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*, Alessandro Della Corte, Arturo Evangelista, Joseph J. Maleszewski, William D. Edwards, Mary J. Roman, Richard B. Devereux, Borja Fernández, Federico M. Asch, Alex J. Barker, Lilia M. Sierra-Galan, Laurent De Kerchove, Susan M. Fernandes, Paul W.M. Fedak, Evaldas Girdauskas, Victoria Delgado, Suhny Abbara, Emmanuel Lansac, Siddharth K. Prakash, Malenka M. BissellBogdan A. Popescu, Michael D. Hope, Marta Sitges, Vinod H. Thourani, Phillippe Pibarot, Krishnaswamy Chandrasekaran, Patrizio Lancellotti, Michael A. Borger, John K. Forrest, John Webb, Dianna M. Milewicz, Raj Makkar, Martin B. Leon, Stephen P. Sanders, Michael Markl, Victor A. Ferrari, William C. Roberts, Jae Kwan Song, Philipp Blanke, Charles S. White, Samuel Siu, Lars G. Svensson, Alan C. Braverman, Joseph Bavaria, Thoralf M. Sundt, Gebrine El Khoury, Ruggero De Paulis, Maurice Enriquez-Sarano, Jeroen J. Bax, Catherine M. Otto, Hans Joachim Schäfers

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

57 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)e383-e414
JournalJournal of Thoracic and Cardiovascular Surgery
Volume162
Issue number3
DOIs
StatePublished - Sep 2021

Funding

The authors wish to thank Carl Clingman, Senior Medical Illustrator, Biomedical & Scientific Visualization, Mayo Clinic, and Pavel Zacek, Department of Cardiac Surgery, Charles University Hospital, Hradec Kralove, Czech Republic, for their illustrations. European Journal of Cardio-Thoracic Surgery thanks Martin Misfeld, Patrick Myers, Ulrich Otto von Oppell, Richard Weisel, and the other, anonymous reviewer(s) for their contribution to the peer review process of this article. Victoria Delgado discloses a financial relationship with Abbott Vascular, Edwards Lifesciences, GE Healthcare, MSD, Medtronic, and Novartis. Emmanuel Lansac: patent Extra Aortic ring annuloplasty device with Coroneo Inc. Phillippe Pibarot: funding from Edwards Lifesciences and Medtronic for echocardiography core laboratory services with no personal compensation. Michael A. Borger: discloses a financial relationship with Edwards Lifesciences, Medtronic, Abbott, and CryoLife. John K. Forrest: grant support and consultant to Edwards Lifesciences, Medtronic Inc. John Webb: consultant to Edwards Lifesciences, Abbott, Boston Scientific. Martin B. Leon: institutional clinical research grants from Abbott, BSC, Edwards, and Medtronic. Michael Markl: research support—Siemens Healthineers, Research Grant—Circle Cardiovascular Imaging, Consulting—Circle Cardiovascular Imaging, Research Grant—Cryolife Inc. Victor A. Ferrari: Senior Advisory Board, Journal of Cardiovascular Magnetic Resonance. Philipp Blanke: consultant for Edwards Lifesciences and Circle Cardiovascular Imaging; and provides CT core lab services for Edwards Lifesciences, Medtronic, Neovasc, and Tendyne Holdings, for which he receives no direct compensation. Ruggero De Paulis: patent on aortic root graft with Terumo Aortic. Consultant for Edwards Lifesciences, Medtronic, and Terumo Aortic. Maurice Enriquez-Sarano: consulting fees from Edwards LLC, Cryolife, and ChemImage, Inc. The Department of Cardiology of the LUMC received unrestricted research grants from Abbott Vascular, Bayer, Biotronik, Bioventrix, Boston Scientific, Edwards Lifesciences, GE Healthcare, and Medtronic. The other authors report no conflict of interest.

Keywords

  • aortopathy
  • bicuspid aortic valve
  • classification
  • nomenclature

ASJC Scopus subject areas

  • Surgery
  • Pulmonary and Respiratory Medicine
  • Cardiology and Cardiovascular Medicine

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