Skip to main content
SpringerLink
Log in

Mitral Valve Congenital Abnormalities and Stenosis

  • Chapter
  • First Online:
Textbook of Three-Dimensional Echocardiography

  • 1273 Accesses

Abstract

Three-dimensional echocardiography (3DE) has tremendously improved our ability to diagnose and assess the severity of mitral valve congenital abnormalities such as congenital mitral cleft, congenital double orifice mitral valve and parachute mitral valve.

Rheumatic mitral valve stenosis is still a high-prevalent heart valve disease in many countries and degenerative, calcific, mitral stenosis is an emerging heart valve disease in the elderly. 3DE has become the reference imaging modality to assess the mitral valve stenosis qualitatively in terms of leaflet visualization, quantitation of the reduced diastolic opening, commissural calcification, leaflet mobility/pliability and assessment of the subvalvular apparatus. Moreover, 3DE provides accurate quantitative analysis of mitral stenosis severity and of mitral valve scoring system in order to assess the suitability for percutaneous mitral balloon valvuloplasty.

Finally, transesophageal 3DE is used to guide and assess the results of percutaneous mitral balloon valvuloplasty in the catheterization laboratory.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 129.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Hardcover Book
USD 169.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Seguela PE, Houyel L, Acar P. Congenital malformations of the mitral valve. Arch Cardiovasc Dis. 2011;104:465–79.

    Article  PubMed  Google Scholar 

  2. Banerjee A, Kohl T, Silverman NH. Echocardiographic evaluation of congenital mitral valve anomalies in children. Am J Cardiol. 1995;76:1284–91.

    Article  CAS  PubMed  Google Scholar 

  3. Oppido G, Davies B, McMullan DM, et al. Surgical treatment of congenital mitral valve disease: midterm results of a repair oriented policy. J Thorac Cardiovasc Surg. 2008;135:1313–20.

    Article  PubMed  Google Scholar 

  4. Mantovani F, Clavel MA, Vatury O, et al. Cleft-like indentations in myxomatous mitral valves by three-dimensional echocardiographic imaging. Heart. 2015;101:1111–7.

    Article  CAS  PubMed  Google Scholar 

  5. McCarthy KP, Ring L, Rana BS. Anatomy of the mitral valve: understanding the mitral valve complex in mitral regurgitation. Eur J Echocardiogr. 2010;11:i3–9.

    Article  PubMed  Google Scholar 

  6. Agricola E, Oppizzi M, Maisano F, et al. Detection of mechanisms of immediate failure by transesophageal echocardiography in quadrangular resection mitral valve repair technique for severe mitral regurgitation. Am J Cardiol. 2003;91:175–9.

    Article  PubMed  Google Scholar 

  7. Manganaro R, Zito C, Khandheria BK, et al. Accessory mitral valve tissue: an updated review of the literature. Eur Heart J Cardiovasc Imaging. 2014;15:489–97.

    Article  PubMed  Google Scholar 

  8. Prifti E, Bonacchi M, Bartolozzi F, Frati G, Leacche M, Vanini V. Postoperative outcome in patients with accessory mitral valve tissue. Med Sci Monit. 2003;9:RA146–53.

    Google Scholar 

  9. Anderson RH, Zuberhuler JR, Penkoske PA, Neches WH. Of clefts, commissures, and things. J Thorac Cardiovasc Surg. 1985;90:605–10.

    CAS  PubMed  Google Scholar 

  10. Miglioranza MH, Muraru D, Mihaila S, Haertel JC, Iliceto S, Badano LP. Isolated anterior mitral valve leaflet cleft: 3D transthoracic echocardiography-guided surgical strategy. Arq Bras Cardiol. 2015;104:e49–52.

    PubMed  PubMed Central  Google Scholar 

  11. Seguela P-E, Brosset P, Acar P. Isolated cleft of the posterior mitral valve leaflet assessed by real-time 3D echocardiography. Arch Cardiovasc Dis. 2011;104:365–6.

    Article  PubMed  Google Scholar 

  12. Wyss CA, Enseleit F, van der Loo B, et al. Isolated cleft of the posterior mitral valve leaflet: a congenital form of mitral regurgitation. Clin Cardiol. 2009;32:553–60.

    Article  PubMed  PubMed Central  Google Scholar 

  13. Kent SM, Markwood TT, Vernalis MN, et al. Cleft posterior mitral valve leaflet associated with counterclockwise papillary muscle rotation. J Am Soc Echocardiogr. 2001;14:303–4.

    Article  CAS  PubMed  Google Scholar 

  14. Ziani AB, Latcu DG, Abadir S, et al. Assessment of proximal isovelocity surface area (PISA) shape using three-dimensional echocardiography in a paediatric population with mitral regurgitation or ventricular shunt. Arch Cardiovasc Dis. 2009;102:185–91.

    Article  PubMed  Google Scholar 

  15. Zalstein E, Hamilton R, Zucker N, Levitas A, Gross GJ. Presentation, natural history, and outcome in children and adolescents with double orifice mitral valve. Am J Cardiol. 2004;93:1067–9.

    Article  Google Scholar 

  16. Ermacora D, Muraru D, Cecchetto A, Cucchini U, Badano LP. Transthoracic three-dimensional echocardiography visualization of functional anatomy of double orifice mitral regurgitation. Eur Heart J Cardiovasc Imaging. 2015;16:862.

    Article  PubMed  Google Scholar 

  17. Trowitzsch E, Bano-Rodrigo A, Burger BM, Colan SD, Sanders SP. Twodimensional echocardiographic findings in double orifice mitral valve. J Am Coll Cardiol. 1985;6:383–7.

    Article  CAS  PubMed  Google Scholar 

  18. Séguéla P-E, Dulac Y, Acar P. Double-orifice mitral valve assessed by two- and three-dimensional echocardiography in a newborn. Arch Cardiovasc Dis. 2011;104:361–2.

    Article  PubMed  Google Scholar 

  19. Shone JD, Sellers RC, Anderson RC, et al. The developmental complex of “parachute mitral valve” supravalvular ring of left atrium, subaortic stenosis and coartaction of aorta. Am J Cardiol. 1963;11:714–25.

    Article  CAS  PubMed  Google Scholar 

  20. Marino BS, Kruge LE, Cho CJ, Tomlinson RS, Shera D, Weinberg PM, et al. Parachute mitral valve: morphologic descriptors, associated lesions, and outcomes after biventricular repair. J Thorac Cardiovasc Surg. 2009;137:385–93.e4.

    Article  PubMed  Google Scholar 

  21. Purvis JA, Smyth S, Barr SH. Multi-modality imaging of an adult parachute mitral valve. J Am Soc Echocardiogr. 2011;24:351.e1–3.

    Article  Google Scholar 

  22. Wood P. An appreciation of mitral stenosis. I: clinical features. Br Med J. 1954;4870:1051.

    Article  Google Scholar 

  23. Marijon E, Ou P, Celermajer DS, et al. Prevalence of rheumatic heart disease detected by echocardiographic screening. N Engl J Med. 2007;357:470–6.

    Article  CAS  PubMed  Google Scholar 

  24. Rusted IE, Scheifley CH, Edwards JE. Studies of the mitral valve: II. Certain anatomic features of the mitral valve and associated structures in mitral stenosis. Circulation. 1956;14:398–406.

    Article  PubMed  Google Scholar 

  25. Faletra FF, Perk G, Pandian NG, Nesser H-J, Kronzon I. Real-time 3D interventional echocardiography. London, Heidelberg, New York, Dordrecht: Springer; 2014. https://doi.org/10.1007/978-1-4471-4745-9.

    Book  Google Scholar 

  26. Lang RM, Badano LP, Tsang W, et al. EAE/ASE recommendations for image acquisition and display using three-dimensional echocardiography. J Am Soc Echocardiogr. 2012;25:3–46.

    Article  PubMed  Google Scholar 

  27. Pérez de Isla L, Casanova C, Almería C, Rodrigo JL, Cordeiro P, Mataix L, Aubele AL, Lang R, Zamorano JL. Which method should be the reference method to evaluate the severity of rheumatic mitral stenosis? Gorlin’s method versus 3D-echo. Eur J Echocardiogr. 2007;8:470–3.

    Article  PubMed  Google Scholar 

  28. Baumgartner H, Falk V, Bax JJ, et al. 2017 ESC/EACTS guidelines for the management of valvular heart disease. Eur Heart J. 2017;38:2739–91.

    Article  PubMed  Google Scholar 

  29. Nishimura RA, Otto CM, Bonow RO, et al. 2017 AHA/ACC focused update of the 2014 AHA/ACC guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. J Am Coll Cardiol. 2017;70:252–89.

    Article  PubMed  Google Scholar 

  30. Mannaerts HF, Kamp O, Visser CA. Should mitral valve area assessment in patients with mitral stenosis be based on anatomical or on functional evaluation? A plea for 3D echocardiography as the new clinical standard. Eur Heart J. 2004;25:2073–4.

    Article  PubMed  Google Scholar 

  31. Langerveld J, Valocik G, Plokker HW, et al. Additional value of three-dimensional transesophageal echocardiography for patients with mitral valve stenosis undergoing balloon valvuloplasty. J Am Soc Echocardiogr. 2003;16:841–9.

    Article  PubMed  Google Scholar 

  32. Zamorano J, de Augustn JA. Three-dimensional echocardiography for assessment of mitral valve stenosis. Curr Opin Cardiol. 2009;24:415–9.

    Article  PubMed  Google Scholar 

  33. Anwar AM, Attia WM, Nosir YF, et al. Validation of a new score for the assessment of mitral stenosis using real-time three-dimensional echocardiography. J Am Soc Echocardiogr. 2010;23:13–22.

    Article  PubMed  Google Scholar 

  34. Hildick-Smith DJ, Taylor GJ, Shapiro LM. Inoue balloon mitral valvuloplasty: long-term clinical and echocardiographic follow-up of a predominantly unfavourable population. Eur Heart J. 2000;21:1690–7.

    Article  CAS  PubMed  Google Scholar 

  35. Chu JW, Levine RA, Chua S, et al. Assessing mitral valve area and orifice geometry in calcific mitral stenosis: a new solution by real-time three-dimensional echocardiography. J Am Soc Echocardiogr. 2008;21:1006–9.

    Article  PubMed  PubMed Central  Google Scholar 

  36. Gorlin R, Gorlin SG. Hydraulic formula for calculation of the area of the stenotic mitral valve, other cardiac valves, and central circulatory shunts. Am Heart J. 1951;41:1–29.

    Article  CAS  PubMed  Google Scholar 

  37. Cannan CR, Nishimura RA, Reeder GS, Ilstrup DR, Larson DR, Holmes DR, et al. Echocardiographic assessment of commissural calcium: a simple predictor of outcome after percutaneous mitral balloon valvotomy. J Am Coll Cardiol. 1997;29:175–80.

    Article  CAS  PubMed  Google Scholar 

  38. Zamorano J, Perez de Isla L, Sugeng L, et al. Real-time three-dimensional echocardiography for rheumatic mitral valve stenosis evaluation: an accurate and novel approach. J Am Coll Cardiol. 2004;43:2091–6.

    Article  PubMed  Google Scholar 

  39. Xie MX, Wang XF, Cheng TO, Wang J, Lu Q. Comparison of accuracy of mitral valve area in mitral stenosis by real-time, three-dimensional echocardiography versus two-dimensional echocardiography versus Doppler pressure half-time. Am J Cardiol. 2005;95:1496–9.

    Article  PubMed  Google Scholar 

  40. Roberts WC, Perloff JK. Mitral valvular disease: a clinicopathologic survey of the conditions causing the mitral valve to function abnormally. Ann Intern Med. 1972;77:939–75.

    Article  CAS  PubMed  Google Scholar 

  41. Turgeman Y, Atar S, Rosenfeld T. The subvalvular apparatus in rheumatic mitral stenosis: methods of assessment and therapeutic implications. Chest. 2003;124:1929–36.

    Article  PubMed  Google Scholar 

  42. Wilkins GT, Weyman AE, Abascal VM, et al. Percutaneous balloon dilatation of the mitral valve: an analysis of echocardiographic variables related to outcome and the mechanism of dilatation. Br Heart J. 1988;60:299–308.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  43. Carpentier A, Pellerin M, Fuzellier J, Relland J. Extensive calcification of the mitral valve annulus: pathology and surgical management. J Thorac Cardiovasc Surg. 1996;11:718–30.

    Article  Google Scholar 

  44. Inoue K, Owaki T, Nakamura T, Kitamura F, Miyamoto N. Clinical application of transvenous mitral commissurotomy by a new balloon catheter. J Thorac Cardiovasc Surg. 1984;87:394–402.

    CAS  PubMed  Google Scholar 

  45. Iung BL, Garbarz E, Michaud P, et al. Late results of percutaneous mitral commissurotomy in a series of 1024 patients: analysis of late clinical deterioration: frequency, anatomic findings and predictive factors. Circulation. 1999;99:3272–8.

    Article  CAS  PubMed  Google Scholar 

  46. Mahmoud HM, Al-Ghamdi MA, Ghabashi AE, Anwar AM. A proposed maneuver to guide transseptal puncture using real-time three-dimensional transesophageal echocardiography: pilot study. Cardiol Res Pract. 2015;174051.

    Google Scholar 

  47. Zamorano J, Perez de Isla L, Sugeng L, et al. Non-invasive assessment of mitral valve area during percutaneous balloon mitral valvuloplasty: role of real-time 3D echocardiography. Eur Heart J. 2004;25:2086–91.

    Article  PubMed  Google Scholar 

  48. Applebaum RM, Kasliwal RR, Kanojia A, Seth A, Bhandari S, Trehan N, et al. Utility of three-dimensional echocardiography during balloon mitral valvuloplasty. J Am Coll Cardiol. 1998;32:1405–9.

    Article  CAS  PubMed  Google Scholar 

  49. Messika-Zeitoun D, Blanc J, Iung B, et al. Impact of degree of commissural opening after percutaneous mitral commissurotomy on long-term outcome. J Am Coll Cardiol Img. 2009;2:1–7.

    Article  Google Scholar 

  50. Mahmoud Elsayed HM, Hassan M, Nagy M, et al. A novel method to measure mitral valve area in patients with rheumatic mitral stenosis using three dimensional transesophageal echocardiography: feasibility and validation. Echocardiography. 2018;35(3):368–74.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. CardioVascular Imaging/Cardiology Department, Queen Elizabeth Hospital, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK

    Hani Mahmoud-Elsayed

Authors
  1. Hani Mahmoud-Elsayed
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Hani Mahmoud-Elsayed .

Editor information

Editors and Affiliations

  1. University of Milano-Bicocca and Istituto Auxologico Italiano, IRCCS, San Luca Hospital, Milano, Italy

    Luigi P. Badano

  2. Department of Medicine, University of Chicago Medical Center, Chicago, IL, USA

    Roberto M. Lang

  3. University of Milano-Bicocca and Istituto Auxologico Italiano, IRCCS, San Luca Hospital, Milano, Italy

    Denisa Muraru

Electronic Supplementary Material

Transesophageal 3DE zoom acquisition of the mitral valve which was cropped to show the mitral valve from both the left atrial (left panel) and the left ventricular (right panel) perspectives. The posterior mitral leaflet is divided into four distinct scallops (P1, P2, P3 and P4) by three cleft-like indentations (WMV 896 kb)

(Left) Transversal cut plane of a transthoracic 3DE full volume acquisition showing the mitral valve from the ventricular perspective and the accessory mitral valve tissue attached to the boby of anterior mitral valve leaflet (AVI 16104 kb)

(Right) Longitudinal cut plane of a transthoracic 3DE full volume acquisition showing the motion of the accessory mitral valve tissue in the left ventricular outflow tract (AVI 13851 kb)

(Left) Transthoracic 3DE visualization of the mitral valve from the left ventricular perspective showing a cleft of the anterior mitral leaflet in a patient with ostium primum atrial septal defect (AVI 2463 kb)

(Right) Transthoracic 3DE visualization of the mitral valve from the left atrial perspective showing a cleft of the anterior mitral leaflet in a patient with ostium primum atrial septal defect (AVI 2838 kb)

Transesophageal 3DE visualization of the mitral valve from the atrial perspective showing a cleft which splits the posterior leaflet in two parts (AVI 10059 kb)

(Left) Transthoracic 3DE visualization of the mitral valve from the ventricular perspective clearly showing the two orifices with a central bridge of abnormal tissue connecting the anterior with the posterior leaflet (AVI 7887 kb)

(Right) Transthoracic 3DE visualization of the mitral valve from the atrial perspective clearly showing the two orifices with a central bridge of abnormal tissue connecting the anterior with the posterior leaflet (AVI 8514 kb)

(Left) Transthoracic 3DE visualization of a parachute mitral valve. A longitudinal cut plane shows a fused papillary muscle. All chordae tendineae are short and thickened and are inserted into this single papillary muscle (AVI 4089 kb)

(Right) En face view of a parachute mitral valve from the ventricular perspective (showing thickened valve leaflets with reduced diastolic excursion (AVI 5145 kb)

(Left) En face view of the stenotic mitral valve orifice as it appears from the left ventricular perspective. The doming of the anterior mitral leaflet is visualized (AVI 16291 kb)

(Right) Longitudinal cut plane of a full-volume 3DE transthoracic data set showing the various lesions of the mitral valve apparatus occurring in rheumatic mitral stenosis (reduced excursion of thickened leaflets, shortened and thickened chordae tendineae; enlarged left atrium) (AVI 20406 kb)

(Left) Transesophageal 3DE visualization of the stenotic mitral valve orifice as it appears from the left ventricular perspective. There is extensive fusion of the commissures (AVI 15048 kb)

(Right) Longitudinal cut plane obtained from a transesophageal full-volume 3DE data set showing the thickened leaflets and the anatomical relationships of the stenotic valve with the surrounding cardiac structures (AVI 12855 kb)

(Left) Thickness of the leaflets of a stenotic mitral valve as they are visualized with conventional two-dimensional echocardiography (AVI 14358 kb)

(Right) Thickness of the leaflets of a stenotic mitral valve as they are visualized with volume rendered three-dimensional echocardiography (AVI 10842 kb)

Transesophageal 3DE data set of a stenotic rheumatic mitral valve displayed in an en face view from the ventricular perspective using volume rendering and rotate display modalities to visualize both the posteromedial and the anterolateral commissure (AVI 4529 kb)

Transesophageal 3DE showing a stenotic rheumatic mitral valve in an en face view from the ventricular perspective. The commissures are partially fused with the medial commissure being less involved than the lateral one (AVI 31806 kb)

Transesophageal 3DE showing a stenotic rheumatic mitral valve with complete fusion of bot commissures in an en face view from ventricular perspective (AVI 15093 kb)

Transthoracic 3DE obtained from the parasternal approach in a patient with severe mitral stenosis. The longitudinal cut plane shows the extent of the involvement of the subvalvular apparatus with thickened, fused and shortened chordae tendineae (AVI 17173 kb)

En face view of a stenotic mitral valve obtained from a full volume transthoracic 3DE data set showing that the orifice is clearly delimited by leaflet margins that are at different depth in the data set making unreliable a planimetry of the residual orifice area on the volume rendered images since planimetry will occur on a flat plane that cannot account for depth differences (AVI 21806 kb)

(Upper) Transesophageal two-dimensional long axis view showing the extensive calcification of the annulus involving the basal two thirds of the mitral leaflets in a patients with degenerative mitral stenosis (AVI 2185 kb)

(Left) Transesophageal 3DE en face view of the mitral valve from the atrial perspective showing the massive calcifications of the annulus narrowing the mitral valve orifice in a patients with degenerative mitral stenosis (AVI 2940 kb)

(Right) Transesophageal 3DE en face view of the mitral valve from the ventricular perspective showing the normal opening of both the commissures in a patients with degenerative mitral stenosis (AVI 2855 kb)

RATLe-90 maneuver; starting by two-dimensional, mid-esophageal 90° bicaval view, activate the 3D-zoom mode. The zoom box should be optimized to include the openings of the superior vena cava (SVC), inferior vena cava (IVC), and aortic root (Ao), with enough depth to include the whole interatrial septum (IAS) from both atrial perspectives excluding extra atrial tissues. Then, acquiring this volume will give us a truncated volume with the SVC (arrow head) pointing to the right of the screen, IVC pointing to the left, left atrial perspective of the IAS is up, and right atrial perspective is down. Then we will rotate anticlockwise this volume for 90° around the z-axis to have the SVC pointing superiorly (arrow head) and the IVC pointing inferiorly. Then we will Tilt-Left this volume for 90° around the y-axis to have the anatomically oriented en face view of the IAS from the right atrial (RA) perspective. Then, reducing the gain will remove the blood signals and will allow clear identification of the right atrial structures, for example, SVC opening, IVC opening, Eustachian valve (EV), coronary sinus (CS) opening, and aortic root (Ao). Further gain reduction will cause dropout artifact in the thin area of the fossa ovalis (arrow head) that will help determining its location to guide septal puncture (MOV 4950 kb)

Transesophageal 3DE. Reducing the gain will remove the noise and will allow clear identification of the right atrial structures, for example, superior (SVC) and inferior (IVC) vena cava opening, Eustachian valve (EV), coronary sinus (CS) opening, and aortic root (Ao). Further gain reduction will cause dropout artifact in the thin area of the fossa ovalis (arrow head) that will help determining its location to guide septal puncture (MOV 4273 kb)

Transesophageal 3DE anatomically oriented view of the interatrial septum (IAS) from the right atrial perspective during guidance of transseptal puncture. By tilting the data set to the left along the Y axis, the catheter could be clearly appreciated from the left atrial side (WMV 2247 kb)

Transesophageal 3DE (zoom mode) anatomically oriented view of the mitral valve from the atrial perspective showing the positioning of the balloon which crosses crossing the stenosed valve and it is inflated to dilate the valve (WMV 2122 kb)

Live/real-time transesophageal 3DE during balloon inflation. The simultaneous visualization of two orthogonal two-dimensional views (four-chamber, upper left panel, and bicommissural, lower left panel) together with the volume rendered en face view from the left atrium allows the full control of balloon positioning during inflation (AVI 16345 kb)

Rights and permissions

Reprints and permissions

Copyright information

© 2019 Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Mahmoud-Elsayed, H. (2019). Mitral Valve Congenital Abnormalities and Stenosis. In: Badano, L., Lang, R., Muraru, D. (eds) Textbook of Three-Dimensional Echocardiography. Springer, Cham. https://doi.org/10.1007/978-3-030-14032-8_8

Download citation

  • DOI: https://doi.org/10.1007/978-3-030-14032-8_8

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-14030-4

  • Online ISBN: 978-3-030-14032-8

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation