Role of Imaging Modalities in the Evaluation of Nonischemic Cardiomyopathy

A special issue of Diagnostics (ISSN 2075-4418). This special issue belongs to the section "Medical Imaging and Theranostics".

Deadline for manuscript submissions: closed (30 April 2021) | Viewed by 80188

Special Issue Editor


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Guest Editor
Department of Cardiology, Onassis Cardiac Surgery Center, 17674 Athens, Greece
Interests: genes in genetic and inflammatory cardiomyopathies; imaging in genetic and inflammatory cardiomyopathies; brain and heart in genetic and inflammatory cardiomyopathies
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Special Issue Information

Dear Colleagues,

Patients with nonischemic cardiomyopathies (NICM) constitute 50% of the total number of patients with cardiovascular diseases. Until recently, the only way to evaluate them was the use of left ventricular ejection fraction, which gives us a rough idea about the disease pathophysiology.

Recently, the tremendous development of various noninvasive imaging modalities allowed the detailed evaluation not only of the patients with NICM but also the identification of carriers and those with phenotypically mild disease. Furthermore, they facilitated the classification of various types of NICM according to disease severity and provided useful information about disease prediction.

In this Special Issue of Diagnostics, we will describe the strong and weak points of each modality in parallel with the cost–benefit ratio for both patients and health care system. Finally, we will focus on the clinical role of various imaging modalities in the evaluation of NICM and give an overview of the present and future perspectives of these modalities in both diagnosis and prognosis of NICM.

Prof. Dr. Sophie Mavrogeni
Guest Editor

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Keywords

  • Nonischemic cardiomyopathy
  • Echocardiography
  • SPECT
  • PET
  • Cardiovascular Magnetic Resonance
  • CT
  • Dilated cardiomyopathy
  • Hypertrophic cardiomyopathy
  • restrictive cardiomyopathy
  • neuromuscular disorders
  • myocardial inflammation
  • Fabry disease
  • Danon disease
  • Autoimmune rheumatic diseases
  • metabolic diseases

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Published Papers (17 papers)

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Research

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11 pages, 1186 KiB  
Article
Pitfalls of the Semi-Quantitative Analyzing 99mTc-Pyrophosphate Planar Images for Diagnosing Transthyretin Cardiac Amyloidosis: A Possible Solution
by Yuankai Zhu, Ruping Pan, Dan Peng, Qingjian Dong and Xiaohua Zhu
Diagnostics 2022, 12(1), 94; https://doi.org/10.3390/diagnostics12010094 - 1 Jan 2022
Viewed by 4547
Abstract
Background: Two different approaches, 1-h heart-to-contralateral (H/CL) ratio and 3-h visual grading scale relative to ribs (VGSr), have been established to interpret 99mTc-PYP planar images for the detection of amyloid transthyretin cardiac amyloidosis (ATTR-CA). Since they are prone to pitfalls, this pilot [...] Read more.
Background: Two different approaches, 1-h heart-to-contralateral (H/CL) ratio and 3-h visual grading scale relative to ribs (VGSr), have been established to interpret 99mTc-PYP planar images for the detection of amyloid transthyretin cardiac amyloidosis (ATTR-CA). Since they are prone to pitfalls, this pilot study aimed to explore the diagnostic practicality of the 3-h visual grading scale relative to the upper segment of sternum (VGSs) approach for interpreting 99mTc-PYP planar images. Methods: A total of 42 patients were enrolled in this retrospective study. SPECT/CT approach and planar approaches including H/CL ratio, VGSr, and VGSs were utilized to interpret the 99mTc-PYP images obtained at both 1 and 3 h. The classification criteria of the latest expert consensus recommendations were considered as the gold standard. The concordance between the interpretation of each approach and the gold standard was investigated. Results: In addition to 1- and 3-h SPECT/CT approaches, the interpretation of planar images using the 3-h VGSs approach was also applicable, which turns identical to the gold standard (κ = 1.000; p < 0.001). Conclusions: For the interpretation of 99mTc-PYP planar images, the 3-h VGSs approach should be the optimal method, particularly in the case without available or feasible tomography imaging. Only one imaging session (planar and SPECT/CT) at 3 h would be sufficient for the detection of ATTR-CA, and favorable for patient satisfaction. Full article
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12 pages, 706 KiB  
Article
Anthropometric and Biochemical Markers as Possible Indicators of Left Ventricular Abnormal Geometric Pattern and Function Impairment in Obese Normotensive Children
by Filippina Giannisi, Anastasia Keivanidou, Ioanna Sakellari, Sofia Balala, Maria Hassapidou, Areti Hitoglou-Makedou and Andreas Giannopoulos
Diagnostics 2020, 10(7), 468; https://doi.org/10.3390/diagnostics10070468 - 10 Jul 2020
Cited by 4 | Viewed by 2452
Abstract
Εmerging data indicate that various effects of obesity on the cardiovascular system can be evident during childhood. The aim of this study was to detect early changes in left ventricular structure and function in obese normotensive children and explore possible associations of these [...] Read more.
Εmerging data indicate that various effects of obesity on the cardiovascular system can be evident during childhood. The aim of this study was to detect early changes in left ventricular structure and function in obese normotensive children and explore possible associations of these changes with anthropometric and biochemical parameters. Normotensive 8–11-year-old obese and normal weight children were included in the study. They all underwent anthropometric measurements, laboratory tests, and echocardiography study by conventional and tissue Doppler to assess geometric pattern and function of left ventricle. Statistically significant differences in most anthropometric and metabolic parameters were noticed between groups. Obese children showed higher left ventricular mass index (LVMI) (40.05 ± 9.44 vs. 28.31 ± 6.22), lower E/A ratio (1.76 ± 0.33 vs. 2.08 ± 0.56), and higher E/e’ (6.04 ± 1.13 vs. 5.43 ± 0.96) compared to lean peers. Waist-to-height ratio and hs-CRP correlated significantly with E/A in the obese group. Left ventricular hypertrophy was present in 47.2% of obese children and eccentric was the prominent type. Waist-to-height ratio and serum cortisol levels in plasma increased the odds of having any type of abnormal ventricular geometric pattern. Echocardiographic evaluation of left ventricle and diastolic function could be considered for obese normotensive children based on waist-to-height ratio, hs-CRP, and serum cortisol. Full article
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14 pages, 1339 KiB  
Article
The Double-Edged Sword of T1-Mapping in Systemic Sclerosis—A Comparison with Infectious Myocarditis Using Cardiovascular Magnetic Resonance
by George Markousis-Mavrogenis, Loukia Koutsogeorgopoulou, Gikas Katsifis, Theodoros Dimitroulas, Genovefa Kolovou, George D. Kitas, Petros P. Sfikakis and Sophie I. Mavrogeni
Diagnostics 2020, 10(5), 335; https://doi.org/10.3390/diagnostics10050335 - 24 May 2020
Cited by 12 | Viewed by 2994
Abstract
Aims: T1-mapping is considered a surrogate marker of acute myocardial inflammation. However, in diffuse cutaneous systemic sclerosis (dcSSc) this might be confounded by coexisting myocardial fibrosis. We hypothesized that T1-based indices should not by themselves be considered as indicators of myocardial inflammation in [...] Read more.
Aims: T1-mapping is considered a surrogate marker of acute myocardial inflammation. However, in diffuse cutaneous systemic sclerosis (dcSSc) this might be confounded by coexisting myocardial fibrosis. We hypothesized that T1-based indices should not by themselves be considered as indicators of myocardial inflammation in dcSSc patients. Methods/Results: A cohort of 59 dcSSc and 34 infectious myocarditis patients was prospectively evaluated using a 1.5-Tesla system for an indication of suspected myocardial inflammation and was compared with 31 healthy controls. Collectively, 33 (97%) and 57 (98%) of myocarditis and dcSSc patients respectively had ≥1 pathologic T2-based index. However, 33 (97%) and 45 (76%) of myocarditis and dcSSc patients respectively had ≥1 pathologic T2-based index. T2-signal ratio was significantly higher in myocarditis patients compared with dcSSc patients (2.5 (0.6) vs. 2.1 (0.4), p < 0.001). Early gadolinium enhancement, late gadolinium enhancement and T2-mapping did not differ significantly between groups. However, both native T1-mapping and extracellular volume fraction were significantly lower in myocarditis compared with dcSSc patients (1051.0 (1027.0, 1099.0) vs. 1120.0 (1065.0, 1170.0), p < 0.001 and 28.0 (26.0, 30.0) vs. 31.5 (30.0, 33.0), p < 0.001, respectively). The original Lake Louise criteria (LLc) were positive in 34 (100%) myocarditis and 40 (69%) dcSSc patients, while the updated LLc were positive in 32 (94%) and 44 (76%) patients, respectively. Both criteria had good agreement with greater but nonsignificant discordance in dcSSc patients. Conclusions: ~25% of dcSSc patients with suspected myocardial inflammation had no CMR evidence of acute inflammatory processes. T1-based indices should not be used by themselves as surrogates of acute myocardial inflammation in dcSSc patients. Full article
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9 pages, 578 KiB  
Article
High-Sensitivity Troponin I and Creatinine Kinase-Myocardial Band in Screening for Myocardial Injury in Patients with Carbon Monoxide Poisoning
by June-Sung Kim, Byuk Sung Ko, Chang Hwan Sohn, Youn-Jung Kim and Won Young Kim
Diagnostics 2020, 10(4), 242; https://doi.org/10.3390/diagnostics10040242 - 21 Apr 2020
Cited by 10 | Viewed by 3816
Abstract
Myocardial dysfunction due to acute carbon monoxide (CO) poisoning is common and associated with poor outcomes. The role of cardiac markers, including creatine kinase-myocardial band (CK-MB), high-sensitivity troponin I (hsTnI), and brain natriuretic peptide (BNP), in identifying patients with CO-induced cardiomyopathy were evaluated. [...] Read more.
Myocardial dysfunction due to acute carbon monoxide (CO) poisoning is common and associated with poor outcomes. The role of cardiac markers, including creatine kinase-myocardial band (CK-MB), high-sensitivity troponin I (hsTnI), and brain natriuretic peptide (BNP), in identifying patients with CO-induced cardiomyopathy were evaluated. This single-center, retrospective cohort study included 905 consecutive adult patients in the CO poisoning registry from February 2009 to December 2019. Cardiomyopathy was defined as any abnormality on transthoracic echocardiography (TTE), including left ventricular systolic and diastolic dysfunction, right ventricular dysfunction, and wall motion abnormalities. The areas under receiver operating curves (AUCs) for biomarkers were compared. Of the 850 included patients, 101 (11.9%) had CO-induced cardiomyopathy. Initial and peak hsTnI and CK-MB concentrations, and initial BNP concentrations were significantly higher in patients with than without cardiomyopathy (all P-values < 0.01), but the AUCs were higher for hsTnI (0.894) and CK-MB (0.864) than for BNP (0.796). Initial TnI > 0.01 ng/mL and CK-MB > 1.5 ng/mL each had 95% sensitivity and 97% negative predictive value for CO-induced cardiomyopathy. Higher hsTnI or CK-MB levels on admission can identify patients at high-risk of CO-induced cardiomyopathy and can be a screening tool for CO poisoning. Full article
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11 pages, 569 KiB  
Article
Cardiovascular Magnetic Resonance Identifies High-Risk Systemic Sclerosis Patients with Normal Echocardiograms and Provides Incremental Prognostic Value
by George Markousis-Mavrogenis, Vasiliki-Kalliopi Bournia, Stylianos Panopoulos, Loukia Koutsogeorgopoulou, George Kanoupakis, Dimitrios Apostolou, Gikas Katsifis, Michail Polychroniadis, Theodoros Dimitroulas, Genovefa Kolovou, George D. Kitas, Sophie I. Mavrogeni and Petros P. Sfikakis
Diagnostics 2019, 9(4), 220; https://doi.org/10.3390/diagnostics9040220 - 11 Dec 2019
Cited by 26 | Viewed by 3278
Abstract
Background: Acute cardiac events are a significant contributor to mortality in systemic sclerosis (SSc). However, echocardiographic evaluation may be deceptively normal during an acute presentation. We hypothesized that in diffuse SSc patients presenting with acute cardiac events and a normal echocardiogram, cardiovascular magnetic [...] Read more.
Background: Acute cardiac events are a significant contributor to mortality in systemic sclerosis (SSc). However, echocardiographic evaluation may be deceptively normal during an acute presentation. We hypothesized that in diffuse SSc patients presenting with acute cardiac events and a normal echocardiogram, cardiovascular magnetic resonance (CMR) would have incremental diagnostic/prognostic value. Methods. 50 consecutive diffuse SSc patients with normal echocardiograms were evaluated using a 1.5T system. A total of 27 (63%) had experienced an acute cardiac event three to tendays before CMR evaluation (rhythm disturbances, angina pectoris, shortness of breath). Left/right ventricular (LV/RV) volumes and ejection fractions (EF), as well as LV mass, the T2-signal ratio, early/late gadolinium enhancement (EGE/LGE), native/post-contrast T1-mapping, T2-mapping and extracellular volume fraction (ECV) were compared between the event and no-event groups. Results: No differences were identified in LV/RV volumes/EF/mass. In logistic regression analyses, independent predictors of belonging to the event group were EGE (odds ratio (95% CI): 1.55 (1.06–2.26), p = 0.024), LGE (1.81 (1.23–2.67), p = 0.003), T2 mapping (1.20 (1.06–1.36), p = 0.004) and native/post-contrast T1 mapping (1.17 (1.04–1.32), p = 0.007 and 0.86 (0.75–0.98), p = 0.025). At a median follow-up of ~1.2 years, 42% vs. 11% of the event/no-event group respectively reached a combined endpoint of event occurrence/recurrence or cardiovascular mortality. Of the independent predictors resulting from logistic regression analyses, only LGE (hazard ratio (95% CI): 1.20 (1.11–1.30), p < 0.001), T2-mapping (1.07 (1.01–1.14), p = 0.025) and native T1-mapping (1.08 (1.01–1.15), p = 0.017) independently predicted the combined endpoint. Conclusions: A normal echocardiogram does not preclude myocardial lesions in diffuse SSc patients, which can be detected by CMR especially in symptomatic patients. Full article
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Review

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22 pages, 2766 KiB  
Review
Sclerodermic Cardiomyopathy—A State-of-the-Art Review
by Adrian Giucă, Tea Gegenava, Carmen Marina Mihai, Ciprian Jurcuţ, Adrian Săftoiu, Diana Monica Gȋrniţă, Bogdan Alexandru Popescu, Nina Ajmone Marsan and Ruxandra Jurcuț
Diagnostics 2022, 12(3), 669; https://doi.org/10.3390/diagnostics12030669 - 9 Mar 2022
Cited by 8 | Viewed by 5721
Abstract
Systemic sclerosis (SSc) is a chronic autoimmune disorder with unknown triggering factors, and complex pathophysiologic links which lead to fibrosis of skin and internal organs, including the heart, lungs, and gut. However, more than 100 years after the first description of cardiac disease [...] Read more.
Systemic sclerosis (SSc) is a chronic autoimmune disorder with unknown triggering factors, and complex pathophysiologic links which lead to fibrosis of skin and internal organs, including the heart, lungs, and gut. However, more than 100 years after the first description of cardiac disease in SSc, sclerodermic cardiomyopathy (SScCmp) is an underrecognized, occult disease with important adverse long-term prognosis. Laboratory tests, electrocardiography (ECG) and cardiovascular multimodality imaging techniques (transthoracic 2D and 3D echocardiography, cardiac magnetic resonance (CMR), and novel imaging techniques, including myocardial deformation analysis) provide new insights into the cardiac abnormalities in patients with SSc. This state-of-the-art review aims to stratify all the cardiac investigations needed to diagnose and follow-up the SScCmp, and discusses the epidemiology, risk factors and pathophysiology of this important cause of morbidity of the SSc patient. Full article
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21 pages, 2267 KiB  
Review
A Review of the Role of Imaging Modalities in the Evaluation of Viral Myocarditis with a Special Focus on COVID-19-Related Myocarditis
by Adedayo Adeboye, Deya Alkhatib, Asra Butt, Neeraja Yedlapati and Nadish Garg
Diagnostics 2022, 12(2), 549; https://doi.org/10.3390/diagnostics12020549 - 21 Feb 2022
Cited by 25 | Viewed by 9896
Abstract
Viral myocarditis is inflammation of the myocardium secondary to viral infection. The clinical presentation of viral myocarditis is very heterogeneous and can range from nonspecific symptoms of malaise and fatigue in subclinical disease to a more florid presentation, such as acute cardiogenic shock [...] Read more.
Viral myocarditis is inflammation of the myocardium secondary to viral infection. The clinical presentation of viral myocarditis is very heterogeneous and can range from nonspecific symptoms of malaise and fatigue in subclinical disease to a more florid presentation, such as acute cardiogenic shock and sudden cardiac death in severe cases. The accurate and prompt diagnosis of viral myocarditis is very challenging. Endomyocardial biopsy is considered to be the gold standard test to confirm viral myocarditis; however, it is an invasive procedure, and the sensitivity is low when myocardial involvement is focal. Cardiac imaging hence plays an essential role in the noninvasive evaluation of viral myocarditis. The current coronavirus disease 2019 (COVID-19) pandemic has generated considerable interest in the use of imaging in the early detection of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2)-related myocarditis. This article reviews the role of various cardiac imaging modalities used in the diagnosis and assessment of viral myocarditis, including COVID-19-related myocarditis. Full article
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13 pages, 1704 KiB  
Review
The Role of Cardiovascular Magnetic Resonance Imaging in the Evaluation of Hypertrophic Cardiomyopathy
by Sanjay Sivalokanathan
Diagnostics 2022, 12(2), 314; https://doi.org/10.3390/diagnostics12020314 - 26 Jan 2022
Cited by 9 | Viewed by 5246
Abstract
Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiac disorder, affecting 1 out of 500 adults globally. It is a widely heterogeneous disorder characterized by a range of phenotypic expressions, and is most often identified by non-invasive imaging that includes echocardiography and cardiovascular [...] Read more.
Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiac disorder, affecting 1 out of 500 adults globally. It is a widely heterogeneous disorder characterized by a range of phenotypic expressions, and is most often identified by non-invasive imaging that includes echocardiography and cardiovascular magnetic resonance imaging (CMR). Within the last two decades, cardiac magnetic resonance imaging (MRI) has emerged as the defining tool for the characterization and prognostication of cardiomyopathies. With a higher image quality, spatial resolution, and the identification of morphological variants of HCM, CMR has become the gold standard imaging modality in the assessment of HCM. Moreover, it has been crucial in its management, as well as adding prognostic information that clinical history nor other imaging modalities may not provide. This literature review addresses the role and current applications of CMR, its capacity in evaluating HCM, and its limitations. Full article
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15 pages, 1526 KiB  
Review
Cardiac Resynchronization Therapy in Non-Ischemic Cardiomyopathy: Role of Multimodality Imaging
by Cristian Stătescu, Carina Ureche, Ștefana Enachi, Rodica Radu and Radu A. Sascău
Diagnostics 2021, 11(4), 625; https://doi.org/10.3390/diagnostics11040625 - 30 Mar 2021
Cited by 4 | Viewed by 2803
Abstract
Non-ischemic cardiomyopathy encompasses a heterogeneous group of diseases, with a generally unfavorable long-term prognosis. Cardiac resynchronization therapy (CRT) is a useful therapeutic option for patients with symptomatic heart failure, currently recommended by all available guidelines, with outstanding benefits, especially in non-ischemic dilated cardiomyopathy. [...] Read more.
Non-ischemic cardiomyopathy encompasses a heterogeneous group of diseases, with a generally unfavorable long-term prognosis. Cardiac resynchronization therapy (CRT) is a useful therapeutic option for patients with symptomatic heart failure, currently recommended by all available guidelines, with outstanding benefits, especially in non-ischemic dilated cardiomyopathy. Still, in spite of clear indications based on identifying a dyssynchronous pattern on the electrocardiogram (ECG,) a great proportion of patients are non-responders. The idea that multimodality cardiac imaging can play a role in refining the selection criteria and the implant technique and help with subsequent system optimization is promising. In this regard, predictors of CRT response, such as apical rocking and septal flash have been identified. Promising new data come from studies using cardiac magnetic resonance and nuclear imaging for showcasing myocardial dyssynchrony. Still, to date, no single imaging predictor has been included in the guidelines, probably due to lack of validation in large, multicenter cohorts. This review provides an up-to-date synthesis of the latest evidence of CRT use in non-ischemic cardiomyopathy and highlights the potential additional value of multimodality imaging for improving CRT response in this population. By incorporating all these findings into our clinical practice, we can aim toward obtaining a higher proportion of responders and improve the success rate of CRT. Full article
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24 pages, 4344 KiB  
Review
The Importance of Multimodality Imaging in the Diagnosis and Management of Patients with Infiltrative Cardiomyopathies: An Update
by Radu Sascău, Larisa Anghel, Alexandra Clement, Mădălina Bostan, Rodica Radu and Cristian Stătescu
Diagnostics 2021, 11(2), 256; https://doi.org/10.3390/diagnostics11020256 - 7 Feb 2021
Cited by 4 | Viewed by 3884
Abstract
Infiltrative cardiomyopathies (ICMs) comprise a broad spectrum of inherited and acquired conditions (mainly amyloidosis, sarcoidosis, and hemochromatosis), where the progressive buildup of abnormal substances within the myocardium results in left ventricular hypertrophy and manifests as restrictive physiology. Noninvasive multimodality imaging has gradually eliminated [...] Read more.
Infiltrative cardiomyopathies (ICMs) comprise a broad spectrum of inherited and acquired conditions (mainly amyloidosis, sarcoidosis, and hemochromatosis), where the progressive buildup of abnormal substances within the myocardium results in left ventricular hypertrophy and manifests as restrictive physiology. Noninvasive multimodality imaging has gradually eliminated endomyocardial biopsy from the diagnostic workup of infiltrative cardiac deposition diseases. However, even with modern imaging techniques’ widespread availability, these pathologies persist in being largely under- or misdiagnosed. Considering the advent of novel, revolutionary pharmacotherapies for cardiac amyloidosis, the archetypal example of ICM, a standardized diagnostic approach is warranted. Therefore, this review aims to emphasize the importance of contemporary cardiac imaging in identifying specific ICM and improving outcomes via the prompt initiation of a targeted treatment. Full article
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12 pages, 2258 KiB  
Review
Cardiac MRI and Myocardial Injury in COVID-19: Diagnosis, Risk Stratification and Prognosis
by Saagar K. Sanghvi, Logan S. Schwarzman and Noreen T. Nazir
Diagnostics 2021, 11(1), 130; https://doi.org/10.3390/diagnostics11010130 - 15 Jan 2021
Cited by 24 | Viewed by 7222
Abstract
Myocardial injury is a common complication of the COVID-19 illness and is associated with a worsened prognosis. Systemic hyperinflammation seen in the advanced stage of COVID-19 likely contributes to myocardial injury. Cardiac magnetic resonance imaging (CMR) is the preferred imaging modality for non-invasive [...] Read more.
Myocardial injury is a common complication of the COVID-19 illness and is associated with a worsened prognosis. Systemic hyperinflammation seen in the advanced stage of COVID-19 likely contributes to myocardial injury. Cardiac magnetic resonance imaging (CMR) is the preferred imaging modality for non-invasive evaluation in acute myocarditis, enabling risk stratification and prognostication. Modified scanning protocols in the pandemic setting reduce risk of exposure while providing critical data regarding cardiac tissue inflammation and fibrosis, chamber remodeling, and contractile function. The growing use of CMR in clinical practice to assess myocardial injury will improve understanding of the acute and chronic sequelae of myocardial inflammation from various pathological etiologies. Full article
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20 pages, 13230 KiB  
Review
Role of CMR Mapping Techniques in Cardiac Hypertrophic Phenotype
by Andrea Baggiano, Alberico Del Torto, Marco Guglielmo, Giuseppe Muscogiuri, Laura Fusini, Mario Babbaro, Ada Collevecchio, Rocco Mollace, Stefano Scafuri, Saima Mushtaq, Edoardo Conte, Andrea Daniele Annoni, Alberto Formenti, Maria Elisabetta Mancini, Giulia Mostardini, Daniele Andreini, Andrea Igoren Guaricci, Mauro Pepi, Marianna Fontana and Gianluca Pontone
Diagnostics 2020, 10(10), 770; https://doi.org/10.3390/diagnostics10100770 - 29 Sep 2020
Cited by 24 | Viewed by 5346
Abstract
Non-ischemic cardiomyopathies represent a heterogeneous group of myocardial diseases potentially leading to heart failure, life-threatening arrhythmias, and eventually death. Myocardial dysfunction is associated with different underlying pathological processes, ultimately inducing changes in morphological appearance. Thus, classification based on presenting morphological phenotypes has been [...] Read more.
Non-ischemic cardiomyopathies represent a heterogeneous group of myocardial diseases potentially leading to heart failure, life-threatening arrhythmias, and eventually death. Myocardial dysfunction is associated with different underlying pathological processes, ultimately inducing changes in morphological appearance. Thus, classification based on presenting morphological phenotypes has been proposed, i.e., dilated, hypertrophic, restrictive, and right ventricular cardiomyopathies. In light of the key diagnostic and prognostic role of morphological and functional features, cardiovascular imaging has emerged as key element in the clinical workflow of suspected cardiomyopathies, and above all, cardiovascular magnetic resonance (CMR) represents the ideal technique to be used: thanks to its physical principles, besides optimal spatial and temporal resolutions, incomparable contrast resolution allows to assess myocardial tissue abnormalities in detail. Traditionally, weighted images and late enhancement images after gadolinium-based contrast agent administration have been used to perform tissue characterization, but in the last decade quantitative assessment of pre-contrast longitudinal relaxation time (native T1), post-contrast longitudinal relaxation time (post-contrast T1) and transversal relaxation time (T2), all displayed with dedicated pixel-wise color-coded maps (mapping), has contributed to give precious knowledge insight, with positive influence of diagnostic accuracy and prognosis assessment, mostly in the setting of the hypertrophic phenotype. This review aims to describe the available evidence of the role of mapping techniques in the assessment of hypertrophic phenotype, and to suggest their integration in the routine CMR evaluation of newly diagnosed cardiomyopathies with increased wall thickness. Full article
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20 pages, 1201 KiB  
Review
Myocardial Function, Heart Failure and Arrhythmia in Marfan Syndrome: A Systematic Literature Review
by Anthony Demolder, Yskert von Kodolitsch, Laura Muiño-Mosquera and Julie De Backer
Diagnostics 2020, 10(10), 751; https://doi.org/10.3390/diagnostics10100751 - 25 Sep 2020
Cited by 18 | Viewed by 8008
Abstract
Marfan syndrome (MFS) is a heritable systemic connective tissue disease with important cardiovascular involvement, including aortic root dilatation and mitral valve prolapse. Life expectancy in patients with MFS is mainly determined by cardiovascular complications, among which aortic dissection or rupture are most dreaded. [...] Read more.
Marfan syndrome (MFS) is a heritable systemic connective tissue disease with important cardiovascular involvement, including aortic root dilatation and mitral valve prolapse. Life expectancy in patients with MFS is mainly determined by cardiovascular complications, among which aortic dissection or rupture are most dreaded. In recent years, heart failure and ventricular arrhythmia have drawn attention as extra-aortic cardiovascular manifestations and as additional reported causes of death. Imaging studies have provided data supporting a primary myocardial impairment in the absence of valvular disease or cardiovascular surgery, while studies using ambulatory ECG have demonstrated an increased susceptibility to ventricular arrhythmia. In this paper, current literature was reviewed in order to provide insights in characteristics, pathophysiology and evolution of myocardial function, heart failure and ventricular arrhythmia in MFS. Full article
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17 pages, 4527 KiB  
Review
The Advantages of New Multimodality Imaging in Choosing the Optimal Management Strategy for Patients with Hypertrophic Cardiomyopathy
by Larisa Anghel, Cristian Stătescu, Ionela-Lăcrămioara Șerban, Minela Aida Mărănducă, Doina Butcovan, Alexandra Clement, Mădălina Bostan and Radu Sascău
Diagnostics 2020, 10(9), 719; https://doi.org/10.3390/diagnostics10090719 - 19 Sep 2020
Cited by 1 | Viewed by 3772
Abstract
In recent years, significant advances have been made in the diagnosis and therapeutic management of hypertrophic cardiomyopathy (HCM) patients, which has led to an important improvement in their longevity and quality of life. The use of multimodality imaging has an essential role in [...] Read more.
In recent years, significant advances have been made in the diagnosis and therapeutic management of hypertrophic cardiomyopathy (HCM) patients, which has led to an important improvement in their longevity and quality of life. The use of multimodality imaging has an essential role in the diagnosis, assessing the regional distribution and severity of the disease, with important prognostic implications. At the same time, imaging contributes to the identification of optimal treatment for patients with hypertrophic cardiomyopathy, whether it is pharmaceutical, interventional or surgical treatment. Novel pharmacotherapies (like myosin inhibitors), minimally invasive procedures (such as transcatheter mitral valve repair, high-intensity focused ultrasound or radiofrequency ablation) and gene-directed approaches, may soon become alternatives for HCM patients. However, there are only few data on the early diagnosis of patients with HCM, in order to initiate treatment as soon as possible, to reduce the risk of sudden cardiac death (SCD). The aim of our review is to highlight the advantages of contemporary imaging in choosing the optimal management strategies for HCM patients, considering the novel therapies which are currently applied or studied for these patients. Full article
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21 pages, 2621 KiB  
Review
The Assessment of Myocardial Strain by Cardiac Imaging in Healthy Infants with Acute Bronchiolitis: A Systematic Review and Meta-Analysis
by Moises Rodriguez-Gonzalez, Alvaro Antonio Perez-Reviriego, Ana Castellano-Martinez and Helena Maria Cascales-Poyatos
Diagnostics 2020, 10(6), 382; https://doi.org/10.3390/diagnostics10060382 - 8 Jun 2020
Cited by 9 | Viewed by 3101
Abstract
This study aims to systematically review the incidence of myocardial strain detected by echocardiography in previously healthy infants with acute bronchiolitis and its role as a predictor for adverse outcomes in this setting. Methods: Pubmed/Medline, Excerpta Medica Data Base (EMBASE), and Cochrane Library [...] Read more.
This study aims to systematically review the incidence of myocardial strain detected by echocardiography in previously healthy infants with acute bronchiolitis and its role as a predictor for adverse outcomes in this setting. Methods: Pubmed/Medline, Excerpta Medica Data Base (EMBASE), and Cochrane Library were searched in April 2020 to identify original observational prospective studies that systematically performed echocardiography for the screening of myocardial strain in healthy infants with acute bronchiolitis. Pooled estimates were generated using random-effects models. Heterogeneity within studies was assessed using Cochran’s Q and I2 statistics. Funnel plots and Egger´s regression method were constructed to evaluate publication bias. Sensitivity analyses were also conducted to evaluate potential sources of heterogeneity. Results: After a detailed screening of 305 articles, a total of 10 studies with 395 participants (mean of 40 participants per study) was included. Five of them were classified as high-quality studies. Up to 28% of cases presented adverse outcomes. The echocardiographic screening for myocardial strain was performed within the first 24 h of admission in 92% cases. Tissue Doppler imaging and Speckle-Tracking echocardiography were performed only in 20% of cases. The presence of pulmonary hypertension was evaluated with methods different from the tricuspid regurgitation jet in 64% of cases. Seven studies found some grade of myocardial strain with a pooled incidence of 21% (CI 95%, 11–31%), in the form of pulmonary hypertension (pooled incidence of 20% (CI 95%, 11–30%)), and myocardial dysfunction (pooled incidence of 5% (CI 95%, 1–9%)). The presence of these echocardiographic alterations was associated with adverse outcomes (pooled relative risk = 16; CI 95%, 8.2–31.5). After a subgroup analysis based on the echocardiographic techniques used, no significant heterogeneity across the studies was observed. There was no evidence of publication bias when assessed by Egger´s test. Cardiac biomarkers to assess myocardial strain were used in five studies. Only N-terminal-pro-brain natriuretic peptide accurately predicted the presence of myocardial strain by echocardiography. Conclusions: Myocardial strain is not infrequent in previously healthy infants with acute bronchiolitis, and it could be present at the early stages of the disease with prognostic implications. There is a need for sufficiently powered prospective studies with a similar methodology, preferably employing advanced imaging techniques, to conclusively address the usefulness of the assessment of myocardial strain in this setting. Full article
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14 pages, 4338 KiB  
Review
Arrhythmogenic Inflammatory Cardiomyopathy in Autoimmune Rheumatic Diseases: A Challenge for Cardio-Rheumatology
by Sophie I. Mavrogeni, George Markousis-Mavrogenis, Constantina Aggeli, Dimitris Tousoulis, George D. Kitas, Genovefa Kolovou, Efstathios K. Iliodromitis and Petros P. Sfikakis
Diagnostics 2019, 9(4), 217; https://doi.org/10.3390/diagnostics9040217 - 10 Dec 2019
Cited by 12 | Viewed by 4543
Abstract
Ventricular arrhythmia (VA) in autoimmune rheumatic diseases (ARD) is an expression of autoimmune inflammatory cardiomyopathy (AIC), caused by structural, electrical, or inflammatory heart disease, and has a serious impact on a patient’s outcome. Myocardial scar of ischemic or nonischemic origin through a re-entry [...] Read more.
Ventricular arrhythmia (VA) in autoimmune rheumatic diseases (ARD) is an expression of autoimmune inflammatory cardiomyopathy (AIC), caused by structural, electrical, or inflammatory heart disease, and has a serious impact on a patient’s outcome. Myocardial scar of ischemic or nonischemic origin through a re-entry mechanism facilitates the development of VA. Additionally, autoimmune myocardial inflammation, either isolated or as a part of the generalized inflammatory process, also facilitates the development of VA through arrhythmogenic autoantibodies and inflammatory channelopathies. The clinical presentation of AIC varies from oligo-asymptomatic presentation to severe VA and sudden cardiac death (SCD). Both positron emission tomography (PET) and cardiovascular magnetic resonance (CMR) can diagnose AIC early and be useful tools for the assessment of therapies during follow-ups. The AIC treatment should be focused on the following: (1) early initiation of cardiac medication, including ACE-inhibitors, b-blockers, and aldosterone antagonists; (2) early initiation of antirheumatic medication, depending on the underlying disease; and (3) potentially implantable cardioverter–defibrillator (ICD) and/or ablation therapy in patients who are at high risk for SCD. Full article
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6 pages, 850 KiB  
Case Report
Acute Myocarditis-Like Episode in a Curly-Haired Young Boy—Red Flags for Familial Arrhythmogenic Cardiomyopathy
by Alina Elena Pătru, Sebastian Onciul, Adrian Sturzu, Eliza Cinteză, Eleonora Gima, Bogdan A. Popescu, Philippe Chevalier and Ruxandra Jurcuț
Diagnostics 2020, 10(9), 651; https://doi.org/10.3390/diagnostics10090651 - 31 Aug 2020
Cited by 1 | Viewed by 2272
Abstract
The present case report describes a mother and son with arrhythmogenic cardiomyopathy (ACM) with early and greater left ventricle (LV) involvement. The presence of curly hair in both, together with the resuscitated sudden cardiac death of the mother, allowed timely genetic testing, which [...] Read more.
The present case report describes a mother and son with arrhythmogenic cardiomyopathy (ACM) with early and greater left ventricle (LV) involvement. The presence of curly hair in both, together with the resuscitated sudden cardiac death of the mother, allowed timely genetic testing, which found a pathogenic nonsense mutation of the desmoplakin gene. While asymptomatic from an arrhythmic point of view, the son’s evolution was characterized by a well-documented exercise-induced myocarditis-like stage. Full article
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