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 Fernandes¹¹, Paul W M Fedak¹², Evaldas Girdauskas¹³, Victoria Delgado¹⁴, Suhny Abbara¹⁵, Emmanuel Lansac¹⁶, Siddharth K Prakash¹⁷, Malenka M Bissell¹⁸, Bogdan A Popescu¹⁹, Michael D Hope²⁰, Marta Sitges²¹, Vinod H Thourani²², Phillippe Pibarot²³, Krishnaswamy Chandrasekaran²⁴, Patrizio Lancellotti²⁵, Michael A Borger²⁶, John K Forrest²⁷, John Webb²⁸, Dianna M Milewicz¹⁷, Raj Makkar²⁹, Martin B Leon³⁰, Stephen P Sanders³¹, Michael Markl³², Victor A Ferrari³³, William C Roberts³⁴, Jae-Kwan Song³⁵, Philipp Blanke³⁶, Charles S White³⁷, Samuel Siu³⁸, Lars G Svensson³⁹, Alan C Braverman⁴⁰, Joseph Bavaria⁴¹, Thoralf M Sundt⁴², Gebrine El Khoury¹⁰, Ruggero De Paulis⁴³, Maurice Enriquez-Sarano²⁴, Jeroen J Bax¹⁴, Catherine M Otto⁴⁴, Hans-Joachim Schäfers⁴⁵

Affiliations

¹ Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota. Electronic address: michelena.hector@mayo.edu.
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, Minnesota.
⁵ Division of Cardiology, Weill Cornell Medicine, New York, New York.
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.
⁸ Department of Radiology, Children's Hospital Colorado, University of Colorado, Anschutz Medical Campus, Aurora, Colorado.
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, California; Division of Cardiovascular Medicine, Department of Medicine, Stanford University, Palo Alto, California.
¹² Department of Cardiac Sciences, Libin Cardiovascular Institute, Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada.
¹³ Department of Cardiovascular Surgery, University Heart and Vascular Center Hamburg, Hamburg, Germany.
¹⁴ Department of Cardiology, Leiden University Medical Center, Leiden, Netherlands.
¹⁵ Cardiothoracic Imaging Division, Department of Radiology, UT Southwestern Medical Center, Dallas, Texas.
¹⁶ Department of Cardiac Surgery, Institute Mutualiste Montsouris, Paris, France.
¹⁷ Department of Internal Medicine, McGovern Medical School, The University of Texas Health Science Center at Houston, Houston, Texas.
¹⁸ Department of Biomedical Imaging Science, Leeds Institute to Cardiovascular and Metabolic Medicine, University of Leeds, Leeds, United Kingdom.
¹⁹ Department of Cardiology, University of Medicine and Pharmacy "Carol Davila"-Euroecolab, Emergency Institute for Cardiovascular Diseases "Prof. Dr. C. C. Iliescu", Bucharest, Romania.
²⁰ Department of Radiology and Biomedical Imaging, University of California San Francisco, San Francisco, California.
²¹ Cardiovascular Institute, Hospital Clínic, Universitat de Barcelona, IDIBAPS, CIBERCV, ISCIII (CB16/11/00354), CERCA Programme, Barcelona, Spain.
²² Department of Cardiovascular Surgery, Marcus Valve Center, Piedmont Heart Institute, Atlanta, Georgia.
²³ Department of Cardiology, Québec Heart & Lung Institute, Laval University Québec, Québec, Canada.
²⁴ Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota.
²⁵ Department of Cardiology, University of Liège Hospital, GIGA Cardiovascular Sciences, CHU Sart Tilman, Liège, Belgium; Gruppo Villa Maria Care and Research, Maria Cecilia Hospital, Cotignola, and Anthea Hospital, Bari, Italy.
²⁶ University Clinic of Cardiac Surgery, Leipzig Heart Center, Leipzig, Germany.
²⁷ Yale University School of Medicine & Yale New Haven Hospital, New Haven, Connecticut.
²⁸ St Paul's Hospital, University of British Columbia, Vancouver, British Columbia, Canada.
²⁹ Cedars Sinai Heart Institute, Los Angeles, California.
³⁰ Division of Cardiology, Columbia University Irving Medical Center/NY Presbyterian Hospital, New York, New York.
³¹ Cardiac Registry, Departments of Cardiology, Pathology and Cardiac Surgery, Boston Children's Hospital, Boston, Massachusetts; Department of Pediatrics, Harvard Medical School, Boston, Massachusetts.
³² Department of Radiology, Feinberg School of Medicine, Northwestern University, Chicago, Illinois.
³³ Cardiovascular Medicine Division, University of Pennsylvania Medical Center and Penn Cardiovascular Institute, Philadelphia, Pennsylvania.
³⁴ Baylor Heart and Vascular Institute, Baylor University Medical Center, Texas A & M School of Medicine, Dallas Campus, Dallas, Texas.
³⁵ University of Ulsan College of Medicine, Asan Medical Center, Seoul, South Korea.
³⁶ Department of Radiology, St. Paul's Hospital, Vancouver, British Columbia, Canada.
³⁷ Department of Radiology, University of Maryland School of Medicine, Baltimore, Maryland.
³⁸ Schulich School of Medicine and Dentistry, London, Ontario, Canada.
³⁹ Heart, Vascular and Thoracic Institute, Cleveland Clinic, Cleveland, Ohio.
⁴⁰ Cardiovascular Division, Department of Medicine, Washington University School of Medicine, St. Louis, Missouri.
⁴¹ Division of Cardiac Surgery, University of Pennsylvania, Philadelphia, Pennsylvania.
⁴² Division of Cardiac Surgery, Massachusetts General Hospital, Boston, Massachusetts.
⁴³ Department of Cardiac Surgery, European Hospital and Unicamillus University Rome, Rome, Italy.
⁴⁴ Division of Cardiology, University of Washington, Seattle, Washington.
⁴⁵ Department of Thoracic and Cardiovascular Surgery, Saarland University Medical Center, Homburg/Saar, Germany.

PMID: 34304860
DOI: 10.1016/j.athoracsur.2020.08.119

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. Ann Thorac Surg. 2021 Sep.

. 2021 Sep;112(3):e203-e235.

doi: 10.1016/j.athoracsur.2020.08.119. Epub 2021 Jul 22.

Authors

Affiliations

¹ Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota. Electronic address: michelena.hector@mayo.edu.
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, Minnesota.
⁵ Division of Cardiology, Weill Cornell Medicine, New York, New York.
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.
⁸ Department of Radiology, Children's Hospital Colorado, University of Colorado, Anschutz Medical Campus, Aurora, Colorado.
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, California; Division of Cardiovascular Medicine, Department of Medicine, Stanford University, Palo Alto, California.
¹² Department of Cardiac Sciences, Libin Cardiovascular Institute, Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada.
¹³ Department of Cardiovascular Surgery, University Heart and Vascular Center Hamburg, Hamburg, Germany.
¹⁴ Department of Cardiology, Leiden University Medical Center, Leiden, Netherlands.
¹⁵ Cardiothoracic Imaging Division, Department of Radiology, UT Southwestern Medical Center, Dallas, Texas.
¹⁶ Department of Cardiac Surgery, Institute Mutualiste Montsouris, Paris, France.
¹⁷ Department of Internal Medicine, McGovern Medical School, The University of Texas Health Science Center at Houston, Houston, Texas.
¹⁸ Department of Biomedical Imaging Science, Leeds Institute to Cardiovascular and Metabolic Medicine, University of Leeds, Leeds, United Kingdom.
¹⁹ Department of Cardiology, University of Medicine and Pharmacy "Carol Davila"-Euroecolab, Emergency Institute for Cardiovascular Diseases "Prof. Dr. C. C. Iliescu", Bucharest, Romania.
²⁰ Department of Radiology and Biomedical Imaging, University of California San Francisco, San Francisco, California.
²¹ Cardiovascular Institute, Hospital Clínic, Universitat de Barcelona, IDIBAPS, CIBERCV, ISCIII (CB16/11/00354), CERCA Programme, Barcelona, Spain.
²² Department of Cardiovascular Surgery, Marcus Valve Center, Piedmont Heart Institute, Atlanta, Georgia.
²³ Department of Cardiology, Québec Heart & Lung Institute, Laval University Québec, Québec, Canada.
²⁴ Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota.
²⁵ Department of Cardiology, University of Liège Hospital, GIGA Cardiovascular Sciences, CHU Sart Tilman, Liège, Belgium; Gruppo Villa Maria Care and Research, Maria Cecilia Hospital, Cotignola, and Anthea Hospital, Bari, Italy.
²⁶ University Clinic of Cardiac Surgery, Leipzig Heart Center, Leipzig, Germany.
²⁷ Yale University School of Medicine & Yale New Haven Hospital, New Haven, Connecticut.
²⁸ St Paul's Hospital, University of British Columbia, Vancouver, British Columbia, Canada.
²⁹ Cedars Sinai Heart Institute, Los Angeles, California.
³⁰ Division of Cardiology, Columbia University Irving Medical Center/NY Presbyterian Hospital, New York, New York.
³¹ Cardiac Registry, Departments of Cardiology, Pathology and Cardiac Surgery, Boston Children's Hospital, Boston, Massachusetts; Department of Pediatrics, Harvard Medical School, Boston, Massachusetts.
³² Department of Radiology, Feinberg School of Medicine, Northwestern University, Chicago, Illinois.
³³ Cardiovascular Medicine Division, University of Pennsylvania Medical Center and Penn Cardiovascular Institute, Philadelphia, Pennsylvania.
³⁴ Baylor Heart and Vascular Institute, Baylor University Medical Center, Texas A & M School of Medicine, Dallas Campus, Dallas, Texas.
³⁵ University of Ulsan College of Medicine, Asan Medical Center, Seoul, South Korea.
³⁶ Department of Radiology, St. Paul's Hospital, Vancouver, British Columbia, Canada.
³⁷ Department of Radiology, University of Maryland School of Medicine, Baltimore, Maryland.
³⁸ Schulich School of Medicine and Dentistry, London, Ontario, Canada.
³⁹ Heart, Vascular and Thoracic Institute, Cleveland Clinic, Cleveland, Ohio.
⁴⁰ Cardiovascular Division, Department of Medicine, Washington University School of Medicine, St. Louis, Missouri.
⁴¹ Division of Cardiac Surgery, University of Pennsylvania, Philadelphia, Pennsylvania.
⁴² Division of Cardiac Surgery, Massachusetts General Hospital, Boston, Massachusetts.
⁴³ Department of Cardiac Surgery, European Hospital and Unicamillus University Rome, Rome, Italy.
⁴⁴ Division of Cardiology, University of Washington, Seattle, Washington.
⁴⁵ Department of Thoracic and Cardiovascular Surgery, Saarland University Medical Center, Homburg/Saar, Germany.

PMID: 34304860
DOI: 10.1016/j.athoracsur.2020.08.119

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.

Copyright © 2021 Jointly between The Society of Thoracic Surgeons, the American Association for Thoracic Surgery, the European Association for Cardio-Thoracic Surgery, and the Radiological Society of North America. Published by Elsevier Inc. All rights reserved.

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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

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LinkOut - more resources

Full Text Sources