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The role of echocardiographic deformation imaging in hypertrophic myopathies

Nature Reviews Cardiology volume 7, pages 384–396 (2010)Cite this article

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Abstract

Echocardiography has a leading role in the routine assessment and diagnosis of hypertrophic ventricles. However, the use of M-mode echocardiography and measurement of global left ventricular function may be misleading. Traditionally, systolic function was thought to be preserved in patients with hypertrophic myopathies until the late stages of the disease, and hypertrophic myopathies were thought to affect the myocardium more diffusely than ischemic heart disease. Ultrasound deformation imaging, either by Doppler myocardial imaging or speckle tracking, provides more-sensitive detection of regional myocardial motion and deformation than standard echocardiography. Basic and clinical studies that apply these techniques have revealed early, often subclinical impairment in systolic function. This information allows the detection and treatment of myocardial dysfunction at an early stage, which is of high clinical importance. Physiological hypertrophic remodeling seen in athletes differs from pathological myocardial hypertrophy, which can be caused by compensatory reactive hypertrophy owing to pressure overload in patients with aortic stenosis or hypertension, as well as amyloidosis, Fabry disease or Friedreich ataxia. Each of the etiologies associated with hypertrophy demonstrate distinct regional changes in myocardial deformation, which allows identification of the underlying processes, and will improve the assessment and follow-up of patients with hypertrophic myopathies.

Key Points

  • Hypertrophic myopathies represent a heterogeneous group of diseases with specific myocardial regions that are affected

  • Characteristic changes in regional deformation can be used to determine the underlying hypertrophic processes, and improve the assessment and follow-up of treatment

  • Pressure overload mainly affects the basal septum, whereas systemic cardiomyopathies that induce hypertrophy affect the left ventricular free wall, hypertrophic cardiomyopathy shows patient-specific abnormal segments, and amyloidosis affects the myocardium diffusely

  • Conventional echocardiography routinely assesses radial function of the myocardium, which might mask the early onset of myocardial disease as functional (systolic) impairment primarily affects the longitudinal myocardial fibers

  • Doppler myocardial imaging and speckle tracking are more-sensitive imaging modalities than conventional echocardiography, thus enabling the detection of hypertrophic myopathies at an earlier stage

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Figure 1: Balance of cardiac contractility and loading.
Figure 2: Normal myocardial motion and deformation, and typical profiles of strain rate and strain traces.9
Figure 3: Schematic overview of typical longitudinal strain curves observed in different types of hypertrophic remodeling.

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Acknowledgements

This work was partially funded by the Ministry of Science, Education and Sports of the Republic of Croatia (project number 108-1081875-1991), and the L'Oréal-UNESCO Scholarship for Women in Science.

Charles P. Vega, University of California, Irvine, CA, is the author of and is solely responsible for the content of the learning objectives, questions and answers of the MedscapeCME-accredited continuing medical education activity associated with this article.

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Authors and Affiliations

  1. Department of Cardiovascular Diseases, University of Zagreb School of Medicine, University Hospital Center Zagreb, Kišpaticeva 12, 10000, Zagreb, Croatia

    Maja Cikes

  2. St. George's Hospital, Blackshaw Road, Tooting, SW17 0QT, London, UK

    George R. Sutherland & Lisa J. Anderson

  3. Catalan Institution for Research and Advanced Studies, Universitat Pompeu Fabra, Carrer Tànger 122–140, 08018, Barcelona, Spain

    Bart H. Bijnens

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Cikes, M., Sutherland, G., Anderson, L. et al. The role of echocardiographic deformation imaging in hypertrophic myopathies. Nat Rev Cardiol 7, 384–396 (2010). https://doi.org/10.1038/nrcardio.2010.56

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