Skip to main content
Springer Nature Link
Log in

Review of Congenital Mitral Valve Stenosis: Analysis, Repair Techniques and Outcomes

  • Published:
Cardiovascular Engineering and Technology Aims and scope Submit manuscript

Abstract

The spectrum of congenital mitral valve stenosis (MS) consists of a complex of defects that result in obstruction to left ventricular inflow. This spectrum includes patients with underdeveloped left heart structures (Fig. 1) to those with isolated congenital MS. The specific mitral valve defects can further be divided into categories based on the relationship to the mitral valve annulus including valvar, supravalvar and subvalvar components. Clinically, these patients present based on the degree of obstruction, associated mitral regurgitation, secondary pulmonary hypertension, associated lung disease and/or associated cardiac lesions. There are a number of factors that contribute to the successful outcomes in these patients including pre-operative imaging, aggressive surgical techniques and peri-operative management.

(a) Image representing a parachute mitral valve with a small left ventricular cavity. Supra-mitral ring with a fibrous rim of tissue attaches to the mitral annulus and extends on the surfaces of the mitral leaflets. Stenosing mitral membranes or the thickened fribrotic rim of tissue grows on to the atrial aspects of the mitral leaflets restricting the effective orifice area. This thickened fibrous anterior leaflet tissue can extend down onto the chordae tendenae towards the papillary muscle creating an “arcade” like structure. Additional valvar components include commissural fusion and thickened fibrous leaflets that can lead to restricted leaflet motion. Note the sub-valvar or papillary muscle components of congenital MS including the shortened chordae tendenae, reduced inter-chordal space and tethering of the papillary muscles to the ventricle. (b) Pathologic specimen of a parachute mitral valve with a single papillary muscle.

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

Access this article

Log in via an institution

Subscribe and save

Springer+
from €37.37 /Month
  • Starting from 10 chapters or articles per month
  • Access and download chapters and articles from more than 300k books and 2,500 journals
  • Cancel anytime
View plans

Buy Now

Price includes VAT (Netherlands)

Instant access to the full article PDF.

Institutional subscriptions

Figure 2
Figure 3

Similar content being viewed by others

Explore related subjects

Discover the latest articles, books and news in related subjects, suggested using machine learning.

References

  1. Ruckman, R. N., and R. Van Praagh. Anatomic types of congenital mitral stenosis: report of 49 autopsy cases with consideration of diagnosis and surgical implications. Am. J. Cardiol. 42:592–601, 1978.

    Article  Google Scholar 

  2. Carpentier, A., S. Chauvaud, and S. Mihaileanu. Classification of congenital malformations of the mitral valve and their surgical management. In: Perspectives in Pediatric Cardiology. Part 3: Pediatric Cardiac Surgery, edited by G. Crupi, L. Parenzan, and R. G. Anderson. Mt. Kisco, NY: Futura Publishing, 1990, pp. 97–102.

    Google Scholar 

  3. Layman, T. E., and J. E. Edwards. Anomalous mitral arcade. A type of congenital mitral insufficiency. Circulation 35:389–395, 1967.

    Article  Google Scholar 

  4. Acar, P., K. Warner, N. Pandian, J. Rhodes, and G. Marx. Three-dimensional echocardiographic analysis of valve anatomy as a determinant of mitral regurgitation after surgery for atrioventricular septal defects. Am. J. Cardiol. 83(5):745–749, 1999.

    Article  Google Scholar 

  5. Anderson, R. H., and M. Kanani. Mitral valve repair: critical analysis of the anatomy discussed. Multimed. Man. Cardiothorac. Surg. 2006. doi:10.1510/mmcts.2006.002147.

    Google Scholar 

  6. del Nido, P. J., and C. Baird. Congenital mitral valve stenosis: anatomic variants and surgical reconstruction. Semin. Thorac. Cardiovasc. Surg. 15(1):69–74, 2012. doi:10.1053/j.pcsu.2012.01.011.

    Article  Google Scholar 

  7. Caldarone, C. A., G. Raghuveer, C. B. Hills, D. L. Atkins, T. L. Burns, D. M. Behrendt, and J. H. Moller. Long-term survival after mitral valve replacement in children aged. Circulation 104(90001):I-143–I-147, 2001. doi:10.1161/hc37t1.09484.

    Google Scholar 

  8. Eble, B. K., W. P. Fiser, P. Simpson, J. Dugan, J. J. Drummond-Webb, and A. T. Yetman. Mitral valve replacement in children: predictors of long-term outcome. Ann Thorac Surg 76(3):853–859, 2003; (discussion 859–860).

    Article  Google Scholar 

  9. David, T., S. Armstrong, and Z. Sun. Left ventricular function after mitral valve surgery. J. Heart Valve Dis. 4:S175–S180, 1995.

    Google Scholar 

  10. Baird, C., P. Myers, G. Marx, and P. del Nido. Mitral valve operations at a high-volume pediatric heart center: Evolving techniques and improved survival with mitral valve repair versus replacement. Ann. Pediatr. Card. 5(1):13, 2012. doi:10.4103/0974-2069.93704.

    Article  Google Scholar 

  11. Toscano, A., L. Pasquini, R. Iacobelli, et al. Congenital supravalvar mitral ring: an underestimated anomaly. J. Thorac. Cardiovasc. Surg. 137:538–542, 2009.

    Article  Google Scholar 

  12. McElhinney, D. B., M. C. Sherwood, J. F. Keane, et al. Current management of severe congenital mitral stenosis: outcomes of transcatheter and surgical therapy in 108 infants and children. Circulation 112:707–714, 2005.

    Article  Google Scholar 

  13. Myers, P. O., Z. Khalpey, C. W. Baird, et al. In vivo valvulogenesis: Initial experience with atrioventricular valve leaflet augmentation with CorMatrix® extracellular matrix scaffolds. Circulation 126:16130, 2012.

    Google Scholar 

  14. Zaidi, A. H., M. Nathan, S. Emani, C. Baird, P. J. del Nido, K. Gauvreau, M. Harris, S. P. Sanders, and R. F. Padera. Preliminary experience with porcine intestinal submucosa (CorMatrix) for valve reconstruction in congenital heart disease: histologic evaluation of explanted valves. J. Thorac. Cardiovasc. Surg. 2014. doi:10.1016/j.jtcvs.2014.02.081.

    Google Scholar 

  15. Abdullah, I., F. B. Ramirez, D. B. McElhinney, J. E. Lock, P. J. del Nido, and S. Emani. Modification of a stented bovine jugular vein conduit (melody valve) for surgical mitral valve replacement. Ann. Thorac. Surg. 94(4):e97–e98, 2012. doi:10.1016/j.athoracsur.2012.02.101.

    Article  Google Scholar 

  16. Quinonez, L. G., R. Breitbart, W. Tworetsky, J. E. Lock, A. C. Marshall, and S. M. Emani. Stented bovine jugular vein graft (Melody valve) for surgical mitral valve replacement in infants and children. J. Thorac. Cardiovasc. Surg. 2013. doi:10.1016/j.jtcvs.2013.10.059.

    Google Scholar 

Download references

Conflict of interest

Christopher W. Baird, Gerald R. Marx, Michele Borisuk, Sitaram Emani and Pedro J. del Nido declare that they have no conflicts of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

Author information

Authors and Affiliations

  1. Boston Children’s Hospital, Boston, MA, USA

    Christopher W. Baird, Gerald R. Marx, Michele Borisuk, Sitram Emani & Pedro J. del Nido

Authors
  1. Christopher W. Baird
    View author publications

    Search author on:PubMed Google Scholar

  2. Gerald R. Marx
    View author publications

    Search author on:PubMed Google Scholar

  3. Michele Borisuk
    View author publications

    Search author on:PubMed Google Scholar

  4. Sitram Emani
    View author publications

    Search author on:PubMed Google Scholar

  5. Pedro J. del Nido
    View author publications

    Search author on:PubMed Google Scholar

Corresponding author

Correspondence to Christopher W. Baird.

Additional information

Associate Editor Ajit P. Yoganathan oversaw the review of this article.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Baird, C.W., Marx, G.R., Borisuk, M. et al. Review of Congenital Mitral Valve Stenosis: Analysis, Repair Techniques and Outcomes. Cardiovasc Eng Tech 6, 167–173 (2015). https://doi.org/10.1007/s13239-015-0223-0

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue date:

  • DOI: https://doi.org/10.1007/s13239-015-0223-0

Keywords