International consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional and research purposes
Héctor 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-Galán, Laurent de Kerchove, Susan M. Fernandes, Paul W.M. Fedak, Evaldas Girdauskas, Victoria Delgado, Suhny Abbara, Emmanuel Lansac, Siddharth K. Prakash, Malenka M. Bissell, Bogdan A. Popescu, Michael D. Hope, Marta Sitges, Vinod H. Thourani, Phillippe Pibarot, Krishnaswamy Chandrasekaran, Patrizio Lancellotti, Michael A. Borger, John K. Forrest, John G. 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 C. Siu, Lars G. Svensson, Alan C. Braverman, Joseph E. Bavaria, Thoralf M. Sundt, Gébrine El Khoury, Ruggero De Paulis, Maurice Enriquez‐Sarano, Jeroen J. Bax, Catherine M Otto, Hans‐Joachim Schäfers
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.