Use Of Cardiac Magnetic Resonance Research Articles

Association between Left Ventricular Wall Thickness and Heart Rate Variability with Complex Ventricular Arrhythmia in Hypertrophic Cardiomyopathy

Objective: To demonstrate the association between the maximal left ventricular (LV) wall thickness and the late gadolinium enhancement (LGE) by cardiac magnetic resonance (CMR) and heart rate variability (HRV) with complex ventricular arrhythmia (VA) in patients with hypertrophic cardiomyopathy (HCM). Materials and Methods: The present study was a retrospective study. Fifty-seven HCM patients who underwent CMR and Holter monitoring at Siriraj Hospital, Bangkok, Thailand, were identified and included. Complex VA was defined as Lown grading system type 4a or 4b as couplet or non-sustained ventricular tachycardia. Standard deviation of normal RR-interval (SDNN) was used to assess HRV. Results: The authors studied 57 patients, 40 (70.2%) patients had LGE, and 13 (22.8%) patients had complex VA. Complex VA was demonstrated in 11 (27.5%) patients in the LGE group compared with two (11.8%) in those without (p=0.304), and four (57.1%), and nine (18.0%) in patients had a maximal LV wall thickness of 25 mm or more and less than 25 mm, respectively (p=0.041). SDNN was 100.98±29.82 ms versus 123.85±35.12 ms (p=0.015) in the LGE group compared with the no-LGE group, and 89.08±25.39 ms versus 113.34±33.06 ms (p=0.018) in patients with and without complex VA. Multivariate analysis showed significant associations of maximal LV wall thickness of 25 mm or more and SDNN of less than 105 ms with complex VA with odds ratios of 6.71 (95% confidence interval [CI] 1.09 to 41.14; p=0.040) and 5.15 (95% CI 1.14 to 23.30; p=0.033), respectively. Conclusion: In HCM patients, increased LV wall thickness and a reduction in SDNN are associated with complex VA. The present study supports the utility of CMR and HRV for risk stratification of patients with HCM. Keywords: Heart rate variability; Maximal left ventricular wall thickness; Complex ventricular arrhythmia; Hypertrophic cardiomyopathy

Society for Cardiovascular Magnetic Resonance/European Society of Cardiovascular Imaging/American Society of Echocardiography/Society for Pediatric Radiology/North American Society for Cardiovascular Imaging Guidelines for the Use of Cardiac Magnetic Resonance in Pediatric Congenital and Acquired Heart Disease: Endorsed by The American Heart Association.

Cardiovascular magnetic resonance has been utilized in the management and care of pediatric patients for nearly 40 years. It has evolved to become an invaluable tool in the assessment of the littlest of hearts for diagnosis, pre-interventional management and follow-up care. Although mentioned in a number of consensus and guidelines documents, an up-to-date, large, stand-alone guidance work for the use of cardiovascular magnetic resonance in pediatric congenital 36 and acquired 35 heart disease endorsed by numerous Societies involved in the care of these children is lacking. This guidelines document outlines the use of cardiovascular magnetic resonance in this patient population for a significant number of heart lesions in this age group and although admittedly, is not an exhaustive treatment, it does deal with an expansive list of many common clinical issues encountered in daily practice.

Diagnostic Yield of Cardiac Magnetic Resonance in Athletes with and without Features of the Athlete's Heart and Suspected Structural Heart Disease.

Cardiac magnetic resonance (CMR) is a second-line imaging test in cardiology. Balanced enlargement of heart chambers called athlete’s heart (AH) is a part of physiological adaptation to regular physical activity. The aim of this study was to evaluate the diagnostic utility of CMR in athletes with suspected structural heart disease (SHD) and to analyse the relation between the coexistence of AH and SHD. We wanted to assess whether the presence of AH phenotype could be considered as a sign of a healthy heart less prone to development of SHD. This retrospective, single centre study included 154 consecutive athletes (57 non-amateur, all sports categories, 87% male, mean age 34 ± 12 years) referred for CMR because of suspected SHD. The suspicion was based on existing guidelines including electrocardiographic and/or echocardiographic changes suggestive of abnormality but without a formal diagnosis. CMR permitted establishment of a new diagnosis in 66 patients (42%). The main diagnoses included myocardial fibrosis typical for prior myocarditis (n = 21), hypertrophic cardiomyopathy (n = 17, including 6 apical forms), other cardiomyopathies (n = 10) and prior myocardial infarction (n = 6). Athlete’s heart was diagnosed in 59 athletes (38%). The presence of pathologic late gadolinium enhancement (LGE) was found in 41 patients (27%) and was not higher in athletes without AH (32% vs. 19%, p = 0.08). Junction-point LGE was more prevalent in patients with AH phenotype (22% vs. 9%, p = 0.02). Patients without AH were not more likely to be diagnosed with SHD than those with AH (49% vs. 32%, p = 0.05). Based on the results of CMR and other tests, three patients (2%) were referred for ICD implantation for the primary prevention of sudden cardiac death with one patient experiencing adequate intervention during follow-up. The inclusion of CMR into the diagnostic process leads to a new diagnosis in many athletes with suspicion of SHD and equivocal routine tests. Athletes with AH pattern are equally likely to be diagnosed with SHD in comparison to those without AH phenotype. This shows that the development of AH and SHD can occur in parallel, which makes differential diagnosis in this group of patients more challenging.

Myocardial oedema: pathophysiological basis and implications for the failing heart.

Myocardial fluid homeostasis relies on a complex interplay between microvascular filtration, interstitial hydration, cardiomyocyte water uptake and lymphatic removal. Dysregulation of one or more of these mechanisms may result in myocardial oedema. Interstitial and intracellular fluid accumulation disrupts myocardial architecture, intercellular communication, and metabolic pathways, decreasing contractility and increasing myocardial stiffness. The widespread use of cardiac magnetic resonance enabled the identification of myocardial oedema as a clinically relevant imaging finding with prognostic implications in several types of heart failure. Furthermore, growing experimental evidence has contributed to a better understanding of the physical and molecular interactions in the microvascular barrier, myocardial interstitium and lymphatics and how they might be disrupted in heart failure. In this review, we summarize current knowledge on the factors controlling myocardial water balance in the healthy and failing heart and pinpoint the new potential therapeutic avenues.

Symptomatic Young Adults with ST-Segment Elevation-Acute Coronary Syndrome or Myocarditis: The Three-Factor Diagnostic Model.

Myocarditis may mimic myocardial infarction (MI) due to a similar clinical presentation, including chest pain, electrocardiography changes, and laboratory findings. The purpose of the study was to investigate the diagnostic value of clinical, laboratory, and electrocardiography characteristics of patients with acute coronary syndrome - like myocarditis and MI. We analysed 90 patients (≤45 years old) with an initial diagnosis of ST-segment elevation myocardial infarction; 40 patients (44.4%), through the use of cardiac magnetic resonance, were confirmed to have myocarditis, and 50 patients (55.6%) were diagnosed with MI. Patients with myocarditis were younger and had fewer cardiovascular risk factors than those with MI. The cutoff value distinguishing between myocarditis and MI was defined as the age of 36 years. The history of recent infections (82.5% vs. 6%) and C-reactive protein (CRP) levels on admission (Me 45.9 vs. 3.4) was more associated with myocarditis. Further, the QTc interval was inversely correlated with the echocardiographic ejection fraction in both groups but was significantly longer in patients with MI. Non-invasive diagnostics based on clinical features and laboratory findings are basic but still essential tools for differentiation between MI and myocarditis. The three-factor model including the criteria of age, abnormal CRP, and history of recent infections might become a valuable clinical indication.

From Other Journals: A Review of Recent Articles by Our Editorial Team.

In this review, we provide a brief description of recently published articles addressing topics relevant to pediatric cardiologists. Our hope is to provide a summary of the latest articles published recently in other journals in our field. The articles address the extracardiac anomalies in fetuses with congenital heart disease, post COVID-19 vaccination myocarditis, the use of cardiac magnetic resonance after the Fontan operation, congenitally corrected transposition in adults, robitically assisted congenital heart surgery and the increased risk of congenital heart surgery in patients receiving tracheal surgery during the same admission.

A case report of myocardial infarction with non-obstructive coronary arteries as the initial presentation of eosinophilic granulomatosis with polyangiitis

BackgroundEosinophilic granulomatosis with polyangiitis (EGPA) is a multisystem disorder commonly affecting the lung and skin, with cardiovascular involvement found in up to 60% of patients. We present a case of myocardial infarction with non-obstructive coronary arteries (MINOCA) as the initial presentation of EGPA.Case summaryA 52-year-old female with past medical history of asthma, recurrent sinusitis, and peripheral neuropathy presented to our hospital with chest pain, rash, acute vision loss, elevated troponin, and peripheral eosinophilia. Electrocardiogram showed no ischaemic changes and coronary angiography displayed normal coronary anatomy. On a subsequent visit, cardiac magnetic resonance (CMR) showed predominant focal anteroseptal and inferoseptal akinesis with focal sub-endocardial delayed enhancement, indicative of a myocardial infarction involving the septal branches of the left anterior descending artery. Due to the focal findings on CMR, peripheral eosinophilia, and rash, the patient was evaluated for EGPA. Rheumatologic workup and skin biopsy were suggestive of small vessel vasculitis. The patient was diagnosed with multi-organ EGPA, involving the coronaries, which was ultimately thought to be the aetiology of her MINOCA. Following steroid and monoclonal antibody therapy, the patient experienced notable improvement in her cardiac function at follow-up appointments.DiscussionThis is a unique case MINOCA as the initial presentation of EGPA. Considering the heterogeneous disease presentation of those diagnosed with MINOCA, utilization of CMR is essential to guide diagnosis and management of such patients.

Multiparametric Cardiac Magnetic Resonance Imaging Detects Altered Myocardial Tissue and Function in Heart Transplantation Recipients Monitored for Cardiac Allograft Vasculopathy.

Cardiac allograft vasculopathy (CAV) is a complication beyond the first-year post-heart transplantation (HTx). We aimed to test the utility of cardiac magnetic resonance (CMR) to detect functional/structural changes in HTx recipients with CAV. Seventy-seven prospectively recruited HTx recipients beyond the first-year post-HTx and 18 healthy controls underwent CMR, including cine imaging of ventricular function and T1- and T2-mapping to assess myocardial tissue changes. Data analysis included quantification of global cardiac function and regional T2, T1 and extracellular volume based on the 16-segment model. International Society for Heart and Lung Transplantation criteria was used to adjudicate CAV grade (0-3) based on coronary angiography. The majority of HTx recipients (73%) presented with CAV (1: n = 42, 2/3: n = 14, 0: n = 21). Global and segmental T2 (49.5 ± 3.4 ms vs 50.6 ± 3.4 ms, p < 0.001;16/16 segments) were significantly elevated in CAV-0 compared to controls. When comparing CAV-2/3 to CAV-1, global and segmental T2 were significantly increased (53.6 ± 3.2 ms vs. 50.6 ± 2.9 ms, p < 0.001; 16/16 segments) and left ventricular ejection fraction was significantly decreased (54 ± 9% vs. 59 ± 9%, p < 0.05). No global, structural, or functional differences were seen between CAV-0 and CAV-1. Transplanted hearts display functional and structural alteration compared to native hearts, even in those without evidence of macrovasculopathy (CAV-0). In addition, CMR tissue parameters were sensitive to changes in CAV-1 vs. 2/3 (mild vs. moderate/severe). Further studies are warranted to evaluate the diagnostic value of CMR for the detection and classification of CAV.

Disclosing the cardiomyopathic substrate within the heart muscles in amyloidosis by cardiac magnetic resonance: diagnostic and prognostic implications

The use of cardiac magnetic resonance (CMR) for accurate morphological assessment of cardiomyopathies is well described. CMR tissue characterization with late gadolinium enhancement and parametric mapping is highly promising in differentiating key etiologies of left ventricular hypertrophy, diagnosing cardiac involvement in systemic amyloidosis, detecting early disease, and tracking changes over time, as well as providing valuable prognostic information. This review focuses on the typical imaging findings in cardiac amyloidosis by CMR, highlighting its potentials with respect to traditional imaging techniques. Furthermore, the diagnostic and prognostic role of CMR parameters and the future perspectives related to the newest applications are addressed.

Electromagnetic Exposure of Personnel Involved in Cardiac MRI Examinations in 1.5T, 3T and 7T Scanners.

(1) Background: It has been hypothesised that a significant increase in the use of cardiac magnetic resonance (CMR), for example, when examining COVID-19 convalescents using magnetic resonance imaging (MRI), has an influence the exposure profiles of medical personnel to static magnetic fields (STmf). (2) Methods: Static exposure to STmf (SEmf) was recorded during activities that modelled performing CMR by radiographers. The motion-induced time variability of that exposure (TVEmf) was calculated from SEmf samples. The results were compared with: (i) labour law requirements; (ii) the distribution of vertigo perception probability near MRI magnets; and (iii) the exposure profile when actually performing a head MRI. (3) Results: The exposure profiles of personnel managing 42 CMR scans (modelled using medium (1.5T), high (3T) and ultrahigh (7T) field scanners) were significantly different than when managing a head MRI. The majority of SEmf and TVEmf samples (up to the 95th percentile) were at low vertigo perception probability (SEmf < 500 mT, TVEmf < 600 mT/s), but a small fraction were at medium/high levels; (4) Conclusion: Even under the “normal working conditions” defined for SEmf (STmf < 2T) by labour legislation (Directive 2013/35/EC), increased CMR usage increases vertigo-related hazards experienced by MRI personnel (a re-evaluation of electromagnetic safety hazards is suggested in the case of these or similar changes in work organisation).

Diagnostic Utility of Cardiac Magnetic Resonance in Recurrent "Third Time" Myocarditis Without Electrocardiographic Changes

A few studies have reported on recurrent myocarditis occurring more than twice in one patient. In this study, we present a recurrent "third time" acute myocarditis in a young female Japanese patient with a history of a definitive diagnosis of lymphocytic myocarditis by endomyocardial biopsy, cardiac magnetic resonance imaging (CMR), and catheter examination twice in the past. Although chest pain and an increase in the cardiac enzymes were observed the third time, no significant changes were noted in the 12-lead electrocardiogram (ECG), and a definitive diagnosis could be achieved by CMR. This case suggested that in patients with a history of myocarditis, if there is chest pain and elevated cardiac enzymes even without any changes in the 12-lead ECG, acute myocarditis should be considered, and CMR is useful for the differentiation.Only four case reports including this present case were found through the previous literatures. More than two recurrent episodes of myocarditis have been extremely rare, but all cases have typical chest symptoms and a troponin level increase, leading to a relatively benign prognosis.

Abstract 9693: Predicting Biventricular Repair in Common Atrioventricular Canal by Cardiac Magnetic Resonance Imaging: The New Normal?

Introduction: Patients with malaligned common atrioventricular (AV) canal (CAVC) are difficult to manage when one ventricle is borderline hypoplastic. The decision between the single ventricle pathway or attempting a biventricular repair depends upon pre-operative parameters which have previously been studied only by echocardiography. The purpose of this study was to determine the utility of cardiac magnetic resonance (CMR) in predicting success of a biventricular repair in CAVC. Methods: We performed a single center, retrospective cohort study of children with varying degrees of CAVC malalignment who underwent CMR prior to definitive repair (7/1/10 to 2/28/21). The primary outcome was successful biventricular repair. CMR measurements were performed by the investigators. To account for both dominant right ventricles (RV) and left ventricles (LV), ratios of ventricular and AV valve size were normalized as differences from their expected values. Associations were tested using univariable logistic regression. Multivariable regression models using combinations of two variables with p-values less than 0.2 were tested. Results: We included 28 patients (median age at CMR: 5.2 months, 43% male). Clinical and CMR variables are shown in Table 1. The optimal multivariable predictive model included the normalized LV end diastolic volume (EDV) to total ventricular EDV ratio and the LV-RV angle in diastole. Area under the receiver operator characteristic curve was 0.83 (p = 0.041). An LV EDV to total EDV ratio between 0.30 and 0.66 had a sensitivity of 92% and specificity of 63% for predicting successful biventricular repair. Including an LV-RV angle in diastole of less than 72 degrees resulted in a sensitivity of 83% and specificity of 81%. Conclusions: Multiple CMR measures were associated with surgical outcomes. CMR measures such as LV EDV to total ventricular EDV ratio and LV-RV angle in diastole can accurately predict successful biventricular canal repair.

Left ventricular myocardial deformation assessment in asymptomatic patients with recently diagnosed sarcoidosis of the respiratory tract and/or extrapulmonary sarcoidosis

BackgroundSarcoidosis is a systemic granulomatous disease affecting different organs including the heart. Myocardial strain analysis could potentially detect the early stages of cardiac dysfunction in sarcoidosis patients. The present study aims to assess the use of cardiac magnetic resonance (CMR) strain analysis using feature tracking (FT) in the detection of early cardiac involvement in asymptomatic patients with sarcoidosis.MethodsOne hundred and thirteen CMR studies of patients with sarcoidosis of the respiratory tract and/or extrapulmonary sarcoidosis without pre-existing known cardiovascular disease were included in the study and analysed using FT and compared to 22 age and gender-matched controls. Global longitudinal strain (GLS), global circumferential strain (GCS) and global radial strain (GRS) of the left ventricle (LV) were measured.ResultsThe sarcoidosis patients did not significantly differ from the controls in basic demographic data and had normal global and regional systolic LV function—LV ejection fraction (EF) 66 ± 7% vs 65 ± 5% in the controls (p = NS). No statistically significant differences were found in all strain parameters between patients and controls: GLS (− 13.9 ± 3.1 vs. − 14.2 ± 2.5), GCS (− 23.4 ± 4.0 vs. − 22.2 ± 2.9) and GRS (53.4 ± 13.5 vs. 51.2 ± 13.6%) (p = NS).ConclusionPatients with sarcoidosis of the respiratory tract and/or extrapulmonary sarcoidosis had normal myocardial deformation measured by CMR-FT derived global strain

Cardiac magnetic resonance in athletes with ventricular arrhythmias

Abstract Funding Acknowledgements Type of funding sources: None. Background Ventricular arrhythmias in athletes represent an important issue in sport eligibility as they may be a marker of a concealed cardiomyopathy, potentially at risk of sudden cardiac death during sport activity. Purpose to assess the utility of cardiac magnetic resonance (CMR) to identify cardiac abnormalities in athletes with documented frequent premature ventricular beats (PVB) or complex ventricular arrhythmias (VA) undergoing competitive sport eligibility screening. Methods We enrolled 65 athletes with documented ventricular arrhythmias as follows: 1) frequent PVB at 24-hour ambulatory ECG monitoring (&amp;gt;100 PVB/24h); 2) polymorphic PVB; 3) repetitive PVB (couplets, triplets, or nonsustained ventricular tachicardia). All athletes were studied with rest ECG, exercise stress testing, 24-hour ambulatory ECG monitoring, transthoracic echocardiography and contrast-enhanced CMR. Results Sixty-five athletes (56 males, 9 females, age 30 ± 15years) underwent CMR with gadolinium contrast. In 55 patients (84.6%) CMR was normal, in 10 patients (15.4%) the following abnormalities were reported at CMR: four patients with isolated late gadolinium enhancement (LGE) of whom 3 patients with subepicardial LGE in the inferolateral wall and 1 patient with intramyocardial basal inferior LGE; 1 patient with hypertrophic cardiomyopathy with septal LGE; 1 patient with left ventricular non compaction; 3 patients with right ventricular abnormalities (a 13-year-old patient with dilated right ventricle and LGE located at the superior interventricular insertion point, 1 patient with a dilated and hypokinetic right ventricle, 1 patient with a focal right ventricular aneurysm). Transthoracic echocardiography was normal in 7/10 (70%) athletes with CMR abnormalities. Conclusion In athletes with frequent or complex ventricular arrhythmias cardiac magnetic resonance identifies cardiac abnormalities in a significant proportion of subjects and it may offer a diagnostic incremental value.

Could we use cardiac MRIfor diagnosis of infective endocarditis

Abstract Funding Acknowledgements Type of funding sources: None. Introduction Echocardiography is a reliable imaging tool in diagnosing infective endocarditis (IE) according to "modified Dukes criteria"; however, it lacks tissue characterization of cardiac masses, and transthoracic echocardiography (TTE) has low sensitivity in detection of vegetations. Moreover, transesophageal echo (TEE) is considered semi-invasive and intolerable in some situations .On the other hand, cardiac magnetic resonance (CMR) is a powerful true 3D imaging tool with wide field of view, and superior capability of tissue characterization of cardiac masses; however, modest information is available about utility of CMR in diagnosing IE. Purpose To assess the feasibility of CMR to identify vegetations and complications of IE, and compare obtained findings with those obtained from echocardiography regarding: presence, number, size of vegetations, and detection of cardiac complications (e.g. aortic root abscess, periannular abscess, and shunts). Materials and Methods Eighty consecutive patients with suspected IE were enrolled in the study. All patients underwent TTE; only those with left-sided lesions underwent TEE. When clinical situation allowed, CMR examination using 1.5 Tesla magnet were performed using the following sequences: Steady-state free precession (SSFP), T1W (± fat suppression) and T2W, first-pass perfusion, and delayed hyperenhancement. Moreover, chest and abdominal survey was done. Results Sixty-one patients (45 males and 16 females) were able to undergo and complete CMR study. Affection of tricuspid valve was seen in 39.3%, mitral 31.1%, aortic 24.6%, and pulmonary 4.9% of cases. All vegetations visualized by echocardiography were also clearly detected and confirmed by CMR. The sensitivity, specificity, accuracy and Kappa agreement of CMR with echocardiography in depicting &amp;gt;0.5cm vegetations were all 100%. By tissue characterization, vegetations resemble features of thrombi (with variation in signal intensity according to age of vegetation). However, in some cases, masses have a unique pattern that is different from vegetations of IE, and subsequently other diagnoses were suggested (e.g. fibroelastoma, Libman-Saks endocarditis). In 22 patients, CMR provided more information than echocardiography, and in 6 patients, CMR made a paradigm shift of diagnosis. Conclusion Cardiac MRI is a powerful imaging tool in diagnosis of IE and its complications. In comparison with echocardiography, CMR can identify the presence, numbers, and size of vegetations accurately. Moreover, with its unique ability of tissue characterization, CMR helps distinguish vegetations from other masses; therefore changes the diagnosis of IE and subsequent management in some patients.

Description and Clinical Implications of Myocardial Clefts Using Echocardiography.

BackgroundMyocardial clefts (MCs) are rare anomalies with debatable clinical significance. Increased use of cardiac magnetic resonance (CMR) has led to the appreciation of subtle left ventricular (LV) wall structural defects, and studies showed varying clinical significance, ranging from asymptomatic incidental findings to being considered a novel imaging marker of hypertrophic cardiomyopathy. Sparse data are available about the utility of two-dimensional echocardiography (2DE) to visualize these anomalies. We describe our institutional experience categorizing MCs using 2DE.MethodsThe echocardiography database was retrospectively queried for diagnosing MCs using Synapse® Cardiovascular Picture Archiving and Communication System (PACS) (Fujifilm, Tokyo, Japan). Identified patients were admitted to Detroit Medical Center (DMC) between January 2012 and May 2019. MCs were defined as recesses filled with luminal blood, obliterate during systole, and have U, wedge, and tunnel shapes. Images were interpreted by a cardiologist blinded to the data. Baseline demographics and clinical characteristics were documented. The study was descriptive; no intervention was done.ResultsSixteen patients with a mean age of 62.43 were included; 68.75% were women, and 81.25% were African American. The prevalence of cardiac comorbidities was primary hypertension 12 (75%), coronary artery disease 5 (31.25%), heart failure with reduced ejection fraction (HFrEF) 4 (25.0%), valvular heart disease 4 (25.0%), arrhythmia/heart block 4 (25.0%), and heart failure with preserved ejection fraction (HFpEF) 2 (12.5%). The indications for 2DE evaluation were heart failure/cardiogenic shock 2 (12.5%), acute coronary syndrome 2 (12.5%), syncope/presyncope 2 (12.5%), atypical chest pain 2 (12.5%), and others 8 (50.0%). Twenty-one MCs were visualized in eight segments of LV walls and septum as follows: basal inferior 7, mid inferoseptal 6, mid inferior 3, mid anteroseptal 2, mid inferolateral 1, mid anterolateral 1, basal inferoseptal 1, apical inferoseptal 1, and apical septal 1. Morphology was classified as tunnel in 66.66%, wedge in 23.8%, and U in 9.5%.ConclusionIn various LV and septal walls, MCs detected on 2DE were benign and incidental findings without significant implications for preclinical hypertrophic cardiomyopathy (HCM).

Arrhythmogenic Left Ventricular Cardiomyopathy: Genotype-Phenotype Correlations and New Diagnostic Criteria.

Arrhythmogenic cardiomyopathy (ACM) is an inherited heart muscle disease characterized by loss of ventricular myocardium and fibrofatty replacement, which predisposes to scar-related ventricular arrhythmias and sudden cardiac death, particularly in the young and athletes. Although in its original description the disease was characterized by an exclusive or at least predominant right ventricle (RV) involvement, it has been demonstrated that the fibrofatty scar can also localize in the left ventricle (LV), with the LV lesion that can equalize or even overcome that of the RV. While the right-dominant form is typically associated with mutations in genes encoding for desmosomal proteins, other (non-desmosomal) mutations have been showed to cause the biventricular and left-dominant variants. This has led to a critical evaluation of the 2010 International Task Force criteria, which exclusively addressed the right phenotypic manifestations of ACM. An International Expert consensus document has been recently developed to provide upgraded criteria (“the Padua Criteria”) for the diagnosis of the whole spectrum of ACM phenotypes, particularly left-dominant forms, highlighting the use of cardiac magnetic resonance. This review aims to offer an overview of the current knowledge on the genetic basis, the phenotypic expressions, and the diagnosis of left-sided variants, both biventricular and left-dominant, of ACM.

Tricuspid Annulus Disjunction: Novel Findings by Cardiac Magnetic Resonance in Patients With Mitral Annulus Disjunction

ObjectivesThis study aimed to assess whether patients with MAD also have disjunction of the tricuspid annulus. BackgroundMitral annulus disjunction (MAD) is an abnormal atrial displacement of the mitral annulus. Whether the disjunction extends to the right side of the heart is not known. MethodsIn a cohort of patients with MAD, we assessed the presence of tricuspid annulus disjunction (TAD) with the use of cardiac magnetic resonance. We explored the associations between TAD and MAD characteristics and the relationship to ventricular arrhythmias (nonsustained/sustained ventricular tachycardias and aborted cardiac arrest). ResultsWe included 84 patients (mean age: 48 ± 16 years; 63% female). We observed TAD in 42 (50%). Patients with TAD were older (age 52 ± 16 years vs. 43 ± 15 years; p = 0.02), had greater circumferential extent of MAD (164 ± 57° vs. 115 ± 58°; p = 0.002), greater maximum longitudinal MAD distance (9.4 ± 2.9 mm vs. 6.2 ± 2.8 mm; p < 0.001), and more frequent mitral valve prolapse (n = 39 [92%] vs. n = 24 [57%]; p < 0.001). Ventricular arrhythmias had occurred in 34 patients (41%), who were younger (age 39 ± 14 years vs. 54 ± 14 years; p < 0.001) and had lower prevalence of TAD (n = 22 [29%] vs. n = 12 [52%]; p = 0.03). TAD was not associated with ventricular arrhythmias when adjusted for age (odds ratio adjusted for age: 0.54; 95% confidence interval: 0.20 to 1.45; p = 0.22). ConclusionsWe report for the first time the existence of right-sided annulus disjunction as a common finding in patients with MAD. TAD was associated with more severe left-sided annulus disjunction and mitral valve prolapse, but not with ventricular arrhythmias.

Identifying Myocardial Infarction Using Hierarchical Template Matching-Based Myocardial Strain: Algorithm Development and Usability Study.

BackgroundMyocardial infarction (MI; location and extent of infarction) can be determined by late enhancement cardiac magnetic resonance (CMR) imaging, which requires the injection of a potentially harmful gadolinium-based contrast agent (GBCA). Alternatively, emerging research in the area of myocardial strain has shown potential to identify MI using strain values.ObjectiveThis study aims to identify the location of MI by developing an applied algorithmic method of circumferential strain (CS) values, which are derived through a novel hierarchical template matching (HTM) method.MethodsHTM-based CS H-spread from end-diastole to end-systole was used to develop an applied method. Grid-tagging magnetic resonance imaging was used to calculate strain values in the left ventricular (LV) myocardium, followed by the 16-segment American Heart Association model. The data set was used with k-fold cross-validation to estimate the percentage reduction of H-spread among infarcted and noninfarcted LV segments. A total of 43 participants (38 MI and 5 healthy) who underwent CMR imaging were retrospectively selected. Infarcted segments detected by using this method were validated by comparison with late enhancement CMR, and the diagnostic performance of the applied algorithmic method was evaluated with a receiver operating characteristic curve test.ResultsThe H-spread of the CS was reduced in infarcted segments compared with noninfarcted segments of the LV. The reductions were 30% in basal segments, 30% in midventricular segments, and 20% in apical LV segments. The diagnostic accuracy of detection, using the reported method, was represented by area under the curve values, which were 0.85, 0.82, and 0.87 for basal, midventricular, and apical slices, respectively, demonstrating good agreement with the late-gadolinium enhancement–based detections.ConclusionsThe proposed applied algorithmic method has the potential to accurately identify the location of infarcted LV segments without the administration of late-gadolinium enhancement. Such an approach adds the potential to safely identify MI, potentially reduce patient scanning time, and extend the utility of CMR in patients who are contraindicated for the use of GBCA.

Current use of cardiac magnetic resonance in tertiary referral centres for the diagnosis of cardiomyopathy: the ESC EORP Cardiomyopathy/Myocarditis Registry.

Aims Cardiac magnetic resonance (CMR) is recommended in the diagnosis of cardiomyopathies, but it is time-consuming, expensive, and limited in availability in some European regions. The aim of this study was to determine the use of CMR in cardiomyopathy patients enrolled into the European Society of Cardiology (ESC) cardiomyopathy registry [part of the EURObservational Research Programme (EORP)].Methods and results Three thousand, two hundred, and eight consecutive adult patients (34.6% female; median age: 53.0 ± 15 years) with cardiomyopathy were studied: 1260 with dilated (DCM), 1739 with hypertrophic (HCM), 66 with restrictive (RCM), and 143 with arrhythmogenic right ventricular cardiomyopathy (ARVC). CMR scans were performed at baseline in only 29.4% of patients. CMR utilization was variable according to cardiomyopathy subtypes: from 51.1% in ARVC to 36.4% in RCM, 33.8% in HCM, and 20.6% in DCM (P < 0.001). CMR use in tertiary referral centres located in different European countries varied from 1% to 63.2%. Patients undergoing CMR were younger, less symptomatic, less frequently had implantable cardioverter-defibrillator (ICD)/pacemaker implanted, had fewer cardiovascular risk factors and comorbidities (P < 0.001). In 28.6% of patients, CMR was used along with transthoracic echocardiography (TTE); 67.6% patients underwent TTE alone, and 0.9% only CMR.Conclusion Less than one-third of patients enrolled in the registry underwent CMR and the use varied greatly between cardiomyopathy subtypes, clinical profiles of patients, and European tertiary referral centres. This gap with current guidelines needs to be considered carefully by scientific societies to promote wider availability and use of CMR in patients with cardiomyopathies.