Musella F, D'Amato M, D'Andrea A, Izzo R, Esposito G. La Mura L, et al. J Clin Med. 2024 Jul 4;13(13):3924. doi: 10.3390/jcm13133924. J Clin Med. 2024. PMID: 38999489 Free PMC article. Review.
Progression of the ascending aorta diameter after surgical or transcatheter bicuspid aortic valve replacement.
Chiariello GA, Di Mauro M, Pasquini A, Bruno P, Nesta M, Fabiani L, Mazza A, Meloni M, Baldo E, Ponzo M, Ferraro F, Conserva AD, D'Acierno E, Villa E, Trani C, Burzotta F, Massetti M. Chiariello GA, et al. Interdiscip Cardiovasc Thorac Surg. 2024 May 2;38(5):ivae100. doi: 10.1093/icvts/ivae100. Interdiscip Cardiovasc Thorac Surg. 2024. PMID: 38775458 Free PMC article.
Severe acute recoil following transcatheter aortic valve replacement with a self-expanding prosthesis in a heavily calcified bicuspid aortic valve.
García-Escobar A, Jiménez-Valero S, Galeote G, Jurado-Román A, Cabrera JÁ, Moreno R. García-Escobar A, et al. Future Cardiol. 2024 Dec-Dec;20(15-16):823-826. doi: 10.1080/14796678.2024.2421688. Epub 2024 Nov 19. Future Cardiol. 2024. PMID: 39560006
Bicuspid aortic valve repair-current techniques, outcomes, challenges, and future perspectives.
Zhang H. Zhang H. Front Cardiovasc Med. 2024 Jan 3;10:1295146. doi: 10.3389/fcvm.2023.1295146. eCollection 2023. Front Cardiovasc Med. 2024. PMID: 38235290 Free PMC article. Review.

See all "Cited by" articles

MeSH terms

Actions
Actions
Actions
Actions
Actions
Actions
Actions

LinkOut - more resources

Full Text Sources

Save citation to file

Email citation

Add to Collections

Add to My Bibliography

Your saved search

Create a file for external citation management software

Your RSS Feed

International consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional and research purposes

Affiliations

International consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional and research purposes

Authors

Affiliations

Abstract

Comment in

Similar articles

Cited by

MeSH terms

LinkOut - more resources

Full Text Sources

Abstract

Comment in

Similar articles

Cited by

MeSH terms

Related information

LinkOut - more resources

Full Text Sources