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The Left Ventricular Outflow in Hypertrophic Cardiomyopathy: From Structure to Function

Journal of Cardiovascular Translational Research volume 2, pages 510–517 (2009)Cite this article

Abstract

Left ventricular outflow tract obstruction (LVOTO) is one of the defining features of hypertrophic cardiomyopathy (HCM) and one of the main determinants of prognosis. Although the importance of obstruction was recognized since the original description by Teare and Brock, its exact cause and methods for its relief are still being hotly debated. We believe that a rational approach to solving these issues depends on thorough understanding of the specific structure and functions of the left ventricular outflow tract (LVOT) in health and disease. There is now compelling evidence that the LVOT performs a series of vital sophisticated functions which are mediated by the design characteristics, structure, and biological properties of its component parts and that dysregulation of one or more of these functions results in obstruction and other abnormalities. We here review the integrated functions of the LVOT, its structural and functional relationships, with particular reference to its component parts (the major players) and their role in HCM. This knowledge is essential to evolve tailored restorative techniques for treating HCM.

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References

  1. Maron, M. S., Olivotto, I., Zenovich, A. G., et al. (2006). Hypertrophic cardiomyopathy is predominantly a disease of left ventricular outflow tract obstruction. Circulation, 114, 2232–2239.

    Article  PubMed  Google Scholar 

  2. Maron, M. S., Olivotto, I., Betocchi, S., et al. (2003). Effect of left ventricular outflow tract obstruction on clinical outcome in hypertrophic cardiomyopathy. The New England journal of medicine, 348, 295–303.

    Article  PubMed  Google Scholar 

  3. Brock, R. (1959). Functional obstruction of the left ventricle (acquired aortic subvalvar stenosis). Guy’s Hospital Reports, 108, 126–143.

    CAS  PubMed  Google Scholar 

  4. Brock, R. (1957). Functional obstruction of the left ventricle; acquired aortic subvalvar stenosis. Guy’s Hospital Reports, 106, 221–238.

    CAS  PubMed  Google Scholar 

  5. Teare, D. (1958). Asymmetrical hypertrophy of the heart in young adults. British heart journal, 20, 1–8.

    Article  CAS  PubMed  Google Scholar 

  6. Yacoub, M. H., Kilner, P. J., Birks, E. J., & Misfeld, M. (1999). The aortic outflow and root: a tale of dynamism and crosstalk. Annals of Thoracic Surgery, 68, S37–S43.

    Article  CAS  PubMed  Google Scholar 

  7. Kilner, P. J., Yang, G. Z., Wilkes, A. J., Mohiaddin, R. H., Firmin, D. N., & Yacoub, M. H. (2000). Asymmetric redirection of flow through the heart. Nature, 404, 759–761.

    Article  CAS  PubMed  Google Scholar 

  8. Helderman, F., Segers, D., de Crom, R., et al. (2007). Effect of shear stress on vascular inflammation and plaque development. Current Opinion in Lipidology, 18, 527–533.

    Article  CAS  PubMed  Google Scholar 

  9. El-Hamamsy, I., & Yacoub, M. H. (2009). Cellular and molecular mechanisms of thoracic aortic aneurysms. Nature Reviews Cardiology, 6, 771–786.

    Article  CAS  PubMed  Google Scholar 

  10. Davies, J. E., Parker, K. H., Francis, D. P., Hughes, A. D., & Mayet, J. (2008). What is the role of the aorta in directing coronary blood flow? Heart, 94, 1545–1547.

    Article  PubMed  Google Scholar 

  11. Lie-Venema, H., van den Akker, N. M. S., Bax, N. A. M., et al. (2007). Origin, fate, and function of epicardium-derived cells (EPDCs) in normal and abnormal cardiac development. TheScientificWorldJOURNAL, 7, 1777–1798.

    CAS  PubMed  Google Scholar 

  12. Olivotto, I., Cecchi, F., Poggesi, C., & Yacoub, M. H. (2009). Developmental origins of hypertrophic cardiomyopathy phenotypes: a unifying hypothesis. Nature Reviews Cardiology, 6, 317–321.

    Article  CAS  PubMed  Google Scholar 

  13. Bertho, E., & Gagnon, G. (1964). A comparative study in three dimension of the blood supply of the normal interventricular septum in human, canine, bovine, porcine, ovine and equine heart. Diseases of the chest, 46, 251–262.

    CAS  PubMed  Article  Google Scholar 

  14. Merrick, A. F., Yacoub, M. H., Ho, S. Y., & Anderson, R. H. (2000). Anatomy of the muscular subpulmonary infundibulum with regard to the Ross procedure. Annals of Thoracic Surgery, 69, 556–561.

    Article  CAS  PubMed  Google Scholar 

  15. Muresian, H. (2006). The Ross procedure: new insights into the surgical anatomy. Annals of Thoracic Surgery, 81, 495–501.

    Article  PubMed  Google Scholar 

  16. Sigwart, U. (1995). Non-surgical myocardial reduction for hypertrophic obstructive cardiomyopathy. Lancet, 346, 211–214.

    Article  CAS  PubMed  Google Scholar 

  17. Zimmerman, J. (1966). The functional and surgical anatomy of the heart. Annals of the Royal College of Surgeons of England, 39, 348–366.

    CAS  PubMed  Google Scholar 

  18. Icardo, J. M. (1996). Developmental biology of the vertebrate heart. The Journal of experimental zoology, 275, 144–161.

    Article  CAS  PubMed  Google Scholar 

  19. Yang, G. Z., Merrifield, R., Masood, S., & Kilner, P. J. (2007). Flow and myocardial interaction: an imaging perspective. Philosophical Transactions of the Royal Society of London. Series B: Biological Sciences, 362, 1329–1341.

    Article  PubMed  Google Scholar 

  20. Dagum, P., Green, G. R., Nistal, F. J., et al. (1999). Deformational dynamics of the aortic root: modes and physiologic determinants. Circulation, 100, II54–II62.

    CAS  PubMed  Google Scholar 

  21. Lansac, E., Lim, H. S., Shomura, Y., et al. (2005). Aortic root dynamics are asymmetric. Journal of heart valve disease, 14, 400–407.

    PubMed  Google Scholar 

  22. Lansac, E., Lim, H. S., Shomura, Y., et al. (2002). A four-dimensional study of the aortic root dynamics. European Journal of Cardio-Thoracic Surgery, 22, 497–503.

    Article  CAS  PubMed  Google Scholar 

  23. El-Hamamsy, I., Balachandran, K., Yacoub, M. H., et al. (2009). Endothelium-dependent regulation of the mechanical properties of aortic valve cusps. Journal of the American College of Cardiology, 53, 1448–1455.

    Article  CAS  PubMed  Google Scholar 

  24. Markl, M., Draney, M. T., Hope, M. D., et al. (2004). Time-resolved 3-dimensional velocity mapping in the thoracic aorta: visualization of 3-directional blood flow patterns in healthy volunteers and patients. Journal of computer assisted tomography, 28, 459–468.

    Article  PubMed  Google Scholar 

  25. Yacoub, M. H. (2005). Surgical versus alcohol septal ablation for hypertrophic obstructive cardiomyopathy: the pendulum swings. Circulation, 112, 450–452.

    Article  PubMed  Google Scholar 

  26. Binder, J., Ommen, S. R., Gersh, B. J., et al. (2006). Echocardiography-guided genetic testing in hypertrophic cardiomyopathy: septal morphological features predict the presence of myofilament mutations. Mayo Clinic Proceedings, 81, 459–467.

    Article  PubMed  Google Scholar 

  27. Bujak, M., & Frangogiannis, N. G. (2007). The role of TGF-{beta} signaling in myocardial infarction and cardiac remodeling. Cardiovascular Research, 74, 184–195.

    Article  CAS  PubMed  Google Scholar 

  28. He, S., Hopmeyer, J., Lefebvre, X. P., Schwammenthal, E., Yoganathan, A. P., & Levine, R. A. (1997). Importance of leaflet elongation in causing systolic anterior motion of the mitral valve. Journal of heart valve disease, 6, 149–159.

    CAS  PubMed  Google Scholar 

  29. Klues, H. G., Proschan, M. A., Dollar, A. L., Spirito, P., Roberts, W. C., & Maron, B. J. (1993). Echocardiographic assessment of mitral valve size in obstructive hypertrophic cardiomyopathy. Anatomic validation from mitral valve specimen. Circulation, 88, 548–555.

    CAS  PubMed  Google Scholar 

  30. Klues, H. G., Maron, B. J., Dollar, A. L., & Roberts, W. C. (1992). Diversity of structural mitral valve alterations in hypertrophic cardiomyopathy. Circulation, 85, 1651–1660.

    CAS  PubMed  Google Scholar 

  31. Levine, R. A., Vlahakes, G. J., Lefebvre, X., et al. (1995). Papillary muscle displacement causes systolic anterior motion of the mitral valve. Experimental validation and insights into the mechanism of subaortic obstruction. Circulation, 91, 1189–1195.

    CAS  PubMed  Google Scholar 

  32. Klues, H. G., Roberts, W. C., & Maron, B. J. (1991). Anomalous insertion of papillary muscle directly into anterior mitral leaflet in hypertrophic cardiomyopathy. Significance in producing left ventricular outflow obstruction. Circulation, 84, 1188–1197.

    CAS  PubMed  Google Scholar 

  33. Sherrid, M. V., Chaudhry, F. A., & Swistel, D. G. (2003). Obstructive hypertrophic cardiomyopathy: echocardiography, pathophysiology, and the continuing evolution of surgery for obstruction. Annals of Thoracic Surgery, 75, 620–632.

    Article  PubMed  Google Scholar 

  34. Klues, H. G., Roberts, W. C., & Maron, B. J. (1993). Morphological determinants of echocardiographic patterns of mitral valve systolic anterior motion in obstructive hypertrophic cardiomyopathy. Circulation, 87, 1570–1579.

    CAS  PubMed  Google Scholar 

  35. Cecchi, F., Olivotto, I., Nistri, S., Antoniucci, D., & Yacoub, M. H. (2006). Midventricular obstruction and clinical decision-making in obstructive hypertrophic cardiomyopathy. Herz, 31, 871–876.

    Article  PubMed  Google Scholar 

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Acknowledgment

The authors would like to acknowledge Ms. Sherine George for producing the illustrations.

Author information

Affiliations

  1. Harefield Heart Science Center, Harefield Hospital, London, UK

    Magdi H. Yacoub, Ismail El-Hamamsy & Robert George

  2. Aswan Heart Center, Aswan, Egypt

    Karim Said, Gehan Magdi & Fatma Abul Enein

  3. Azienda Ospedaliera Universitaria Careggi, Florence, Italy

    Alessandra Rossi, Iacopo Olivotto & Franco Cecchi

Authors
  1. Magdi H. Yacoub
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  2. Ismail El-Hamamsy
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  3. Karim Said
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  4. Gehan Magdi
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  5. Fatma Abul Enein
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  6. Robert George
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  7. Alessandra Rossi
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  8. Iacopo Olivotto
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  9. Franco Cecchi
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Corresponding author

Correspondence to Magdi H. Yacoub.

Additional information

Magdi H. Yacoub and Ismail El-Hamamsy contributed equally to this manuscript.

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Yacoub, M.H., El-Hamamsy, I., Said, K. et al. The Left Ventricular Outflow in Hypertrophic Cardiomyopathy: From Structure to Function. J. of Cardiovasc. Trans. Res. 2, 510–517 (2009). https://doi.org/10.1007/s12265-009-9153-2

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  • DOI: https://doi.org/10.1007/s12265-009-9153-2

Keywords

  • Hypertrophic Cardiomyopathy
  • Outflow Obstruction
  • Surgery
  • Systolic Anterior Movement (SAM)