Pattern Of Gadolinium Enhancement Research Articles

P982 Correlation between electrocardiographic and imaging data in patients with acute myocarditis: a single centre retrospective analysis

Abstract Funding Acknowledgements None Background Acute myocarditis is a clinical and pathological condition defined as an inflammation of the myocardium. Its diagnosis is often challenging and requires multiple information derived from different diagnostic modalities. Purpose The aim of the study is to evaluate the correlation between electrocardiographic and imaging data in patients with acute myocarditis. Methods We made a retrospective analysis of 102 patients admitted to our Centre between January 2012 and April 2019 for suspected acute myocarditis. Diagnosis was confirmed with cardiac magnetic resonance (CMR) by identification of myocardial edema in T2-weighted images and/or typical subepicardial or midwall pattern of late gadolinium enhancement (LGE). Significant coronary artery disease was ruled out with coronary angiography. Electrocardiogram (ECG) was analysed on admission - in order to evaluate the presence of ST segment abnormalities, atrio-ventricular or bundle-branch block and heart rhythm disorders - and at the time of discharge. Every patient underwent echocardiography and CMR: from both these exams we reported the presence of regional wall motion abnormalities and left ventricular ejection fraction (LVEF). Results Mean age of our population was 39 ± 18 years; 92 people (90%) were males. At admission, 85 patients (83%) presented ECG abnormalities; the most frequent was ST-segment elevation (65 cases). Conduction or rhythm disorders were observed in 26 cases (25%). At the time of discharge, 41 out of 85 patients had complete regression of ECG changes. Mean value of LVEF measured with echocardiography was 56.4 ± 7.6%. In patients with normal ECG on admission it was 59.9 ± 3.1%, whereas in patients with abnormal ECG 55.7 ± 7.9% (p = 0.045). Considering CMR, mean LVEF in the population was 58.5 ± 8.6%, varying between 64.0 ± 8.9% in the group with normal ECG and 57.4 ± 10.1% in the group with ECG abnormalities (p = 0.02). Moreover, subjects with altered ECG on admission had a higher prevalence of wall motion abnormalities both in echocardiography (47/85 – 55% vs 3/17 – 18%, p < 0.01) and in CMR (45/85 – 53% vs 3/17 – 18%, p < 0.01). Patients with ECG normalization at discharge had a higher prevalence of ST-segment elevation (88 vs 66%, p = 0.02), while the group with persistent ECG alterations had a higher incidence of AV or bundle-branch block (23 vs 7%, p = 0.048). No statistical difference was noted between these two groups regarding echocardiographic or CMR values. Conclusion In our experience evaluation of ECG at admission in patients with suspected acute myocarditis identifies a subgroup of individuals with lower values of LVEF and a higher prevalence of wall motion abnormalities both in echocardiography and in CMR, while data derived by imaging techniques had no significant predictive value on ECG evolution at the time of discharge.

321 Differentiating Myocarditis from Myocardial Infarction in Infarct-Like Presentations Using Troponin and Cardiac MRI

Infarct-like presentations are usually well differentiated on cardiac MRI by the pattern of late gadolinium enhancement (LGE), but transmural LGE can occur. The aim of this study is to determine if there is a difference in the relationship between the peak troponin and the amount of LGE in acute myocarditis and acute myocardial infarction in infarct-like presentations. If so, we aim to define a ratio that can be used as an objective differentiator. 111 patients across two sites were included as they had an infarct-like presentation, troponin rise and cardiac MRI between 2010 and 2018. CVI42 was used for MRI tissue characterisation. Of the 51 patients with myocarditis, 36 (59%) were male and the mean peak troponin I was 10872 ng/L. Of the 61 patients with a myocardial infarction, 47 (77%) were male and the mean peak troponin I was 74133 ng/L. Preliminary results from the analysis of 26 patients showed the LGE mass was significantly greater in the infarct group compared to the myocarditis group (23.8 vs 9.8 grams, p=0.02). There was also a significant greater ratio of peak troponin to LGE mass in the infarct group compared with the myocarditis group (mean ratio 4306:1 vs 557:1, p=0.01). Preliminary results suggest there is a significantly greater peak troponin for the same amount of LGE in infarction compared with myocarditis. Further analysis is underway to confirm these findings over the larger sample size, and validate an objective cut off to differentiate myocarditis from infarction.

P887 Two sides of the same mass

Abstract Echocardiography (echo) remains the first-line imaging modality for the evaluation of cardiac masses. Three-dimensional (3D) echo, either transthoracic or transesophageal (TTE and TEE respectively), has allowed for better definition imaging, providing more information about the size, mobility, attachment and relation of these lesions with cardiac structures. Nevertheless, due to its superior tissue characterization capability, other imaging techniques, such as cardiac magnetic resonance (CMR), are very helpful in the differential diagnosis, making multimodality imaging the most attractive option for the study of intracardiac masses. We present the case of a 85 year-old male, with paroxysmal atrial fibrillation (under effective anticoagulation), type 2 diabetes mellitus, hypertension, referred for the study of an asymptomatic cardiac mass found in a routine TTE. There were no relevant findings on physical examination. The TTE showed a bilobar spheroid mass, in the right atrium, attached to the interatrial septum, with 33x23mm and regular edges. A 3D TEE was performed confirming the previous findings, but also showing extension of this mass through the fossa ovalis membrane, reaching the left atrium; this aspect raised the doubt about either protrusion or invasion of the left atrium and, respectively, a benign (like a myxoma) versus malignant behaviour (such as a sarcoma). To better characterize this lesion, a CMR was ordered, which revealed a bilobar heterogeneous mass, attached to the right side of the interatrial septum, at the fossa ovalis membrane, without signs of adjacent tissue invasion, namely unequivocal invasion of the left atrium; it presented with intermediate T1 signal, hyperintense T2 signal and heterogeneous pattern of gadolinium enhancement, features mostly in favour of a right atrial myxoma. The complimentary study found no other relevant changes, namely no findings suggestive of endocarditis (negative blood cultures), autoimmune disease or malignancy. The patient refused undergoing heart surgery and, therefore, kept follow-up with clinical and echocardiographic stability. Although histological examination remains the only tool for definitive diagnosis, multimodality imaging allows a quite comprehensive evaluation of intracardiac masses, enlightening the differential diagnosis. Here the imaging findings helped to establish a benign origin as the most likely, very important in this case of a probable right atrial myxoma, due to its peculiar protrusion to the left atrium through the fossa ovalis membrane. Abstract P887 Figure. atrial myxoma

P1332 Breaking the dichotomy of myocardial infarction and stress cardiomyopathy

Abstract INTRODUCTION An 83-year-old woman with a past medical history of hypertension, dyslipidaemia and recent diagnosis of disseminated breast cancer was admitted at Emergency department for acute heart failure. She complained about short chest pain episodes on previous days, the ECG showed new T negative waves on precordial leads and high sensitive troponin serum levels were modestly increased (TropT 1200ng/l). Echocardiographic assessment documented moderate left ventricular dysfunction (LVEF 40%) with apical and midventricular dyskinesia associated with hypercontractility off basal segments. The following day the patient underwent a coronary angiography that showed an occlusion in the distal segment of a large obtuse marginal branch (Panel A) with no other significant stenosis in the remaining vessels. Ventriculography showed a typical apical ballooning pattern (Panel B). Despite the occluded coronary artery was reaching the apex, the myocardial wall motion abnormalities extended beyond the ischemic territory. Therefore, a cardiac magnetic resonance (CMR) was performed a few days later in order to clarify the diagnosis. CMR confirmed the mid LVEF dysfunction with apical dyskinesia and the T2 weighted spin echo images showed myocardial oedema in all the apical segments (Panel C). Remarkably, a subedocardial late gadolinium enhancement (LGE) restricted to the apical segment of the inferolateral wall was detected, being consistent with the ischaemic territory of the occluded obtuse marginal branch (Panel D). These findings supported the concomitant diagnosis of an apical stress cardiomyopathy and an acute inferolateral myocardial infarction. The patient was successfully discharged one week later with a normal LVEF and no signs of congestion. DISCUSSION Apical ballooning in the scenario of an acute coronary syndrome may be secondary to both an acute coronary artery occlusion or to stress cardiomyopathy. Cardiac MR has a key role to resolve the differential diagnosis of these two aetiologies, since ischemia typically produces a subendocardial late gadolinium enhancement pattern and stress cardiomyopathy shows an apical oedema without coronary stenosis or extending beyond a localized ischemic territory. Our case is astonishing because in the clinical practice these two diagnoses frequently excludes each other, but as demonstrated in our case, the differential diagnosis between myocardial infarction and stress cardiomyopathy is not a proper dichotomy. Abstract P1332 Figure

Delayed myocardial enhancement in children with different types of cardiomyopathy: a diagnostic and prognostic tool

BackgroundThe pattern of late gadolinium enhancement (LGE) in cardiomyopathy is quite different in children compared to adults. In addition, the data about LGE imaging in children are still restricted, so the goal was to study the role of cardiac magnetic resonance (CMR) with different techniques, including LGE images in diagnosis and evaluation of different types of cardiomyopathy in children.ResultsIn group A (enhancement group), LVEDV 146.2 (144) ml, indexed LVEDV 81.8 (195) ml, LVESV 50 (357) ml, indexed LVESV 47.5 (243) ml, and LVEF 36% (64%), and a major adverse effect was found in 12 out of 15 cases (80%). However, in group B (non-enhancement group), the results were LVEDV 72 (303) ml, indexed LVEDV 75 (318) ml, LVESV 30 (220) ml, indexed LVESV 37.1 (189) ml, and LVEF 45.79% (65%), and a major adverse cardiac effect was found in 2 out of 16 cases (12.5%). The LVEF was lower, and LV volume indices including LVEDV and LVESV were higher in patients with LGE compared to those without LGE with a statistically significant difference (p value = 0.001, p value = 0.003, and p value = 0.005, respectively). Furthermore, it was also found that a major adverse effect occurs with higher incidence in enhancement cases (92%) as compared to non-enhancement cases (12.5%) with a statistically significant difference (p value ≤ 0.001). LGE was found in 15 cases out of 31 cases (48.4%); however, the remaining 13 cases had no contrast study.ConclusionCardiac MRI can be considered as an important non-invasive imaging modality, not only for assessment but also for differentiation between ischemic and non-ischemic cardiomyopathy in the pediatric age group. Using its different techniques allows a better assessment of morphologic and functional parameters in cardiomyopathy. Moreover, the late gadolinium enhancement is regarded as a promising non-invasive tool in the detection and quantification of myocardial scars. That is considered of high importance in diagnosis, categorization, and detection of etiology in most cases of different types of cardiomyopathy, in addition to risk stratification that can be an essential step in patient management.

4088Diagnostic specificity of triangular basal inferoseptal late gadolinium enhancement for identification of cardiac sarcoidosis

Abstract Background/Introduction Sarcoidosis, a multisystem disorder characterized by infiltration of noncaseating granulomas, can manifest as left ventricular (LV) dysfunction and fatal ventricular arrhythmias. The clinical diagnosis of cardiac sarcoidosis (CS) can be challenging and often requires multiple imaging modalities including cardiac magnetic resonance (CMR). The characterization of unique late gadolinium enhancement (LGE) patterns may assist in the diagnosis on CMR. Purpose We sought to examine the diagnostic value of inferoseptal triangular LGE for CS. Methods The retrospective cohort included 149 non-ischemic cardiomyopathy (NICM) patients referred to our hospital for a CMR from January 2012 to December 2018. Left ventricular LGE was identified in 86 patients (56.4±10.4 years, 84.9% male). Amongst them, 73 patients with septal LGE. We classified the various septal LGE patterns into four categories: (A) Mid-wall septal and basal inferoseptal triangular LGE; (B) Endocardial right ventricular (RV) septal LGE; (C) Basal inferoseptal triangular with endocardial RV septal LGE; (D) Mid-wall septal LGE (Figure). The diagnosis of sarcoidosis was confirmed by the Japanese Circulation Society/Japanese Society of Nuclear Cardiology guidelines. Results Individual diagnoses and imaging characteristics of non-ischemic cardiomyopathy types with septal LGE are summarized in the Table. LV ejection fraction, LV end-diastolic volume index (EDVI) and end-systolic volume index (ESVI) did not significantly differ between sarcoidosis and non-sarcoidosis patients. Pattern A and Pattern B were highly specific but insensitive (Specificity/Sensitivity- A: 98.4/8.3%, p=0.19; B: 95.1%/8.3%, p=0.64) for CS. The only pattern with statistically significantly diagnostic indices was pattern C: specificity of 100% and sensitivity of 58.3% (p<0.001). Pattern D revealed both low specificity of 6.6% and sensitivity of 25% (p<0.001). Imaging characteristics and diagnosis Septal LGE/Diagnosis Sarcoidosis Idiopathic Pulmonary hypertension Giant cell myocarditis Amyloidosis Systemic lupus erythematous Myeloproliferative neoplasm Large cell lymphoma Mid-wall septal and basal inferoseptal triangular LGE 1 1 0 0 0 0 0 0 Endocardial RV septal LGE 1 2 0 1 0 0 0 0 Basal inferoseptal triangular andendocardial RV septal LGE 7 0 0 0 0 0 0 0 Mid-wall septal LGE 3 50 3 0 1 1 1 1 Individual diagnosis and patterns of septal late gadolinium enhancement. Septal LGE patterns Conclusions The presence of basal inferoseptal triangular LGE pattern with endocardial RV involvement enables an accurate diagnosis of CS. The absence of this triangular septal LGE pattern, however, cannot exclude the diagnosis of CS. Acknowledgement/Funding Taipei Veterans General Hospital-National Yang-Ming University Excellent Physician Scientists Cultivation Program, No. 106-V-A-009

P5560Assessing etiology in a cohort of patients with myocarditis presenting with complex ventricular arrhythmias: can the percutaneous endomyocardial biopsy help?

Abstract Background Myocarditis represents a common but often under-diagnosed disease, with a wide range of clinical presentations; diagnosis is often presumptive and a clear etiology leading to a specific therapeutic approach is usually not identified. Purpose To describe and assess disease etiology in a cohort of myocarditis patients (pts) with arrhythmic presentation undergoing an invasive diagnostic work-up. Methods All pts with myocarditis presenting with ventricular arrhythmias undergoing an electro-anatomical mapping (EAM) guided endo-myocardial biopsy (EMB) at our institution were enrolled. All enrolled pts also underwent cardiac magnetic resonance imaging (MRI) and an electrophysiological study (EPS). Demographics, arrhythmic presentation, MRI data, arrhythmic inducibility at EPS, EAM and EMB biopsy data were retrieved and analyzed. Molecular biology testing for cardio-tropic virus genome as well as leukocyte immunohistochemical typization were routinely performed on all EMB samples. Results Twenty-six pts were enrolled (85% male, 39±6 y.o.). Clinical presentation was an organized ventricular arrhythmia in 16 (62%) pts (n=3 non-sustained ventricular arrhythmia; n=9 sustained ventricular arrhythmia; n=4 ventricular fibrillation) while frequent (>10.000) premature ventricular complexes (PVCs) in the remaining 10 (38%) pts. MRI showed a late gadolinium enhancement (LGE) pattern consistent with myocarditis in all pts (35% left LGE; 65% right LGE). At the EPS, 10 (38%) pts showed inducibility for SVTs and underwent an intra-cardiac defibrillator (ICD) implant, while 4 (16%) more were implanted for secondary arrhythmic prevention. EAM was performed in 18 (70%), 6 (22%) and 2 (8%) pts in the right, left and in both ventricle respectively; in all cases, abnormal myocardial voltages were retrieved in the area showing LGE at MRI. Extensive myocardial scarring was detected in 7 (27%) pts. All EMB were performed without peri-procedural complications; inflammatory infiltrate and substrate alteration consistent with myocarditis were retrieved in 100% of the bioptic samples. Viral genome was identified in 13 (50%) samples (n=5 Human Herpes Virus 6; n=2 Parvovirus B 19; n=3 Adenovirus; n=1 Ebstein Barr Virus; n=1 Cytomegalovirus; n=1 Rhinovirus) and specific human immunoglobulin treatment was undergone by a single pt; eosinophilic infiltration was found in 2 (8%) patients; lymphocite invasion and auto-antibodies consistent with auto-immune myocarditis were detected in 2 (8%) patients and appropriate immunosuppressive therapy was started, while a myocardial band contraction pattern typical of toxic myocarditis was found in a single (4%) patient [Figure 1]. Different Myocarditis Etiology Rates Conclusion In our myocarditis cohort, EMB confirmed viruses to represented the first myocarditis etiological agent. Despite an invasive work-out, 31% of the cohort etiology still remains unclear.

P3684Detecting true left dominant arrhythmogenic cardiomyopathy: cardiac magnetic resonance imaging and an invasive diagnostic assessment to go beyond current diagnostic criteria

Abstract Background Left-dominant arrhythmogenic cardiomyopathy (LDACM) represents an underdiagnosed subtype of the classical right-dominant ACM, with a fibro-fatty infiltration of the left ventricle ab disease initio. To date, ACM diagnosing criteria do not include any paradigm for LDACM and no shared consensus or position statement has been issued yet. Purpose To analyse the diagnostic work-up needed to reach a definite diagnosis in LDACM patients (pts). Methods All pts with a high clinical suspicion of ACM admitted at our institution were evaluated. Disease and familiar history, and both baseline ECG and cardiac ultrasound (US) were retrieved in all pts. Before invasive evaluation, all pts underwent cardiac magnetic resonance imaging (MRI) for morphology assessment and tissue characterization by late gadolinium enhancement (LGE). An invasive evaluation with an electrophysiological study (EPS) and an endo-cavitary electro-anatomical mapping (EAM) was then subsequently performed; EAM-guided endo-myocardial biopsy (EMB) was performed at physician discretion, for direct histological evaluation of myocardial substrate. Results 30 ACM pts (53±6 y.o.; 66% male) were defined as LDACM; 22 (73%) pts presented unspecific ECG abnormalities, with 8 (27%) pts instead presenting negative t-waves in V4-V6. Cardiac US resulted unremarkable in 27 (90%) pts. Sustained ventricular arrhythmia with right bundle brunch block were experienced in 4 (14%) pts, while frequent premature ventricular beats with the same morphology in 10 (33%). LDACM diagnosis was mainly suspected upon MRI evaluation: all 30 pts presented a late gadolinium enhancement (LGE) pattern revealing an isolate left ventricle fibro-fatty infiltration, with normal biventricular contractility (LV and RV ejection fraction 57±9% and 53±2%, respectively). Right ventricular, left ventricular and biventricular endo-cavitary EAM was performed in 10 (33%), 11 (37%) and 9 (30%) pts respectively, revealing pathologically low unipolar voltages in 7 (23%) and both unipolar and bipolar low voltages in 15 (50%) pts. In 18 (60%) pts an EMB was performed, revealing in 15 (83%) a fibro-fatty infiltrate and a fibro-fatty infiltrated with a superimposed viral myocarditis in a single pt. Genetic testing was performed in 16 (53%) pts, of which 10 (33%) showed causative mutation of desmosomal genes. If strictly adhering to the existing criteria, only 7 (23%) LDACM definite diagnosis would have been reached, even when using EMB and genetic testing. LDACM EAM with late potentials Conclusion LDACM is an underestimated ACM subtype that require MRI evaluation and an invasive work-up for definite diagnosis. Although EMB and genetic testing being the most effective diagnostic tools currently at disposal adhering to existing criteria, a definite diagnosis could be reached only in a fraction of patient population. Existing diagnostic criteria should be revised, mainly to take in consideration EAM specific role and to properly define the LDACM entity.

Magnetic resonance imaging in immune-mediated myelopathies.

Immune-mediated myelopathies are a heterogeneous group of inflammatory spinal cord disorders including autoimmune disorders with known antibodies, e.g. aquaporin-4 IgG channelopathy or anti-myelin oligodendrocyte glycoprotein-associated myelitis, myelopathies in the context of multiple sclerosis and systemic autoimmune disorders with myelopathy, as well as post-infectious and paraneoplastic myelopathies. Although magnetic resonance imaging of the spinal cord is still challenging due to the small dimension of the cord cross-section and frequent movement and susceptibility artifacts, recent methodological advances have led to improved diagnostic evaluation and characterization of immune-mediated myelopathies. Topography, length and width of the lesion, gadolinium enhancement pattern, and changes in morphology over time help in narrowing the broad differential diagnosis. In this review, we give an overview of recent advances in magnetic resonance imaging of immune-mediated myelopathies and its role in the differential diagnosis and monitoring of this heterogeneous group of disorders.

Myocarditis with checkpoint inhibitor immunotherapy: case report of late gadolinium enhancement on cardiac magnetic resonance with pathology correlate

BackgroundNivolumab is a human IgG4 anti-programmed cell death protein-1 (PD-1) monoclonal antibody that works by augmenting the immune response against tumour cells. It has the potential of causing multiple autoimmune-related events, including cardiac. However, the real incidence and diagnosis of cardiac complications remains unclear.Case summaryA 47-year-old woman with a history of carotid artery dissection and metastatic melanoma presented with acute heart failure. One year prior to presentation, she had received one cycle only of checkpoint inhibitor therapy with both ipilimumab and nivolumab, and nivolumab only was restarted 4 months prior to presentation. On admission, she was hypotensive, tachycardic due to atrial tachycardia and with pulmonary oedema. An echocardiogram revealed a left ventricular ejection fraction of 26%. Cardiovascular magnetic resonance (CMR) demonstrated a non-ischaemic pattern of late gadolinium enhancement (LGE), most consistent with myocarditis. The diagnosis of immunotherapy-mediated cardiac toxicity was highly considered and immunosuppressive therapy was initiated. However, she went into refractory cardiogenic shock and did not survive. An autopsy performed with samples from areas of myocardium with and without LGE on the CMR, found correlation.DiscussionAccording to the literature, cardiac complications develop in less than 1% of patients treated with checkpoint inhibitors, with a 0.06% incidence reported in nivolumab specifically. However, it may be higher, given the lack of cardiac monitoring during treatment. We present the first case demonstrating direct histological correlation of T-lymphocytic infiltration with areas of LGE on CMR. Future investigation using CMR for early detection of inflammation and left ventricular dysfunction may help to diagnosis disease earlier.

Regional amyloid distribution and impact on mortality in light-chain amyloidosis: a T1 mapping cardiac magnetic resonance study

Background: T1 mapping allows quantitative assessment of “diffuse” deposition of amyloid protein in the myocardium. Early detection of cardiac involvement and potential prognostic improvement could benefit patients with AL amyloidosis.Objectives: This study aims to evaluate the regional variation of amyloid infiltration in the left ventricle and the prognostic value of T1 mapping in patients with AL amyloidosis.Methods: We prospectively enrolled 77 patients with AL amyloidosis who underwent cardiac magnetic resonance on a 3.0-T scanner. Native T1 and extracellular volume (ECV) were quantitated on the basal, mid, and apical levels of the left ventricle. Late gadolinium enhancement (LGE) pattern (no or non-specific LGE, sub-endocardial LGE, and transmural LGE) was also assessed. Forty healthy subjects served as controls. The primary end point was all-cause mortality.Results: Basal ECV (26.9 ± 2.8% versus 31.1 ± 4.9%, p < .001) were lower than apical ECV in the healthy controls; however, basal ECV (60.6 ± 11.5% versus 53.0 ± 9.6%, p = .003) were significantly higher than apical ECV in patients with transmural LGE. During the follow-up period (median duration, 28 months; 25th–75th percentile, 13.5–38.0 months), 46 patients died. Basal ECV has the largest area under the curve of 0.845 (95% CI, 0.747–0.917) to predict all-cause mortality. Multivariable Cox analysis indicated that basal ECV was an independent prognostic factor and showed incremental prognostic value beyond NYHA class, Mayo stage, and LGE pattern.Conclusion: We demonstrated that T1 mapping may have the potential to detect a characteristic amyloid deposition with a decreasing gradient from base to apex. Furthermore, myocardial ECV indicated that basal amyloid infiltration provided robust and incremental prognostic value in patients with AL amyloidosis.

The Evolving Role of Cardiovascular Magnetic Resonance Imaging in the Evaluation of Systemic Amyloidosis.

Systemic amyloidosis is a serious multiorgan disease with reduced life expectancy, irrespective of type. The impact of magnetic resonance imaging (MRI) in managing this condition has been immense. The last decade in particular has seen a surge of interest in the assessment and evaluation of the heart in patients with systemic amyloidosis by cardiovascular magnetic resonance imaging (CMR), with approximately 85% of all publications on this subject arising in the last 10 years. This has been largely driven by the creation of new sequences and their subsequent modernisation and technical development, thereby rendering previously prohibitive methods clinically more relevant and applicable. In turn, this has led to an increased awareness and recognition of the disease. This review demonstrates how MRI has become a pivotal diagnostic tool in the assessment of cardiac amyloidosis over the last 2 decades, with the ability to track disease and predict mortality. Several different pathognomonic patterns of late gadolinium enhancement (LGE) are now recognised and are able to prognosticate. T1 mapping and extracellular volume (ECV) techniques have resulted in even earlier disease detection before LGE is even visible and along with T2 mapping, provide new insights into biology. As newer therapies also evolve and become available, the need for accurate tracking of cardiac disease response to treatment carries increasing importance. All these are examined in this review, mainly focussing on light-chain (AL) and transthyretin (ATTR) amyloidosis.

Prognostic value of novel imaging parameters derived from standard cardiovascular magnetic resonance in high risk patients with systemic light chain amyloidosis

BackgroundThe differentiated assessment of functional parameters besides morphological changes is essential for the evaluation of prognosis in systemic immunoglobulin light chain (AL) amyloidosis.MethodsSeventy-four subjects with AL amyloidosis and presence of late gadolinium enhancement (LGE) pattern typical for cardiac amyloidosis were analyzed. Long axis strain (LAS) and myocardial contraction fraction (MCF), as well as morphological and functional markers, were measured. The primary endpoint was death, while death and heart transplantation served as a composite secondary endpoint.ResultsAfter a median follow-up of 41 months, 29 out of 74 patients died and 10 received a heart transplant. Left ventricular (LV) functional parameters were reduced in patients, who met the composite endpoint (LV ejection fraction 51% vs. 61%, LAS − 6.9% vs − 10%, GLS − 12% vs − 15% and MCF 42% vs. 69%; p < 0.001 for all). In unadjusted univariate analysis, LAS (HR = 1.05, p < 0.001) and MCF (HR = 0.96, p < 0.001) were associated with reduced transplant-free survival. Kaplan-Meier analyses showed a significantly lower event-free survival in patients with reduced MCF. MCF and LAS performed best to identify high risk patients for secondary endpoint (Log-rank test p < 0.001) in a combined model. Using sequential Cox regression analysis, the addition of LAS and MCF to LV ejection fraction led to a significant increase in the predictive power of the model (χ2 (df = 1) = 28.2, p < 0.001).ConclusionsLAS and MCF as routinely available and robust CMR-derived parameters predict outcome in LGE positive AL amyloidosis. Patients with impaired LV function in combination with reduced LAS and MCF are at the highest risk for death and heart transplantation.

Gadolinium and Multiple Sclerosis: Vessels, Barriers of the Brain, and Glymphatics.

The pathogenesis of multiple sclerosis is characterized by a cascade of pathobiologic events, ranging from focal lymphocytic infiltration and microglia activation to demyelination and axonal degeneration. MS has several of the hallmarks of an inflammatory autoimmune disorder, including breakdown of the BBB. Gadolinium-enhanced MR imaging is currently the reference standard to detect active inflammatory lesions in MS. Knowledge of the patterns and mechanisms of contrast enhancement is vital to limit the radiologic differential diagnosis in the staging and evaluation of MS lesion activity. The aim of this review was the following: 1) to outline the pathophysiology of the effect of lymphocyte-driven inflammation in MS, 2) to describe the effects of gadolinium on the BBB and glymphatic system, and 3) to describe gadolinium enhancement patterns and artifacts that can mimic lesions in MS.

Outcome in Dilated Cardiomyopathy Related to the Extent, Location, and Pattern of Late Gadolinium Enhancement

ObjectivesThis study sought to investigate the association between the extent, location, and pattern of late gadolinium enhancement (LGE) and outcome in a large dilated cardiomyopathy (DCM) cohort. BackgroundThe relationship between LGE and prognosis in DCM is incompletely understood. MethodsThe authors examined the association between LGE and all-cause mortality and a sudden cardiac death (SCD) composite based on the extent, location, and pattern of LGE in DCM. ResultsOf 874 patients (588 men, median age 52 years) followed for a median of 4.9 years, 300 (34.3%) had nonischemic LGE. Estimated adjusted hazard ratios for patients with an LGE extent of 0 to 2.55%, 2.55% to 5.10%, and >5.10%, respectively, were 1.59 (95% confidence interval [CI]: 0.99 to 2.55), 1.56 (95% CI: 0.96 to 2.54), and 2.31 (95% CI: 1.50 to 3.55) for all-cause mortality, and 2.79 (95% CI: 1.42 to 5.49), 3.86 (95% CI: 2.09 to 7.13), and 4.87 (95% CI: 2.78 to 8.53) for the SCD endpoint. There was a marked nonlinear relationship between LGE extent and outcome such that even small amounts of LGE predicted a substantial increase in risk. The presence of septal LGE was associated with increased mortality, but SCD was most associated with the combined presence of septal and free-wall LGE. Predictive models using LGE presence and location were superior to models based on LGE extent or pattern. ConclusionsIn DCM, the presence of septal LGE is associated with a large increase in the risk of death and SCD events, even when the extent is small. SCD risk is greatest with concomitant septal and free-wall LGE. The incremental value of LGE extent beyond small amounts and LGE pattern is limited.

Aspergilloma mimicking olfactory groove meningioma

Central nervous system aspergillosis is rare with reported mortality rate of 13–50% involving foci in paranasal sinuses and even higher mortality rates (80–100%) in patients of immune-compromised status. Modern day imaging offers an opportunity for early diagnosis, but findings are minimal. A typical finding is that of a space occupying mass lesion with iso-hypointense signal on T1-weighted sequences and extreme hypointense appearance on T2-weighted. This results from the concentration of ferromagnetic substances (iron/zinc/magnesium/manganese) within the lesion. Gadolinium enhancement pattern varies from homogeneous to peripheral ring enhancement. The immune-competent hosts present with homogeneous enhancement while those with immune compromise bear more variable radiological appearance. Due to such radiological appearance, meningioma or tuberculoma is considered in differential earlier than fungal granuloma, if not for the hypointense appearance on T2-weighted sequences which strongly points to fungal granuloma. Through this case report, we attempt to highlight the unusual radiological presentation of the entity.

Approach to Myelopathy.

Myelopathy is commonly encountered in clinical practice and is associated with a large number of causes. This article reviews the anatomy of the spinal cord and discusses how the clinical findings, time course, and radiographic patterns can help to identify the causes of myelopathy. Imaging observations such as MRI "pancake" gadolinium enhancement pattern with spondylotic myelopathy and subpial enhancement with neurosarcoidosis have improved diagnostic specificity. On the other hand, common diseases such as degenerative spine disease are now recognized as presenting much more variably. Improved imaging and the identification of genetic markers enhance the specificity of diagnosis. The improved identification of biomarkers has shown that the same cause, such as compressive myelopathy, can present more variably than previously appreciated. Spinal cord dysfunction, or myelopathy, remains a clinical diagnosis, and determining the cause requires integration of clinical, laboratory, and imaging parameters, none of which have great specificity individually. Most cases of myelopathy will require further neuroimaging, and some require CSF analysis for diagnosis. This article presents an approach to the diagnosis of myelopathy based on excluding compressive myelopathy initially and then differentiating between acute and subacute processes and chronic causes.

PROGNOSTIC SIGNIFICANCE OF NON-ISCHAEMIC MYOCARDIAL FIBROSIS IN PATIENTS WITH NORMAL LV SIZE AND FUNCTION: A LARGE CMR REGISTRY STUDY

With the increasing utilisation of cardiovascular magnetic resonance (CMR), increasing numbers of patients are identified with normal left ventricular (LV) volumes and function but with non-ischaemic (mid-wall and subepicardial) patterns of late gadolinium enhancement (LGE). The underlying aetiology

Fluorescein sodium-guided resection of intracranial lesions in 22 dogs.

To evaluate the safety of an intraoperative fluorescein sodium (FS) injection and elucidate the relationships between the MRI findings, pathological diagnoses, and intraoperative staining characteristics of intracranial lesions in 22 dogs. Prospective case series. Twenty-two dogs with intracranial lesions. FS was intravenously administered to 22 dogs undergoing craniotomy for suspected intracranial tumors to evaluate perioperative and postoperative adverse effects. The intensities and patterns of gadolinium (Gd) enhancement on preoperative magnetic resonance imaging and intraoperative FS staining were graded, and their relationship was evaluated. Intraoperative FS staining characteristics and pathological diagnoses were compared. The only adverse effect noted was repetitive vomiting in 2 dogs. The intensities and patterns between preoperative Gd enhancement and intraoperative FS staining appeared to agree. High-grade glioma and histiocytic sarcoma had more intense FS staining. Lesions with strong Gd enhancement, including meningiomas and choroid plexus tumors, also had intense FS staining. The intraoperative use of FS is a simple and safe technique to guide the resection of intracranial lesions in dogs. The findings on Gd enhancement, FS staining, and histopathology appeared to agree but require validation in a larger set of cases. The intraoperative use of FS may improve the prognosis of dogs with brain tumors by guiding the resection of lesions.

Increased Prognostic Value of Query Amyloid Late Enhancement Score in Light-Chain Cardiac Amyloidosis.

Late gadolinium enhancement (LGE) pattern is a powerful imaging biomarker for prognosis of cardiac amyloidosis. It is unknown if the query amyloid late enhancement (QALE) score in light-chain (AL) amyloidosis could provide increased prognostic value compared with LGE pattern.Methods and Results:Seventy-eight consecutive patients with AL amyloidosis underwent contrast-enhanced cardiovascular magnetic resonance imaging. Patients with cardiac involvement were grouped by LGE pattern and analyzed using QALE score. Receiver operating characteristic curve was used to identify the optimal cut-off for QALE score in predicting all-cause mortality. Survival of these patients was analyzed with the Kaplan-Meier method and multivariate Cox regression. During a median follow-up of 34 months, 53 of 78 patients died. The optimal cut-off for QALE score to predict mortality at 12-month follow-up was 9.0. On multivariate Cox analysis, QALE score ≥9 (HR, 5.997; 95% CI: 2.665-13.497; P<0.001) and log N-terminal pro-brain natriuretic peptide (HR, 1.525; 95% CI: 1.112-2.092; P=0.009) were the only 2 independent predictors of all-cause mortality. On Kaplan-Meier analysis, patients with subendocardial LGE can be further risk stratified using QALE score ≥9. The QALE scoring system provides powerful independent prognostic value in AL cardiac amyloidosis. QALE score ≥9 has added value to differentiate prognosis in AL amyloidosis patients with a subendocardial LGE pattern.