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

Author:

Michelena Hector I1ORCID,Della Corte Alessandro2ORCID,Evangelista Arturo3,Maleszewski Joseph J4ORCID,Edwards William D4,Roman Mary J5,Devereux Richard B5,Fernández Borja6,Asch Federico M7,Barker Alex J8ORCID,Sierra-Galan Lilia M9ORCID,De Kerchove Laurent10ORCID,Fernandes Susan M1112,Fedak Paul W M13ORCID,Girdauskas Evaldas14,Delgado Victoria15ORCID,Abbara Suhny16ORCID,Lansac Emmanuel17ORCID,Prakash Siddharth K18ORCID,Bissell Malenka M19ORCID,Popescu Bogdan A20ORCID,Hope Michael D21,Sitges Marta22ORCID,Thourani Vinod H23,Pibarot Phillippe24ORCID,Chandrasekaran Krishnaswamy1,Lancellotti Patrizio2526ORCID,Borger Michael A27,Forrest John K28,Webb John29,Milewicz Dianna M18,Makkar Raj30ORCID,Leon Martin B31,Sanders Stephen P3233,Markl Michael34,Ferrari Victor A35,Roberts William C36,Song Jae-Kwan37,Blanke Philipp38,White Charles S39,Siu Samuel40ORCID,Svensson Lars G41,Braverman Alan C42ORCID,Bavaria Joseph43,Sundt Thoralf M44,El Khoury Gebrine10,De Paulis Ruggero45ORCID,Enriquez-Sarano Maurice1ORCID,Bax Jeroen J15ORCID,Otto Catherine M46,Schäfers Hans-Joachim47ORCID,

Affiliation:

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

Abstract

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.

Publisher

Oxford University Press (OUP)

Subject

Cardiology and Cardiovascular Medicine,Pulmonary and Respiratory Medicine,General Medicine,Surgery

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