Summary: 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 Alessandro2,Evangelista Arturo3,Maleszewski Joseph J14,Edwards William D4,Roman Mary J5,Devereux Richard B5,Fernández Borja6,Asch Federico M7,Barker Alex J8,Sierra-Galan Lilia M9,De Kerchove Laurent10,Fernandes Susan M1112,Fedak Paul W M13,Girdauskas Evaldas14,Delgado Victoria15,Abbara Suhny16,Lansac Emmanuel17,Prakash Siddharth K18,Bissell Malenka M19,Popescu Bogdan A20,Hope Michael D21,Sitges Marta22,Thourani Vinod H23,Pibarot Phillippe24,Chandrasekaran Krishnaswamy1,Lancellotti Patrizio252627,Borger Michael A28,Forrest John K29,Webb John30,Milewicz Dianna M18,Makkar Raj29,Leon Martin B30,Sanders Stephen P3132,Markl Michael33,Ferrari Victor A34,Roberts William C3536,Song Jae-Kwan37,Blanke Philipp38,White Charles S39,Siu Samuel40,Svensson Lars G41,Braverman Alan C42,Bavaria Joseph43,Sundt Thoralf M44,Khoury Gebrine El10,De Paulis Ruggero45,Enriquez-Sarano Maurice1,Bax Jeroen J15,Otto Catherine M46,Schäfers Hans-Joachim47

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, Colorado, 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. Department of Pediatrics, Division of Pediatric Cardiology, Stanford University, Palo Alto, CA, USA

12. ?>Department of Medicine, Division of Cardiovascular 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

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, 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, Italy

27. Anthea Hospital, Bari, Italy

28. University Clinic of Cardiac Surgery, Leipzig Heart Center, Leipzig, Germany

29. Yale University School of Medicine & Yale New Haven Hospital, New Haven, CT, USA

30. St Paul's Hospital, University of British Columbia, Vancouver, Canada

31. Cardiac Registry, Departments of Cardiology, Pathology and Cardiac Surgery, Boston Children’s Hospital, Boston, MA, USA

32. Department of Pediatrics, Harvard Medical School, Boston, MA, USA

33. Department of Radiology, Feinberg School of Medicine, Northwestern University, Chicago, IL, USA

34. Cardiovascular Medicine Division, University of Pennsylvania Medical Center and Penn Cardiovascular Institute, Philadelphia, PA, USA

35. Baylor Heart and Vascular Institute, Baylor University Medical Center, Dallas, TX, USA

36. Texas A & M School of Medicine, 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, Italy

46. Division of Cardiology, University of Washington, Seattle, WA, USA

47. Division of Thoracic and Cardiovascular Surgery, Saarland University Medical Center, Homburg, Saar, Germany

Abstract

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.

Funder

the Heart Valve Society

European Association of Cardiovascular Imaging

Society of Thoracic Surgeons

American Association for Thoracic Surgery

Society for Cardiovascular Magnetic Resonance

Society of Cardiovascular Computed Tomography

North American Society for Cardiovascular Imaging

International Bicuspid Aortic Valve Consortium

Publisher

Oxford University Press (OUP)

Subject

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

Reference53 articles.

1. International consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional and research purposes;Hi,2020

2. Bicuspid aortic valve: identifying knowledge gaps and rising to the challenge from the International Bicuspid Aortic Valve Consortium (BAVCon);Michelena;Circulation,2014

3. Sex differences and survival in adults with bicuspid aortic valves: verification in 3 contemporary echocardiographic cohorts;Michelena;J Am Heart Assoc,2016

4. The congenitally bicuspid aortic valve. A study of 85 autopsy cases;Roberts;Am J Cardiol,1970

5. Nosology spectrum of the bicuspid aortic valve condition: complex-presentation valvulo-aortopathy;Michelena;Circulation,2020

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