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Pediatrics
Diagnosis
Cardiac
Congenital Heart Disease
Aortic Stenosis
Aortic Stenosis
Randy R. Richardson, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Abbreviations

            • Aortic stenosis (AS)

          • Synonyms

            • Aortic valvar stenosis, aortic valvular stenosis

          • Definitions

            • Spectrum of aortic valve abnormalities that ranges from asymptomatic bicuspid aortic valve to thickened, obstructing aortic valve stenosis to severe neonatal aortic atresia & hypoplastic left heart syndrome (HLHS)
            • AS may be valvar, supravalvar, or subvalvar (subaortic)
              • Valvar stenosis most common at 80%

          IMAGING

          • General Features

            • Radiographic Findings

              • CT Findings

                • MR Findings

                  • Echocardiographic Findings

                    • Angiographic Findings

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Staging, Grading, & Classification

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    Selected References

                                    1. Antequera-González B et al: Bicuspid aortic valve and endothelial dysfunction: current evidence and potential therapeutic targets. Front Physiol. 11:1015, 2020
                                    2. Mylonas KS et al: Rapid right ventricular pacing for balloon valvuloplasty in congenital aortic stenosis: a systematic review. World J Cardiol. 12(11):540-9, 2020
                                    3. Correction: Diagnosis, imaging and clinical management of aortic coarctation. Heart. 105(14):e6, 2019
                                    4. Ancona MB et al: Impact of ascending aorta dilation on mid-term outcome after transcatheter aortic valve implantation. J Invasive Cardiol. 31(10):278-81, 2019
                                    5. De Rubeis G et al: Aortic valvular imaging with cardiovascular magnetic resonance: seeking for comprehensiveness. Br J Radiol. 92(1101):20170868, 2019
                                    6. Lee JC et al: Evaluation of aortic regurgitation with cardiac magnetic resonance imaging: a systematic review. Heart. 104(2):103-10, 2018
                                    7. Guner A et al: Evaluation of the congenital supravalvular aortic stenosis by different imaging modalities. Echocardiography. 34(9):1376-8, 2017
                                    8. Opotowsky AR et al: Imaging adult patients with discrete subvalvar aortic stenosis. Curr Opin Cardiol. 32(5):513-20, 2017
                                    9. Ren X et al: The significance of aortic valve calcification in patients with bicuspid aortic valve disease. Int J Cardiovasc Imaging. 32(3):471-8, 2016
                                    10. Looi JL et al: Morphology of congenital and acquired aortic valve disease by cardiovascular magnetic resonance imaging. Eur J Radiol. 84(11):2144-54, 2015
                                    11. Dusenbery SM et al: Myocardial extracellular remodeling is associated with ventricular diastolic dysfunction in children and young adults with congenital aortic stenosis. J Am Coll Cardiol. 63(17):1778-85, 2014
                                    12. Printz BF: The 30-year road of noninvasive imaging for congenital aortic stenosis: new insights from cardiac magnetic resonance imaging. J Am Coll Cardiol. 63(17):1786-7, 2014
                                    13. Wassmuth R et al: Cardiac magnetic resonance imaging of congenital bicuspid aortic valves and associated aortic pathologies in adults. Eur Heart J Cardiovasc Imaging. 15(6):673-9, 2014
                                    14. 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
                                    15. Yuan J et al: Follow-up by cardiac magnetic resonance imaging in patients with hypertrophic cardiomyopathy who underwent percutaneous ventricular septal ablation. Am J Cardiol. 106(10):1487-91, 2010
                                    Related Anatomy
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                                    Related Differential Diagnoses
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                                    References
                                    Tables

                                    Tables

                                    KEY FACTS

                                    • Terminology

                                      • Imaging

                                        • Pathology

                                          • Clinical Issues

                                            TERMINOLOGY

                                            • Abbreviations

                                              • Aortic stenosis (AS)

                                            • Synonyms

                                              • Aortic valvar stenosis, aortic valvular stenosis

                                            • Definitions

                                              • Spectrum of aortic valve abnormalities that ranges from asymptomatic bicuspid aortic valve to thickened, obstructing aortic valve stenosis to severe neonatal aortic atresia & hypoplastic left heart syndrome (HLHS)
                                              • AS may be valvar, supravalvar, or subvalvar (subaortic)
                                                • Valvar stenosis most common at 80%

                                            IMAGING

                                            • General Features

                                              • Radiographic Findings

                                                • CT Findings

                                                  • MR Findings

                                                    • Echocardiographic Findings

                                                      • Angiographic Findings

                                                        DIFFERENTIAL DIAGNOSIS

                                                          PATHOLOGY

                                                          • General Features

                                                            • Staging, Grading, & Classification

                                                              CLINICAL ISSUES

                                                              • Presentation

                                                                • Demographics

                                                                  • Natural History & Prognosis

                                                                    • Treatment

                                                                      Selected References

                                                                      1. Antequera-González B et al: Bicuspid aortic valve and endothelial dysfunction: current evidence and potential therapeutic targets. Front Physiol. 11:1015, 2020
                                                                      2. Mylonas KS et al: Rapid right ventricular pacing for balloon valvuloplasty in congenital aortic stenosis: a systematic review. World J Cardiol. 12(11):540-9, 2020
                                                                      3. Correction: Diagnosis, imaging and clinical management of aortic coarctation. Heart. 105(14):e6, 2019
                                                                      4. Ancona MB et al: Impact of ascending aorta dilation on mid-term outcome after transcatheter aortic valve implantation. J Invasive Cardiol. 31(10):278-81, 2019
                                                                      5. De Rubeis G et al: Aortic valvular imaging with cardiovascular magnetic resonance: seeking for comprehensiveness. Br J Radiol. 92(1101):20170868, 2019
                                                                      6. Lee JC et al: Evaluation of aortic regurgitation with cardiac magnetic resonance imaging: a systematic review. Heart. 104(2):103-10, 2018
                                                                      7. Guner A et al: Evaluation of the congenital supravalvular aortic stenosis by different imaging modalities. Echocardiography. 34(9):1376-8, 2017
                                                                      8. Opotowsky AR et al: Imaging adult patients with discrete subvalvar aortic stenosis. Curr Opin Cardiol. 32(5):513-20, 2017
                                                                      9. Ren X et al: The significance of aortic valve calcification in patients with bicuspid aortic valve disease. Int J Cardiovasc Imaging. 32(3):471-8, 2016
                                                                      10. Looi JL et al: Morphology of congenital and acquired aortic valve disease by cardiovascular magnetic resonance imaging. Eur J Radiol. 84(11):2144-54, 2015
                                                                      11. Dusenbery SM et al: Myocardial extracellular remodeling is associated with ventricular diastolic dysfunction in children and young adults with congenital aortic stenosis. J Am Coll Cardiol. 63(17):1778-85, 2014
                                                                      12. Printz BF: The 30-year road of noninvasive imaging for congenital aortic stenosis: new insights from cardiac magnetic resonance imaging. J Am Coll Cardiol. 63(17):1786-7, 2014
                                                                      13. Wassmuth R et al: Cardiac magnetic resonance imaging of congenital bicuspid aortic valves and associated aortic pathologies in adults. Eur Heart J Cardiovasc Imaging. 15(6):673-9, 2014
                                                                      14. 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
                                                                      15. Yuan J et al: Follow-up by cardiac magnetic resonance imaging in patients with hypertrophic cardiomyopathy who underwent percutaneous ventricular septal ablation. Am J Cardiol. 106(10):1487-91, 2010