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Cardiac
Diagnosis
Valvular
Bicuspid Aortic Valve
Bicuspid Aortic Valve
Suhny Abbara, MD, FACR, FSCCT; Christopher M. Walker, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Bicuspid aortic valve (BAV)

            • Definitions

              • Raphe represents visible fusion point between 2 underdeveloped coronary cusps
              • Commissure represents opening between aortic valve cusps (leaflets)
              • BAV is most common congenital cardiovascular malformation
                • Occurs in 1-2% of world population
                • There are 2 functional cusps caused by fusion during embryology (raphe present) or de novo development of 2 cusps (raphe absent)
                • Raphe in middle of 1 fused cusp often mimics trileaflet aortic valve in diastole
              • Acquired bicuspid valves are secondary to inflammatory processes (e.g., rheumatic fever) or calcification of normal trileaflet aortic valve resulting in fusion of 2 valve cusps

            IMAGING

            • General Features

              • Imaging Recommendations

                • Radiographic Findings

                  • CT Findings

                    • MR Findings

                      • Echocardiographic Findings

                        • Angiographic Findings

                          DIFFERENTIAL DIAGNOSIS

                            PATHOLOGY

                            • General Features

                              • Staging, Grading, & Classification

                                • Gross Pathologic & Surgical Features

                                  • Microscopic Features

                                    CLINICAL ISSUES

                                    • Presentation

                                      • Demographics

                                        • Natural History & Prognosis

                                          • Treatment

                                            Selected References

                                            1. Rodríguez-Palomares JF et al: Aortic flow patterns and wall shear stress maps by 4D-flow cardiovascular magnetic resonance in the assessment of aortic dilatation in bicuspid aortic valve disease. J Cardiovasc Magn Reson. 20(1):28, 2018
                                            2. Yoon SH et al: Diagnosis and outcomes of transcatheter aortic valve implantation in bicuspid aortic valve stenosis. Interv Cardiol. 13(2):62-5, 2018
                                            3. Ridley CH et al: The Sievers classification of the bicuspid aortic valve for the perioperative echocardiographer: the importance of valve phenotype for aortic valve repair in the era of the functional aortic annulus. J Cardiothorac Vasc Anesth. 30(4):1142-51, 2016
                                            4. Hoey ET et al: Multi-detector CT angiography of the aortic valve-Part 2: disease specific findings. Quant Imaging Med Surg. 4(4):273-81, 2014
                                            5. Ko SM et al: Bicuspid aortic valve: spectrum of imaging findings at cardiac MDCT and cardiovascular MRI. AJR Am J Roentgenol. 198(1):89-97, 2012
                                            6. Alkadhi H et al: Cardiac CT for the differentiation of bicuspid and tricuspid aortic valves: comparison with echocardiography and surgery. AJR Am J Roentgenol. 195(4):900-8, 2010
                                            7. Tanaka R et al: Diagnostic value of cardiac CT in the evaluation of bicuspid aortic stenosis: comparison with echocardiography and operative findings. AJR Am J Roentgenol. 195(4):895-9, 2010
                                            8. Ryan R et al: Cardiac valve disease: spectrum of findings on cardiac 64-MDCT. AJR Am J Roentgenol. 190(5):W294-303, 2008
                                            9. Sievers HH et al: A classification system for the bicuspid aortic valve from 304 surgical specimens. J Thorac Cardiovasc Surg. 133(5):1226-33, 2007
                                            10. Lewin MB et al: The bicuspid aortic valve: adverse outcomes from infancy to old age. Circulation. 111(7):832-4, 2005
                                            11. Cripe L et al: Bicuspid aortic valve is heritable. J Am Coll Cardiol. 44(1):138-43, 2004
                                            12. Ward C: Clinical significance of the bicuspid aortic valve. Heart. 83(1):81-5, 2000
                                            13. Arai AE et al: Visualization of aortic valve leaflets using black blood MRI. J Magn Reson Imaging. 10(5):771-7, 1999
                                            14. Sabet HY et al: Congenitally bicuspid aortic valves: a surgical pathology study of 542 cases (1991 through 1996) and a literature review of 2,715 additional cases. Mayo Clin Proc. 74(1):14-26, 1999
                                            15. Beppu S et al: Rapidity of progression of aortic stenosis in patients with congenital bicuspid aortic valves. Am J Cardiol. 71(4):322-7, 1993
                                            16. Brandenburg RO Jr et al: Accuracy of 2-dimensional echocardiographic diagnosis of congenitally bicuspid aortic valve: echocardiographic-anatomic correlation in 115 patients. Am J Cardiol. 51(9):1469-73, 1983
                                            17. Waller BF et al: Bicuspid aortic valve. Comparison of congenital and acquired types. Circulation. 48(5):1140-50, 1973
                                            Related Anatomy
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                                            Related Differential Diagnoses
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                                            References
                                            Tables

                                            Tables

                                            KEY FACTS

                                            • Terminology

                                              • Imaging

                                                • Top Differential Diagnoses

                                                  • Pathology

                                                    • Clinical Issues

                                                      TERMINOLOGY

                                                      • Abbreviations

                                                        • Bicuspid aortic valve (BAV)

                                                      • Definitions

                                                        • Raphe represents visible fusion point between 2 underdeveloped coronary cusps
                                                        • Commissure represents opening between aortic valve cusps (leaflets)
                                                        • BAV is most common congenital cardiovascular malformation
                                                          • Occurs in 1-2% of world population
                                                          • There are 2 functional cusps caused by fusion during embryology (raphe present) or de novo development of 2 cusps (raphe absent)
                                                          • Raphe in middle of 1 fused cusp often mimics trileaflet aortic valve in diastole
                                                        • Acquired bicuspid valves are secondary to inflammatory processes (e.g., rheumatic fever) or calcification of normal trileaflet aortic valve resulting in fusion of 2 valve cusps

                                                      IMAGING

                                                      • General Features

                                                        • Imaging Recommendations

                                                          • Radiographic Findings

                                                            • CT Findings

                                                              • MR Findings

                                                                • Echocardiographic Findings

                                                                  • Angiographic Findings

                                                                    DIFFERENTIAL DIAGNOSIS

                                                                      PATHOLOGY

                                                                      • General Features

                                                                        • Staging, Grading, & Classification

                                                                          • Gross Pathologic & Surgical Features

                                                                            • Microscopic Features

                                                                              CLINICAL ISSUES

                                                                              • Presentation

                                                                                • Demographics

                                                                                  • Natural History & Prognosis

                                                                                    • Treatment

                                                                                      Selected References

                                                                                      1. Rodríguez-Palomares JF et al: Aortic flow patterns and wall shear stress maps by 4D-flow cardiovascular magnetic resonance in the assessment of aortic dilatation in bicuspid aortic valve disease. J Cardiovasc Magn Reson. 20(1):28, 2018
                                                                                      2. Yoon SH et al: Diagnosis and outcomes of transcatheter aortic valve implantation in bicuspid aortic valve stenosis. Interv Cardiol. 13(2):62-5, 2018
                                                                                      3. Ridley CH et al: The Sievers classification of the bicuspid aortic valve for the perioperative echocardiographer: the importance of valve phenotype for aortic valve repair in the era of the functional aortic annulus. J Cardiothorac Vasc Anesth. 30(4):1142-51, 2016
                                                                                      4. Hoey ET et al: Multi-detector CT angiography of the aortic valve-Part 2: disease specific findings. Quant Imaging Med Surg. 4(4):273-81, 2014
                                                                                      5. Ko SM et al: Bicuspid aortic valve: spectrum of imaging findings at cardiac MDCT and cardiovascular MRI. AJR Am J Roentgenol. 198(1):89-97, 2012
                                                                                      6. Alkadhi H et al: Cardiac CT for the differentiation of bicuspid and tricuspid aortic valves: comparison with echocardiography and surgery. AJR Am J Roentgenol. 195(4):900-8, 2010
                                                                                      7. Tanaka R et al: Diagnostic value of cardiac CT in the evaluation of bicuspid aortic stenosis: comparison with echocardiography and operative findings. AJR Am J Roentgenol. 195(4):895-9, 2010
                                                                                      8. Ryan R et al: Cardiac valve disease: spectrum of findings on cardiac 64-MDCT. AJR Am J Roentgenol. 190(5):W294-303, 2008
                                                                                      9. Sievers HH et al: A classification system for the bicuspid aortic valve from 304 surgical specimens. J Thorac Cardiovasc Surg. 133(5):1226-33, 2007
                                                                                      10. Lewin MB et al: The bicuspid aortic valve: adverse outcomes from infancy to old age. Circulation. 111(7):832-4, 2005
                                                                                      11. Cripe L et al: Bicuspid aortic valve is heritable. J Am Coll Cardiol. 44(1):138-43, 2004
                                                                                      12. Ward C: Clinical significance of the bicuspid aortic valve. Heart. 83(1):81-5, 2000
                                                                                      13. Arai AE et al: Visualization of aortic valve leaflets using black blood MRI. J Magn Reson Imaging. 10(5):771-7, 1999
                                                                                      14. Sabet HY et al: Congenitally bicuspid aortic valves: a surgical pathology study of 542 cases (1991 through 1996) and a literature review of 2,715 additional cases. Mayo Clin Proc. 74(1):14-26, 1999
                                                                                      15. Beppu S et al: Rapidity of progression of aortic stenosis in patients with congenital bicuspid aortic valves. Am J Cardiol. 71(4):322-7, 1993
                                                                                      16. Brandenburg RO Jr et al: Accuracy of 2-dimensional echocardiographic diagnosis of congenitally bicuspid aortic valve: echocardiographic-anatomic correlation in 115 patients. Am J Cardiol. 51(9):1469-73, 1983
                                                                                      17. Waller BF et al: Bicuspid aortic valve. Comparison of congenital and acquired types. Circulation. 48(5):1140-50, 1973