Abstract
Tissue Doppler and deformation imaging, including Doppler-derived strain and speckle tracking, have significantly improved our understanding of cardiac mechanics in both physiological and pathological states. The various modes of left ventricular deformation (longitudinal, circumferential, radial and twist) leading to systolic contraction can nowadays be quantified. One of the best applications of deformation imaging is in the area of hypertrophic cardiomyopathies. Deformation imaging allows the evaluation of global and regional myocardial performance and the noninvasive characterization of abnormal intramural myocardial mechanics. In this review, we discuss the role of myocardial deformation imaging derived by echocardiography in the assessment of rare hypertrophic phenotype including Fabry disease, Friedreich ataxia and amyloidosis. Deformation imaging allows for early identification of myocardial dysfunction in many hypertrophic disorders, at an earlier stage than that provided by standard imaging or echocardiographic techniques. This allows for the implementation of appropriate therapy before significant disease progression has occurred and prior to the development of advanced myocardial fibrosis. Thus therapy would likely be more effective and may potentially lead to improvement in patient outcome. Additionally strain imaging allows to better monitoring the efficacy of therapy by assessing the progression and regression of myocardial involvement. Finally, findings on strain imaging carry important prognostic information in many hypertrophic disorders.
Highlights
-c for early identification of myocardial dysfunction in many hypertrophic disorders, at an earlier n stage than that provided by standard imaging or o echocardiographic techniques
We discuss the role of myocardial deformation imaging derived by echocardiography in the assessment of rare hypertrophic phenotype including Fabry disease, Friedreich ataxia and amyloidosis
The difference between myocardial motion assessed by tissue velocities and myocardial deformation assessed by strain and strain rate may be explained by this example: a moving object will change its position over time but will not deform if all of its parts move at the same velocity
Summary
Several comprehensive reviews have been published regarding the technical aspects and clinical applications of strain imaging.[1,2,3] Briefly, the difference between myocardial motion assessed by tissue velocities and myocardial deformation assessed by strain and strain rate may be explained by this example: a moving object will change its position (motion) over time but will not deform if all of its parts move at the same velocity. Newer echocardiographic modalities allow detection of abnormalities in myocardial function in the pre-clinical stage and prior to the development of morphological abnormalities on standard echocardiography (Table 1).[14] basal inferolateral segment with focal reduction of peak longitudinal strain in this area on deformation imaging.[20,21,22,23] A characteristic pattern is noted on strain rate imaging consisting of an early systolic peak followed by a rapid fall and a second peak during the isovolumic relaxation phase indicative of post-systolic shortening.[20,21,22,23] Whereas this double peak sign (Figure 1) is first noted in the basal inferolateral segment, it could as well be identified in other myocardial segments of FC where no fibrosis is initially detected by late enhancement on CMR.[20,21,22,23] These segments demonstrate late enhancement indicative of fibrosis on follow-up CMR. Main features concerning standard and new echocardiographic findings in Fabry disease, Friedreich ataxia and amyloidosis
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