TY - JOUR
AU - Weytjens,, Caroline
AB - Abstract Dilated cardiomyopathy (DCM) is defined by the presence of left ventricular or biventricular dilatation and systolic dysfunction in the absence of abnormal loading conditions or coronary artery disease sufficient to explain these changes. This is a heterogeneous disease frequently having a genetic background. Imaging is important for the diagnosis, the prognostic assessment and for guiding therapy. A multimodality imaging approach provides a comprehensive evaluation of all the issues related to this disease. The present document aims to provide recommendations for the use of multimodality imaging according to the clinical question. Selection of one or another imaging technique should be based on the clinical condition and context. Techniques are presented with the aim to underscore what is ‘clinically relevant’ and what are the tools that ‘can be used’. There remain some gaps in evidence on the impact of multimodality imaging on the management and the treatment of DCM patients where ongoing research is important. dilated cardiomyopathy, prognosis, treatment, echocardiography, cardiac magnetic resonance, nuclear imaging A definition for dilated cardiomyopathy Dilated cardiomyopathy (DCM) is defined by the presence of left ventricular (LV) or biventricular dilatation and systolic dysfunction in the absence of abnormal loading conditions (hypertension and valve disease) or coronary artery disease sufficient to cause global systolic impairment (Figure 1 and Tables 1and2).1–3 Figure 1 Open in new tabDownload slide Clinical spectrum of the DCM with the important pre-clinical period. From Pinto et al.1aShown by two independent imaging modalities. bMutation carrier or not; anti-heart autoantibody (AHA) positive or negative. Figure 1 Open in new tabDownload slide Clinical spectrum of the DCM with the important pre-clinical period. From Pinto et al.1aShown by two independent imaging modalities. bMutation carrier or not; anti-heart autoantibody (AHA) positive or negative. Table 1 Key points of the position paper based on scientific background and experts’ consensus Key points 1 Dilated cardiomyopathy (DCM) is defined by the presence of left ventricular (LV) or biventricular dilatation and systolic dysfunction in the absence of abnormal loading conditions (hypertension and valve disease) or coronary artery disease sufficient to cause global systolic impairment. 2 All the imaging techniques should not be performed and repeated in every single DCM-patient. They should be used to answer a specific clinical question. 3 Imaging techniques (echocardiography first) should be used for screening individuals with risk factors for non-familial DCM and for early diagnosis of first-degree relatives in familial DCM. 4 Echocardiography is the ‘first step’ imaging technique. It provides information about anatomy, function, and haemodynamics, as well as prognostic information, for the best treatment selection. 5 Cardiac magnetic resonance (CMR) is an important tool to consider (at least once) in every patient with DCM. It is the gold standard for measuring LV-, RV volumes, and ejection fraction. It also provides tissue characterization and may suggest the cause of ventricular dysfunction. 6 Nuclear imaging is not used in the routine assessment of every DCM. It is the reference standard for the non-invasive evaluation of myocardial adrenergic tone. 7 Cardiac-computed tomography (CT) is highly valuable to exclude significant epicardial coronary artery disease. Additionally, the good spatial resolution and ease of navigation make cardiac-CT suitable when device implantation is proposed (e.g. transcatheter prosthesis, ventricular assist device, or left ventricular pacing lead). 8 Left ventricular (LV) longitudinal dysfunction is a sensitive marker of subclinical, early myocardial dysfunction, usually assessed with the measurement of long-axis myocardial velocities, and by longitudinal deformation. The measurement of s’ and the use of global longitudinal strain are recommended. 9 In DCM patients at risk for ventricular arrhythmias, though the level of evidence remains insufficient, there are strong elements encouraging the use of speckle tracking echocardiography, CMR, or MIBG-SPECT imaging for best assessing. 10 When cardiac resynchronization therapy (CRT) is a therapeutic option, early systolic septal shortening with inward motion (septal bounce and septal flash) followed by late systolic stretch of the septum, and an apex motion towards the late contracting lateral wall (apical rocking) are considered strong predictors of CRT-response. New semi-automatic approaches based on the use of regional longitudinal strain curves are highly promising. 11 The quantification of right ventricular (RV) function is mandatory as well as the assessment of diastolic function and valvular function during the follow-up of a DCM-patient. Imaging of DCM should not be limited to the LV size and function. 12 For LVADs carriers: echocardiographic (and sometimes haemodynamic) testing provides an objective means of optimizing the medical management and the LVAD pump speed. 13 Secondary mitral regurgitation (MR) is a key prognostic marker in DCM. It should be quantified carefully and systematically integrated with the other haemodynamic data and with the adequation between the degree of regurgitation and the degree of LV enlargement. Key points 1 Dilated cardiomyopathy (DCM) is defined by the presence of left ventricular (LV) or biventricular dilatation and systolic dysfunction in the absence of abnormal loading conditions (hypertension and valve disease) or coronary artery disease sufficient to cause global systolic impairment. 2 All the imaging techniques should not be performed and repeated in every single DCM-patient. They should be used to answer a specific clinical question. 3 Imaging techniques (echocardiography first) should be used for screening individuals with risk factors for non-familial DCM and for early diagnosis of first-degree relatives in familial DCM. 4 Echocardiography is the ‘first step’ imaging technique. It provides information about anatomy, function, and haemodynamics, as well as prognostic information, for the best treatment selection. 5 Cardiac magnetic resonance (CMR) is an important tool to consider (at least once) in every patient with DCM. It is the gold standard for measuring LV-, RV volumes, and ejection fraction. It also provides tissue characterization and may suggest the cause of ventricular dysfunction. 6 Nuclear imaging is not used in the routine assessment of every DCM. It is the reference standard for the non-invasive evaluation of myocardial adrenergic tone. 7 Cardiac-computed tomography (CT) is highly valuable to exclude significant epicardial coronary artery disease. Additionally, the good spatial resolution and ease of navigation make cardiac-CT suitable when device implantation is proposed (e.g. transcatheter prosthesis, ventricular assist device, or left ventricular pacing lead). 8 Left ventricular (LV) longitudinal dysfunction is a sensitive marker of subclinical, early myocardial dysfunction, usually assessed with the measurement of long-axis myocardial velocities, and by longitudinal deformation. The measurement of s’ and the use of global longitudinal strain are recommended. 9 In DCM patients at risk for ventricular arrhythmias, though the level of evidence remains insufficient, there are strong elements encouraging the use of speckle tracking echocardiography, CMR, or MIBG-SPECT imaging for best assessing. 10 When cardiac resynchronization therapy (CRT) is a therapeutic option, early systolic septal shortening with inward motion (septal bounce and septal flash) followed by late systolic stretch of the septum, and an apex motion towards the late contracting lateral wall (apical rocking) are considered strong predictors of CRT-response. New semi-automatic approaches based on the use of regional longitudinal strain curves are highly promising. 11 The quantification of right ventricular (RV) function is mandatory as well as the assessment of diastolic function and valvular function during the follow-up of a DCM-patient. Imaging of DCM should not be limited to the LV size and function. 12 For LVADs carriers: echocardiographic (and sometimes haemodynamic) testing provides an objective means of optimizing the medical management and the LVAD pump speed. 13 Secondary mitral regurgitation (MR) is a key prognostic marker in DCM. It should be quantified carefully and systematically integrated with the other haemodynamic data and with the adequation between the degree of regurgitation and the degree of LV enlargement. Open in new tab Table 1 Key points of the position paper based on scientific background and experts’ consensus Key points 1 Dilated cardiomyopathy (DCM) is defined by the presence of left ventricular (LV) or biventricular dilatation and systolic dysfunction in the absence of abnormal loading conditions (hypertension and valve disease) or coronary artery disease sufficient to cause global systolic impairment. 2 All the imaging techniques should not be performed and repeated in every single DCM-patient. They should be used to answer a specific clinical question. 3 Imaging techniques (echocardiography first) should be used for screening individuals with risk factors for non-familial DCM and for early diagnosis of first-degree relatives in familial DCM. 4 Echocardiography is the ‘first step’ imaging technique. It provides information about anatomy, function, and haemodynamics, as well as prognostic information, for the best treatment selection. 5 Cardiac magnetic resonance (CMR) is an important tool to consider (at least once) in every patient with DCM. It is the gold standard for measuring LV-, RV volumes, and ejection fraction. It also provides tissue characterization and may suggest the cause of ventricular dysfunction. 6 Nuclear imaging is not used in the routine assessment of every DCM. It is the reference standard for the non-invasive evaluation of myocardial adrenergic tone. 7 Cardiac-computed tomography (CT) is highly valuable to exclude significant epicardial coronary artery disease. Additionally, the good spatial resolution and ease of navigation make cardiac-CT suitable when device implantation is proposed (e.g. transcatheter prosthesis, ventricular assist device, or left ventricular pacing lead). 8 Left ventricular (LV) longitudinal dysfunction is a sensitive marker of subclinical, early myocardial dysfunction, usually assessed with the measurement of long-axis myocardial velocities, and by longitudinal deformation. The measurement of s’ and the use of global longitudinal strain are recommended. 9 In DCM patients at risk for ventricular arrhythmias, though the level of evidence remains insufficient, there are strong elements encouraging the use of speckle tracking echocardiography, CMR, or MIBG-SPECT imaging for best assessing. 10 When cardiac resynchronization therapy (CRT) is a therapeutic option, early systolic septal shortening with inward motion (septal bounce and septal flash) followed by late systolic stretch of the septum, and an apex motion towards the late contracting lateral wall (apical rocking) are considered strong predictors of CRT-response. New semi-automatic approaches based on the use of regional longitudinal strain curves are highly promising. 11 The quantification of right ventricular (RV) function is mandatory as well as the assessment of diastolic function and valvular function during the follow-up of a DCM-patient. Imaging of DCM should not be limited to the LV size and function. 12 For LVADs carriers: echocardiographic (and sometimes haemodynamic) testing provides an objective means of optimizing the medical management and the LVAD pump speed. 13 Secondary mitral regurgitation (MR) is a key prognostic marker in DCM. It should be quantified carefully and systematically integrated with the other haemodynamic data and with the adequation between the degree of regurgitation and the degree of LV enlargement. Key points 1 Dilated cardiomyopathy (DCM) is defined by the presence of left ventricular (LV) or biventricular dilatation and systolic dysfunction in the absence of abnormal loading conditions (hypertension and valve disease) or coronary artery disease sufficient to cause global systolic impairment. 2 All the imaging techniques should not be performed and repeated in every single DCM-patient. They should be used to answer a specific clinical question. 3 Imaging techniques (echocardiography first) should be used for screening individuals with risk factors for non-familial DCM and for early diagnosis of first-degree relatives in familial DCM. 4 Echocardiography is the ‘first step’ imaging technique. It provides information about anatomy, function, and haemodynamics, as well as prognostic information, for the best treatment selection. 5 Cardiac magnetic resonance (CMR) is an important tool to consider (at least once) in every patient with DCM. It is the gold standard for measuring LV-, RV volumes, and ejection fraction. It also provides tissue characterization and may suggest the cause of ventricular dysfunction. 6 Nuclear imaging is not used in the routine assessment of every DCM. It is the reference standard for the non-invasive evaluation of myocardial adrenergic tone. 7 Cardiac-computed tomography (CT) is highly valuable to exclude significant epicardial coronary artery disease. Additionally, the good spatial resolution and ease of navigation make cardiac-CT suitable when device implantation is proposed (e.g. transcatheter prosthesis, ventricular assist device, or left ventricular pacing lead). 8 Left ventricular (LV) longitudinal dysfunction is a sensitive marker of subclinical, early myocardial dysfunction, usually assessed with the measurement of long-axis myocardial velocities, and by longitudinal deformation. The measurement of s’ and the use of global longitudinal strain are recommended. 9 In DCM patients at risk for ventricular arrhythmias, though the level of evidence remains insufficient, there are strong elements encouraging the use of speckle tracking echocardiography, CMR, or MIBG-SPECT imaging for best assessing. 10 When cardiac resynchronization therapy (CRT) is a therapeutic option, early systolic septal shortening with inward motion (septal bounce and septal flash) followed by late systolic stretch of the septum, and an apex motion towards the late contracting lateral wall (apical rocking) are considered strong predictors of CRT-response. New semi-automatic approaches based on the use of regional longitudinal strain curves are highly promising. 11 The quantification of right ventricular (RV) function is mandatory as well as the assessment of diastolic function and valvular function during the follow-up of a DCM-patient. Imaging of DCM should not be limited to the LV size and function. 12 For LVADs carriers: echocardiographic (and sometimes haemodynamic) testing provides an objective means of optimizing the medical management and the LVAD pump speed. 13 Secondary mitral regurgitation (MR) is a key prognostic marker in DCM. It should be quantified carefully and systematically integrated with the other haemodynamic data and with the adequation between the degree of regurgitation and the degree of LV enlargement. Open in new tab Table 2 Diagnostic criteria of DCM LV or biventricular systolic dysfunction (defined as LVEF <45%) and dilatationa that are not explained by abnormal loading conditions or coronary artery disease. Left ventricular or biventricular global systolic dysfunction (defined as LVEF <45%) without dilatation, not explained by abnormal loading conditions or coronary artery disease. LV or biventricular systolic dysfunction (defined as LVEF <45%) and dilatationa that are not explained by abnormal loading conditions or coronary artery disease. Left ventricular or biventricular global systolic dysfunction (defined as LVEF <45%) without dilatation, not explained by abnormal loading conditions or coronary artery disease. From Pinto et al.1 a LV dilatation is defined by LV end-diastolic (ED) volumes or diameters >2 SD from normal according to normograms (Z scores >2 SD) corrected for body surface area (BSA) and age or BSA and gender. Open in new tab Table 2 Diagnostic criteria of DCM LV or biventricular systolic dysfunction (defined as LVEF <45%) and dilatationa that are not explained by abnormal loading conditions or coronary artery disease. Left ventricular or biventricular global systolic dysfunction (defined as LVEF <45%) without dilatation, not explained by abnormal loading conditions or coronary artery disease. LV or biventricular systolic dysfunction (defined as LVEF <45%) and dilatationa that are not explained by abnormal loading conditions or coronary artery disease. Left ventricular or biventricular global systolic dysfunction (defined as LVEF <45%) without dilatation, not explained by abnormal loading conditions or coronary artery disease. From Pinto et al.1 a LV dilatation is defined by LV end-diastolic (ED) volumes or diameters >2 SD from normal according to normograms (Z scores >2 SD) corrected for body surface area (BSA) and age or BSA and gender. Open in new tab DCM has an estimated prevalence of one case in 2500 individuals, is a major cause of heart failure (HF) with reduced ejection fraction (EF) and is the leading indication for heart transplantation worldwide.1–3 This heterogeneous disease encompasses a broad range of underlying causes, including genetic and acquired disorders (Table 3) that have been revisited within recent years with a growing proportion of familial/genetic causes (about one-third and up to half of cases) and increasing identification of inflammatory cardiomyopathy that may be related to concealed myocarditis or unrecognized autoimmune diseases.1,2,6 Table 3 Main causes of a DCM Causes Sub-type of causes Genetic causes Main genes, such as titin, are related to predominant cardiac expression Neuromuscular disorders Syndromic diseases1 Infectious causes (chronic myocarditis) Viral, bacterial, fungal, and parasitic causes Toxic and overload Such as ethanol, cocaine, and iron overload Electrolyte disturbance Such as hypocalcaemia Endocrinology causes Such as dysthyroidism and acromegaly Nutritional deficiency Such as selenium, thiamine, and carnitine deficiencies Autoimmune diseases Organ-specific (such as inflammatory cardiomyopathy) or not (such as polymyositis) Drugs induced Such as antineoplastic and psychiatric drugs Tachycardia-induced cardiomyopathy4 Peripartum cardiomyopathy5 Causes Sub-type of causes Genetic causes Main genes, such as titin, are related to predominant cardiac expression Neuromuscular disorders Syndromic diseases1 Infectious causes (chronic myocarditis) Viral, bacterial, fungal, and parasitic causes Toxic and overload Such as ethanol, cocaine, and iron overload Electrolyte disturbance Such as hypocalcaemia Endocrinology causes Such as dysthyroidism and acromegaly Nutritional deficiency Such as selenium, thiamine, and carnitine deficiencies Autoimmune diseases Organ-specific (such as inflammatory cardiomyopathy) or not (such as polymyositis) Drugs induced Such as antineoplastic and psychiatric drugs Tachycardia-induced cardiomyopathy4 Peripartum cardiomyopathy5 Open in new tab Table 3 Main causes of a DCM Causes Sub-type of causes Genetic causes Main genes, such as titin, are related to predominant cardiac expression Neuromuscular disorders Syndromic diseases1 Infectious causes (chronic myocarditis) Viral, bacterial, fungal, and parasitic causes Toxic and overload Such as ethanol, cocaine, and iron overload Electrolyte disturbance Such as hypocalcaemia Endocrinology causes Such as dysthyroidism and acromegaly Nutritional deficiency Such as selenium, thiamine, and carnitine deficiencies Autoimmune diseases Organ-specific (such as inflammatory cardiomyopathy) or not (such as polymyositis) Drugs induced Such as antineoplastic and psychiatric drugs Tachycardia-induced cardiomyopathy4 Peripartum cardiomyopathy5 Causes Sub-type of causes Genetic causes Main genes, such as titin, are related to predominant cardiac expression Neuromuscular disorders Syndromic diseases1 Infectious causes (chronic myocarditis) Viral, bacterial, fungal, and parasitic causes Toxic and overload Such as ethanol, cocaine, and iron overload Electrolyte disturbance Such as hypocalcaemia Endocrinology causes Such as dysthyroidism and acromegaly Nutritional deficiency Such as selenium, thiamine, and carnitine deficiencies Autoimmune diseases Organ-specific (such as inflammatory cardiomyopathy) or not (such as polymyositis) Drugs induced Such as antineoplastic and psychiatric drugs Tachycardia-induced cardiomyopathy4 Peripartum cardiomyopathy5 Open in new tab The appropriate recognition of DCM is of paramount importance. First, the correct identification of the cause through a dedicated diagnostic workup will lead to an aetiology-oriented approach to therapy, which was illustrated and detailed in a recent Consensus document from the ESC Working Group on Myocardial & Pericardial diseases.1 Second, over recent decades, research has shed new light on the natural history of DCM, and it is recognized that many patients have a long preclinical phase characterized by few (if any) symptoms and minor cardiac abnormalities that fall outside current disease definitions.1 The clinical spectrum of cardiac expression in DCM is described in Figure 1. Genes have been identified. But there are many forms of DCM that are isolated/sporadic cases and ‘idiopathic’. In some relatives, there is a preclinical phase without cardiac expression that subsequently progresses towards mild cardiac abnormalities, such as isolated LV dilatation (present in ∼25% of relatives of familial DCM) or arrhythmogenic features (ventricular or supraventricular arrhythmia or conduction defects) that can be observed in myocarditis or in the early phase of genetic diseases, such as Lamin A/C mutation DCM and neuromuscular disorders. The overt phase of systolic dysfunction is usually associated with LV dilatation though in some cases it may be absent, leading to diagnostic confusion. For this reason, a new category of hypokinetic non-DCM was recently proposed (Table 2) as well as a scoring system for characterization of clinical status in the early stage.1 Imaging methods for diagnosing a DCM and for excluding ischaemic aetiology Symptoms of HF are the most common presenting clinical manifestations. Atrial or ventricular arrhythmias or even sudden death can occur at any stage of the disease but are more common in advanced disease. Imaging plays a key role in these patients. Imaging techniques should be used for the diagnosis and for excluding ischaemic aetiology. A comprehensive echocardiography is mandatory. A ‘Focused cardiac ultrasound (FoCUS) exam’ (eventually using handheld ultrasound device) can only raise the suspicion of DCM and should always be complemented by a complete echocardiographic examination, integrating strain measurements, and—increasingly—3D imaging. Only comprehensive echocardiography provides all relevant information on haemodynamics, global ventricular anatomy and function, regional function, dyssynchrony, valvular heart disease, right heart function, atrial characteristics, and geometry (remodelling) that should be obtained.7–9 Contrast agents could be considered to exclude a mural thrombus or evoking a non-compaction DCM for instance. Transoesophageal echocardiography may be considered for assessing valvular function, presence of atrial thrombi and for guiding transcatheter therapy in patients with concomitant valvular heart disease (mostly secondary mitral and tricuspid regurgitation). Stress echocardiography might also be used for dynamicity of secondary valvular disease in addition to the important goal of exploring the potential ischaemic aetiology. Excluding ischaemic aetiology is fundamental, but other conditions have to be listed: a tachycardiomyopathy should be also diagnosed by repeating the comprehensive echocardiography after correction of a rapid tachyarrhythmia. In pregnant women, peripartum cardiomyopathy and screening for cardiomyopathy should be proposed when a heart dysfunction has been reported during a previous pregnancy. In patients treated for cancer, treatments might induce a DCM but can also facilitate the expression of a DCM in patients at risk. Myocarditis or iron overload are potentially reversible causes of DCM. Toxic like alcohol should not be forgotten. To exclude coronary artery disease, one of the three modalities listed below may be required: Cardiac computed tomography (CT) is highly valuable for excluding significant epicardial coronary artery disease. Additionally, its spatial resolution and ease of navigation make cardiac CT suitable when device implantation is proposed (e.g. prosthesis, mechanical assist device, or LV pacing lead). In patients with atrial fibrillation, cardiac CT has high accuracy for excluding left atrial (LA) thrombus and guiding ablation procedures using electroanatomical mapping of the left atrium. Perfusion could be evaluated but also fractional flow reserve via CT has demonstrated a substantial improvement in the identification of haemodynamically significant coronary artery disease.10 Radionuclide imaging techniques allow non-invasive assessment of myocardial perfusion and metabolism and even cardiac innervation through injection of radio-labelled targeted imaging compounds. Myocardial perfusion techniques are clinically relevant especially for distinguishing DCM from ischaemic cardiomyopathy. Cardiovascular magnetic resonance (CMR) is clinically relevant. CMR could be used for excluding the ischaemic component of LV dysfunctions.11 Its main value is on the myocardial tissue characterization. It detects the presence and extent of myocardial oedema, scarring, fibrosis, and infiltration (as well as an iron overload) in the dysfunctional myocardium. This additional unique non-invasive information can aid the identification of the final underlying diagnosis and provide prognostic value. Specific issues—clinical scenarios De novo diagnosis of unrecognized ventricular dysfunction/HF The early detection of DCM can be done in still asymptomatic patients. It has to be based on risk factors (importance of the family tree and of the family history, uncontrolled cardiovascular risk factors like diabetes could be considered as well). The disease often has a long asymptomatic phase, with normal left ventricular ejection fraction (LVEF) and or, sometimes dilated LV cavity dimensions.1 The subclinical phase of early myocardial dysfunction may, however, be identified with advanced imaging techniques.12 The importance of the detection of subclinical disease [by careful analysis of LV size, diastolic function, and global longitudinal strain (GLS)] is important as it allows the institution of early preventive and therapeutic measures, such as lifestyle changes or medical treatments. It may alter the course of the disease2,12–14; and it may result in a substantial reduction of morbidity and mortality.7 Early phenotypes Decreased LVEF is a late and insensitive finding in the natural history of DCM, often reflecting irreversible myocardial dysfunction. Considering echocardiography, tissue Doppler imaging with the measurement of the positive peak mid-systolic velocity (averaging septal and lateral side of mitral annulus; normal value 8.9 + 1.6 cm15) can be considered as a clinically relevant early marker of LV longitudinal dysfunction.12,15,16 Additionally, GLS by 2D speckle tracking echocardiography is the most commonly studied parameter for detecting preclinical disease and is highly reproducible when performed by trained operators.8,17–19 The current recommendation is to use the same vendor for serial surveillance. Inter-vendor variability has improved after the work performed by the standardization Task Force initiated by EACVI and American Society of Echocardiography.20,21 Abnormal circumferential and radial deformation parameters, as well as abnormal torsion, have also been described in preclinical DCM patients.22 Nevertheless, major limitations are the lack of reliable cut-off values and the lack of large studies. If these more advanced echocardiographic techniques are not available for preclinical screening,3,6 echocardiography is limited in only performing LVEF measurements. Quality of the acquisitions of the apical views should be optimized. The apex foreshortening should be carefully avoided. The relatively high variability of manually traced 2D LVEF (biplane Simpson’s method), the concomitant use of LV cavity opacification or the use of automated 2D EF or 3D EF has to be considered for more reliable and reproducible assessments of small changes in LV volumes and function.8 More recent data are also encouraging the use of 3D transthoracic echocardiographic (TTE) for the right ventricular (RV) function and volumes.23 CMR may impact preclinical diagnosis, as it is golden standard for LV and RV quantification. CMR should be considered in the case of suboptimal, borderline or doubtful echocardiographic data, and in high-risk families when the diagnosis of DCM is still in doubt and would have direct implications on management.24 Despite its relatively low availability and high cost, CMR may be used in the assessment of myocardial longitudinal strain and helps in early diagnosis of specific aetiologies (sarcoidosis and post-myocarditis DCM).25 The tissue characterization [early gadolinium enhancement, T2- and T1-weighted sequences or mapping, and late gadolinium enhancement (LGE)] are a key clinical feature of CMR.26 The clinical value of CMR in the early detection of the disease must be further explored in larger trials. Cardiac CT: Despite its excellent spatial resolution, the role of cardiac CT for early diagnosis of DCM is limited due to its lower temporal resolution, radiation and the need for iodinated contrast. It can be useful when echocardiographic images are suboptimal (and CMR contraindicated) and concomitant coronary artery or pericardial disease have to be excluded.13,27 Cardiac CT can make the diagnosis by demonstrating dilatation of left and right ventricles, pulmonary oedema, dilatation of pulmonary arteries, and absence of coronary artery disease. Gated radionuclide imaging studies provide an accurate alternative to echocardiography or CMR to assess LV systolic function and regional contractility. Radionuclide ventriculography can be used to assess LV systolic (and diastolic) function without any geometrical assumptions of the LV. Due to its low intraobserver variability, this technique has been used but it is no more recommended.28,29 RV systolic function can be assessed with radionuclide ventriculography (particularly using first-pass or equilibrium gated blood pool techniques). It requires an expertise. Diagnostic criteria for relatives of familial DCM DCM is idiopathic in 50% of cases, about one-third of which are hereditary. There are already more than 50 genes identified that are associated with DCM, many related to the cytoskeleton. The most frequent ones are titin, lamin, and desmin. The ESC working group on myocardial and pericardial diseases recently proposed diagnostic criteria for relatives of familial DCM patients,1 integrating at least imaging methods and 12-lead electrocardiogram (ECG) (Table 4). Table 4 Diagnostic criteria for relatives of familial DCM1 Major 1. Unexplained decrease of LVEF ≤50% but >45% OR 2. Unexplained LVED dilatation (diameter or volume) according to nomograms (LVED diameter/volume 2 SD + 5% since this more specific echocardiographic criterion was used in studies that demonstrated the predictive impact of isolated dilatation in relatives). Minor 1. Complete LBBB or AV block (PR ≥200 ms or higher degree of AV block). 2. Unexplained ventricular arrhythmia (100 ventricular premature beats per hour in 24 h or non-sustained ventricular tachycardia, ≥3 beats at a rate of ≥120 bpm). 3. Segmental wall motion abnormalities in the left ventricle in the absence of intraventricular conduction defect. 4. Late enhancement (LGE) of non-ischaemic origin on cardiac magnetic resonance imaging. 5. Evidence of non-ischaemic myocardial abnormalities (inflammation, necrosis, and/or fibrosis) on EMB. 6. Presence of serum organ-specific and disease-specific AHA by one or more autoantibody tests. Major 1. Unexplained decrease of LVEF ≤50% but >45% OR 2. Unexplained LVED dilatation (diameter or volume) according to nomograms (LVED diameter/volume 2 SD + 5% since this more specific echocardiographic criterion was used in studies that demonstrated the predictive impact of isolated dilatation in relatives). Minor 1. Complete LBBB or AV block (PR ≥200 ms or higher degree of AV block). 2. Unexplained ventricular arrhythmia (100 ventricular premature beats per hour in 24 h or non-sustained ventricular tachycardia, ≥3 beats at a rate of ≥120 bpm). 3. Segmental wall motion abnormalities in the left ventricle in the absence of intraventricular conduction defect. 4. Late enhancement (LGE) of non-ischaemic origin on cardiac magnetic resonance imaging. 5. Evidence of non-ischaemic myocardial abnormalities (inflammation, necrosis, and/or fibrosis) on EMB. 6. Presence of serum organ-specific and disease-specific AHA by one or more autoantibody tests. Note: Feature shown by two independent imaging modalities.1 Open in new tab Table 4 Diagnostic criteria for relatives of familial DCM1 Major 1. Unexplained decrease of LVEF ≤50% but >45% OR 2. Unexplained LVED dilatation (diameter or volume) according to nomograms (LVED diameter/volume 2 SD + 5% since this more specific echocardiographic criterion was used in studies that demonstrated the predictive impact of isolated dilatation in relatives). Minor 1. Complete LBBB or AV block (PR ≥200 ms or higher degree of AV block). 2. Unexplained ventricular arrhythmia (100 ventricular premature beats per hour in 24 h or non-sustained ventricular tachycardia, ≥3 beats at a rate of ≥120 bpm). 3. Segmental wall motion abnormalities in the left ventricle in the absence of intraventricular conduction defect. 4. Late enhancement (LGE) of non-ischaemic origin on cardiac magnetic resonance imaging. 5. Evidence of non-ischaemic myocardial abnormalities (inflammation, necrosis, and/or fibrosis) on EMB. 6. Presence of serum organ-specific and disease-specific AHA by one or more autoantibody tests. Major 1. Unexplained decrease of LVEF ≤50% but >45% OR 2. Unexplained LVED dilatation (diameter or volume) according to nomograms (LVED diameter/volume 2 SD + 5% since this more specific echocardiographic criterion was used in studies that demonstrated the predictive impact of isolated dilatation in relatives). Minor 1. Complete LBBB or AV block (PR ≥200 ms or higher degree of AV block). 2. Unexplained ventricular arrhythmia (100 ventricular premature beats per hour in 24 h or non-sustained ventricular tachycardia, ≥3 beats at a rate of ≥120 bpm). 3. Segmental wall motion abnormalities in the left ventricle in the absence of intraventricular conduction defect. 4. Late enhancement (LGE) of non-ischaemic origin on cardiac magnetic resonance imaging. 5. Evidence of non-ischaemic myocardial abnormalities (inflammation, necrosis, and/or fibrosis) on EMB. 6. Presence of serum organ-specific and disease-specific AHA by one or more autoantibody tests. Note: Feature shown by two independent imaging modalities.1 Open in new tab In this proposal, imaging criteria may be major (LVEF and LV dilatation) or minor (abnormal regional wall motion in the absence of conduction defects and non-ischaemic LGE CMR). The measurement of GLS is encouraged, as mentioned in key point 3. Timing of screening A general time frame to perform echocardiography in first-degree relatives of patients with cardiomyopathy, when genetic results are not available, has been proposed.30 More recently, specific recommendations were provided for familial DCM, in which echocardiography and ECG should be performed in all first-degree relatives starting in childhood (∼10 years of age) and repeated every 2–3 years if cardiovascular tests are normal and every year if minor abnormalities are detected.1 When to stop the screening remains an unresolved issue and it might differ according to the family history. The limit of 60–65 years of age has been proposed.30 The screening intervals will also depend on the course of the specific types of DCM. For instance, in cardio-oncology patients, this screening will follow specific recommendations.29,31,32 Prognosis and risk stratification: new parameters that can be used in clinical practice; a pragmatic approach Despite advances in DCM-treatments, 10-year survival remains <60%, with death preceeded by numerous HF exacerbations, reflecting the difficulty in assessing the individual risk. Remarkably, the clinical course of DCM patients varies widely, ranging from rapidly progressive HF or sudden cardiac death (SCD) to LV reverse remodelling (RR), i.e. significant reduction of LV volumes along with sustained recovery of LVEF. Nearly 40% of newly diagnosed DCM patients experience LV RR under optimal medical therapy (OMT) at a median of 2 years of follow-up, foreseeing a favourable long-term outcome.33,34 This evidence questioned the appropriateness of at least 3 months of OMT in newly diagnosed DCM patients with HF before proceeding to device(s) implantation, as proposed by the current guidelines.35 Additionally, the LV ejection-fraction cut-off of ≤35% in symptomatic [New York Heart Association (NYHA) Class II and III] DCM patients for primary prevention implantable cardioverter-defibrillator (ICD) placement (Class I, Level of evidence B)35 is subject of controversies,36 considering its low sensitivity and specificity in identifying high-risk patients as well as the poor cost-effectiveness profile. Prognostic markers LV dilatation and impaired contractile function are major prognosticators (for cardiovascular death and hospitalization) in DCM (whatever the imaging technique used). While dilatation is associated with adverse outcome, RR and normalization of the LV dimensions are associated with improved survival.33,37 RR is a therapeutic objective that may take months/years to reach and is monitored by serial imaging. Other imaging parameters, associated with the risk of death or hospitalization for HF, include LA enlargement, RV dilatation, and RV contractile dysfunction.38,39 The latter may be caused by the intrinsic disease or develop secondary to left HF. LV strain has also been repeatedly demonstrated as a key and independent prognostic marker in DCM.40–42 Recently, RV strain imaging has been suggested as a tool of choice to consider to best define the risk of death and hospitalization in patients with DCM.43 The quantification of RV function and size should be systematically reported in DCM patients.9,44 LV filling pressure and diastolic function should be assessed and reported. The necessary parameters comprise at least LA volume, E/A ratio and E velocity deceleration time, e’, E/e’, maximal velocity of tricuspid regurgitation have to be reported when a DCM-patient is scanned by echocardiography.45 LA strain is a new promising approach tested but still under investigation.46,47 Secondary (functional) MR (Carpentier I + IIIb) is a potentially reversible consequence and aggravator of ventricular remodelling that is incrementally associated with adverse outcome.48 In clinical practice, TTE is used for quantification of secondary MR severity and potential response to therapy.49–51 Stress echocardiography parameters, but also nuclear imaging measurements such as contractile reserve and coronary flow reserve, predict RR, and functional recovery in patients with DCM.52,53 Coronary flow reserve assessment could be assessed also by echocardiography in DCM patients with left bundle branch block.54,55 Also, the presence of microvascular dysfunction (as assessed by positron emission tomography) is associated with poorer outcomes and a higher risk of progression to overt HF and death.56 Specific predictors for ventricular arrhythmias Ventricular arrhythmias are the most feared complications in DCM. Compared to patients with ischaemic cardiomyopathy, the incidence of ventricular arrhythmias in patients with DCM is lower. ICD implantation is the standard of care for prevention of SCD in high-risk patients.57 The identification of high-risk individuals is difficult. Current guidelines recommend ICD for primary prevention, as a Class IB indication in patients with non-ischaemic DCM and LVEF ≤35%, on OMT, and with more than 1-year life expectancy.57 However, adherence to current guidelines has been questioned, and previous trials have not been convincing in the beneficial effect of primary prevention ICD in non-ischaemic patients.58–60 Primary prevention ICD in patients with non-ischaemic DCM was less efficient at preventing total mortality compared to patients with ischaemic heart disease.61,62 A beneficial effect on all-cause mortality has only been shown in one randomized trial including patients with non-ischaemic heart disease (SCD-HeFT), even if a predefined SCD-HeFT subgroup analysis demonstrated that the benefit was significant only for the ischaemic subgroup.63 The most recent study on this topic, the DANISH study, further showed the limited effect of primary prevention ICD on total mortality in patients with non-ischaemic DCM,60 indicating that recommendations for primary prevention ICD in these patients need to be improved. Despite its known limitations, EF still remains the only imaging parameter to guide decisions on primary prevention ICD therapy in non-ischaemic DCM. Echocardiographic parameters have been proposed as risk markers of ventricular tachycardia/VF, which are additive to EF. However, none of these echocardiographic markers have emerged to substantially influence patient care. The most important emerging parameters from echocardiography include GLS64,65 and mechanical dispersion.66 GLS has shown to be a better marker of ventricular arrhythmias in patients with DCM and remains a good predictor in patients with relatively preserved EF.64 Reversed apical rotation and loss of LV torsion are also associated with significant LV remodelling and more impaired LV function, indicating a more advanced disease stage.67 Mechanical dispersion has been suggested as a marker of unfavourable arrhythmic outcome64,66 (Figures 2 and 3). Mechanical dispersion is measured as the standard deviation of time from Q/R on ECG to peak strain by longitudinal strain in a 16 LV segment model. Mechanical dispersion reflects heterogeneous myocardial contraction and might be associated with increased myocardial interstitial fibrosis.68 CMR holds promises in this context by showing that newly diagnosed DCM patients without mid-wall LGE are more likely to experience LV RR than those with LGE, irrespective of the severity of clinical status and of LV dilatation and dysfunction at initial evaluation.34 Moreover, CMR renders available important risk markers at multiple levels in addition to LV functional parameters. As an example, RV systolic dysfunction (ejection-fraction ≤45%), as quantified by CMR, is a powerful and independent adverse predictor of transplant-free survival and other HF outcomes.69 About one-third of DCM patients show mid-wall LGE, reflecting replacement fibrosis, and this has been shown to be a strong and independent predictor of all-cause mortality, cardiovascular death/transplantation, and SCD37,70–73 with incremental prognostic value to LV ejection-fraction.37,70,71 DCM patients with mid-wall LGE had been reported with a four-fold increased risk of SCD or aborted SCD after correction for other confounders, refining the arrhythmic risk estimation with potential important implications for public health and resource utilization (Figure 4).37,71–73 Mid-wall fibrosis has been shown to be an effective prognosticator amongst a wide range of disease severity, including in DCM patients without history of HF (Class B of HF) and in candidates for device(s) treatment.70,71,73–75 Patients with DCM and mid-wall fibrosis receiving cardiac resynchronization therapy (CRT) were less likely to exhibit LV RR and had worse clinical outcomes compared to non-LGE patients, and these outcomes were similar to those of ischaemic cardiomyopathy patients.74 These data are in line with a meta-analysis on nine studies, including nearly 1500 patients with DCM, which reported that LGE has an excellent prognostic value for all-cause mortality, HF hospitalization, and SCD.76 Several studies have proposed diverse cut-off values for fibrosis extent for predicting clinical outcomes, but currently, there is no consensus about which cut-off can effectively stratify DCM patients.71,72 Nonetheless, mid-wall fibrosis retained its prognostic value when considered as a continuous variable, supporting the concept that the extent, the location and not only the presence of fibrosis may be a prognostic marker.37,77,78 Figure 2 Open in new tabDownload slide Example of a DCM with a typical pattern of mechanical dyssynchrony: too early contraction of the septum before the aortic valve opening and lengthening of the anterolateral wall leading to a delayed shortening of this wall. (Longitudinal strain imaging—take care at the timing and at the colour according to each left ventricular wall). Figure 2 Open in new tabDownload slide Example of a DCM with a typical pattern of mechanical dyssynchrony: too early contraction of the septum before the aortic valve opening and lengthening of the anterolateral wall leading to a delayed shortening of this wall. (Longitudinal strain imaging—take care at the timing and at the colour according to each left ventricular wall). Figure 3 Open in new tabDownload slide Mechanical dispersion: the longitudinal peaks of longitudinal deformation are not reaching their peak at the same period of time in patients with DCM at increased risk of ventricular arrhythmias. Figure 3 Open in new tabDownload slide Mechanical dispersion: the longitudinal peaks of longitudinal deformation are not reaching their peak at the same period of time in patients with DCM at increased risk of ventricular arrhythmias. Figure 4 Open in new tabDownload slide A 62-year-old woman with idiopathic cardiomyopathy and a history of ventricular arrhythmias presenting recurrent episodes of ventricular tachycardia. (A) The scintigraphic perfusion images show homogeneous perfusion in the whole left ventricle, with the exception of a minimum reduction of perfusion in the proximal portion of the inferior wall (SRS 1, not significant). The innervation images (lower rows) reveal an extensive area of denervation involving the lateral and inferior walls (SS-MIBG 17) with a clear innervation/perfusion mismatch. (B) At EP study located the sites of origin of the arrhythmia at the level of the inferior and inferolateral LV walls. Figure 4 Open in new tabDownload slide A 62-year-old woman with idiopathic cardiomyopathy and a history of ventricular arrhythmias presenting recurrent episodes of ventricular tachycardia. (A) The scintigraphic perfusion images show homogeneous perfusion in the whole left ventricle, with the exception of a minimum reduction of perfusion in the proximal portion of the inferior wall (SRS 1, not significant). The innervation images (lower rows) reveal an extensive area of denervation involving the lateral and inferior walls (SS-MIBG 17) with a clear innervation/perfusion mismatch. (B) At EP study located the sites of origin of the arrhythmia at the level of the inferior and inferolateral LV walls. Parametric mapping sequences have been applied in DCM cohorts to quantify myocardial native T1 and T2 relaxation times as well as extracellular volume fraction (ECV). The results from different studies using different T1 mapping sequences at diverse magnetic fields were concordant in their reporting of higher native T1 and ECV values in DCM patients compared to controls.79,80 In DCM patients, myocardial ECV reflects histology-verified collagen content and may serve as a potential non-invasive marker of diffuse interstitial fibrosis and for monitoring the response to anti-remodelling treatments.81 Recently, a higher native T1 value of myocardium was demonstrated as an independent predictor of all-cause mortality and HF events in a cohort of 637 patients with DCM.80 Despite the adoption of parametric imaging as a promising tool in DCM patients and potentially providing diagnostic as well prognostic information in addition to LGE, multicentre, multivendor, multi-sequence studies in large cohorts of normal subjects, and DCM patients are still warranted. Cardiac radionuclide imaging techniques Single photon emission computed tomography (SPECT): DCM is among the major predisposing factor for ventricular arrhythmias, whose genesis relies on the combined presence of a triggering mechanism that initiates the arrhythmia and of an anatomic substrate that maintains the arrhythmia once it is initiated (i.e. islands of scar tissue after myocarditis). One of the most relevant factors that may trigger ventricular arrhythmias is represented by an abnormality of cardiac sympathetic tone. Preliminary data indicated that impairment of cardiac adrenergic innervation may represent a relevant marker of adverse prognosis, particularly predisposing to the development of malignant ventricular arrhythmias.82 Nuclear imaging might offer the chance to shed light on cardiac sympathetic tone through the use of a dedicated nervous radiotracer [123I-metaiodobenzyl-guanidine (123I-MIBG)] (Figure 5). From planar images, 123I-MIBG uptake is semi-quantitatively assessed by calculating the heart-to-mediastinum (H/M) ratio and the washout rate, which estimates cardiac global adrenergic receptor density and has been associated with adverse prognosis.83 However, despite their excellent reproducibility, those planar scintigraphic measures are unable to unmask regional alterations of cardiac adrenergic tone, whose presence has been shown to be associated with different cardiac pathologies, independently predicting patient outcomes. Some studies have suggested that a regional 123I-MIBG defect score, derived from SPECT images, may be superior to the H/M ratio in predicting patients’ adverse prognosis, highlighting the independent detrimental effect of regional adrenergic innervation heterogeneity.84 Figure 5 Open in new tabDownload slide About one-third of DCM patients show mid-wall late gadolinium enhancement (arrow—LGE), reflecting replacement fibrosis, and this has been shown to be a strong and independent predictor of all-cause mortality, CV death/transplantation, and sudden cardiac death (see the text). MRI cine (A and B); LGE images (C). *Pericardium. Figure 5 Open in new tabDownload slide About one-third of DCM patients show mid-wall late gadolinium enhancement (arrow—LGE), reflecting replacement fibrosis, and this has been shown to be a strong and independent predictor of all-cause mortality, CV death/transplantation, and sudden cardiac death (see the text). MRI cine (A and B); LGE images (C). *Pericardium. The use of new solid-state cardiac cameras with cadmium–zinc–telluride detectors, characterized by higher photon sensitivity and spatial resolution than standard cameras allow a comprehensive assessment of myocardial innervation and perfusion in a single imaging session and with a limited radiation burden.85,86 However, more data are needed in order to use 123I-MIBG in clinical routine. Positron emission tomography (PET) remains the reference standard for the non-invasive evaluation of myocardial adrenergic tone, allowing the absolute quantification of sympathetic nerve terminal activity.87 The versatility of PET radiotracers allows performance of a combined investigation of both pre-synaptic and post-synaptic receptor density. Accordingly, the positron tracers [11C]hydroxyephedrine and [11C]epinephrine permit quantification of the density of sympathetic nerve terminals,87 while post-synaptic receptor density can be assessed with [11C]CGP12177, which has been shown to independently predict patients’ adverse prognosis, particularly related to the incidence of symptomatic HF. Specificity for familial DCM A particular subset of patients with familial non-ischaemic DCM has a genetic aetiology, especially patients with Lamin A/C (LMNA) mutations. These patients with LMNA mutations typically have early onset of atrioventricular (AV) block, supraventricular and ventricular arrhythmias, and progressive DCM. SCD due to ventricular arrhythmias is frequent and often occurs before the development of DCM.88–90 Compared to patients with DCM of another aetiology, risk stratification of ventricular arrhythmias in these patients requires a different approach since these patients have a significantly higher risk of SCD. Reduced EF is a late symptom and cannot be used as the decision tool for ICD. Conduction block, male gender, septal LGE, non-sustained ventricular tachycardia, reduced functional capacity, genotype, and previous competitive sports are suggested as risk markers, and ICD implantation for primary prevention in LMNA patients1 should be considered quite early.88,90,91 Additional imaging markers from echocardiography in these patients include septal strain and mechanical dispersion.92 The role of cardiac imaging in the decision of HF interventions CRT/Left ventricular assistance devices Resynchronization therapy Global LV function assessment LVEF below 35% is a prerequisite for CRT according to current guidelines.25 Although GLS has emerged as a sensitive and robust measure of global LV function, there is currently no sufficient evidence for recommending a certain cut-off value for this parameter for patient selection. No randomized study with a control group has demonstrated that GLS-based implantation of a CRT-device change the outcomes. Regional LV functional assessment CRT resynchronizes the contraction of the cardiac walls, which improves cardiac performance and induces RR.93 Consequently, the assessment of mechanical dyssynchrony has been proposed as selection criteria in CRT candidates. Unlike nonspecific parameters, which showed no added predictive value over ECG criteria,94,95 parameters reflecting the typical deformation patterns amendable to CRT can accurately identify responders to CRT.96–98 In particular, early systolic septal shortening with inward motion (septal bounce and septal flash)99,100 followed by late systolic stretch of the septum and an apex motion towards the late contracting lateral wall (apical rocking)96,101–103 are strong predictors of CRT success.96,98 These patterns are visually recognizable.96,100–102 If needed, less experienced readers may benefit from quantitative assessments.104 A low-dose dobutamine challenge can unmask apical rocking and septal flash in a minority of patients where typical dyssynchrony patterns are difficult to recognize.102,105 The modality of choice for the assessment of mechanical dyssynchrony is echocardiography, as it combines the best temporal resolution with the option of quantification by tissue Doppler or speckle tracking techniques.8 CMR and radionuclide imaging techniques may also serve this purpose.106 Unlike echocardiography, SPECT myocardial perfusion imaging provides a single parameter to define mechanical dyssynchrony [phase analysis derived standard deviation (SD)] which is reproducible, repeatable on serial imaging testing, and easy to derive.107 Regional myocardial work can be estimated from echocardiographic pressure strain loops108,109 and has been shown to be related to RR after CRT.110,111 To what extent these methods predict CRT success beyond dyssynchrony assessment remains to be determined with a control group and not on patients that are all implanted according to current guidelines111,112 (Figures 2and6). Figure 6 Open in new tabDownload slide New approach of longitudinal strain (globally and regionally). The strain curves are computed according to the blood pressure and the calculation of the intra-left ventricular pressures (pressure–strain loops). Promising approach for calculating the myocardial work and the potential clinical value for predicting better the response to cardiac resynchronization therapy. AVC, aortic valve closure; AVO, aortic valve opening; MVC, mitral valve closure; MVO, mitral valve opening. Figure 6 Open in new tabDownload slide New approach of longitudinal strain (globally and regionally). The strain curves are computed according to the blood pressure and the calculation of the intra-left ventricular pressures (pressure–strain loops). Promising approach for calculating the myocardial work and the potential clinical value for predicting better the response to cardiac resynchronization therapy. AVC, aortic valve closure; AVO, aortic valve opening; MVC, mitral valve closure; MVO, mitral valve opening. Scar burden reduces the effect of CRT and must be assessed before device implantation. This is much less important in DCM (and much more complicated to quantify) than in ischaemic heart disease. Nevertheless, CMR is the method of choice as it shows interstitial fibrosis (T1 mapping) but also authentic scar tissue in post-myocarditis cardiomyopathies for instance.113,114 The level of evidence and the inter-machine variability justify to abstain from a recommendation to use T1-mapping approaches in daily routine practice at the present time. Upon availability, SPECT or a combined [18]-Fluoro-2-désoxy-D-glucose/ammonia-PET study may also serve to assess myocardial viability prior to CRT implantation. Procedure planning Cardiac CT can visualize the coronary veins non-invasively if pre-procedural planning of LV lead placement is needed.115 Hybrid imaging methods may be used to overlay coronary vein anatomy with myocardial viability from PET and cardiac phase analysis from gated SPECT studies, thereby guide non-invasively the implantation of LV pacing leads. Therapy response and RR AV and VV optimization could be performed to increase the response rate to CRT. AV optimization can be guided during imaging by aiming at a maximal transmitral filling time or stroke volume.116,117 VV optimization may be attempted by means of regional deformation analysis. However, there is limited evidence on the effect on patient outcome.117 Cessation of apical rocking and of septal flash is an immediate marker of successful CRT implantation and predicts RR and survival benefit.96 Echocardiography is the method of choice for all functional assessments following CRT implantation. In addition to clinical improvement and survival benefit, increases in LV function and decreases in LV volume are long-term signs of favourable CRT-response. The latter is frequently accompanied by a normalization of wall thickness, i.e. an increase in septal and decrease in lateral wall thickness. Echocardiography is the ‘first-line method’ to document this so-called ‘reverse remodelling’. Although CMR might have higher accuracy, it is usually not a convenient approach to perform a routine CMR scan in a patient with an implanted electronic device (image quality could be impaired due to the metal artefact of the device).118 However, CMR in patients with pacemakers and ICD both MR-conditional, and more recently also in non-conditional devices, can be performed safely in expert CMR centres.119 An LV end-systolic volume decrease of more than 15% within the first year is a commonly accepted cut-off for successful CRT. It must be assumed, however, that in certain patients, less RR might also be related to survival benefit, while in some patients, the pure stabilization of LV size, i.e. the prevention of further remodelling, might be a therapeutic success.120 Left ventricular assist devices Patient assessment The absence of severe RV and tricuspid valve dysfunction are relevant criteria to determine the eligibility of patients for the implantation of a left ventricular assist device (LVAD).25,121 RV longitudinal strain has demonstrated useful and independently predicts RV failure after LVAD implant.122,123 Echocardiography is the first line method of choice for the initial assessment of cardiac morphology and function of an LVAD candidate (Tables 1 and 4).23,121 RV size should be routinely assessed by conventional 2D echocardiography using multiple acoustic windows, and the report should include both qualitative and quantitative parameters.124 Three-dimensional echocardiography may be used in laboratories with experience and the necessary equipment.8,25 Extra-cardiac anatomic structures, such as the great vessels, may be imaged with CMR or, in case of implanted devices, CT.115 Patient follow-up In addition to the assessment of left and RV morphology and function, the 2D and Doppler examination of the LVAD cannula within the LV is relevant for the functional assessment of the device125–127 (Table 5 and 6). Table 5 LVAD preimplantation echocardiographic workup 1. Left ventricle and interventricular septum  LV size and morphology: should not be too small and with increased LV trabeculation or thrombi.  Make sure that there is no LV apical aneurysm and no ventricular septal defect. 2. Right ventricle  RV dilatation.  RV systolic dysfunction: that is challenging and that should consider the pulmonary pressures (afterload) and all the qualitative and quantitative parameters available (including the subcostal window). 3. Atrial, interatrial septum, and inferior vena cava  Left atrial appendage thrombus, patent foramen ovale (PFO), or atrial septal defect should be looked for. 4. Valvular abnormalities  Any prosthetic valve (mechanical should be avoided).  The degree of aortic regurgitation should be assessed extremely carefully. TOE could be necessary.  All the other valves should not be significantly abnormal or be planned for correction at the time of the LVAD implantation (tricuspid regurgitation especially). 5. Aorta and make sure there is no congenital heart disease.  Aortic aneurysm, dissection, atheroma, coarctation but also mobile mass lesion should be looked for (consider TOE). 1. Left ventricle and interventricular septum  LV size and morphology: should not be too small and with increased LV trabeculation or thrombi.  Make sure that there is no LV apical aneurysm and no ventricular septal defect. 2. Right ventricle  RV dilatation.  RV systolic dysfunction: that is challenging and that should consider the pulmonary pressures (afterload) and all the qualitative and quantitative parameters available (including the subcostal window). 3. Atrial, interatrial septum, and inferior vena cava  Left atrial appendage thrombus, patent foramen ovale (PFO), or atrial septal defect should be looked for. 4. Valvular abnormalities  Any prosthetic valve (mechanical should be avoided).  The degree of aortic regurgitation should be assessed extremely carefully. TOE could be necessary.  All the other valves should not be significantly abnormal or be planned for correction at the time of the LVAD implantation (tricuspid regurgitation especially). 5. Aorta and make sure there is no congenital heart disease.  Aortic aneurysm, dissection, atheroma, coarctation but also mobile mass lesion should be looked for (consider TOE). LV, left ventricular; LVAD, left ventricular assistance device; PFO, patent foramen ovale; RV, right ventricular; TOE, transoesophageal echocardiography. Open in new tab Table 5 LVAD preimplantation echocardiographic workup 1. Left ventricle and interventricular septum  LV size and morphology: should not be too small and with increased LV trabeculation or thrombi.  Make sure that there is no LV apical aneurysm and no ventricular septal defect. 2. Right ventricle  RV dilatation.  RV systolic dysfunction: that is challenging and that should consider the pulmonary pressures (afterload) and all the qualitative and quantitative parameters available (including the subcostal window). 3. Atrial, interatrial septum, and inferior vena cava  Left atrial appendage thrombus, patent foramen ovale (PFO), or atrial septal defect should be looked for. 4. Valvular abnormalities  Any prosthetic valve (mechanical should be avoided).  The degree of aortic regurgitation should be assessed extremely carefully. TOE could be necessary.  All the other valves should not be significantly abnormal or be planned for correction at the time of the LVAD implantation (tricuspid regurgitation especially). 5. Aorta and make sure there is no congenital heart disease.  Aortic aneurysm, dissection, atheroma, coarctation but also mobile mass lesion should be looked for (consider TOE). 1. Left ventricle and interventricular septum  LV size and morphology: should not be too small and with increased LV trabeculation or thrombi.  Make sure that there is no LV apical aneurysm and no ventricular septal defect. 2. Right ventricle  RV dilatation.  RV systolic dysfunction: that is challenging and that should consider the pulmonary pressures (afterload) and all the qualitative and quantitative parameters available (including the subcostal window). 3. Atrial, interatrial septum, and inferior vena cava  Left atrial appendage thrombus, patent foramen ovale (PFO), or atrial septal defect should be looked for. 4. Valvular abnormalities  Any prosthetic valve (mechanical should be avoided).  The degree of aortic regurgitation should be assessed extremely carefully. TOE could be necessary.  All the other valves should not be significantly abnormal or be planned for correction at the time of the LVAD implantation (tricuspid regurgitation especially). 5. Aorta and make sure there is no congenital heart disease.  Aortic aneurysm, dissection, atheroma, coarctation but also mobile mass lesion should be looked for (consider TOE). LV, left ventricular; LVAD, left ventricular assistance device; PFO, patent foramen ovale; RV, right ventricular; TOE, transoesophageal echocardiography. Open in new tab Table 6 Post-LVAD implantation complications One has to be systematic and specialized in the assessment of patients with LVAD (being aware of the device implanted, the patient and the history): 1. Pericardial effusion or haematoma  Cardiac tamponade will lead to RV compression and decrease in RV outflow tract velocity time integral. Check for the pericardium using all the echocardiographic windows (TOE if needed) and assess right heart output. 2. LV failure related to LV overloading  Important of serial exam comparison  a. 2D/3D: increasing LV size; increased AV opening duration, increased left atrial volume.  b. Doppler: increased mitral inflow peak E-wave diastolic velocity, increased E/A and E/e’ ratio, decreased deceleration time of mitral E velocity, worsening functional MR, and elevated pulmonary artery systolic pressure. 3. RV failure  a. 2D: increased RV size, decreased RV systolic function, high RAP (dilated IVC/leftward atrial septal shift), and leftward deviation of ventricular septum.  b. Doppler: increased TR severity, reduced RVOT SV, reduced LVAD inflow cannula, and/or outflow-graft velocities (<0.5 m/s with severe failure); inflow-cannula high velocities if associated with a suction event.  Of Note: a ‘too-high’ LVAD pump speed may contribute to RV failure by increasing TR (septal shift) and/or by increasing RV preload. 4. Inadequate LV filling or excessive LV unloading  Small LV dimensions (typically <3 cm and/or marked deviation of interventricular septum towards LV). Danger to not misinterpret an RV failure and/or pump speed too high for loading conditions. 5. LVAD suction with induced ventricular ectopy  Underfilled LV and mechanical impact of inflow cannula with LV endocardium, typically septum, and resolves with speed turndown. 6. LVAD-related continuous aortic insufficiency  Clinically significant—at least moderate and possibly severe—characterized by an AR vena contracta >3 mm; increased LV size and relatively decreased RVOT SV despite normal/increased inflow cannula and/or outflow-graft flows. 7. LVAD-related mitral regurgitation  a. Primary: inflow cannula interference with mitral apparatus.  b. Secondary: MR-functional, related to partial LV unloading/persistent heart failure. 8. Intracardiac thrombus  Including right and left atrial, LV apical, and aortic root thrombus 9. Inflow-cannula abnormality  a. 2D/3D: small or crowded inflow zone with or without evidence of localized obstructive muscle trabeculation, adjacent MV apparatus or thrombus; mispositioned inflow cannula.  b. High-velocity colour or spectral Doppler at inflow orifice. Results from malposition, suction event/other inflow obstruction: aliased colour-flow Doppler, and CW Doppler velocity >1.5 m/s.  c. Low-velocity inflow (markedly reduced peak systolic and nadir diastolic velocities) may indicate internal inflow-cannula thrombosis or more distal obstruction within the system. Doppler flow velocity profile may appear relatively ‘continuous’ (decreased phasic/pulsatile pattern). 10. Outflow-graft abnormality  Typically, due to obstruction/pump cessation.  a. 2D imaging (TOE): visible kink or thrombus.  b. Doppler: peak outflow-graft velocity >2 m/s at the obstruction site; but diminished or no spectral Doppler signal if sample volume is remote from obstruction location, combined with lack of RVOT SV change and/or expected LV dimension change with pump-speed changes. 11. Pump malfunction/pump arrest  a. Reduced inflow-cannula or outflow-graft flow velocities on colour and spectral Doppler or, with pump arrest, show diastolic flow reversal.  b. Signs of worsening HF: including dilated LV, worsening MR, worsened TR, and/or increased TR velocity; attenuated speed-change responses: decrease or absence of expected changes in LV linear dimension. One has to be systematic and specialized in the assessment of patients with LVAD (being aware of the device implanted, the patient and the history): 1. Pericardial effusion or haematoma  Cardiac tamponade will lead to RV compression and decrease in RV outflow tract velocity time integral. Check for the pericardium using all the echocardiographic windows (TOE if needed) and assess right heart output. 2. LV failure related to LV overloading  Important of serial exam comparison  a. 2D/3D: increasing LV size; increased AV opening duration, increased left atrial volume.  b. Doppler: increased mitral inflow peak E-wave diastolic velocity, increased E/A and E/e’ ratio, decreased deceleration time of mitral E velocity, worsening functional MR, and elevated pulmonary artery systolic pressure. 3. RV failure  a. 2D: increased RV size, decreased RV systolic function, high RAP (dilated IVC/leftward atrial septal shift), and leftward deviation of ventricular septum.  b. Doppler: increased TR severity, reduced RVOT SV, reduced LVAD inflow cannula, and/or outflow-graft velocities (<0.5 m/s with severe failure); inflow-cannula high velocities if associated with a suction event.  Of Note: a ‘too-high’ LVAD pump speed may contribute to RV failure by increasing TR (septal shift) and/or by increasing RV preload. 4. Inadequate LV filling or excessive LV unloading  Small LV dimensions (typically <3 cm and/or marked deviation of interventricular septum towards LV). Danger to not misinterpret an RV failure and/or pump speed too high for loading conditions. 5. LVAD suction with induced ventricular ectopy  Underfilled LV and mechanical impact of inflow cannula with LV endocardium, typically septum, and resolves with speed turndown. 6. LVAD-related continuous aortic insufficiency  Clinically significant—at least moderate and possibly severe—characterized by an AR vena contracta >3 mm; increased LV size and relatively decreased RVOT SV despite normal/increased inflow cannula and/or outflow-graft flows. 7. LVAD-related mitral regurgitation  a. Primary: inflow cannula interference with mitral apparatus.  b. Secondary: MR-functional, related to partial LV unloading/persistent heart failure. 8. Intracardiac thrombus  Including right and left atrial, LV apical, and aortic root thrombus 9. Inflow-cannula abnormality  a. 2D/3D: small or crowded inflow zone with or without evidence of localized obstructive muscle trabeculation, adjacent MV apparatus or thrombus; mispositioned inflow cannula.  b. High-velocity colour or spectral Doppler at inflow orifice. Results from malposition, suction event/other inflow obstruction: aliased colour-flow Doppler, and CW Doppler velocity >1.5 m/s.  c. Low-velocity inflow (markedly reduced peak systolic and nadir diastolic velocities) may indicate internal inflow-cannula thrombosis or more distal obstruction within the system. Doppler flow velocity profile may appear relatively ‘continuous’ (decreased phasic/pulsatile pattern). 10. Outflow-graft abnormality  Typically, due to obstruction/pump cessation.  a. 2D imaging (TOE): visible kink or thrombus.  b. Doppler: peak outflow-graft velocity >2 m/s at the obstruction site; but diminished or no spectral Doppler signal if sample volume is remote from obstruction location, combined with lack of RVOT SV change and/or expected LV dimension change with pump-speed changes. 11. Pump malfunction/pump arrest  a. Reduced inflow-cannula or outflow-graft flow velocities on colour and spectral Doppler or, with pump arrest, show diastolic flow reversal.  b. Signs of worsening HF: including dilated LV, worsening MR, worsened TR, and/or increased TR velocity; attenuated speed-change responses: decrease or absence of expected changes in LV linear dimension. Value of echocardiography. Adapted from Estep et al.141 2D, two-dimensional; 3D, three-dimensional; AR, aortic regurgitation; AV, aortic valve; BP, blood pressure; CW, continuous-wave; E, mitral valve early peak diastolic velocity; e’, mitral annular velocity; IVC, inferior vena cava; LV, left ventricular; LVAD, left ventricular assist device; LVOT, left ventricular outflow tract; MR, mitral regurgitation; MV, mitral valve; RAP, right atrial pressure; RV, right ventricular; RVOT, right ventricular outflow tract; SV, stroke volume; TR, tricuspid regurgitation. Open in new tab Table 6 Post-LVAD implantation complications One has to be systematic and specialized in the assessment of patients with LVAD (being aware of the device implanted, the patient and the history): 1. Pericardial effusion or haematoma  Cardiac tamponade will lead to RV compression and decrease in RV outflow tract velocity time integral. Check for the pericardium using all the echocardiographic windows (TOE if needed) and assess right heart output. 2. LV failure related to LV overloading  Important of serial exam comparison  a. 2D/3D: increasing LV size; increased AV opening duration, increased left atrial volume.  b. Doppler: increased mitral inflow peak E-wave diastolic velocity, increased E/A and E/e’ ratio, decreased deceleration time of mitral E velocity, worsening functional MR, and elevated pulmonary artery systolic pressure. 3. RV failure  a. 2D: increased RV size, decreased RV systolic function, high RAP (dilated IVC/leftward atrial septal shift), and leftward deviation of ventricular septum.  b. Doppler: increased TR severity, reduced RVOT SV, reduced LVAD inflow cannula, and/or outflow-graft velocities (<0.5 m/s with severe failure); inflow-cannula high velocities if associated with a suction event.  Of Note: a ‘too-high’ LVAD pump speed may contribute to RV failure by increasing TR (septal shift) and/or by increasing RV preload. 4. Inadequate LV filling or excessive LV unloading  Small LV dimensions (typically <3 cm and/or marked deviation of interventricular septum towards LV). Danger to not misinterpret an RV failure and/or pump speed too high for loading conditions. 5. LVAD suction with induced ventricular ectopy  Underfilled LV and mechanical impact of inflow cannula with LV endocardium, typically septum, and resolves with speed turndown. 6. LVAD-related continuous aortic insufficiency  Clinically significant—at least moderate and possibly severe—characterized by an AR vena contracta >3 mm; increased LV size and relatively decreased RVOT SV despite normal/increased inflow cannula and/or outflow-graft flows. 7. LVAD-related mitral regurgitation  a. Primary: inflow cannula interference with mitral apparatus.  b. Secondary: MR-functional, related to partial LV unloading/persistent heart failure. 8. Intracardiac thrombus  Including right and left atrial, LV apical, and aortic root thrombus 9. Inflow-cannula abnormality  a. 2D/3D: small or crowded inflow zone with or without evidence of localized obstructive muscle trabeculation, adjacent MV apparatus or thrombus; mispositioned inflow cannula.  b. High-velocity colour or spectral Doppler at inflow orifice. Results from malposition, suction event/other inflow obstruction: aliased colour-flow Doppler, and CW Doppler velocity >1.5 m/s.  c. Low-velocity inflow (markedly reduced peak systolic and nadir diastolic velocities) may indicate internal inflow-cannula thrombosis or more distal obstruction within the system. Doppler flow velocity profile may appear relatively ‘continuous’ (decreased phasic/pulsatile pattern). 10. Outflow-graft abnormality  Typically, due to obstruction/pump cessation.  a. 2D imaging (TOE): visible kink or thrombus.  b. Doppler: peak outflow-graft velocity >2 m/s at the obstruction site; but diminished or no spectral Doppler signal if sample volume is remote from obstruction location, combined with lack of RVOT SV change and/or expected LV dimension change with pump-speed changes. 11. Pump malfunction/pump arrest  a. Reduced inflow-cannula or outflow-graft flow velocities on colour and spectral Doppler or, with pump arrest, show diastolic flow reversal.  b. Signs of worsening HF: including dilated LV, worsening MR, worsened TR, and/or increased TR velocity; attenuated speed-change responses: decrease or absence of expected changes in LV linear dimension. One has to be systematic and specialized in the assessment of patients with LVAD (being aware of the device implanted, the patient and the history): 1. Pericardial effusion or haematoma  Cardiac tamponade will lead to RV compression and decrease in RV outflow tract velocity time integral. Check for the pericardium using all the echocardiographic windows (TOE if needed) and assess right heart output. 2. LV failure related to LV overloading  Important of serial exam comparison  a. 2D/3D: increasing LV size; increased AV opening duration, increased left atrial volume.  b. Doppler: increased mitral inflow peak E-wave diastolic velocity, increased E/A and E/e’ ratio, decreased deceleration time of mitral E velocity, worsening functional MR, and elevated pulmonary artery systolic pressure. 3. RV failure  a. 2D: increased RV size, decreased RV systolic function, high RAP (dilated IVC/leftward atrial septal shift), and leftward deviation of ventricular septum.  b. Doppler: increased TR severity, reduced RVOT SV, reduced LVAD inflow cannula, and/or outflow-graft velocities (<0.5 m/s with severe failure); inflow-cannula high velocities if associated with a suction event.  Of Note: a ‘too-high’ LVAD pump speed may contribute to RV failure by increasing TR (septal shift) and/or by increasing RV preload. 4. Inadequate LV filling or excessive LV unloading  Small LV dimensions (typically <3 cm and/or marked deviation of interventricular septum towards LV). Danger to not misinterpret an RV failure and/or pump speed too high for loading conditions. 5. LVAD suction with induced ventricular ectopy  Underfilled LV and mechanical impact of inflow cannula with LV endocardium, typically septum, and resolves with speed turndown. 6. LVAD-related continuous aortic insufficiency  Clinically significant—at least moderate and possibly severe—characterized by an AR vena contracta >3 mm; increased LV size and relatively decreased RVOT SV despite normal/increased inflow cannula and/or outflow-graft flows. 7. LVAD-related mitral regurgitation  a. Primary: inflow cannula interference with mitral apparatus.  b. Secondary: MR-functional, related to partial LV unloading/persistent heart failure. 8. Intracardiac thrombus  Including right and left atrial, LV apical, and aortic root thrombus 9. Inflow-cannula abnormality  a. 2D/3D: small or crowded inflow zone with or without evidence of localized obstructive muscle trabeculation, adjacent MV apparatus or thrombus; mispositioned inflow cannula.  b. High-velocity colour or spectral Doppler at inflow orifice. Results from malposition, suction event/other inflow obstruction: aliased colour-flow Doppler, and CW Doppler velocity >1.5 m/s.  c. Low-velocity inflow (markedly reduced peak systolic and nadir diastolic velocities) may indicate internal inflow-cannula thrombosis or more distal obstruction within the system. Doppler flow velocity profile may appear relatively ‘continuous’ (decreased phasic/pulsatile pattern). 10. Outflow-graft abnormality  Typically, due to obstruction/pump cessation.  a. 2D imaging (TOE): visible kink or thrombus.  b. Doppler: peak outflow-graft velocity >2 m/s at the obstruction site; but diminished or no spectral Doppler signal if sample volume is remote from obstruction location, combined with lack of RVOT SV change and/or expected LV dimension change with pump-speed changes. 11. Pump malfunction/pump arrest  a. Reduced inflow-cannula or outflow-graft flow velocities on colour and spectral Doppler or, with pump arrest, show diastolic flow reversal.  b. Signs of worsening HF: including dilated LV, worsening MR, worsened TR, and/or increased TR velocity; attenuated speed-change responses: decrease or absence of expected changes in LV linear dimension. Value of echocardiography. Adapted from Estep et al.141 2D, two-dimensional; 3D, three-dimensional; AR, aortic regurgitation; AV, aortic valve; BP, blood pressure; CW, continuous-wave; E, mitral valve early peak diastolic velocity; e’, mitral annular velocity; IVC, inferior vena cava; LV, left ventricular; LVAD, left ventricular assist device; LVOT, left ventricular outflow tract; MR, mitral regurgitation; MV, mitral valve; RAP, right atrial pressure; RV, right ventricular; RVOT, right ventricular outflow tract; SV, stroke volume; TR, tricuspid regurgitation. Open in new tab Secondary (functional) mitral regurgitation Secondary mitral regurgitation (MR) is an important issue in DCM patients. A clear prognostic value of this type of MR has been reported. MR in DCM is not mainly due to a disease of the leaflets but to the symmetrical or asymmetrical dilation of the left ventricle. The detection of the MR should not wait that the LV dysfunction become too severe. When the LV is too enlarged and the function to decrease, MR loses its prognostic value.128 Secondary MR needs to be carefully assessed50 (Figures 7and8). Medical treatment including CRT will impact on the severity of the MR. Figure 7 Open in new tabDownload slide Four-chamber cine of patient affected by DCM and severe LV systolic dysfunction and LBBB in diastole (A) and systole (B); mid-ventricle LV short-axis view used for tissue tracking analysis (C); representation of the radial strai curves showing the amount of dyssynchrony (D); schematic representation of radial strain (%) curves: the two arrows represent the dyssynchrony between the septum and the lateral walls. Figure 7 Open in new tabDownload slide Four-chamber cine of patient affected by DCM and severe LV systolic dysfunction and LBBB in diastole (A) and systole (B); mid-ventricle LV short-axis view used for tissue tracking analysis (C); representation of the radial strai curves showing the amount of dyssynchrony (D); schematic representation of radial strain (%) curves: the two arrows represent the dyssynchrony between the septum and the lateral walls. Figure 8 Open in new tabDownload slide A woman 75 years old, idiopathic DCM, who justified a cardiac resynchronization therapy and an ICD. After few years, despite an OMT she is still NYHA II+ and recently hospitalized for acute HF. The transthoracic echocardiography completed by a transoesophageal exam allows to describe the spherization of the left ventricle (LVEF 35% and LV end-diastolic diameter 64 mm). The tethering effect related to this LV remodelling on both mitral leaflets. The leaflets are thin without any large indentation, without any calcification, and the regurgitant jet is greater than 45 mL/beat (regurgitant orifice area >20 mm2) and maximal in regard to A2P2. Figure 8 Open in new tabDownload slide A woman 75 years old, idiopathic DCM, who justified a cardiac resynchronization therapy and an ICD. After few years, despite an OMT she is still NYHA II+ and recently hospitalized for acute HF. The transthoracic echocardiography completed by a transoesophageal exam allows to describe the spherization of the left ventricle (LVEF 35% and LV end-diastolic diameter 64 mm). The tethering effect related to this LV remodelling on both mitral leaflets. The leaflets are thin without any large indentation, without any calcification, and the regurgitant jet is greater than 45 mL/beat (regurgitant orifice area >20 mm2) and maximal in regard to A2P2. If MR remains severe and symptomatic, surgery and percutaneous correction of the regurgitation could be considered. The ESC guidelines in valvular heart disease provide a Class IIbC indication for the percutaneous edge-to-edge procedure or valve surgery after careful evaluation for ventricular assist device or heart transplant, according to individual patient characteristics in patients with severe secondary mitral regurgitation and LVEF <30% who remain symptomatic despite optimal medical management (heart team decision). The discrepancy between European and American approaches for defining a severe secondary MR exist.51,129 This issue is related to a gap in evidence. The recent Mitra-FR and COAPT trials are encouraging the use of regurgitant volume >45 mL and/or regurgitant orifice area >30 mm2 for deciding for the implantation of clips in symptomatic patients and having a LVEF >20% especially when the degree of the regurgitation is greater than expected according to the degree of LV dilatation.130–134 Appropriateness of each imaging technique to assess patients with DCM European appropriateness criteria for the use of cardiovascular imaging (CVI) in HF have been developed using a rigorous process described elsewhere.135 This document provides a framework for decisions regarding judicious utilization of imaging in the management of patients with HF seen in clinical practice. However, the appropriate use of each non-invasive CVI technique in DCM has not been studied extensively. As in HF patients, CVI can be used for DCM patients in various clinical scenarios and settings: (i) for the diagnosis of the DCM, (ii) for the planning of treatment (CRT/LVAD), and (iii) for the follow-up of the DCM patients (Figure 9). The appropriateness of use for each technique may be dependent on the mode of presentation (urgent or not), the stage of the DCM (early vs. clinical), the symptomatic status, and the need to perform a screening. It should also reflect practice heterogeneity across Europe, with broad variations in access to modern technology and imaging facilities, educational platforms, training requirements, certification guidelines, and reimbursement systems. Figure 9 Open in new tabDownload slide Native T1 mapping (MOLLI) of basal LV short axis showing increased values in the septum. Region of interest for T1 mapping measurement before gadolinium: 1136 ms (A). Post-contrast T1 mapping of basal LV short axis. Region of interest for T1 mapping measurement post gadolinium enhancement: 456 ms (B). Post-contrast basal LV short axis, showing mid-wall myocardial late enhancement (yellow arrows) (C). Basal LV short-axis cine (D). Figure 9 Open in new tabDownload slide Native T1 mapping (MOLLI) of basal LV short axis showing increased values in the septum. Region of interest for T1 mapping measurement before gadolinium: 1136 ms (A). Post-contrast T1 mapping of basal LV short axis. Region of interest for T1 mapping measurement post gadolinium enhancement: 456 ms (B). Post-contrast basal LV short axis, showing mid-wall myocardial late enhancement (yellow arrows) (C). Basal LV short-axis cine (D). Challenges and gaps in evidence Large studies testing imaging-based approach to disease treatment vs. non-imaging-based approach are lacking. The literature suggests that imaging, especially echocardiography, which was tested, was unsuccessful to improve patients’ selection for CRT. Nevertheless, imaging techniques are becoming more mature in the precision and the potential clinical value of parameters offered. Scientific Associations, like the EACVI, are committed to define the most appropriate imaging approach and patients’ pathways.136 Individual modalities and multimodality imaging appropriateness criteria are warranted, as well as randomized prospective large studies involving imaging strategy scenarios. In an era of precision medicine, imaging phenotyping might play a key role in therapeutic decisions and management. Perspectives Despite several imaging and genetic improvements, several challenges persist concerning the diagnosis, genetics and other aetiologies, prognosis, and even definition of DCM. Although a revised definition of DCM has recently been proposed,1 including the creation of a new category of hypokinetic non-dilated cardiomyopathies, several uncertainties persist. Multimodality imaging combined with genetic studies could have a central role in the evaluation of DCM (Table 1 and Figure 10). Figure 10 Open in new tabDownload slide A proposed flowchart for the use of a multimodality approach for assessing dilated cardiomyopathies. Figure 10 Open in new tabDownload slide A proposed flowchart for the use of a multimodality approach for assessing dilated cardiomyopathies. In the present document, the differential diagnoses of DCM (excepting the ischaemic aetiology) are not specifically addressed. One of the major challenges is being able to both make an early diagnosis of DCM, leading to earlier and more effective preventive and therapeutic strategies, but to avoid erroneous diagnosis and misinterpretation of physiological variants. Two such examples are the ‘grey-zone’ LV modifications observed in athletes137 and the frequently difficult diagnosis of LV non-compaction, with the known risk of both over- and under-diagnosis. A unified definition of the diagnostic criteria for LV non-compaction is awaited pending results from ongoing studies.138,139 In all these difficult situations, the combined use of two different imaging modalities is recommended, including preferable echocardiography and CMR. These techniques give additional information and should frequently be used in combination in the same patient to maximize diagnostic performance. Additional studies are warranted to select the most appropriate utilization of each imaging technique when facing a patient with suspected or definite DCM.1,140 Finally, additional investigations such as familial screening, and genetic studies are frequently necessary. Patients with suspected DCM should be referred to specialized centres that can provide a multidisciplinary team approach for early diagnosis, avoiding over-diagnosis, providing adequate familial counselling, prognostic stratification, and finally optimal patients’ management. Funding C.B.D. is supported by the Bristol National Institute of Health Research (NIHR) Biomedical Research Centre (BRC). The views expressed in this publication are those of the authors and not necessarily those of the NHS, the National Institute for Health Research, or the Department of Health. Conflict of interest: Fees for V.D. from Abbott and E.D. from Bristol Myer Squibb and Novartis. All other authors declared no conflict of interest. References 1 Pinto YM , Elliott PM , Arbustini E , Adler Y , Anastasakis A , Bohm M. Proposal for a revised definition of dilated cardiomyopathy, hypokinetic non-dilated cardiomyopathy, and its implications for clinical practice: a position statement of the ESC working group on myocardial and pericardial diseases . Eur Heart J 2016 ; 37 : 1850 – 8 . Google Scholar Crossref Search ADS PubMed WorldCat 2 Weintraub RG , Semsarian C , Macdonald P. Dilated cardiomyopathy . Lancet 2017 ; 390 : 400 – 14 . Google Scholar Crossref Search ADS PubMed WorldCat 3 Elliott P , Andersson B , Arbustini E , Bilinska Z , Cecchi F , Charron P et al. Classification of the cardiomyopathies: a position statement from the European Society of Cardiology Working Group on Myocardial and Pericardial Diseases . Eur Heart J 2007 ; 29 : 270 – 6 . Google Scholar Crossref Search ADS PubMed WorldCat 4 Donal E , Lip GY , Galderisi M , Goette A , Shah D , Marwan M et al. EACVI/EHRA Expert Consensus Document on the role of multi-modality imaging for the evaluation of patients with atrial fibrillation . Eur Heart J Cardiovasc Imaging 2016 ; 17 : 355 – 83 . Google Scholar Crossref Search ADS PubMed WorldCat 5 Bouabdallaoui N , Mouquet F , Lebreton G , Demondion P , Le Jemtel TH , Ennezat PV. Current knowledge and recent development on management of peripartum cardiomyopathy . Eur Heart J Acute Cardiovasc Care 2017 ; 6 : 359 – 66 . Google Scholar Crossref Search ADS PubMed WorldCat 6 Caforio ALP , Adler Y , Agostini C , Allanore Y , Anastasakis A , Arad M et al. Diagnosis and management of myocardial involvement in systemic immune-mediated diseases: a position statement of the European Society of Cardiology Working Group on Myocardial and Pericardial Disease . Eur Heart J 2017 ; 38 : 2649 – 62 . Google Scholar Crossref Search ADS PubMed WorldCat 7 Japp AG , Gulati A , Cook SA , Cowie MR , Prasad SK. The diagnosis and evaluation of dilated cardiomyopathy . J Am Coll Cardiol 2016 ; 67 : 2996 – 3010 . Google Scholar Crossref Search ADS PubMed WorldCat 8 Lang RM , Badano LP , Mor-Avi V , Afilalo J , Armstrong A , Ernande L et al. Recommendations for cardiac chamber quantification by echocardiography in adults: an update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging . Eur Heart J Cardiovasc Imaging 2015 ; 16 : 233 – 70 . Google Scholar Crossref Search ADS PubMed WorldCat 9 Galderisi M , Cosyns B , Edvardsen T , Cardim N , Delgado V , Di Salvo G et al. Standardization of adult transthoracic echocardiography reporting in agreement with recent chamber quantification, diastolic function, and heart valve disease recommendations: an expert consensus document of the European Association of Cardiovascular Imaging . Eur Heart J Cardiovasc Imaging 2017 ; 18 : 1301 – 10 . Google Scholar Crossref Search ADS PubMed WorldCat 10 Celeng C , Leiner T , Maurovich-Horvat P , Merkely B , de Jong P , Dankbaar JW et al. Anatomical and functional computed tomography for diagnosing hemodynamically significant coronary artery disease: a meta-analysis . JACC Cardiovasc Imaging 2019 ; 12 : 1316 – 25 . Google Scholar Crossref Search ADS PubMed WorldCat 11 Soriano CJ , Ridocci F , Estornell J , Jimenez J , Martinez V , De Velasco JA. Noninvasive diagnosis of coronary artery disease in patients with heart failure and systolic dysfunction of uncertain etiology, using late gadolinium-enhanced cardiovascular magnetic resonance . J Am Coll Cardiol 2005 ; 45 : 743 – 8 . Google Scholar Crossref Search ADS PubMed WorldCat 12 Ponikowski P , Voors AA , Anker SD , Bueno H , Cleland JG , Coats AJ et al. 2016 ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure: the Task Force for the diagnosis and treatment of acute and chronic heart failure of the European Society of Cardiology (ESC)Developed with the special contribution of the Heart Failure Association (HFA) of the ESC . Eur Heart J 2016 ; 37 : 2129 – 200 . Google Scholar Crossref Search ADS PubMed WorldCat 13 Fatkin D , Yeoh T , Hayward CS , Benson V , Sheu A , Richmond Z et al. Evaluation of left ventricular enlargement as a marker of early disease in familial dilated cardiomyopathy . Circ Cardiovasc Genet 2011 ; 4 : 342 – 8 . Google Scholar Crossref Search ADS PubMed WorldCat 14 George A , Figueredo VM. Alcoholic cardiomyopathy: a review . J Card Fail 2011 ; 17 : 844 – 9 . Google Scholar Crossref Search ADS PubMed WorldCat 15 Caballero L , Kou S , Dulgheru R , Gonjilashvili N , Athanassopoulos GD , Barone D et al. Echocardiographic reference ranges for normal cardiac Doppler data: results from the NORRE Study . Eur Heart J Cardiovasc Imaging 2015 ; 16 : 1031 – 41 . Google Scholar PubMed WorldCat 16 Sugimoto T , Dulgheru R , Bernard A , Ilardi F , Contu L , Addetia K et al. Echocardiographic reference ranges for normal left ventricular 2D strain: results from the EACVI NORRE study . Eur Heart J Cardiovasc Imaging 2017 ; 18 : 833 – 40 . Google Scholar Crossref Search ADS PubMed WorldCat 17 Farsalinos KE , Daraban AM , Unlu S , Thomas JD , Badano LP , Voigt JU. Head-to-head comparison of global longitudinal strain measurements among nine different vendors: the EACVI/ASE Inter-Vendor Comparison Study . J Am Soc Echocardiogr 2015 ; 28 : 1171 – 81.e2 . Google Scholar Crossref Search ADS PubMed WorldCat 18 Boyd A , Stoodley P , Richards D , Hui R , Harnett P , Vo K et al. Anthracyclines induce early changes in left ventricular systolic and diastolic function: a single centre study . PLoS One 2017 ; 12 : e0175544. Google Scholar Crossref Search ADS PubMed WorldCat 19 Holland DJ , Marwick TH , Haluska BA , Leano R , Hordern MD , Hare JL et al. Subclinical LV dysfunction and 10-year outcomes in type 2 diabetes mellitus . Heart 2015 ; 101 : 1061 – 6 . Google Scholar Crossref Search ADS PubMed WorldCat 20 Voigt JU , Pedrizzetti G , Lysyansky P , Marwick TH , Houle H , Baumann R et al. Definitions for a common standard for 2D speckle tracking echocardiography: consensus document of the EACVI/ASE/Industry Task Force to standardize deformation imaging . Eur Heart J Cardiovasc Imaging 2015 ; 16 : 1 – 11 . Google Scholar Crossref Search ADS PubMed WorldCat 21 Yang H , Marwick TH , Fukuda N , Oe H , Saito M , Thomas JD et al. Improvement in strain concordance between two major vendors after the strain standardization initiative . J Am Soc Echocardiogr 2015 ; 28 : 642 – 8.e7 . Google Scholar Crossref Search ADS PubMed WorldCat 22 Lakdawala NK , Thune JJ , Colan SD , Cirino AL , Farrohi F , Rivero J et al. Subtle abnormalities in contractile function are an early manifestation of sarcomere mutations in dilated cardiomyopathy . Circ Cardiovasc Genet 2012 ; 5 : 503 – 10 . Google Scholar Crossref Search ADS PubMed WorldCat 23 Muraru D , Spadotto V , Cecchetto A , Romeo G , Aruta P , Ermacora D et al. New speckle-tracking algorithm for right ventricular volume analysis from three-dimensional echocardiographic data sets: validation with cardiac magnetic resonance and comparison with the previous analysis tool . Eur Heart J Cardiovasc Imaging 2016 ; 17 : 1279 – 89 . Google Scholar Crossref Search ADS PubMed WorldCat 24 Mavrogeni S , Dimitroulas T , Kitas GD. Multimodality imaging and the emerging role of cardiac magnetic resonance in autoimmune myocarditis . Autoimmun Rev 2012 ; 12 : 305 – 12 . Google Scholar Crossref Search ADS PubMed WorldCat 25 Ponikowski P , Voors AA , Anker SD , Bueno H , Cleland JG , Coats AJ et al. 2016 ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure: the Task Force for the diagnosis and treatment of acute and chronic heart failure of the European Society of Cardiology (ESC)Developed with the special contribution of the Heart Failure Association (HFA) of the ESC . Eur Heart J 2016 ; 37 : 2129 – 200 . Google Scholar Crossref Search ADS PubMed WorldCat 26 Lopez-Fernandez T , Thavendiranathan P. Emerging cardiac imaging modalities for the early detection of cardiotoxicity due to anticancer therapies . Rev Esp Cardiol (Engl Ed) 2017 ; 70 : 487 – 95 . Google Scholar Crossref Search ADS PubMed WorldCat 27 Boulmier D , Audinet C , Heautot JF , Larralde A , Veillard D , Hamonic S et al. Clinical contributions of 64-slice computed tomography in the evaluation of cardiomyopathy of unknown origin . Arch Cardiovasc Dis 2009 ; 102 : 685 – 96 . Google Scholar Crossref Search ADS PubMed WorldCat 28 Naik MM , Diamond GA , Pai T , Soffer A , Siegel RJ. Correspondence of left ventricular ejection fraction determinations from two-dimensional echocardiography, radionuclide angiography and contrast cineangiography . J Am Coll Cardiol 1995 ; 25 : 937 – 42 . Google Scholar Crossref Search ADS PubMed WorldCat 29 Zamorano JL , Lancellotti P , Rodriguez Munoz D , Aboyans V , Asteggiano R , Galderisi M et al. 2016 ESC Position Paper on cancer treatments and cardiovascular toxicity developed under the auspices of the ESC Committee for Practice Guidelines: the Task Force for cancer treatments and cardiovascular toxicity of the European Society of Cardiology (ESC) . Eur Heart J 2016 ; 37 : 2768 – 801 . Google Scholar Crossref Search ADS PubMed WorldCat 30 Charron P , Arad M , Arbustini E , Basso C , Bilinska Z , Elliott P et al. Genetic counselling and testing in cardiomyopathies: a position statement of the European Society of Cardiology Working Group on Myocardial and Pericardial Diseases . Eur Heart J 2010 ; 31 : 2715 – 26 . Google Scholar Crossref Search ADS PubMed WorldCat 31 Lancellotti P , Anker SD , Donal E , Edvardsen T , Popescu BA , Farmakis D et al. EACVI/HFA Cardiac Oncology Toxicity Registry in breast cancer patients: rationale, study design, and methodology (EACVI/HFA COT Registry)—EURObservational Research Program of the European Society of Cardiology . Eur Heart J Cardiovasc Imaging 2015 ; 16 : 466 – 70 . Google Scholar Crossref Search ADS PubMed WorldCat 32 Plana JC , Galderisi M , Barac A , Ewer MS , Ky B , Scherrer-Crosbie M et al. Expert consensus for multimodality imaging evaluation of adult patients during and after cancer therapy: a report from the American Society of Echocardiography and the European Association of Cardiovascular Imaging . Eur Heart J Cardiovasc Imaging 2014 ; 15 : 1063 – 93 . Google Scholar Crossref Search ADS PubMed WorldCat 33 Merlo M , Pyxaras SA , Pinamonti B , Barbati G , Di Lenarda A , Sinagra G. Prevalence and prognostic significance of left ventricular reverse remodeling in dilated cardiomyopathy receiving tailored medical treatment . J Am Coll Cardiol 2011 ; 57 : 1468 – 76 . Google Scholar Crossref Search ADS PubMed WorldCat 34 Masci PG , Schuurman R , Andrea B , Ripoli A , Coceani M , Chiappino S et al. Myocardial fibrosis as a key determinant of left ventricular remodeling in idiopathic dilated cardiomyopathy: a contrast-enhanced cardiovascular magnetic study . Circ Cardiovasc Imaging 2013 ; 6 : 790 – 9 . Google Scholar Crossref Search ADS PubMed WorldCat 35 McMurray JJ , Adamopoulos S , Anker SD , Auricchio A , Bohm M , Dickstein K et al. ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure 2012: the Task Force for the Diagnosis and Treatment of Acute and Chronic Heart Failure 2012 of the European Society of Cardiology. Developed in collaboration with the Heart Failure Association (HFA) of the ESC . Eur Heart J 2012 ; 33 : 1787 – 847 . Google Scholar Crossref Search ADS PubMed WorldCat 36 Alba AC , Foroutan F , Duero Posada J , Battioni L , Schofield T , Alhussein M et al. Implantable cardiac defibrillator and mortality in non-ischaemic cardiomyopathy: an updated meta-analysis . Heart 2018 ; 104 : 230 – 6 . Google Scholar Crossref Search ADS PubMed WorldCat 37 Gulati A , Jabbour A , Ismail TF , Guha K , Khwaja J , Raza S et al. Association of fibrosis with mortality and sudden cardiac death in patients with nonischemic dilated cardiomyopathy . JAMA 2013 ; 309 : 896 – 908 . Google Scholar Crossref Search ADS PubMed WorldCat 38 Merlo M , Gobbo M , Stolfo D , Losurdo P , Ramani F , Barbati G et al. The prognostic impact of the evolution of RV function in idiopathic DCM . JACC Cardiovasc Imaging 2016 ; 9 : 1034 – 42 . Google Scholar Crossref Search ADS PubMed WorldCat 39 Venner C , Selton-Suty C , Huttin O , Erpelding M-L , Aliot E , Juillière Y. Right ventricular dysfunction in patients with idiopathic dilated cardiomyopathy: prognostic value and predictive factors . Arch Cardiovasc Dis 2016 ; 109 : 231 – 41 . Google Scholar Crossref Search ADS PubMed WorldCat 40 Mignot A , Donal E , Zaroui A , Reant P , Salem A , Hamon C et al. Global longitudinal strain as a major predictor of cardiac events in patients with depressed left ventricular function: a multicenter study . J Am Soc Echocardiogr 2010 ; 23 : 1019 – 24 . Google Scholar Crossref Search ADS PubMed WorldCat 41 Saito M , Negishi K , Eskandari M , Huynh Q , Hawson J , Moore A et al. Association of left ventricular strain with 30-day mortality and readmission in patients with heart failure . J Am Soc Echocardiogr 2015 ; 28 : 652 – 66 . Google Scholar Crossref Search ADS PubMed WorldCat 42 Cho GY , Marwick TH , Kim HS , Kim MK , Hong KS , Oh DJ. Global 2-dimensional strain as a new prognosticator in patients with heart failure . J Am Coll Cardiol 2009 ; 54 : 618 – 24 . Google Scholar Crossref Search ADS PubMed WorldCat 43 Carluccio E , Biagioli P , Alunni G , Murrone A , Zuchi C , Coiro S et al. Prognostic value of right ventricular dysfunction in heart failure with reduced ejection fraction: superiority of longitudinal strain over tricuspid annular plane systolic excursion . Circ Cardiovasc Imaging 2018 ; 11 : e006894. Google Scholar PubMed WorldCat 44 Gorter TM , van Veldhuisen DJ , Bauersachs J , Borlaug BA , Celutkiene J , Coats AJS et al. Right heart dysfunction and failure in heart failure with preserved ejection fraction: mechanisms and management. Position statement on behalf of the Heart Failure Association of the European Society of Cardiology . Eur J Heart Fail 2018 ; 20 : 16 – 37 . Google Scholar Crossref Search ADS PubMed WorldCat 45 Nagueh SF , Smiseth OA , Appleton CP , Byrd BF 3rd , Dokainish H , Edvardsen T et al. Recommendations for the evaluation of left ventricular diastolic function by echocardiography: an update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging . Eur Heart J Cardiovasc Imaging 2016 ; 17 : 1321 – 60 . Google Scholar Crossref Search ADS PubMed WorldCat 46 Feneon D , Behaghel A , Bernard A , Fournet M , Mabo P , Daubert JC et al. Left atrial function, a new predictor of response to cardiac resynchronization therapy? Heart Rhythm 2015 ; 12 : 1800 – 6 . Google Scholar Crossref Search ADS PubMed WorldCat 47 Badano LP , Kolias TJ , Muraru D , Abraham TP , Aurigemma G , Edvardsen T et al. Standardization of left atrial, right ventricular, and right atrial deformation imaging using two-dimensional speckle tracking echocardiography: a consensus document of the EACVI/ASE/Industry Task Force to standardize deformation imaging . Eur Heart J Cardiovasc Imaging 2018 ; 19 : 591 – 600 . Google Scholar Crossref Search ADS PubMed WorldCat 48 Rossi A , Dini FL , Faggiano P , Agricola E , Cicoira M , Frattini S et al. Independent prognostic value of functional mitral regurgitation in patients with heart failure. A quantitative analysis of 1256 patients with ischaemic and non-ischaemic dilated cardiomyopathy . Heart 2011 ; 97 : 1675 – 80 . Google Scholar Crossref Search ADS PubMed WorldCat 49 Mack M , Grayburn P. Guideline-directed medical therapy for secondary mitral regurgitation: more questions than answers! JACC Heart Fail 2017 ; 5 : 660 – 2 . Google Scholar Crossref Search ADS PubMed WorldCat 50 Grayburn PA , Carabello B , Hung J , Gillam LD , Liang D , Mack MJ et al. Defining “severe” secondary mitral regurgitation: emphasizing an integrated approach . J Am Coll Cardiol 2014 ; 64 : 2792 – 801 . Google Scholar Crossref Search ADS PubMed WorldCat 51 Lancellotti P , Tribouilloy C , Hagendorff A , Popescu BA , Edvardsen T , Pierard LA et al. Recommendations for the echocardiographic assessment of native valvular regurgitation: an executive summary from the European Association of Cardiovascular Imaging . Eur Heart J Cardiovasc Imaging 2013 ; 14 : 611 – 44 . Google Scholar Crossref Search ADS PubMed WorldCat 52 Naqvi TZ , Goel RK , Forrester JS , Siegel RJ. Myocardial contractile reserve on dobutamine echocardiography predicts late spontaneous improvement in cardiac function in patients with recent onset idiopathic dilated cardiomyopathy . J Am Coll Cardiol 1999 ; 34 : 1537 – 44 . Google Scholar Crossref Search ADS PubMed WorldCat 53 Lee JH , Yang DH , Choi WS , Kim KH , Park SH , Bae MH et al. Prediction of improvement in cardiac function by high dose dobutamine stress echocardiography in patients with recent onset idiopathic dilated cardiomyopathy . Int J Cardiol 2013 ; 167 : 1649 – 50 . Google Scholar Crossref Search ADS PubMed WorldCat 54 Cortigiani L , Rigo F , Gherardi S , Bovenzi F , Molinaro S , Picano E et al. Prognostic implication of Doppler echocardiographic derived coronary flow reserve in patients with left bundle branch block . Eur Heart J 2013 ; 34 : 364 – 73 . Google Scholar Crossref Search ADS PubMed WorldCat 55 Rigo F , Gherardi S , Galderisi M , Pratali L , Cortigiani L , Sicari R et al. The prognostic impact of coronary flow-reserve assessed by Doppler echocardiography in non-ischaemic dilated cardiomyopathy . Eur Heart J 2006 ; 27 : 1319 – 23 . Google Scholar Crossref Search ADS PubMed WorldCat 56 Neglia D , Michelassi C , Trivieri MG , Sambuceti G , Giorgetti A , Pratali L et al. Prognostic role of myocardial blood flow impairment in idiopathic left ventricular dysfunction . Circulation 2002 ; 105 : 186 – 93 . Google Scholar Crossref Search ADS PubMed WorldCat 57 Priori SG , Blomstrom-Lundqvist C , Mazzanti A , Blom N , Borggrefe M , Camm J et al. 2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: the Task Force for the Management of Patients with Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death of the European Society of Cardiology (ESC). Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC) . Eur Heart J 2015 ; 36 : 2793 – 867 . Google Scholar Crossref Search ADS PubMed WorldCat 58 Goldberger JJ , Subacius H , Patel T , Cunnane R , Kadish AH. Sudden cardiac death risk stratification in patients with nonischemic dilated cardiomyopathy . J Am Coll Cardiol 2014 ; 63 : 1879 – 89 . Google Scholar Crossref Search ADS PubMed WorldCat 59 Beggs SAS , Jhund PS , Jackson CE , McMurray JJV , Gardner RS. Non-ischaemic cardiomyopathy, sudden death and implantable defibrillators: a review and meta-analysis . Heart 2018 ; 104 : 144 – 50 . Google Scholar Crossref Search ADS PubMed WorldCat 60 Køber L , Thune JJ , Nielsen JC , Haarbo J , Videbæk L , Korup E et al. Defibrillator implantation in patients with nonischemic systolic heart failure . N Engl J Med 2016 ; 375 : 1221 – 30 . Google Scholar Crossref Search ADS PubMed WorldCat 61 Kadish A , Dyer A , Daubert JP , Quigg R , Estes NA , Anderson KP et al. Prophylactic defibrillator implantation in patients with nonischemic dilated cardiomyopathy . N Engl J Med 2004 ; 350 : 2151 – 8 . Google Scholar Crossref Search ADS PubMed WorldCat 62 Bansch D , Antz M , Boczor S , Volkmer M , Tebbenjohanns J , Seidl K et al. Primary prevention of sudden cardiac death in idiopathic dilated cardiomyopathy: the Cardiomyopathy Trial (CAT) . Circulation 2002 ; 105 : 1453 – 8 . Google Scholar Crossref Search ADS PubMed WorldCat 63 Bardy GH , Lee KL , Mark DB , Poole JE , Toff WD , Tonkin AM et al. Home use of automated external defibrillators for sudden cardiac arrest . N Engl J Med 2008 ; 358 : 1793 – 804 . Google Scholar Crossref Search ADS PubMed WorldCat 64 Haugaa KH , Smedsrud MK , Steen T , Kongsgaard E , Loennechen JP , Skjaerpe T et al. Mechanical dispersion assessed by myocardial strain in patients after myocardial infarction for risk prediction of ventricular arrhythmia . JACC Cardiovasc Imaging 2010 ; 3 : 247 – 56 . Google Scholar Crossref Search ADS PubMed WorldCat 65 Hasselberg NE , Haugaa KH , Sarvari SI , Gullestad L , Andreassen AK , Smiseth OA et al. Left ventricular global longitudinal strain is associated with exercise capacity in failing hearts with preserved and reduced ejection fraction . Eur Heart J Cardiovasc Imaging 2015 ; 16 : 217 – 24 . Google Scholar Crossref Search ADS PubMed WorldCat 66 Haugaa KH , Marek JJ , Ahmed M , Ryo K , Adelstein EC , Schwartzman D et al. Mechanical dyssynchrony after cardiac resynchronization therapy for severely symptomatic heart failure is associated with risk for ventricular arrhythmias . J Am Soc Echocardiogr 2014 ; 27 : 872 – 9 . Google Scholar Crossref Search ADS PubMed WorldCat 67 Popescu BA , Beladan CC , Călin A , Muraru D , Deleanu D , Roşca M et al. Left ventricular remodelling and torsional dynamics in dilated cardiomyopathy: reversed apical rotation as a marker of disease severity . Eur J Heart Fail 2009 ; 11 : 945 – 51 . Google Scholar Crossref Search ADS PubMed WorldCat 68 Haland TF , Almaas VM , Hasselberg NE , Saberniak J , Leren IS , Hopp E et al. Strain echocardiography is related to fibrosis and ventricular arrhythmias in hypertrophic cardiomyopathy . Eur Heart J Cardiovasc Imaging 2016 ; 17 : 613 – 21 . Google Scholar Crossref Search ADS PubMed WorldCat 69 Gulati A , Ismail TF , Jabbour A , Alpendurada F , Guha K , Ismail NA et al. The prevalence and prognostic significance of right ventricular systolic dysfunction in nonischemic dilated cardiomyopathy . Circulation 2013 ; 128 : 1623 – 33 . Google Scholar Crossref Search ADS PubMed WorldCat 70 Masci PG , Doulaptsis C , Bertella E , Del Torto A , Symons R , Pontone G et al. Incremental prognostic value of myocardial fibrosis in patients with non-ischemic cardiomyopathy without congestive heart failure . Circ Heart Fail 2014 ; 7 : 448 – 56 . Google Scholar Crossref Search ADS PubMed WorldCat 71 Neilan TG , Coelho-Filho OR , Danik SB , Shah RV , Dodson JA , Verdini DJ et al. CMR quantification of myocardial scar provides additive prognostic information in nonischemic cardiomyopathy . JACC Cardiovasc Imaging 2013 ; 6 : 944 – 54 . Google Scholar Crossref Search ADS PubMed WorldCat 72 Assomull RG , Prasad SK , Lyne J , Smith G , Burman ED , Khan M et al. Cardiovascular magnetic resonance, fibrosis, and prognosis in dilated cardiomyopathy . J Am Coll Cardiol 2006 ; 48 : 1977 – 85 . Google Scholar Crossref Search ADS PubMed WorldCat 73 Wu KC , Weiss RG , Thiemann DR , Kitagawa K , Schmidt A , Dalal D et al. Late gadolinium enhancement by cardiovascular magnetic resonance heralds an adverse prognosis in nonischemic cardiomyopathy . J Am Coll Cardiol 2008 ; 51 : 2414 – 21 . Google Scholar Crossref Search ADS PubMed WorldCat 74 Leyva F , Taylor RJ , Foley PW , Umar F , Mulligan LJ , Patel K et al. Left ventricular midwall fibrosis as a predictor of mortality and morbidity after cardiac resynchronization therapy in patients with nonischemic cardiomyopathy . J Am Coll Cardiol 2012 ; 60 : 1659 – 67 . Google Scholar Crossref Search ADS PubMed WorldCat 75 Iles L , Pfluger H , Lefkovits L , Butler MJ , Kistler PM , Kaye DM et al. Myocardial fibrosis predicts appropriate device therapy in patients with implantable cardioverter-defibrillators for primary prevention of sudden cardiac death . J Am Coll Cardiol 2011 ; 57 : 821 – 8 . Google Scholar Crossref Search ADS PubMed WorldCat 76 Kuruvilla S , Adenaw N , Katwal AB , Lipinski MJ , Kramer CM , Salerno M. Late gadolinium enhancement on cardiac magnetic resonance predicts adverse cardiovascular outcomes in nonischemic cardiomyopathy: a systematic review and meta-analysis . Circ Cardiovasc Imaging 2014 ; 7 : 250 – 8 . Google Scholar Crossref Search ADS PubMed WorldCat 77 Halliday BP , Cleland JGF , Goldberger JJ , Prasad SK. Personalizing risk stratification for sudden death in dilated cardiomyopathy: the past, present, and future . Circulation 2017 ; 136 : 215 – 31 . Google Scholar Crossref Search ADS PubMed WorldCat 78 Aquaro GD , Perfetti M , Camastra G , Monti L , Dellegrottaglie S , Moro C et al. Cardiac MR with late gadolinium enhancement in acute myocarditis with preserved systolic function: ITAMY Study . J Am Coll Cardiol 2017 ; 70 : 1977 – 87 . Google Scholar Crossref Search ADS PubMed WorldCat 79 Dass S , Suttie JJ , Piechnik SK , Ferreira VM , Holloway CJ , Banerjee R et al. Myocardial tissue characterization using magnetic resonance noncontrast T1 mapping in hypertrophic and dilated cardiomyopathy . Circ Cardiovasc Imaging 2012 ; 5 : 726 – 33 . Google Scholar Crossref Search ADS PubMed WorldCat 80 Puntmann VO , Carr-White G , Jabbour A , Yu CY , Gebker R , Kelle S et al. T1-mapping and outcome in nonischemic cardiomyopathy: all-cause mortality and heart failure . JACC Cardiovasc Imaging 2016 ; 9 : 40 – 50 . Google Scholar Crossref Search ADS PubMed WorldCat 81 Aus Dem Siepen F , Buss SJ , Messroghli D , Andre F , Lossnitzer D , Seitz S et al. T1 mapping in dilated cardiomyopathy with cardiac magnetic resonance: quantification of diffuse myocardial fibrosis and comparison with endomyocardial biopsy . Eur Heart J Cardiovasc Imaging 2015 ; 16 : 210 – 6 . Google Scholar Crossref Search ADS PubMed WorldCat 82 Arora R , Ferrick KJ , Nakata T , Kaplan RC , Rozengarten M , Latif F et al. I-123 MIBG imaging and heart rate variability analysis to predict the need for an implantable cardioverter defibrillator . J Nucl Cardiol 2003 ; 10 : 121 – 31 . Google Scholar Crossref Search ADS PubMed WorldCat 83 Bristow MR. The adrenergic nervous system in heart failure . N Engl J Med 1984 ; 311 : 850 – 1 . Google Scholar Crossref Search ADS PubMed WorldCat 84 Hachamovitch R , Nutter B , Menon V , Cerqueira MD. Predicting risk versus predicting potential survival benefit using 123I-mIBG imaging in patients with systolic dysfunction eligible for implantable cardiac defibrillator implantation: analysis of data from the prospective ADMIRE-HF study . Circ Cardiovasc Imaging 2015 ; 8 :e003110. WorldCat 85 Klein T , Abdulghani M , Smith M , Huang R , Asoglu R , Remo BF et al. Three-dimensional 123I-meta-iodobenzylguanidine cardiac innervation maps to assess substrate and successful ablation sites for ventricular tachycardia: feasibility study for a novel paradigm of innervation imaging . Circ Arrhythm Electrophysiol 2015 ; 8 : 583 – 91 . Google Scholar Crossref Search ADS PubMed WorldCat 86 Gimelli A , Liga R , Bottai M , Pasanisi EM , Giorgetti A , Fucci S et al. Diastolic dysfunction assessed by ultra-fast cadmium-zinc-telluride cardiac imaging: impact on the evaluation of ischaemia . Eur Heart J Cardiovasc Imaging 2015 ; 16 : 68 – 73 . Google Scholar Crossref Search ADS PubMed WorldCat 87 Gimelli A , Liga R , Avogliero F , Coceani M , Marzullo P. Relationships between left ventricular sympathetic innervation and diastolic dysfunction: the role of myocardial innervation/perfusion mismatch . J Nucl Cardiol 2016 ; 25 : 1101 – 9 . Google Scholar Crossref Search ADS PubMed WorldCat 88 Hasselberg NE , Edvardsen T , Petri H , Berge KE , Leren TP , Bundgaard H et al. Risk prediction of ventricular arrhythmias and myocardial function in Lamin A/C mutation positive subjects . Europace 2014 ; 16 : 563 – 71 . Google Scholar Crossref Search ADS PubMed WorldCat 89 van Berlo JH , Duboc D , Pinto YM. Often seen but rarely recognised: cardiac complications of Lamin A/C mutations . Eur Heart J 2004 ; 25 : 812 – 4 . Google Scholar Crossref Search ADS PubMed WorldCat 90 van Rijsingen IA , Arbustini E , Elliott PM , Mogensen J , Hermans-van Ast JF , van der Kooi AJ et al. Risk factors for malignant ventricular arrhythmias in Lamin A/C mutation carriers a European Cohort Study . J Am Coll Cardiol 2012 ; 59 : 493 – 500 . Google Scholar Crossref Search ADS PubMed WorldCat 91 Pasotti M , Klersy C , Pilotto A , Marziliano N , Rapezzi C , Serio A et al. Long-term outcome and risk stratification in dilated cardiolaminopathies . J Am Coll Cardiol 2008 ; 52 : 1250 – 60 . Google Scholar Crossref Search ADS PubMed WorldCat 92 Haugaa KH , Hasselberg NE , Edvardsen T. Mechanical dispersion by strain echocardiography: a predictor of ventricular arrhythmias in subjects with Lamin A/C mutations . JACC Cardiovasc Imaging 2015 ; 8 : 104 – 6 . Google Scholar Crossref Search ADS PubMed WorldCat 93 Leclercq C , Kass DA. Retiming the failing heart: principles and current clinical status of cardiac resynchronization . J Am Coll Cardiol 2002 ; 39 : 194 – 201 . Google Scholar Crossref Search ADS PubMed WorldCat 94 Chung ES , Leon AR , Tavazzi L , Sun JP , Nihoyannopoulos P , Merlino J et al. Results of the predictors of response to CRT (PROSPECT) trial . Circulation 2008 ; 117 : 2608 – 16 . Google Scholar Crossref Search ADS PubMed WorldCat 95 Ruschitzka F , Abraham WT , Singh JP , Bax JJ , Borer JS , Brugada J et al. Cardiac-resynchronization therapy in heart failure with a narrow QRS complex . N Engl J Med 2013 ; 369 : 1395 – 405 . Google Scholar Crossref Search ADS PubMed WorldCat 96 Stankovic I , Prinz C , Ciarka A , Daraban AM , Kotrc M , Aarones M et al. Relationship of visually assessed apical rocking and septal flash to response and long-term survival following cardiac resynchronization therapy (PREDICT-CRT) . Eur Heart J Cardiovasc Imaging 2016 ; 17 : 262 – 9 . Google Scholar Crossref Search ADS PubMed WorldCat 97 Menet A , Bernard A , Tribouilloy C , Leclercq C , Gevaert C , Guyomar Y et al. Clinical significance of septal deformation patterns in heart failure patients receiving cardiac resynchronization therapy . Eur Heart J Cardiovasc Imaging 2017 ; 18 : 1388 – 97 . Google Scholar Crossref Search ADS PubMed WorldCat 98 Risum N , Tayal B , Hansen TF , Bruun NE , Jensen MT , Lauridsen TK et al. Identification of typical left bundle branch block contraction by strain echocardiography is additive to electrocardiography in prediction of long-term outcome after cardiac resynchronization therapy . J Am Coll Cardiol 2015 ; 66 : 631 – 41 . Google Scholar Crossref Search ADS PubMed WorldCat 99 Parsai C , Bijnens B , Sutherland GR , Baltabaeva A , Claus P , Marciniak M et al. Toward understanding response to cardiac resynchronization therapy: left ventricular dyssynchrony is only one of multiple mechanisms . Eur Heart J 2009 ; 30 : 940 – 9 . Google Scholar Crossref Search ADS PubMed WorldCat 100 Dillon JC , Chang S , Feigenbaum H. Echocardiographic manifestations of left bundle branch block . Circulation 1974 ; 49 : 876 – 80 . Google Scholar Crossref Search ADS PubMed WorldCat 101 Stankovic I , Belmans A , Prinz C , Ciarka A , Maria Daraban A , Kotrc M et al. The association of volumetric response and long-term survival after cardiac resynchronization therapy . Eur Heart J Cardiovasc Imaging 2017 ; 18 : 1109 – 17 . Google Scholar PubMed WorldCat 102 Stankovic I , Aarones M , Smith HJ , Voros G , Kongsgaard E , Neskovic AN et al. Dynamic relationship of left-ventricular dyssynchrony and contractile reserve in patients undergoing cardiac resynchronization therapy . Eur Heart J 2014 ; 35 : 48 – 55 . Google Scholar Crossref Search ADS PubMed WorldCat 103 Voigt JU , Schneider TM , Korder S , Szulik M , Gurel E , Daniel WG et al. Apical transverse motion as surrogate parameter to determine regional left ventricular function inhomogeneities: a new, integrative approach to left ventricular asynchrony assessment . Eur Heart J 2008 ; 30 : 959 – 68 . Google Scholar Crossref Search ADS WorldCat 104 Mada RO , Lysyansky P , Duchenne J , Beyer R , Mada C , Muresan L et al. New automatic tools to identify responders to cardiac resynchronization therapy . J Am Soc Echocardiogr 2016 ; 29 : 966 – 72 . Google Scholar Crossref Search ADS PubMed WorldCat 105 Parsai C , Baltabaeva A , Anderson L , Chaparro M , Bijnens B , Sutherland GR. Low-dose dobutamine stress echo to quantify the degree of remodelling after cardiac resynchronization therapy . Eur Heart J 2008 ; 30 : 950 – 8 . Google Scholar Crossref Search ADS WorldCat 106 Claus P , Omar AMS , Pedrizzetti G , Sengupta PP , Nagel E. Tissue tracking technology for assessing cardiac mechanics: principles, normal values, and clinical applications . JACC Cardiovasc Imaging 2015 ; 8 : 1444 – 60 . Google Scholar Crossref Search ADS PubMed WorldCat 107 AlJaroudi W , Chen J , Jaber WA , Lloyd SG , Cerqueira MD , Marwick T. Nonechocardiographic imaging in evaluation for cardiac resynchronization therapy . Circ Cardiovasc Imaging 2011 ; 4 : 334 – 43 . Google Scholar Crossref Search ADS PubMed WorldCat 108 Nowak B , Sinha AM , Schaefer WM , Koch KC , Kaiser HJ , Hanrath P et al. Cardiac resynchronization therapy homogenizes myocardial glucose metabolism and perfusion in dilated cardiomyopathy and left bundle branch block . J Am Coll Cardiol 2003 ; 41 : 1523 – 8 . Google Scholar Crossref Search ADS PubMed WorldCat 109 Urheim S , Rabben SI , Skulstad H , Lyseggen E , Ihlen H , Smiseth OA. Regional myocardial work by strain Doppler echocardiography and LV pressure: a new method for quantifying myocardial function . Am J Physiol Heart Circ Physiol 2005 ; 288 : H2375 – 80 . Google Scholar Crossref Search ADS PubMed WorldCat 110 Galli E , Leclercq C , Fournet M , Hubert A , Bernard A , Smiseth OA et al. Value of myocardial work estimation in the prediction of response to cardiac resynchronization therapy . J Am Soc Echocardiogr 2018 ; 31 : 220 – 30 . Google Scholar Crossref Search ADS PubMed WorldCat 111 Galli E , Leclercq C , Hubert A , Bernard A , Smiseth OA , Mabo P et al. Role of myocardial constructive work in the identification of responders to CRT . Eur Heart J Cardiovasc Imaging 2018 ; 19 : 1010 – 8 . Google Scholar Crossref Search ADS PubMed WorldCat 112 Galli E , Leclercq C , Donal E. Mechanical dyssynchrony in heart failure: still a valid concept for optimizing treatment? Arch Cardiovasc Dis 2017 ; 110 : 60 – 8 . Google Scholar Crossref Search ADS PubMed WorldCat 113 Marsan NA , Westenberg JJ , Ypenburg C , van Bommel RJ , Roes S , Delgado V et al. Magnetic resonance imaging and response to cardiac resynchronization therapy: relative merits of left ventricular dyssynchrony and scar tissue . Eur Heart J 2009 ; 30 : 2360 – 7 . Google Scholar Crossref Search ADS PubMed WorldCat 114 Hendel RC , Friedrich MG , Schulz-Menger J , Zemmrich C , Bengel F , Berman DS et al. CMR first-pass perfusion for suspected inducible myocardial ischemia . JACC Cardiovasc Imaging 2016 ; 9 : 1338 – 48 . Google Scholar Crossref Search ADS PubMed WorldCat 115 Garbi M , Edvardsen T , Bax J , Petersen SE , McDonagh T , Filippatos G et al. EACVI appropriateness criteria for the use of cardiovascular imaging in heart failure derived from European National Imaging Societies voting . Eur Heart J Cardiovasc Imaging 2016 ; 17 : 711 – 21 . Google Scholar Crossref Search ADS PubMed WorldCat 116 Ellenbogen KA , Gold MR , Meyer TE , Fernndez Lozano I , Mittal S , Waggoner AD et al. Primary results from the SmartDelay determined AV optimization: a comparison to other AV delay methods used in cardiac resynchronization therapy (SMART-AV) trial: a randomized trial comparing empirical, echocardiography-guided, and algorithmic atrioventricular delay programming in cardiac resynchronization therapy . Circulation 2010 ; 122 : 2660 – 8 . Google Scholar Crossref Search ADS PubMed WorldCat 117 Auger D , Hoke U , Bax JJ , Boersma E , Delgado V. Effect of atrioventricular and ventriculoventricular delay optimization on clinical and echocardiographic outcomes of patients treated with cardiac resynchronization therapy: a meta-analysis . Am Heart J 2013 ; 166 : 20 – 9 . Google Scholar Crossref Search ADS PubMed WorldCat 118 Nazarian S , Hansford R , Rahsepar AA , Weltin V , McVeigh D , Gucuk Ipek E et al. Safety of magnetic resonance imaging in patients with cardiac devices . N Engl J Med 2017 ; 377 : 2555 – 64 . Google Scholar Crossref Search ADS PubMed WorldCat 119 Sabzevari K , Oldman J , Herrey AS , Moon JC , Kydd AC , Manisty C. Provision of magnetic resonance imaging for patients with ‘MR-conditional’ cardiac implantable electronic devices: an unmet clinical need . Europace 2017 ; 19 : 425 – 31 . Google Scholar PubMed WorldCat 120 Gold MR , Daubert C , Abraham WT , Ghio S , St John Sutton M , Hudnall JH et al. The effect of reverse remodeling on long-term survival in mildly symptomatic patients with heart failure receiving cardiac resynchronization therapy: results of the REVERSE study . Heart Rhythm 2015 ; 12 : 524 – 30 . Google Scholar Crossref Search ADS PubMed WorldCat 121 Jurcut R , Giusca S , La Gerche A , Vasile S , Ginghina C , Voigt JU. The echocardiographic assessment of the right ventricle: what to do in 2010? Eur J Echocardiogr 2010 ; 11 : 81 – 96 . Google Scholar Crossref Search ADS PubMed WorldCat 122 Grant AD , Smedira NG , Starling RC , Marwick TH. Independent and incremental role of quantitative right ventricular evaluation for the prediction of right ventricular failure after left ventricular assist device implantation . J Am Coll Cardiol 2012 ; 60 : 521 – 8 . Google Scholar Crossref Search ADS PubMed WorldCat 123 Cameli M , Lisi M , Righini FM , Focardi M , Lunghetti S , Bernazzali S et al. Speckle tracking echocardiography as a new technique to evaluate right ventricular function in patients with left ventricular assist device therapy . J Heart Lung Transplant 2013 ; 32 : 424 – 30 . Google Scholar Crossref Search ADS PubMed WorldCat 124 Badano LP , Kolias TJ , Muraru D , Abraham TP , Aurigemma G , Edvardsen T et al. Standardization of left atrial, right ventricular, and right atrial deformation imaging using two-dimensional speckle tracking echocardiography: a consensus document of the EACVI/ASE/Industry Task Force to standardize deformation imaging . Eur Heart J Cardiovasc Imaging 2018 ; 19 : 591 – 600 . Google Scholar Crossref Search ADS PubMed WorldCat 125 Addetia K , Uriel N , Maffessanti F , Sayer G , Adatya S , Kim GH et al. 3D Morphological Changes in LV and RV During LVAD Ramp Studies . JACC Cardiovasc Imaging 2018 ; 11 : 159 – 69 . Google Scholar Crossref Search ADS PubMed WorldCat 126 Uriel N , Sayer G , Addetia K , Fedson S , Kim GH , Rodgers D et al. Hemodynamic ramp tests in patients with left ventricular assist devices . JACC Heart Fail 2016 ; 4 : 208 – 17 . Google Scholar Crossref Search ADS PubMed WorldCat 127 Burkhoff D , Sayer G , Doshi D , Uriel N. Hemodynamics of mechanical circulatory support . J Am Coll Cardiol 2015 ; 66 : 2663 – 74 . Google Scholar Crossref Search ADS PubMed WorldCat 128 Patel JB , Borgeson DD , Barnes ME , Rihal CS , Daly RC , Redfield MM. Mitral regurgitation in patients with advanced systolic heart failure . J Card Fail 2004 ; 10 : 285 – 91 . Google Scholar Crossref Search ADS PubMed WorldCat 129 Zoghbi WA , Adams D , Bonow RO , Enriquez-Sarano M , Foster E , Grayburn PA et al. Recommendations for noninvasive evaluation of native valvular regurgitation: a report from the American Society of Echocardiography developed in collaboration with the society for cardiovascular magnetic resonance . J Am Soc Echocardiogr 2017 ; 30 : 303 – 71 . Google Scholar Crossref Search ADS PubMed WorldCat 130 Kamperidis V , van Wijngaarden SE , van Rosendael PJ , Kong WK , Regeer MV , van der Kley F et al. Mitral valve repair for secondary mitral regurgitation in non-ischaemic dilated cardiomyopathy is associated with left ventricular reverse remodelling and increase of forward flow . Eur Heart J Cardiovasc Imaging 2018 ; 19 : 208 – 15 . Google Scholar Crossref Search ADS PubMed WorldCat 131 Nishimura RA , Vahanian A , Eleid MF , Mack MJ. Mitral valve disease–current management and future challenges . Lancet 2016 ; 387 : 1324 – 34 . Google Scholar Crossref Search ADS PubMed WorldCat 132 Stone GW , Lindenfeld J , Abraham WT , Kar S , Lim DS , Mishell JM et al. Transcatheter mitral-valve repair in patients with heart failure . N Engl J Med 2018 ; 379 : 2307. Google Scholar Crossref Search ADS PubMed WorldCat 133 Obadia JF , Messika-Zeitoun D , Leurent G , Iung B , Bonnet G , Piriou N et al. Percutaneous repair or medical treatment for secondary mitral regurgitation . N Engl J Med 2018 ; 379 : 2297. Google Scholar Crossref Search ADS PubMed WorldCat 134 Grayburn PA , Sannino A , Packer M. Proportionate and disproportionate functional mitral regurgitation: a new conceptual framework that reconciles the results of the MITRA-FR and COAPT trials . JACC Cardiovasc Imaging 2019 ; 12 : 353 – 62 . Google Scholar Crossref Search ADS PubMed WorldCat 135 Garbi M , McDonagh T , Cosyns B , Bucciarelli-Ducci C , Edvardsen T , Kitsiou A et al. Appropriateness criteria for cardiovascular imaging use in heart failure: report of literature review . Eur Heart J Cardiovasc Imaging 2015 ; 16 : 147 – 53 . Google Scholar Crossref Search ADS PubMed WorldCat 136 Donal E , Delgado V , Magne J , Bucciarelli-Ducci C , Leclercq C , Cosyns B et al. Rational and design of EuroCRT: an international observational study on multi-modality imaging and cardiac resynchronization therapy . Eur Heart J Cardiovasc Imaging 2017 ; 18 : 1120 – 7 . Google Scholar Crossref Search ADS PubMed WorldCat 137 Galderisi M , Cardim N , D'Andrea A , Bruder O , Cosyns B , Davin L et al. The multi-modality cardiac imaging approach to the Athlete's heart: an expert consensus of the European Association of Cardiovascular Imaging . Eur Heart J Cardiovasc Imaging 2015 ; 16 : 353. Google Scholar Crossref Search ADS PubMed WorldCat 138 Towbin JA , Lorts A , Jefferies JL. Left ventricular non-compaction cardiomyopathy . Lancet 2015 ; 386 : 813 – 25 . Google Scholar Crossref Search ADS PubMed WorldCat 139 Thavendiranathan P , Dahiya A , Phelan D , Desai MY , Tang WH. Isolated left ventricular non-compaction controversies in diagnostic criteria, adverse outcomes and management . Heart 2013 ; 99 : 681 – 9 . Google Scholar Crossref Search ADS PubMed WorldCat 140 Oechslin E , Jenni R. Left ventricular non-compaction revisited: a distinct phenotype with genetic heterogeneity? Eur Heart J 2011 ; 32 : 1446 – 56 . Google Scholar Crossref Search ADS PubMed WorldCat 141 Estep JD , Stainback RF , Little SH , Torre G , Zoghbi WA et al. The role of echocardiography and other imaging modalities in patients with left ventricular assist devices . JACC Cardioavasc Imaging 2010 ; 3 : 1049 – 64 . Google Scholar Crossref Search ADS WorldCat Author notes Member of the European Reference Network on Rare or low prevalence Heart diseases (ERN GUARD-HEART). Bernard Cosyns and Bogdan A. Popescu authors share the senior position in the list of authors. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2019. For permissions, please email: journals.permissions@oup.com. This article is published and distributed under the terms of the Oxford University Press, Standard Journals Publication Model (https://academic.oup.com/journals/pages/open_access/funder_policies/chorus/standard_publication_model)
TI - Multimodality imaging in the diagnosis, risk stratification, and management of patients with dilated cardiomyopathies: an expert consensus document from the European Association of Cardiovascular Imaging
JF - European Heart Journal - Cardiovascular Imaging
DO - 10.1093/ehjci/jez178
DA - 2019-10-01
UR - https://www.deepdyve.com/lp/oxford-university-press/multimodality-imaging-in-the-diagnosis-risk-stratification-and-c8AV269vhh
SP - 1075
VL - 20
IS - 10
DP - DeepDyve
ER -