Abstract
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.
Similar content being viewed by others
References
Maron, B. J. (2002). Hypertrophic cardiomyopathy: A systematic review. JAMA, 287(10), 1308–1320.
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.
Wigle, E. D., Rakowski, H., Kimball, B. P., & Williams, W. G. (1995). Hypertrophic cardiomyopathy. Clinical spectrum and treatment. Circulation, 92(7), 1680–1692.
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.
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.
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.
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.
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.
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.
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.
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.
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.
Pennell, D. J. (2003). Cardiovascular magnetic resonance: Twenty-first century solutions in cardiology. Clinical Medicine, 3(3), 273–278.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Maron, M.S. The Current and Emerging Role of Cardiovascular Magnetic Resonance Imaging in Hypertrophic Cardiomyopathy. J. of Cardiovasc. Trans. Res. 2, 415–425 (2009). https://doi.org/10.1007/s12265-009-9136-3
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12265-009-9136-3