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

J Thorac Cardiovasc Surg. 2021 Sep;162(3):e383-e414. doi: 10.1016/j.jtcvs.2021.06.019. Epub 2021 Jul 22.


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.

Keywords: aortopathy; bicuspid aortic valve; classification; nomenclature.

Publication types

  • Practice Guideline
  • Review
  • Video-Audio Media

MeSH terms

  • Aorta* / diagnostic imaging
  • Aorta* / surgery
  • Aortic Diseases / classification*
  • Aortic Diseases / diagnostic imaging
  • Aortic Diseases / surgery
  • Aortic Valve / abnormalities*
  • Aortic Valve / diagnostic imaging
  • Aortic Valve / surgery
  • Aortography
  • Bicuspid Aortic Valve Disease / classification*
  • Bicuspid Aortic Valve Disease / diagnostic imaging
  • Bicuspid Aortic Valve Disease / surgery
  • Cardiac Imaging Techniques
  • Consensus
  • Humans
  • Phenotype
  • Predictive Value of Tests
  • Prognosis
  • Terminology as Topic*