Journal of Cardiovascular Translational Research

, Volume 2, Issue 4, pp 415–425 | Cite as

The Current and Emerging Role of Cardiovascular Magnetic Resonance Imaging in Hypertrophic Cardiomyopathy

  • Martin S. MaronEmail author


Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiomyopathy with substantial heterogeneity in phenotypic expression and clinical course. Traditionally, two-dimensional echocardiography has been the easiest and most reliable technique for establishing a diagnosis of HCM. However, cardiovascular magnetic resonance (CMR) has emerged as a novel, three-dimensional tomographic imaging technique, which provides high spatial and temporal resolution images of the heart in any plane and without ionizing radiation. As a result, CMR is particularly well suited to provide detailed characterization of the HCM phenotype, including precise assessment of the location and distribution of left ventricular (LV) wall thickening. In this regard, CMR can identify hypertrophy (particularly in the anterolateral free wall and apex), not well appreciated (or underestimated) by two-dimensional echocardiography, with important implications for diagnosis. CMR can also provide detailed characterization of other myocardial structures such as the papillary muscles, which may impact on preoperative management strategies for patients who are candidates for surgical myectomy. Furthermore, CMR enables an accurate assessment of total LV mass, a robust marker of the overall extent of hypertrophy, which may have implications for risk stratification. In addition, a subgroup of HCM patients have normal LV mass (with focal hypertrophy), suggesting that a limited extent of hypertrophy is consistent with a diagnosis of HCM. Finally, following the intravenous administration of gadolinium, first-pass perfusion sequences can identify myocardial perfusion abnormalities, while late gadolinium enhancement (LGE) sequences can characterize areas of myocardial fibrosis/scarring. LGE is associated with systolic dysfunction and likelihood for ventricular tachyarrhythmias on ambulatory Holter monitoring in patients with HCM. However, the precise clinical implications of myocardial perfusion abnormalities and LGE in HCM are still uncertain; this information may have important implications with regard to identifying HCM patients at risk of sudden death and adverse LV remodeling associated with systolic dysfunction. Therefore, at present, CMR provides important information impacting on diagnosis and clinical management strategies in patients with HCM and will likely have an expanding role in the evaluation of patients with this complex disease.


Hypertrophic Cardiomyopathy Cardiovascular Magnetic Resonance 


  1. 1.
    Maron, B. J. (2002). Hypertrophic cardiomyopathy: A systematic review. JAMA, 287(10), 1308–1320.CrossRefPubMedGoogle Scholar
  2. 2.
    Maron, B. J., McKenna, W. J., Danielson, G. K., Kappenberger, L. J., Kuhn, H. J., Seidman, C. E., et al. (2003). American College of Cardiology/European Society of Cardiology clinical expert consensus document on hypertrophic cardiomyopathy. A report of the American College of Cardiology Foundation Task Force on Clinical Expert Consensus Documents and the European Society of Cardiology Committee for Practice Guidelines. Journal of the American College of Cardiology, 42(9), 1687–1713.CrossRefPubMedGoogle Scholar
  3. 3.
    Wigle, E. D., Rakowski, H., Kimball, B. P., & Williams, W. G. (1995). Hypertrophic cardiomyopathy. Clinical spectrum and treatment. Circulation, 92(7), 1680–1692.PubMedGoogle Scholar
  4. 4.
    Shapiro, L. M., & McKenna, W. J. (1983). Distribution of left ventricular hypertrophy in hypertrophic cardiomyopathy: A two-dimensional echocardiographic study. Journal of the American College of Cardiology, 2(3), 437–444.PubMedCrossRefGoogle Scholar
  5. 5.
    Wigle, E. D., Sasson, Z., Henderson, M. A., Ruddy, T. D., Fulop, J., Rakowski, H., et al. (1985). Hypertrophic cardiomyopathy. The importance of the site and the extent of hypertrophy. A review. Progress in Cardiovascular Diseases, 28(1), 1–83.CrossRefPubMedGoogle Scholar
  6. 6.
    Judd, R. M., Lugo-Olivieri, C. H., Arai, M., Kondo, T., Croisille, P., Lima, J. A., et al. (1995). Physiological basis of myocardial contrast enhancement in fast magnetic resonance images of 2-day-old reperfused canine infarcts. Circulation, 92(7), 1902–1910.PubMedGoogle Scholar
  7. 7.
    Kim, R. J., Fieno, D. S., Parrish, T. B., Harris, K., Chen, E. L., Simonetti, O., et al. (1999). Relationship of MRI delayed contrast enhancement to irreversible injury, infarct age, and contractile function. Circulation, 100(19), 1992–2002.PubMedGoogle Scholar
  8. 8.
    Kim, R. J., & Judd, R. M. (2003). Gadolinium-enhanced magnetic resonance imaging in hypertrophic cardiomyopathy: In vivo imaging of the pathologic substrate for premature cardiac death? Journal of the American College of Cardiology, 41(9), 1568–1572.CrossRefPubMedGoogle Scholar
  9. 9.
    Kim, R. J., Wu, E., Rafael, A., Chen, E. L., Parker, M. A., Simonetti, O., et al. (2000). The use of contrast-enhanced magnetic resonance imaging to identify reversible myocardial dysfunction. New England Journal of Medicine, 343(20), 1445–1453.CrossRefPubMedGoogle Scholar
  10. 10.
    Lima, J. A., & Desai, M. Y. (2004). Cardiovascular magnetic resonance imaging: Current and emerging applications. Journal of the American College of Cardiology, 44(6), 1164–1171.CrossRefPubMedGoogle Scholar
  11. 11.
    Moon, J. C., Fisher, N. G., McKenna, W. J., & Pennell, D. J. (2004). Detection of apical hypertrophic cardiomyopathy by cardiovascular magnetic resonance in patients with non-diagnostic echocardiography. Heart, 90(6), 645–649.CrossRefPubMedGoogle Scholar
  12. 12.
    Moon, J. C., McKenna, W. J., McCrohon, J. A., Elliott, P. M., Smith, G. C., & Pennell, D. J. (2003). Toward clinical risk assessment in hypertrophic cardiomyopathy with gadolinium cardiovascular magnetic resonance. Journal of the American College of Cardiology, 41(9), 1561–1567.CrossRefPubMedGoogle Scholar
  13. 13.
    Pennell, D. J. (2003). Cardiovascular magnetic resonance: Twenty-first century solutions in cardiology. Clinical Medicine, 3(3), 273–278.PubMedGoogle Scholar
  14. 14.
    Rickers, C., Wilke, N. M., Jerosch-Herold, M., Casey, S. A., Panse, P., Panse, N., et al. (2005). Utility of cardiac magnetic resonance imaging in the diagnosis of hypertrophic cardiomyopathy. Circulation, 112(6), 855–861.CrossRefPubMedGoogle Scholar
  15. 15.
    Rochitte, C. E., Tassi, E. M., & Shiozaki, A. A. (2006). The emerging role of MRI in the diagnosis and management of cardiomyopathies. Current Cardiology Reports, 8(1), 44–52.CrossRefPubMedGoogle Scholar
  16. 16.
    Maron MS AE, H. C., Buros, J., Gibson, C. M., Hanna, C., Lesser, J. R., Udelson, J. E., et al. (2008). Clinical profile and significance of delayed enhancement in hypertrophic cardiomyopathy. Circulation: Heart Failure, 2008(1), 184–191.Google Scholar
  17. 17.
    Maron, M. S., Maron, B. J., Harrigan, C., Buros, J., Gibson, C. M., Olivotto, I., et al. (2009). Hypertrophic cardiomyopathy phenotype revisited after 50 years with cardiovascular magnetic resonance. Journal of the American College of Cardiology, 54(3), 220–228.CrossRefPubMedGoogle Scholar
  18. 18.
    Harrigan, C. J., Appelbaum, E., Maron, B. J., Buros, J. L., Gibson, C. M., Lesser, J. R., et al. (2008). Significance of papillary muscle abnormalities identified by cardiovascular magnetic resonance in hypertrophic cardiomyopathy. American Journal of Cardiology, 101(5), 668–673.CrossRefPubMedGoogle Scholar
  19. 19.
    Harris, K. M., Spirito, P., Maron, M. S., Zenovich, A. G., Formisano, F., Lesser, J. R., et al. (2006). Prevalence, clinical profile, and significance of left ventricular remodeling in the end-stage phase of hypertrophic cardiomyopathy. Circulation, 114(3), 216–225.CrossRefPubMedGoogle Scholar
  20. 20.
    Moon, J. C., Reed, E., Sheppard, M. N., Elkington, A. G., Ho, S. Y., Burke, M., et al. (2004). The histologic basis of late gadolinium enhancement cardiovascular magnetic resonance in hypertrophic cardiomyopathy. Journal of the American College of Cardiology, 43(12), 2260–2264.CrossRefPubMedGoogle Scholar
  21. 21.
    Adabag, A. S., Maron, B. J., Appelbaum, E., Harrigan, C. J., Buros, J. L., Gibson, C. M., et al. (2008). Occurrence and frequency of arrhythmias in hypertrophic cardiomyopathy in relation to delayed enhancement on cardiovascular magnetic resonance. Journal of the American College of Cardiology, 51(14), 1369–1374.CrossRefPubMedGoogle Scholar
  22. 22.
    Choudhury, L., Mahrholdt, H., Wagner, A., Choi, K. M., Elliott, M. D., Klocke, F. J., et al. (2002). Myocardial scarring in asymptomatic or mildly symptomatic patients with hypertrophic cardiomyopathy. Journal of the American College of Cardiology, 40(12), 2156–2164.CrossRefPubMedGoogle Scholar
  23. 23.
    Kramer, C. M. (2006). The expanding prognostic role of late gadolinium enhanced cardiac magnetic resonance. Journal of the American College of Cardiology, 48(10), 1986–1987.CrossRefPubMedGoogle Scholar
  24. 24.
    Grothues, F., Smith, G. C., Moon, J. C., Bellenger, N. G., Collins, P., Klein, H. U., et al. (2002). Comparison of interstudy reproducibility of cardiovascular magnetic resonance with two-dimensional echocardiography in normal subjects and in patients with heart failure or left ventricular hypertrophy. American Journal of Cardiology, 90(1), 29–34.CrossRefPubMedGoogle Scholar
  25. 25.
    Maron, M. S., Finley, J. J., Bos, J. M., Hauser, T. H., Manning, W. J., Haas, T. S., et al. (2008). Prevalence, clinical significance, and natural history of left ventricular apical aneurysms in hypertrophic cardiomyopathy. Circulation, 118(15), 1541–1549.CrossRefPubMedGoogle Scholar
  26. 26.
    Maron, M. S., Hauser, T. H., Dubrow, E., Horst, T. A., Kissinger, K. V., Udelson, J. E., et al. (2007). Right ventricular involvement in hypertrophic cardiomyopathy. American Journal of Cardiology, 100(8), 1293–1298.CrossRefPubMedGoogle Scholar
  27. 27.
    Maron, B. J., Maron, M. S., Lesser, J. R., Hauser, R. G., Haas, T. S., Harrigan, C. J., et al. (2008). Sudden cardiac arrest in hypertrophic cardiomyopathy in the absence of conventional criteria for high risk status. American Journal of Cardiology, 101(4), 544–547.PubMedGoogle Scholar
  28. 28.
    Kwon, D. H., Setser, R. M., Thamilarasan, M., Popovic, Z. V., Smedira, N. G., Schoenhagen, P., et al. (2008). Abnormal papillary muscle morphology is independently associated with increased left ventricular outflow tract obstruction in hypertrophic cardiomyopathy. Heart (British Cardiac Society), 94(10), 1295–1301.Google Scholar
  29. 29.
    Olivotto, I., Maron, M. S., Autore, C., Lesser, J. R., Rega, L., Casolo, G., et al. (2008). Assessment and significance of left ventricular mass by cardiovascular magnetic resonance in hypertrophic cardiomyopathy. Journal of the American College of Cardiology, 52(7), 559–566.CrossRefPubMedGoogle Scholar
  30. 30.
    Germans, T., Wilde, A. A., Dijkmans, P. A., Chai, W., Kamp, O., Pinto, Y. M., et al. (2006). Structural abnormalities of the inferoseptal left ventricular wall detected by cardiac magnetic resonance imaging in carriers of hypertrophic cardiomyopathy mutations. Journal of the American College of Cardiology, 48(12), 2518–2523.CrossRefPubMedGoogle Scholar
  31. 31.
    Strijack, B., Ariyarajah, V., Soni, R., Jassal, D. S., Greenberg, C. R., McGregor, R., et al. (2008). Late gadolinium enhancement cardiovascular magnetic resonance in genotyped hypertrophic cardiomyopathy with normal phenotype. Journal of Cardiovascular Magnetic Resonance, 10(1), 58.CrossRefPubMedGoogle Scholar
  32. 32.
    Camici, P., Chiriatti, G., Lorenzoni, R., Bellina, R. C., Gistri, R., Italiani, G., et al. (1991). Coronary vasodilation is impaired in both hypertrophied and nonhypertrophied myocardium of patients with hypertrophic cardiomyopathy: a study with nitrogen-13 ammonia and positron emission tomography. Journal of the American College of Cardiology, 17(4), 879–886.PubMedGoogle Scholar
  33. 33.
    Cecchi, F., Olivotto, I., Gistri, R., Lorenzoni, R., Chiriatti, G., & Camici, P. G. (2003). Coronary microvascular dysfunction and prognosis in hypertrophic cardiomyopathy. New England Journal of Medicine, 349(11), 1027–1035.CrossRefPubMedGoogle Scholar
  34. 34.
    Olivotto, I., Cecchi, F., Gistri, R., Lorenzoni, R., Chiriatti, G., Girolami, F., et al. (2006). Relevance of coronary microvascular flow impairment to long-term remodeling and systolic dysfunction in hypertrophic cardiomyopathy. Journal of the American College of Cardiology, 47(5), 1043–1048.CrossRefPubMedGoogle Scholar
  35. 35.
    Petersen, S. E., Jerosch-Herold, M., Hudsmith, L. E., Robson, M. D., Francis, J. M., Doll, H. A., et al. (2007). Evidence for microvascular dysfunction in hypertrophic cardiomyopathy: New insights from multiparametric magnetic resonance imaging. Circulation, 115(18), 2418–2425.CrossRefPubMedGoogle Scholar
  36. 36.
    Sotgia, B., Sciagra, R., Olivotto, I., Casolo, G., Rega, L., Betti, I., et al. (2008). Spatial relationship between coronary microvascular dysfunction and delayed contrast enhancement in patients with hypertrophic cardiomyopathy. Journal of Nuclear Medicine, 49(7), 1090–1096.CrossRefPubMedGoogle Scholar
  37. 37.
    Kwon, D. H., Smedira, N. G., Rodriguez, E. R., Tan, C., Setser, R., Thamilarasan, M., et al. (2009). Cardiac magnetic resonance detection of myocardial scarring in hypertrophic cardiomyopathy: Correlation with histopathology and prevalence of ventricular tachycardia. Journal of the American College of Cardiology, 54(3), 242–249.CrossRefPubMedGoogle Scholar
  38. 38.
    Fieno, D. S., Kim, R. J., Chen, E. L., Lomasney, J. W., Klocke, F. J., & Judd, R. M. (2000). Contrast-enhanced magnetic resonance imaging of myocardium at risk: Distinction between reversible and irreversible injury throughout infarct healing. Journal of the American College of Cardiology, 36(6), 1985–1991.CrossRefPubMedGoogle Scholar
  39. 39.
    Simonetti, O. P., Kim, R. J., Fieno, D. S., Hillenbrand, H. B., Wu, E., Bundy, J. M., et al. (2001). An improved MR imaging technique for the visualization of myocardial infarction. Radiology, 218(1), 215–223.PubMedGoogle Scholar
  40. 40.
    Wilson, J. M., Villareal, R. P., Hariharan, R., Massumi, A., Muthupillai, R., & Flamm, S. D. (2002). Magnetic resonance imaging of myocardial fibrosis in hypertrophic cardiomyopathy. Texas Heart Institute Journal, 29(3), 176–180.PubMedGoogle Scholar
  41. 41.
    Kwon, D. H., Setser, R. M., Popovic, Z. B., Thamilarasan, M., Sola, S., Schoenhagen, P., et al. (2008). Association of myocardial fibrosis, electrocardiography and ventricular tachyarrhythmia in hypertrophic cardiomyopathy: A delayed contrast enhanced MRI study. The International Journal of Cardiovascular Imaging, 24(6), 617–625.CrossRefPubMedGoogle Scholar
  42. 42.
    Valeti, U. S., Nishimura, R. A., Holmes, D. R., Araoz, P. A., Glockner, J. F., Breen, J. F., et al. (2007). Comparison of surgical septal myectomy and alcohol septal ablation with cardiac magnetic resonance imaging in patients with hypertrophic obstructive cardiomyopathy. Journal of the American College of Cardiology, 49(3), 350–357.CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2009

Authors and Affiliations

  1. 1.Division of Cardiology, Hypertrophic Cardiomyopathy CenterTufts Medical CenterBostonUSA

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