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Imaging methods in cardiomyopathies
CRVASA-411; No. of Pages 6 cor et vasa xxx (2016) e1–e6
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Review article
Imaging methods in cardiomyopathies Pavel Gregor *, Hana Línková Cardiocenter, 3rd Medical Faculty, Charles University Prague and University Hospital Královské Vinohrady, Prague, Czech Republic
article info
abstract
Article history:
The echocardiography, as a widely available and relatively inexpensive basic imaging
Received 27 April 2016
method, fulfills an irreplaceable function of a screening method in cardiomyopathies.
Received in revised form
Among new imaging methods, three-dimensional (3D) echocardiography may prove useful,
5 September 2016
particularly in spongious and apical hypertrophic cardiomyopathies; speckletracking echo-
Accepted 7 September 2016
cardiography in differentiation of athletic heart from hypertrophic cardiomyopathy, various types of restrictive cardiomyopathy (including the initial stages of cardiac amyloidosis) and
Available online xxx
distinguishing between stress and spongious cardiomyopathy. More detailed information may be provided by cardiac magnetic resonance imaging, especially in arrhythmogenic right
Keywords: Tissue doppler imaging methods
ventricular cardiomyopathy and the prognostic assessment in all types of cardiomyopa-
Echocardiography
thies. A cardiac CT scan serves particularly for discrimination of ischemic heart disease and
Cardiomyopathy
detection of various extracardiac structures. Nevertheless, an essential disadvantage of this
Cardiovascular magnetic resonance
method is the radiation exposure, preventing its use in long-term follow-up. # 2016 The Czech Society of Cardiology. Published by Elsevier Sp. z o.o. All rights
Arrhythmogenic right ventricular
reserved.
cardiomyopathy Late gadolinium enhancement Cardiac CT
Contents Introduction . . . . . Conflict of interest Ethical statement . Acknowledgments Funding body . . . . References . . . . . . .
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Introduction The development of new imaging methods is thriving. If imaging methods were studied during the era when the
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legendary cardiology textbook was penned by professor Jonáš [1], the imaging methods would comprise cardiovascular radiology, such as orthodiagraphy, orthodiascopy, teleradiography, radiographic kymography and electrokymography,
* Corresponding author at: 3rd Department of Internal Medicine – Cardiology, University Hospital Kralovske Vinohrady and Third Medical Faculty of Charles University, Srobarova 50, 100 34 Prague 10, Czech Republic. E-mail address: [email protected] (P. Gregor). http://dx.doi.org/10.1016/j.crvasa.2016.09.002 0010-8650/# 2016 The Czech Society of Cardiology. Published by Elsevier Sp. z o.o. All rights reserved.
Please cite this article in press as: P. Gregor, H. Línková, Imaging methods in cardiomyopathies, Cor et Vasa (2016), http://dx.doi.org/10.1016/ j.crvasa.2016.09.002
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X-ray cinematography and skiascopy [1]. With the exception of skiascopy, none of the aforementioned methods is being used nowadays. In this review, we discuss the non-invasive imaging methods, as defined by the European Association of Cardiovascular Imaging (EACVI), involving echocardiography, cardiac magnetic resonance, computer tomography and nuclear cardiac imaging. Nuclear cardiac imaging uses intravenous radioactive tracers and includes multi-gated acquisition (MUGA) scans, stress testing with single-photon emission computed tomography (SPECT) and positron emission tomography (PET). Both MUGA and SPECT paging provide information on the right and left ventricular volume, ejection fraction and wall motion assessment. The methods of nuclear cardiology might be sometimes useful in detection of cardiac sarcoidosis or amyloidosis but – on the other hand – these methods do not belong to common imaging methods in cardiomyopathies. Echocardiography is the most established imaging method (introduced by C.H. Herz and I. Edler in 1953), which may be used bedside, applied repeatedly and it is the cheapest of all previously named methods. However, we may ask the following question: is it advancing further or has it reached its zenith? Indeed, in the earliest and most traditional modes, primarily two-dimensional (2D) and Doppler echocardiography, no fundamental innovations have emerged and it is difficult to picture any. However, there have been many advances in 3D echocardiography and in some of the more recent modes, particularly in tissue Doppler and speckle-tracking imaging. 3D echocardiography has proven effective in diagnostics of non-compaction cardiomyopathy, which affects the left or/and right ventricle. It seems that its greatest asset lies in distinguishing isolated left ventricular or right ventricular (or both) forms, in confusing or non-characteristic images, preventing the proper reading [2–4], and as a result the patients are frequently misdiagnosed, i.e. with cardiac tumor. 3D echocardiography may lead to the refinement or correction of previous diagnosis [2]. According to some authors, it enables more accurate assessment of apical hypertrophic cardiomyopathy [5] – Fig. 1, obstruction (particularly mid-ventricular), and recognition of complications following cardiac surgery in hypertrophic
Fig. 1 – 3D echocardiography in hypertrophic cardiomyopathy, with significant interventricular septal hypertrophy (arrow). Apical four-chamber view.
cardiomyopathy, such as fistulae [6,7]. Furthermore, 3D left ventricular volumetry is more precise compared to 2D volumetric analysis [8,9]. Tissue Doppler imaging methods (TDI and TVI) – Fig. 2 – are based on suppression of the high-velocity signals from the blood flow and assessment of the lower-velocity signals of myocardial or vascular tissue motion (Tissue-Tracking Imaging – TTI and Tissue Synchronization Imaging – TSI). They allow the evaluation of the strain (deformation) and measurement of relevant parameters such as strain and strain rate – SR, as an instantaneous velocity difference between two points relative to their distance [s1]. There are several limitations to TDI, such as incidence angle dependence, which are a necessity of higher frame rate, as well as possibility of modification by extracardiac motions [9–11]. A unique imaging method, used in the past few years, is the speckle-tracking echocardiography (STE), where the strain curve is created based on analysis of spatial dislocation of the myocardial tissue spots (speckles) – Fig. 3. It is a non-Doppler parametric imaging with the assessment of deformation in three dimensions. Given the non-Doppler character of this method, it is angle independent in contrast with TDI. Recently, the guidelines for standardization of this method in 2D mode
Fig. 2 – Tissue Doppler echocardiography in a patient with hypertrophic cardiomyopathy. TDI measured at septal mitral annulus, with low velocity of the E0 wave.
Fig. 3 – 2D speckle tracking shows a reduced longitudinal strain in a patient with hypertrophic cardiomyopathy.
Please cite this article in press as: P. Gregor, H. Línková, Imaging methods in cardiomyopathies, Cor et Vasa (2016), http://dx.doi.org/10.1016/ j.crvasa.2016.09.002
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have been published [12]. Nevertheless, more accurate 3D mode may be applied as well [13–15]. STE enables the assessment of tissue shortening and lengthening, expressed as longitudinal, circumferential, radial and transversal strain, as well as shear strain [11]. The advantages of speckle tracking compared to tissue Doppler echocardiography are better 3D resolution, relative angle independence [9,10], and lower interindividual variability [13]. What are the chief clinical application possibilities of speckle tracking? According to the most recent sources, it is the distinguishing of the athletic heart or initial stages of cardiac amyloidosis from hypertrophic cardiomyopathy and differentiation of the stress cardiomyopathy (tako-tsubo), spongious cardiomyopathy (non-compaction), cardiac sarcoidosis and particular types of restrictive cardiomyopathy [13]. In hypertrophic cardiomyopathy, this method may serve for rather reliable left ventricular diastolic function assessment [16,17]. Cardiovascular magnetic resonance (CMR and MRI) is a highresolution imaging method, applicable on beating heart, with an excellent spatial definition. Further advantages include the good reproducibility and tissue resolution (scar, tumor, and inflammation). The main advantages comprise the noninvasivity and the absence of radiation exposure – the examination is repeatable and useful in postoperative follow-up. Its main disadvantage is the impossibility of examining the patients with older pacemaker models. CMR may be employed in diagnostics of all forms of cardiomyopathies. Historically, it plays the most important role in some types of restrictive cardiomyopathies, where other imaging methods do not supply valid information necessary for an early and definitive diagnosis. This applies particularly for cardiac amyloidoses, where timely diagnosis is necessary for the initiation of chemotherapy. Thickening of myocardium and interatrial septum is a characteristic feature and essential information may be provided by late gadolinium enhancement (LGE) positivity – see below. CMR enables an early diagnosis of familiar transthyretin cardiomyopathy – often accompanied by a very discreet cardiac impairment, difficult to demonstrate by other imaging methods, but LGE positive in 60% of the cases [18]. CMR allows the differentiation of endomyocardial fibrosis from other forms of restrictive cardiomyopathies and from constrictive pericarditis [19].
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Fig. 4 – MRI in arrhythmogenic right ventricular cardiomyopathy. (A) Cine sequention in end-diastole and (B) cine sequention in end-systole. Arrows show localization of right ventricular aneurysms. Courtesy of Radka Kočková, M.D., Ph.D., IKEM Praha.
In arrhythmogenic right ventricular cardiomyopathy (Fig. 4), the CMR represents the most accurate imaging method [20]. It allows the precise assessment of global and regional right ventricular dilation, its dysfunction (akinesis and dyskinesis), and discerns the right ventricular aneurysms – essential signs for reliable diagnosis of this entity [21]. Late gadolinium enhancement evaluation (LGE) proves useful as well (see below). In hypertrophic cardiomyopathy, the CMR enables more precise detection of apical or lateral wall endocardium, as well as right ventricular hypertrophy compared to echocardiography [22,23]. It is suitable particularly in cases with suspected hypertrophic cardiomyopathy (i.e. from electrocardiography and personal history), where the echocardiographic finding is negative. This method is more accurate in the case of mid-ventricular obstruction with papillary muscles anomalies, or differentiation between spongious and apical hypertrophic cardiomyopathy – Fig. 5 [19,22–25]. It may be used in dilated cardiomyopathy including myocarditis [24,26], cardiac sarcoidosis [24,27], chemotherapy-induced cardiomyopathy [28], spongious [29] or stress cardiomyopathy [30]. CMR is more accurate compared to transthoracic echocardiography in the left ventricular dimensions and function assessment, as well as myocardial thickness evaluation [31]. Its role in volumetry and evaluation of the right ventricular function is undoubtly dominant [32].
Fig. 5 – MRI – apical form of hypertrophic cardiomyopathy. (A) Cine sequention in four-chamber view at end-diastole. (B) Late enhancement in the same patient 16 min after application of gadolinium. Arrows show localization of increase of signal. Courtesy of Radka Kočková, M.D., Ph.D., IKEM Praha. Please cite this article in press as: P. Gregor, H. Línková, Imaging methods in cardiomyopathies, Cor et Vasa (2016), http://dx.doi.org/10.1016/ j.crvasa.2016.09.002
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Fig. 6 – MRI imaging in hypertrophic cardiomyopathy, late enhancement (LGE). Late enhancement sequention 13 min after application of gadolinium contrast in a patient with hypertrophic cardiomyopathy with septal dominance. Arrows show localization of signal increase. Courtesy of Radka Kočková, M.D., Ph.D., IKEM Praha.
The late gadolinium enhancement – LGE is based on gadolinium accumulation in extracellular tissue characterized by fibrosis and necrosis (Fig. 6). This is applicable for dilated cardiomyopathy, as well as hypertrophic cardiomyopathy, where LGE occurs predominantly in hypertrophic septum [24]. Positive LGE is a valuable prognostic marker in all types of cardiomyopathies [33], such as dilated cardiomyopathy [34–37], where its evaluation has proved more beneficial in outcomes assessment than endomyocardial biopsy [38]. LGE brings valuable prognostic information in myocarditis in adults [39] and children [40], as well as in spongious cardiomyopathy [41]. LGE plays a fundamental role in the outcome prediction in hypertrophic cardiomyopathy, being an independent predictor of morbidity and mortality [22,42–44], including its apical form [45]. Similar results were found in children [46]. It proves useful in the probability assessment for ventricular arrhythmias and sudden cardiac death [47–49] and may likely represent an additional factor (besides the 5 known risk factors) [49,50] in the decision making for an ICD implantation [51]. It has been shown that in patients with the absence of classical risk factors for sudden cardiac death and negative LGE, refraining from ICD implantation was a correct choice [22,33,51,52]. The indications and importance of CMR in children are discussed in the current European guidelines [53]. Cardiac CT is one of the most important imaging methods. The chief advantage of the cardiac CT is a good tissue resolution; the main disadvantage is the radiation exposure. The possibility of differentiation between ischemic heart disease (‘‘ischemic cardiomyopathy’’) and dilated cardiomyopathy, and the visualization of coronary vessels [54] provides the greatest benefit in its clinical use. The visualization of various extracardiac structures or indicators of numerous (mainly systemic) diseases (i.e. lymph nodes in sarcoidosis) is
important as well. Some studies praise the contribution of CT to diagnostics of spongious cardiomyopathy and other entities [55–58], possibilities of left and right ventricular function assessment, measurement of cardiac chamber dimensions and myocardial thickness. A parameter, which serves as an analogue to the ‘‘late enhancement’’ in CMR, may be quantified – according to some studies, both methods showed good correlation [59,60]. Nevertheless, our initial experience with this method does not seem too encouraging. What are the prospects of imaging methods in the future? It is a difficult question to answer, as their progress is hardly predictable for the distant future. The authors of this article believe that echocardiography will remain the main screening method in the near future; the roles of 3D echocardiography and strain assessment methods in routine examination of cardiomyopathies have yet to be defined. The advantages of echocardiography comprise the overall accessibility and an extensive use. However, the latter may not always be of benefit, as the physician experience may vary [61], and in case of poor degree of expertise, it could lead to some discrediting of the method. The star of CMR will probably remain on the rise for some time on; however, echocardiography will likely continue to be the screening method number one. CMR indeed represents a standard method in diagnostics of arrhythmogenic right ventricular cardiomyopathy and certain restrictive cardiomyopathies (particularly cardiac amyloidosis), myocarditis or persisting suspicion for hypertrophic cardiomyopathy with negative echocardiography or its unsatisfactory quality. Nevertheless, the CMR will probably remain the method of supplement to echocardiography for certain diagnoses, even though the non-invasiveness and growing accessibility predestine it for more significant role in cardiomyopathy diagnostics, as well as long-term follow-up. Cardiovascular CT is probably at the top of its potentials in the area of cardiomyopathies nowadays. Recently, new possibilities of use have been emerging, i.e. in percutaneous valve implantation and other invasive procedures [58]. Its most important role in cardiomyopathies is distinguishing the ischemic heart disease from dilated cardiomyopathy thanks to the visualization of the coronary vessels. However, due to the radiation exposure, it will certainly not be appropriate for the long-term follow-up.
Conflict of interest None declared.
Ethical statement Authors state that the research was conducted according to ethical standards.
Acknowledgments The authors gratefully acknowledge Dr. Radka Kočková, Ph.D., Chief of Non-invasive Cardiology Unit Department, Institute
Please cite this article in press as: P. Gregor, H. Línková, Imaging methods in cardiomyopathies, Cor et Vasa (2016), http://dx.doi.org/10.1016/ j.crvasa.2016.09.002
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for Clinical and Experimental Medicine in Prague for Figs. 4–6 on MRI. The work was supported by the Third Faculty of Medicine, Charles University Prague, Research Project PRVOUK P 35/2012.
Funding body
[15]
[16]
None.
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Review article
Imaging methods in cardiomyopathies Pavel Gregor *, Hana Línková Cardiocenter, 3rd Medical Faculty, Charles University Prague and University Hospital Královské Vinohrady, Prague, Czech Republic
article info
abstract
Article history:
The echocardiography, as a widely available and relatively inexpensive basic imaging
Received 27 April 2016
method, fulfills an irreplaceable function of a screening method in cardiomyopathies.
Received in revised form
Among new imaging methods, three-dimensional (3D) echocardiography may prove useful,
5 September 2016
particularly in spongious and apical hypertrophic cardiomyopathies; speckletracking echo-
Accepted 7 September 2016
cardiography in differentiation of athletic heart from hypertrophic cardiomyopathy, various types of restrictive cardiomyopathy (including the initial stages of cardiac amyloidosis) and
Available online xxx
distinguishing between stress and spongious cardiomyopathy. More detailed information may be provided by cardiac magnetic resonance imaging, especially in arrhythmogenic right
Keywords: Tissue doppler imaging methods
ventricular cardiomyopathy and the prognostic assessment in all types of cardiomyopa-
Echocardiography
thies. A cardiac CT scan serves particularly for discrimination of ischemic heart disease and
Cardiomyopathy
detection of various extracardiac structures. Nevertheless, an essential disadvantage of this
Cardiovascular magnetic resonance
method is the radiation exposure, preventing its use in long-term follow-up. # 2016 The Czech Society of Cardiology. Published by Elsevier Sp. z o.o. All rights
Arrhythmogenic right ventricular
reserved.
cardiomyopathy Late gadolinium enhancement Cardiac CT
Contents Introduction . . . . . Conflict of interest Ethical statement . Acknowledgments Funding body . . . . References . . . . . . .
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Introduction The development of new imaging methods is thriving. If imaging methods were studied during the era when the
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legendary cardiology textbook was penned by professor Jonáš [1], the imaging methods would comprise cardiovascular radiology, such as orthodiagraphy, orthodiascopy, teleradiography, radiographic kymography and electrokymography,
* Corresponding author at: 3rd Department of Internal Medicine – Cardiology, University Hospital Kralovske Vinohrady and Third Medical Faculty of Charles University, Srobarova 50, 100 34 Prague 10, Czech Republic. E-mail address: [email protected] (P. Gregor). http://dx.doi.org/10.1016/j.crvasa.2016.09.002 0010-8650/# 2016 The Czech Society of Cardiology. Published by Elsevier Sp. z o.o. All rights reserved.
Please cite this article in press as: P. Gregor, H. Línková, Imaging methods in cardiomyopathies, Cor et Vasa (2016), http://dx.doi.org/10.1016/ j.crvasa.2016.09.002
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X-ray cinematography and skiascopy [1]. With the exception of skiascopy, none of the aforementioned methods is being used nowadays. In this review, we discuss the non-invasive imaging methods, as defined by the European Association of Cardiovascular Imaging (EACVI), involving echocardiography, cardiac magnetic resonance, computer tomography and nuclear cardiac imaging. Nuclear cardiac imaging uses intravenous radioactive tracers and includes multi-gated acquisition (MUGA) scans, stress testing with single-photon emission computed tomography (SPECT) and positron emission tomography (PET). Both MUGA and SPECT paging provide information on the right and left ventricular volume, ejection fraction and wall motion assessment. The methods of nuclear cardiology might be sometimes useful in detection of cardiac sarcoidosis or amyloidosis but – on the other hand – these methods do not belong to common imaging methods in cardiomyopathies. Echocardiography is the most established imaging method (introduced by C.H. Herz and I. Edler in 1953), which may be used bedside, applied repeatedly and it is the cheapest of all previously named methods. However, we may ask the following question: is it advancing further or has it reached its zenith? Indeed, in the earliest and most traditional modes, primarily two-dimensional (2D) and Doppler echocardiography, no fundamental innovations have emerged and it is difficult to picture any. However, there have been many advances in 3D echocardiography and in some of the more recent modes, particularly in tissue Doppler and speckle-tracking imaging. 3D echocardiography has proven effective in diagnostics of non-compaction cardiomyopathy, which affects the left or/and right ventricle. It seems that its greatest asset lies in distinguishing isolated left ventricular or right ventricular (or both) forms, in confusing or non-characteristic images, preventing the proper reading [2–4], and as a result the patients are frequently misdiagnosed, i.e. with cardiac tumor. 3D echocardiography may lead to the refinement or correction of previous diagnosis [2]. According to some authors, it enables more accurate assessment of apical hypertrophic cardiomyopathy [5] – Fig. 1, obstruction (particularly mid-ventricular), and recognition of complications following cardiac surgery in hypertrophic
Fig. 1 – 3D echocardiography in hypertrophic cardiomyopathy, with significant interventricular septal hypertrophy (arrow). Apical four-chamber view.
cardiomyopathy, such as fistulae [6,7]. Furthermore, 3D left ventricular volumetry is more precise compared to 2D volumetric analysis [8,9]. Tissue Doppler imaging methods (TDI and TVI) – Fig. 2 – are based on suppression of the high-velocity signals from the blood flow and assessment of the lower-velocity signals of myocardial or vascular tissue motion (Tissue-Tracking Imaging – TTI and Tissue Synchronization Imaging – TSI). They allow the evaluation of the strain (deformation) and measurement of relevant parameters such as strain and strain rate – SR, as an instantaneous velocity difference between two points relative to their distance [s1]. There are several limitations to TDI, such as incidence angle dependence, which are a necessity of higher frame rate, as well as possibility of modification by extracardiac motions [9–11]. A unique imaging method, used in the past few years, is the speckle-tracking echocardiography (STE), where the strain curve is created based on analysis of spatial dislocation of the myocardial tissue spots (speckles) – Fig. 3. It is a non-Doppler parametric imaging with the assessment of deformation in three dimensions. Given the non-Doppler character of this method, it is angle independent in contrast with TDI. Recently, the guidelines for standardization of this method in 2D mode
Fig. 2 – Tissue Doppler echocardiography in a patient with hypertrophic cardiomyopathy. TDI measured at septal mitral annulus, with low velocity of the E0 wave.
Fig. 3 – 2D speckle tracking shows a reduced longitudinal strain in a patient with hypertrophic cardiomyopathy.
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have been published [12]. Nevertheless, more accurate 3D mode may be applied as well [13–15]. STE enables the assessment of tissue shortening and lengthening, expressed as longitudinal, circumferential, radial and transversal strain, as well as shear strain [11]. The advantages of speckle tracking compared to tissue Doppler echocardiography are better 3D resolution, relative angle independence [9,10], and lower interindividual variability [13]. What are the chief clinical application possibilities of speckle tracking? According to the most recent sources, it is the distinguishing of the athletic heart or initial stages of cardiac amyloidosis from hypertrophic cardiomyopathy and differentiation of the stress cardiomyopathy (tako-tsubo), spongious cardiomyopathy (non-compaction), cardiac sarcoidosis and particular types of restrictive cardiomyopathy [13]. In hypertrophic cardiomyopathy, this method may serve for rather reliable left ventricular diastolic function assessment [16,17]. Cardiovascular magnetic resonance (CMR and MRI) is a highresolution imaging method, applicable on beating heart, with an excellent spatial definition. Further advantages include the good reproducibility and tissue resolution (scar, tumor, and inflammation). The main advantages comprise the noninvasivity and the absence of radiation exposure – the examination is repeatable and useful in postoperative follow-up. Its main disadvantage is the impossibility of examining the patients with older pacemaker models. CMR may be employed in diagnostics of all forms of cardiomyopathies. Historically, it plays the most important role in some types of restrictive cardiomyopathies, where other imaging methods do not supply valid information necessary for an early and definitive diagnosis. This applies particularly for cardiac amyloidoses, where timely diagnosis is necessary for the initiation of chemotherapy. Thickening of myocardium and interatrial septum is a characteristic feature and essential information may be provided by late gadolinium enhancement (LGE) positivity – see below. CMR enables an early diagnosis of familiar transthyretin cardiomyopathy – often accompanied by a very discreet cardiac impairment, difficult to demonstrate by other imaging methods, but LGE positive in 60% of the cases [18]. CMR allows the differentiation of endomyocardial fibrosis from other forms of restrictive cardiomyopathies and from constrictive pericarditis [19].
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Fig. 4 – MRI in arrhythmogenic right ventricular cardiomyopathy. (A) Cine sequention in end-diastole and (B) cine sequention in end-systole. Arrows show localization of right ventricular aneurysms. Courtesy of Radka Kočková, M.D., Ph.D., IKEM Praha.
In arrhythmogenic right ventricular cardiomyopathy (Fig. 4), the CMR represents the most accurate imaging method [20]. It allows the precise assessment of global and regional right ventricular dilation, its dysfunction (akinesis and dyskinesis), and discerns the right ventricular aneurysms – essential signs for reliable diagnosis of this entity [21]. Late gadolinium enhancement evaluation (LGE) proves useful as well (see below). In hypertrophic cardiomyopathy, the CMR enables more precise detection of apical or lateral wall endocardium, as well as right ventricular hypertrophy compared to echocardiography [22,23]. It is suitable particularly in cases with suspected hypertrophic cardiomyopathy (i.e. from electrocardiography and personal history), where the echocardiographic finding is negative. This method is more accurate in the case of mid-ventricular obstruction with papillary muscles anomalies, or differentiation between spongious and apical hypertrophic cardiomyopathy – Fig. 5 [19,22–25]. It may be used in dilated cardiomyopathy including myocarditis [24,26], cardiac sarcoidosis [24,27], chemotherapy-induced cardiomyopathy [28], spongious [29] or stress cardiomyopathy [30]. CMR is more accurate compared to transthoracic echocardiography in the left ventricular dimensions and function assessment, as well as myocardial thickness evaluation [31]. Its role in volumetry and evaluation of the right ventricular function is undoubtly dominant [32].
Fig. 5 – MRI – apical form of hypertrophic cardiomyopathy. (A) Cine sequention in four-chamber view at end-diastole. (B) Late enhancement in the same patient 16 min after application of gadolinium. Arrows show localization of increase of signal. Courtesy of Radka Kočková, M.D., Ph.D., IKEM Praha. Please cite this article in press as: P. Gregor, H. Línková, Imaging methods in cardiomyopathies, Cor et Vasa (2016), http://dx.doi.org/10.1016/ j.crvasa.2016.09.002
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Fig. 6 – MRI imaging in hypertrophic cardiomyopathy, late enhancement (LGE). Late enhancement sequention 13 min after application of gadolinium contrast in a patient with hypertrophic cardiomyopathy with septal dominance. Arrows show localization of signal increase. Courtesy of Radka Kočková, M.D., Ph.D., IKEM Praha.
The late gadolinium enhancement – LGE is based on gadolinium accumulation in extracellular tissue characterized by fibrosis and necrosis (Fig. 6). This is applicable for dilated cardiomyopathy, as well as hypertrophic cardiomyopathy, where LGE occurs predominantly in hypertrophic septum [24]. Positive LGE is a valuable prognostic marker in all types of cardiomyopathies [33], such as dilated cardiomyopathy [34–37], where its evaluation has proved more beneficial in outcomes assessment than endomyocardial biopsy [38]. LGE brings valuable prognostic information in myocarditis in adults [39] and children [40], as well as in spongious cardiomyopathy [41]. LGE plays a fundamental role in the outcome prediction in hypertrophic cardiomyopathy, being an independent predictor of morbidity and mortality [22,42–44], including its apical form [45]. Similar results were found in children [46]. It proves useful in the probability assessment for ventricular arrhythmias and sudden cardiac death [47–49] and may likely represent an additional factor (besides the 5 known risk factors) [49,50] in the decision making for an ICD implantation [51]. It has been shown that in patients with the absence of classical risk factors for sudden cardiac death and negative LGE, refraining from ICD implantation was a correct choice [22,33,51,52]. The indications and importance of CMR in children are discussed in the current European guidelines [53]. Cardiac CT is one of the most important imaging methods. The chief advantage of the cardiac CT is a good tissue resolution; the main disadvantage is the radiation exposure. The possibility of differentiation between ischemic heart disease (‘‘ischemic cardiomyopathy’’) and dilated cardiomyopathy, and the visualization of coronary vessels [54] provides the greatest benefit in its clinical use. The visualization of various extracardiac structures or indicators of numerous (mainly systemic) diseases (i.e. lymph nodes in sarcoidosis) is
important as well. Some studies praise the contribution of CT to diagnostics of spongious cardiomyopathy and other entities [55–58], possibilities of left and right ventricular function assessment, measurement of cardiac chamber dimensions and myocardial thickness. A parameter, which serves as an analogue to the ‘‘late enhancement’’ in CMR, may be quantified – according to some studies, both methods showed good correlation [59,60]. Nevertheless, our initial experience with this method does not seem too encouraging. What are the prospects of imaging methods in the future? It is a difficult question to answer, as their progress is hardly predictable for the distant future. The authors of this article believe that echocardiography will remain the main screening method in the near future; the roles of 3D echocardiography and strain assessment methods in routine examination of cardiomyopathies have yet to be defined. The advantages of echocardiography comprise the overall accessibility and an extensive use. However, the latter may not always be of benefit, as the physician experience may vary [61], and in case of poor degree of expertise, it could lead to some discrediting of the method. The star of CMR will probably remain on the rise for some time on; however, echocardiography will likely continue to be the screening method number one. CMR indeed represents a standard method in diagnostics of arrhythmogenic right ventricular cardiomyopathy and certain restrictive cardiomyopathies (particularly cardiac amyloidosis), myocarditis or persisting suspicion for hypertrophic cardiomyopathy with negative echocardiography or its unsatisfactory quality. Nevertheless, the CMR will probably remain the method of supplement to echocardiography for certain diagnoses, even though the non-invasiveness and growing accessibility predestine it for more significant role in cardiomyopathy diagnostics, as well as long-term follow-up. Cardiovascular CT is probably at the top of its potentials in the area of cardiomyopathies nowadays. Recently, new possibilities of use have been emerging, i.e. in percutaneous valve implantation and other invasive procedures [58]. Its most important role in cardiomyopathies is distinguishing the ischemic heart disease from dilated cardiomyopathy thanks to the visualization of the coronary vessels. However, due to the radiation exposure, it will certainly not be appropriate for the long-term follow-up.
Conflict of interest None declared.
Ethical statement Authors state that the research was conducted according to ethical standards.
Acknowledgments The authors gratefully acknowledge Dr. Radka Kočková, Ph.D., Chief of Non-invasive Cardiology Unit Department, Institute
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for Clinical and Experimental Medicine in Prague for Figs. 4–6 on MRI. The work was supported by the Third Faculty of Medicine, Charles University Prague, Research Project PRVOUK P 35/2012.
Funding body
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[16]
None.
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Please cite this article in press as: P. Gregor, H. Línková, Imaging methods in cardiomyopathies, Cor et Vasa (2016), http://dx.doi.org/10.1016/ j.crvasa.2016.09.002