Summary: 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 Edwards⁵, Mary J Roman⁶, Richard B Devereux⁶, Borja Fernández⁷, Federico M Asch⁸, Alex J Barker⁹, Lilia M Sierra-Galan¹⁰, Laurent De Kerchove¹¹, Susan M Fernandes¹², 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 Chandrasekaran²⁵, Patrizio Lancellotti²⁶, Michael A Borger²⁷, John K Forrest²⁸, John Webb²⁹, Dianna M Milewicz¹⁸, Raj Makkar²⁸, Martin B Leon²⁹, Stephen P Sanders³⁰, Michael Markl³¹, Victor A Ferrari³², William C Roberts³³, Jae-Kwan Song³⁴, Philipp Blanke³⁵, Charles S White³⁶, Samuel Siu³⁷, Lars G Svensson³⁸, Alan C Braverman³⁹, Joseph Bavaria⁴⁰, Thoralf M Sundt⁴¹, Gebrine El Khoury¹¹, Ruggero De Paulis⁴², Maurice Enriquez-Sarano²⁵, Jeroen J Bax¹⁵, Catherine M Otto⁴³, Hans-Joachim Schäfers⁴⁴

Affiliations

¹ Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minn. 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 Cardiovascular Medicine, Mayo Clinic, Rochester, Minn; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minn.
⁵ Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minn.
⁶ Division of Cardiology, Weill Cornell Medicine, New York, NY.
⁷ Departamento de Biología Animal, Facultad de Ciencias, Instituto de Investigación Biomédica de Málaga, Universidad de Málaga, Ciber-CV, Málaga, Spain.
⁸ MedStar Health Research Institute, Washington, DC.
⁹ Department of Radiology, Children's Hospital Colorado, University of Colorado, Anschutz Medical Campus, Colo.
¹⁰ Cardiovascular Division, American British Cowdray Medical Center, Mexico City, Mexico.
¹¹ Division of Cardiothoracic and Vascular Surgery, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium.
¹² Department of Pediatrics, Division of Pediatric Cardiology, Stanford University, Palo Alto, Calif; Department of Medicine, Division of Cardiovascular Medicine, Stanford University, Palo Alto, Calif.
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, The Netherlands.
¹⁶ Cardiothoracic Imaging Division, Department of Radiology, UT Southwestern Medical Center, Dallas, Tex.
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, Tex.
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, Calif.
²² Cardiovascular Institute, Hospital Clínic, Universitat de Barcelona, Spain, IDIBAPS; CIBERCV, ISCIII (CB16/11/00354); and CERCA Programme.
²³ Department of Cardiovascular Surgery, Marcus Valve Center, Piedmont Heart Institute, Atlanta, Ga.
²⁴ Department of Cardiology, Québec Heart & Lung Institute, Laval University, Québec, Canada.
²⁵ Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minn.
²⁶ 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, Italy; 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, Conn.
²⁹ St Paul's Hospital, University of British Columbia, Vancouver, Canada; aeCedars Sinai Heart Institute, Los Angeles, Calif; afDivision of Cardiology, Columbia University Irving Medical Center/NY Presbyterian Hospital, New York, NY.
³⁰ Cardiac Registry, Departments of Cardiology, Pathology and Cardiac Surgery, Boston Children's Hospital, Boston, Mass; Department of Pediatrics, Harvard Medical School, Boston, Mass.
³¹ Department of Radiology, Feinberg School of Medicine, Northwestern University, Chicago, Ill.
³² Cardiovascular Medicine Division, University of Pennsylvania Medical Center and Penn Cardiovascular Institute, Philadelphia, Pa.
³³ Baylor Heart and Vascular Institute, Baylor University Medical Center, Dallas, Tex; Texas A & M School of Medicine, Dallas, Tex.
³⁴ University of Ulsan College of Medicine, Asan Medical Center, Seoul, South Korea.
³⁵ Department of Radiology, St. Paul's Hospital, Vancouver, British Columbia, Canada.
³⁶ Department of Radiology, University of Maryland School of Medicine, Baltimore, Md.
³⁷ Schulich School of Medicine and Dentistry, London, Ontario, Canada.
³⁸ Heart, Vascular and Thoracic Institute, Cleveland Clinic, Cleveland, Ohio.
³⁹ Cardiovascular Division, Department of Medicine, Washington University School of Medicine, St. Louis, Mo.
⁴⁰ Division of Cardiac Surgery, University of Pennsylvania, Philadelphia, Pa.
⁴¹ Division of Cardiac Surgery, Massachusetts General Hospital, Boston, Mass.
⁴² Department of Cardiac Surgery, European Hospital and Unicamillus University, Rome, Italy.
⁴³ Division of Cardiology, University of Washington, Seattle, Wash.
⁴⁴ Division of Thoracic and Cardiovascular Surgery, Saarland University Medical Center, Homburg, Saar, Germany.

PMID: 34304894
DOI: 10.1016/j.jtcvs.2021.05.008

Free article

Review

Summary: 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. J Thorac Cardiovasc Surg. 2021 Sep.

Free article

. 2021 Sep;162(3):781-797.

doi: 10.1016/j.jtcvs.2021.05.008. Epub 2021 Jul 22.

Authors

Affiliations

¹ Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minn. 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 Cardiovascular Medicine, Mayo Clinic, Rochester, Minn; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minn.
⁵ Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minn.
⁶ Division of Cardiology, Weill Cornell Medicine, New York, NY.
⁷ Departamento de Biología Animal, Facultad de Ciencias, Instituto de Investigación Biomédica de Málaga, Universidad de Málaga, Ciber-CV, Málaga, Spain.
⁸ MedStar Health Research Institute, Washington, DC.
⁹ Department of Radiology, Children's Hospital Colorado, University of Colorado, Anschutz Medical Campus, Colo.
¹⁰ Cardiovascular Division, American British Cowdray Medical Center, Mexico City, Mexico.
¹¹ Division of Cardiothoracic and Vascular Surgery, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium.
¹² Department of Pediatrics, Division of Pediatric Cardiology, Stanford University, Palo Alto, Calif; Department of Medicine, Division of Cardiovascular Medicine, Stanford University, Palo Alto, Calif.
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, The Netherlands.
¹⁶ Cardiothoracic Imaging Division, Department of Radiology, UT Southwestern Medical Center, Dallas, Tex.
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, Tex.
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, Calif.
²² Cardiovascular Institute, Hospital Clínic, Universitat de Barcelona, Spain, IDIBAPS; CIBERCV, ISCIII (CB16/11/00354); and CERCA Programme.
²³ Department of Cardiovascular Surgery, Marcus Valve Center, Piedmont Heart Institute, Atlanta, Ga.
²⁴ Department of Cardiology, Québec Heart & Lung Institute, Laval University, Québec, Canada.
²⁵ Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minn.
²⁶ 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, Italy; 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, Conn.
²⁹ St Paul's Hospital, University of British Columbia, Vancouver, Canada; aeCedars Sinai Heart Institute, Los Angeles, Calif; afDivision of Cardiology, Columbia University Irving Medical Center/NY Presbyterian Hospital, New York, NY.
³⁰ Cardiac Registry, Departments of Cardiology, Pathology and Cardiac Surgery, Boston Children's Hospital, Boston, Mass; Department of Pediatrics, Harvard Medical School, Boston, Mass.
³¹ Department of Radiology, Feinberg School of Medicine, Northwestern University, Chicago, Ill.
³² Cardiovascular Medicine Division, University of Pennsylvania Medical Center and Penn Cardiovascular Institute, Philadelphia, Pa.
³³ Baylor Heart and Vascular Institute, Baylor University Medical Center, Dallas, Tex; Texas A & M School of Medicine, Dallas, Tex.
³⁴ University of Ulsan College of Medicine, Asan Medical Center, Seoul, South Korea.
³⁵ Department of Radiology, St. Paul's Hospital, Vancouver, British Columbia, Canada.
³⁶ Department of Radiology, University of Maryland School of Medicine, Baltimore, Md.
³⁷ Schulich School of Medicine and Dentistry, London, Ontario, Canada.
³⁸ Heart, Vascular and Thoracic Institute, Cleveland Clinic, Cleveland, Ohio.
³⁹ Cardiovascular Division, Department of Medicine, Washington University School of Medicine, St. Louis, Mo.
⁴⁰ Division of Cardiac Surgery, University of Pennsylvania, Philadelphia, Pa.
⁴¹ Division of Cardiac Surgery, Massachusetts General Hospital, Boston, Mass.
⁴² Department of Cardiac Surgery, European Hospital and Unicamillus University, Rome, Italy.
⁴³ Division of Cardiology, University of Washington, Seattle, Wash.
⁴⁴ Division of Thoracic and Cardiovascular Surgery, Saarland University Medical Center, Homburg, Saar, Germany.

PMID: 34304894
DOI: 10.1016/j.jtcvs.2021.05.008

Abstract

This International evidence-based nomenclature and classification consensus on the congenital bicuspid aortic valve and its aortopathy recognizes 3 types of bicuspid aortic valve: 1. Fused type, with 3 phenotypes: right-left cusp fusion, right-non cusp fusion and left-non cusp fusion; 2. 2-sinus type with 2 phenotypes: Latero-lateral and antero-posterior; and 3. Partial-fusion or forme fruste. This consensus recognizes 3 bicuspid-aortopathy types: 1. Ascending phenotype; root phenotype; and 3. extended phenotypes.

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

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

Affiliations

Summary: 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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