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Mitral Valve Prolapse
Daniel W. Entrikin, MD; Gudrun Feuchtner, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        TERMINOLOGY

        • Abbreviations

          • Mitral valve prolapse (MVP)
        • Definitions

          • MVP: Systolic extension of mitral valve leaflets > 2 mm into left atrium
            • 2 main forms
              • "Billowing" leaflet: Entire leaflet or portion of leaflet bows posteriorly into left atrium > 2 mm beyond mitral annular plane; tips of leaflets remain in left ventricle
              • "Flail" leaflet: Chordal rupture to portion of leaflet allows retrograde extension of free edge of leaflet into left atrium
          • Mitral regurgitation: Systolic retrograde flow of blood from left ventricle into left atrium
          • Myxomatous mitral valve disease: Inflammatory and infiltrative disease of mitral valve
            • Involves excess collagen and proteoglycan deposition in valve tissue
            • Results in edematous leaflet thickening, elongation, redundancy, and dysfunction
            • Most common cause of mitral prolapse and regurgitation requiring surgery in Western world
            • a.k.a. Barlow syndrome or floppy valve disease

        IMAGING

        • General Features

          • Radiographic Findings

            • CT Findings

              • Echocardiographic Findings

                • Angiographic Findings

                  • Nuclear Medicine Findings

                    • MR Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Staging, Grading, & Classification

                              • Gross Pathologic & Surgical Features

                                • Microscopic Features

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Demographics

                                      • Natural History & Prognosis

                                        • Treatment

                                          DIAGNOSTIC CHECKLIST

                                          • Consider

                                            • Image Interpretation Pearls

                                              Selected References

                                              1. Althunayyan A et al: Mitral valve prolapse. Expert Rev Cardiovasc Ther. ePub, 2018
                                              2. Doherty JU et al: ACC/AATS/AHA/ASE/ASNC/HRS/SCAI/SCCT/SCMR/STS 2017 Appropriate Use Criteria for Multimodality Imaging in Valvular Heart Disease: A Report of the American College of Cardiology Appropriate Use Criteria Task Force, American Association for Thoracic Surgery, American Heart Association, American Society of Echocardiography, American Society of Nuclear Cardiology, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, Society of Cardiovascular Computed Tomography, Society for Cardiovascular Magnetic Resonance, and Society of Thoracic Surgeons. J Am Coll Cardiol. 70(13):1647-2, 2017
                                              3. Edwards NC et al: Quantification of left ventricular interstitial fibrosis in asymptomatic chronic primary degenerative mitral regurgitation. Circ Cardiovasc Imaging. 7(6):946-53, 2014
                                              4. Guy TS et al: Mitral valve prolapse. Annu Rev Med. 63:277-92, 2012
                                              5. Morris MF et al: CT and MR imaging of the mitral valve: radiologic-pathologic correlation. Radiographics. 30(6):1603-20, 2010
                                              6. Han Y et al: Cardiovascular magnetic resonance characterization of mitral valve prolapse. JACC Cardiovasc Imaging. 1(3):294-303, 2008
                                              7. Grau JB et al: The genetics of mitral valve prolapse. Clin Genet. 72(4):288-95, 2007
                                              8. Hepner AD et al: The prevalence of mitral valve prolapse in patients undergoing echocardiography for clinical reason. Int J Cardiol. 2007
                                              9. Pinheiro AC et al: Diagnostic value of color flow mapping and Doppler echocardiography in the quantification of mitral regurgitation in patients with mitral valve prolapse or rheumatic heart disease. J Am Soc Echocardiogr. 20(10):1141-8, 2007
                                              10. Sharma R et al: The evaluation of real-time 3-dimensional transthoracic echocardiography for the preoperative functional assessment of patients with mitral valve prolapse: a comparison with 2-dimensional transesophageal echocardiography. J Am Soc Echocardiogr. 20(8):934-40, 2007
                                              11. Mechleb BK et al: Mitral valve prolapse: relationship of echocardiography characteristics to natural history. Echocardiography. 23(5):434-7, 2006
                                              12. Müller S et al: Comparison of three-dimensional imaging to transesophageal echocardiography for preoperative evaluation in mitral valve prolapse. Am J Cardiol. 98(2):243-8, 2006
                                              13. Pepi M et al: Head-to-head comparison of two- and three-dimensional transthoracic and transesophageal echocardiography in the localization of mitral valve prolapse. J Am Coll Cardiol. 48(12):2524-30, 2006
                                              14. Braunwald E: Valvular heart disease. In Braunwald E: Heart Disease: A Textbook of Cardiovascular Medicine. 6th ed. Philadelphia: W. B. Saunders Company, 2001
                                              15. Becker AE et al: Pathomorphology of mitral valve prolapse. In Boudoulas H et al: Mitral Valve: Floppy Valve, Mitral Valve Prolapse and Mitral Regurgitation. 2nd ed. Armonk, NY: Furuta, 2000
                                              16. Boudoulas H et al: Natural history. In Boudoulas H et al: Mitral Valve: Floppy Valve, Mitral Valve Prolapse and Mitral Regurgitation. 2nd ed. Armonk, NY: Furuta, 2000
                                              Related Anatomy
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                                              Related Differential Diagnoses
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                                              References
                                              Tables

                                              Tables

                                              KEY FACTS

                                              • Terminology

                                                • Imaging

                                                  • Top Differential Diagnoses

                                                    TERMINOLOGY

                                                    • Abbreviations

                                                      • Mitral valve prolapse (MVP)
                                                    • Definitions

                                                      • MVP: Systolic extension of mitral valve leaflets > 2 mm into left atrium
                                                        • 2 main forms
                                                          • "Billowing" leaflet: Entire leaflet or portion of leaflet bows posteriorly into left atrium > 2 mm beyond mitral annular plane; tips of leaflets remain in left ventricle
                                                          • "Flail" leaflet: Chordal rupture to portion of leaflet allows retrograde extension of free edge of leaflet into left atrium
                                                      • Mitral regurgitation: Systolic retrograde flow of blood from left ventricle into left atrium
                                                      • Myxomatous mitral valve disease: Inflammatory and infiltrative disease of mitral valve
                                                        • Involves excess collagen and proteoglycan deposition in valve tissue
                                                        • Results in edematous leaflet thickening, elongation, redundancy, and dysfunction
                                                        • Most common cause of mitral prolapse and regurgitation requiring surgery in Western world
                                                        • a.k.a. Barlow syndrome or floppy valve disease

                                                    IMAGING

                                                    • General Features

                                                      • Radiographic Findings

                                                        • CT Findings

                                                          • Echocardiographic Findings

                                                            • Angiographic Findings

                                                              • Nuclear Medicine Findings

                                                                • MR Findings

                                                                  • Imaging Recommendations

                                                                    DIFFERENTIAL DIAGNOSIS

                                                                      PATHOLOGY

                                                                      • General Features

                                                                        • Staging, Grading, & Classification

                                                                          • Gross Pathologic & Surgical Features

                                                                            • Microscopic Features

                                                                              CLINICAL ISSUES

                                                                              • Presentation

                                                                                • Demographics

                                                                                  • Natural History & Prognosis

                                                                                    • Treatment

                                                                                      DIAGNOSTIC CHECKLIST

                                                                                      • Consider

                                                                                        • Image Interpretation Pearls

                                                                                          Selected References

                                                                                          1. Althunayyan A et al: Mitral valve prolapse. Expert Rev Cardiovasc Ther. ePub, 2018
                                                                                          2. Doherty JU et al: ACC/AATS/AHA/ASE/ASNC/HRS/SCAI/SCCT/SCMR/STS 2017 Appropriate Use Criteria for Multimodality Imaging in Valvular Heart Disease: A Report of the American College of Cardiology Appropriate Use Criteria Task Force, American Association for Thoracic Surgery, American Heart Association, American Society of Echocardiography, American Society of Nuclear Cardiology, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, Society of Cardiovascular Computed Tomography, Society for Cardiovascular Magnetic Resonance, and Society of Thoracic Surgeons. J Am Coll Cardiol. 70(13):1647-2, 2017
                                                                                          3. Edwards NC et al: Quantification of left ventricular interstitial fibrosis in asymptomatic chronic primary degenerative mitral regurgitation. Circ Cardiovasc Imaging. 7(6):946-53, 2014
                                                                                          4. Guy TS et al: Mitral valve prolapse. Annu Rev Med. 63:277-92, 2012
                                                                                          5. Morris MF et al: CT and MR imaging of the mitral valve: radiologic-pathologic correlation. Radiographics. 30(6):1603-20, 2010
                                                                                          6. Han Y et al: Cardiovascular magnetic resonance characterization of mitral valve prolapse. JACC Cardiovasc Imaging. 1(3):294-303, 2008
                                                                                          7. Grau JB et al: The genetics of mitral valve prolapse. Clin Genet. 72(4):288-95, 2007
                                                                                          8. Hepner AD et al: The prevalence of mitral valve prolapse in patients undergoing echocardiography for clinical reason. Int J Cardiol. 2007
                                                                                          9. Pinheiro AC et al: Diagnostic value of color flow mapping and Doppler echocardiography in the quantification of mitral regurgitation in patients with mitral valve prolapse or rheumatic heart disease. J Am Soc Echocardiogr. 20(10):1141-8, 2007
                                                                                          10. Sharma R et al: The evaluation of real-time 3-dimensional transthoracic echocardiography for the preoperative functional assessment of patients with mitral valve prolapse: a comparison with 2-dimensional transesophageal echocardiography. J Am Soc Echocardiogr. 20(8):934-40, 2007
                                                                                          11. Mechleb BK et al: Mitral valve prolapse: relationship of echocardiography characteristics to natural history. Echocardiography. 23(5):434-7, 2006
                                                                                          12. Müller S et al: Comparison of three-dimensional imaging to transesophageal echocardiography for preoperative evaluation in mitral valve prolapse. Am J Cardiol. 98(2):243-8, 2006
                                                                                          13. Pepi M et al: Head-to-head comparison of two- and three-dimensional transthoracic and transesophageal echocardiography in the localization of mitral valve prolapse. J Am Coll Cardiol. 48(12):2524-30, 2006
                                                                                          14. Braunwald E: Valvular heart disease. In Braunwald E: Heart Disease: A Textbook of Cardiovascular Medicine. 6th ed. Philadelphia: W. B. Saunders Company, 2001
                                                                                          15. Becker AE et al: Pathomorphology of mitral valve prolapse. In Boudoulas H et al: Mitral Valve: Floppy Valve, Mitral Valve Prolapse and Mitral Regurgitation. 2nd ed. Armonk, NY: Furuta, 2000
                                                                                          16. Boudoulas H et al: Natural history. In Boudoulas H et al: Mitral Valve: Floppy Valve, Mitral Valve Prolapse and Mitral Regurgitation. 2nd ed. Armonk, NY: Furuta, 2000