Late Gadolinium Enhancement Research Articles

Specific locations of myocardial inflammation and fibrosis are associated with higher risk of events in cardiac sarcoidosis

Background18F-Fluorodeoxyglucose (18F-FDG) positron emission tomography (PET)/magnetic resonance (MR) can identify inflammation and fibrosis, which are high-risk features in cardiac sarcoidosis. ObjectiveThe purpose of this study was to evaluate whether the involvement of certain myocardial segments is associated with higher risk compared to others. MethodsOne hundred twenty-four patients with suspected clinical sarcoidosis underwent 18F-FDG-PET/MR. Late gadolinium enhancement (LGE) and focal 18F-FDG uptake were evaluated globally and in the 16 myocardial segments. Presence of LGE was defined when the percentage of LGE exceeded 5.7% globally (relative to myocardial volume) and in each myocardial segment. Patients were followed up for 5.5 years. Events were defined as ventricular arrhythmia (VA) (including sustained ventricular tachycardia, ventricular fibrillation, and appropriate implantable cardioverter-defibrillator discharge), heart failure hospitalization, or all-cause death. ResultsMean age was 57.1 ± 8.9 years, and 39.5% were female. Twenty-two patients (17.6%) had an event during follow-up, and 9 (7.2%) presented with VA. LGE and 18F-FDG uptake were more frequent in patients with than without events (36.4% vs 7.8%, P = .001). Presence of LGE and 18F-FDG in the basal anterior segment were independent predictors for events after adjustment for left ventricular ejection fraction and relative enhanced volume (LGE: odds ratio [1.2–92.4], P = .034;18F-FDG: odds ratio 5.5 [1.1–27.5], P = .038). LGE presence in basal to mid-anterior, mid-anteroseptal, and basal to mid-inferoseptal segments was an independent predictor of VA. Presence of 18F-FDG in basal to mid-anterior, mid-inferoseptal and mid-inferior segments was an independent predictor of VA. ConclusionInvolvement of specific myocardial segments, particularly basal to mid-anterior and mid-septal segments, is associated with higher rates of events in patients with suspected cardiac sarcoidosis.

Diffuse Interstitial Fibrosis of the Myocardium Predicts Outcome in Moderate and Asymptomatic SevereAortic Stenosis.

Patients with moderate or asymptomatic severe aortic stenosis (AS) are at risk of cardiovascular events. The authors investigated the utility of cardiac magnetic resonance (CMR) to identify drivers of outcome in patients with moderate or asymptomatic severe AS. A prospective, international, multicenter cohort (n=457) of patients with moderate (aortic valve area [AVA]: 1.0-1.5cm2) or asymptomatic severe AS (AVA≤1.0cm2 and NYHA functional class I-II) patients underwent CMR. Diffuse interstitial fibrosis and scar in the myocardium were analyzed with extracellular volume fraction (ECV%) and late gadolinium enhancement (LGE). The outcome was a composite of mortality and heart failure admission. Median ECV% was 26.6% (IQR: 24.4%-29.9%), and LGE was present in 31.5% (median 0.8%; IQR: 0.1%-1.7%). Greater AS severity was associated with greater left ventricular mass and diastolic dysfunction, but not with ECV% or LGE. During a median 5.7 years of follow-up, 83 events occurred. Patients with events had higher ECV% (median ECV% 26.3% vs 28.2%; P = 0.003). Patients in the highest ECV% tertiles (ECV% >28.6%) had worse outcomes both in the entire cohort and in those with NYHA functional class I moderate or severe AS, and ECV% was independently associated with outcome (adjusted HR: 1.05; P = 0.039). The ECV% had significant incremental prognostic value when added to parameters of AS severity and cardiac function, comorbidities, aortic valve replacement, and LGE (P< 0.05). Increased diffuse interstitial fibrosis of the myocardium is associated with poor outcomes in patients with moderate and asymptomatic severe AS and can help identify those who require closer surveillance for adverse outcomes.

Predicting Late Gadolinium Enhancement of Acute Myocardial Infarction in Contrast-Free Cardiac Cine MRI Using Deep Generative Learning.

Late gadolinium enhancement (LGE) cardiac magnetic resonance (CMR) is a standard technique for diagnosing myocardial infarction (MI), which, however, poses risks due to gadolinium contrast usage. Techniques enabling MI assessment based on contrast-free CMR are desirable to overcome the limitations associated with contrast enhancement. We introduce a novel deep generative learning method, termed cine-generated enhancement (CGE), which transforms standard contrast-free cine CMR into LGE-equivalent images for MI assessment. CGE features with multislice spatiotemporal feature extractor, enhancement contrast modulation, and sophisticated loss function. Data from 430 patients with acute MI from 3 centers were collected. After image quality control, 1525 pairs (289 patients) of center I were used for training, and 293 slices (52 patients) of the same center were reserved for internal testing. The 40 patients (401 slices) of the other 2 centers were used for external testing. The CGE robustness was further tested in 20 normal subjects in a public cine CMR data set. CGE images were compared with LGE for image quality assessment and MI quantification regarding scar size and transmurality. The CGE method produced images of superior quality to LGE in both internal and external data sets. There was a significant (P<0.001) correlation between CGE and LGE measurements of scar size (Pearson correlation, 0.79/0.80; intraclass correlation coefficient, 0.79/0.77) and transmurality (Pearson correlation, 0.76/0.64; intraclass correlation coefficient, 0.76/0.63) in internal/external data set. Considering all data sets, CGE demonstrated high sensitivity (91.27%) and specificity (95.83%) in detecting scars. Realistic enhancement images were obtained for the normal subjects in the public data set without false positive subjects. CGE achieved superior image quality to LGE and accurate scar delineation in patients with acute MI of both internal and external data sets. CGE can significantly simplify the CMR examination, reducing scan times and risks associated with gadolinium-based contrasts, which are crucial for acute patients.

Rates and predictors for sustained ventricular tachycardia in patients with cardiac sarcoidosis and AV block as first cardiac presentation: Implications for device implantation

BackgroundAtrioventricular block (AVB) is a frequent initial presentation of cardiac sarcoidosis (CS), but dangerous ventricular arrhythmias (VA) can occur. Despite the scarcity of data, guidelines recommend implantable cardioverter-defibrillator (ICD) rather than a pacemaker implantation whenever a device is needed. ObjectiveIn this study, we aimed to establish predictors for sustained VA in patients with CS presenting with pacing indication because of an AVB. MethodsWe prospectively enrolled 112 patients with CS. Excluding those with VA, 82 patients remained and were divided into 2 groups: 34 individuals with AVB as initial presentation and 48 with other symptoms as first presentation (OSF). Both groups were compared for clinical characteristics, rates of VA, left ventricular assist device (LVAD) implantation, heart transplantation, and mortality. ResultsDuring follow-up, VA was detected in 50% in the AVB and 10.4% in the OSF group (P = .001). Death, LVAD implantation, and heart transplantation occurred in 11.8% in AVB group vs 10.4% in the OSF group (P = .847). Late gadolinium enhancement (LGE) was equally observed in both groups: 70% vs 70.5% (P = .966), whereas more patients in the AVB group exhibited abnormal positron emission tomography (PET) uptake: 86.2% vs 54.3% (P = .007). In multivariate analysis, AVB (hazard ratio [HR], 25.15), right ventricular (RV) LGE in cardiovascular magnetic resonance (CMR) (HR, 7.39) were predictors for VA occurrence, whereas the use of immunosuppressive therapy was associated with less VA (HR, 0.26). ConclusionsPatients with CS presenting with AVB have a high risk of sustained VA. Although immunosuppressive drugs may reduce the occurrence of VA, ICD implantation is reasonable, especially in case of RV LGE.

Right ventricular function and determining factors of dysfunction in ST-segment-elevation myocardial infarction: a cross-sectional study with cardiac magnetic resonance imaging (MRI).

Over the past few decades, left ventricular (LV) dysfunction in ST-segment elevation myocardial infarction (STEMI) patients has been the focus of research. Recently, co-occurring right ventricular (RV) dysfunction has received more attention in clinical practice. We aimed to assess RV function using cardiac magnetic resonance (CMR) imaging and identify factors that may contribute to RV dysfunction in STEMI patients. We retrospectively studied 189 patients with STEMI who underwent CMR 1-7 days after successful percutaneous coronary intervention (PCI). The ejection fraction (EF), wall thickening rate (WTR), peak radial strain (RS), circumferential strain (CS) and longitudinal strain (LS) of the LV, interventricular septum (IVS) and RV were measured with cine images. The location and extent of the infarct were determined using late gadolinium enhancement (LGE) imaging. The differences of function between STEMI patients with right ventricular ejection fraction (RVEF) <50% and those with RVEF ≥50% were compared using an independent-sample t-test. Linear regression analyses were used to determine independent predictors of RVEF. RVEF <50% was observed in 32.28%% STEMI patients, who also demonstrated significantly lower left ventricular ejection fraction (LVEF), WTR, RS, CS, LS and larger infarct sizes than those with RVEF ≥50%. Patients with RVEF <50% also demonstrated a higher incidence of RV infarction, higher RV end-systolic volume (ESV) index, and lower RV RS and CS. Multivariable linear regression analysis revealed LV EF, IVS WTR and IVS RS as significant predictors for RVEF, while male gender, the culprit lesion in the right coronary artery (RCA), peak troponin were negative predictors for RVEF. Notably, peak troponin, LV EF, LV RS, LV CS, LV WTR, and IVS WTR demonstrated higher area under the curve (AUC) values for predicting RV dysfunction. RV dysfunction was detected in 32.28% of STEMI patients. Patients with acute STEMI and RVEF <50% had impaired LV and IVS functions. Systolic function of the LV and IVS, peak troponin, and culprit lesions in the RCA were independent predictors of RV dysfunction in STEMI patients.

Late Cardiac Involvement of the Glycogen Storage Disease Type IIIa with Massive Left Ventricular Hypertrophy and Late Gadolinium Enhancement.

Late Cardiac Involvement of the Glycogen Storage Disease Type IIIa with Massive Left Ventricular Hypertrophy and Late Gadolinium Enhancement.

First report of the clinical characteristics and outcomes of cardiac amyloidosis in Saudi Arabia.

Cardiac amyloidosis (CA) is a potentially fatal multisystemic disease that remains significantly underdiagnosed, particularly in the Middle East. This study aims to evaluate the prevalence and clinical characteristics of CA in a high-risk population at a tertiary centre in Saudi Arabia. This cross-sectional, retrospective, single-centre study was conducted at a tertiary hospital in Riyadh, Saudi Arabia. We reviewed the medical records of heart failure patients seen between August 2018 and July 2022 who exhibited red flags for CA and subsequently underwent CA screening. Red flags that prompted the workup included at least two of the following factors: the presence of unilateral or bilateral carpal tunnel syndrome, a family history of transthyretin amyloid (ATTR) amyloidosis and specific electrocardiographic features (relative/absolute low QRS voltage, pseudoinfarct pattern and atrioventricular/interventricular conduction abnormalities). Echocardiographic red flags included mainly increased wall thickness (≥12mm), significant diastolic dysfunction, reduced left ventricular (LV) longitudinal function, right ventricular (RV) dysfunction and elevated right atrial (RA)/pulmonary artery (PA) pressure. Cardiac magnetic resonance (CMR) red flags included aspects similar to those in an echocardiogram as well as a subendocardial or transmural late gadolinium enhancement (LGE) pattern. These patients were assessed for CA through technetium-99m pyrophosphate ([99mTc]Tc-PYP) bone scintigraphy, serum and urine protein electrophoresis with immunofixation and a serum-free light chain assay. A total of 177 patients were screened, of which 21.0 (11.9%) patients were diagnosed with transthyretin amyloid CA (ATTR-CA) and 13 (7.3%) patients were diagnosed with light chain CA (AL-CA). Compared with patients with negative/equivocal [99mTc]Tc-PYP scans (grades 0-1), patients with positive [99mTc]Tc-PYP scans (grades 2-3) were older (78.0 vs. 68.0years, P<0.001), had higher levels of troponin (P=0.003) and N-terminal pro-brain natriuretic peptide (NT-proBNP) (P<0.001), had a higher LV mass index (P<0.001), displayed a more depressed global longitudinal strain (GLS) (P<0.001) with a greater prevalence of a relative apical sparing pattern (P<0.001) and demonstrated a higher incidence of first-degree atrioventricular block (P=0.008) and low voltage patterns on electrocardiography (P<0.001). Patients with ATTR-CA and AL-CA were more likely to have a subendocardial or transmural LGE pattern on CMR (P<0.001) and had a significantly lower overall survival (P<0.001) when compared with other heart failure aetiologies. This is the first study to describe the clinical characteristics and outcomes of CA in the Middle East and Saudi Arabia. The prevalence of CA among screened heart failure patients here aligns with major international studies, suggesting significant underdiagnosis in the region. Therefore, larger multicentric studies and regional screening programmes are urgently needed to accurately characterize the epidemiology and outcomes of CA in the Middle East.

Визначення предикторів стійких порушень серцевого ритму у військовослужбовців із тяжким перебігом міокардиту

The aim – to study heart rhythm and conduction disturbances in different localization and distribution of myocardial lesions in combatants with severe myocarditis based on the results of a 6-month follow-up.Materials and methods. 46 male military personnel with a severe course of acute myocarditis (AM) with a reduced ejection fraction (EF) of the left ventricle (LV) (≤ 40 %) with an average age of (35,1±2,4) years were examined. Examinations were carried out in the 1st month after the onset of symptoms of myocarditis and after 6 months of observation. The diagnosis of myocarditis and the severe course of the disease were established on the basis of the Recommendations for the diagnosis and treatment of myocarditis of the All-Ukrainian Association of Cardiologists of Ukraine. All patients underwent for 24-hour ECG monitoring with analysis of the frequency and spectrum of rhythm and conduction disturbances and cardiac magnetic resonance (CMR) imaging with contrast analysis of the topography of the lesion and counting the number of LV segments affected by inflammatory changes and LV segments with the presence of late gadolinium enhancement (LGE).Results and discussion. When comparing the results of heart MRI with the data of daily ECG monitoring, a clear association of the presence of frequent ventricular extrasystoles (VE) and paroxysms of non-sustained ventricular tachycardia (NSVT) with the localization of LGE in the interventricular septum (IS) was established – among patients with LGE lesions at the onset of AM more than in a third (37.0 %) had frequent VE, and NSVT paroxysms, which increase the risk of developing life-threatening ventricular arrhythmias, were detected in 25,9 % of cases. After 6 months of observation in the presence of LGE in the IS, the frequency of detection of frequent VE and NSVT paroxysms was also significantly higher compared to other localization of the lesion and amounted to 20,0 and 13,3 %, respectively. With the help of correlation analysis, an associative relationship was revealed between the presence of LGE in the IS and the presence of frequent VE and NSVT paroxysms in the debut of myocarditis – r=0.73 (р&lt;0.01) and r=0.66 (р&lt;0,01) respectively, and also after 6 months of observation – r=0.65 (р&lt;0.01) and r=0.59 (р&lt;0.05), respectively. According to the results of the multivariate regression analysis, predictors of frequent VE persistence after 6 months were: LVEF ≤30 %; LV end-diastolic volume index ≥105 ml/m2; presence of inflammatory changes in ≥5.0 LV segments; presence of LGE in ≥4.0 LV segments and its presence in the IS, determined in the 1st month from the onset of the disease. The predictors NSVT paroxysms persistence after 6 months were the same factors with the exception of LV EF, and, according to the value of the β coefficient (β=1.302; p&lt;0.001), the most significant contribution was the presence of LGE in the IS.Conclusions. In combatants with severe myocarditis, the presence of late gadolinium enhancement in the interventricular septum is an additional risk factor for the persistence of frequent ventricular extrasystoles and paroxysms of non-sustained ventricular tachycardia during 6 months, while the presence of late gadolinium enhancement in the posterior and lateral walls of the left ventricle has no reliable relationship with the presence of rhythm and conduction disorders. On the basis of multivariate regression analysis, predictors of frequent ventricular extrasystoles and paroxysms of non-sustained ventricular tachycardia persistence were established in combatants with myocarditis.

Predicting Long-Term Clinical Outcomes of Patients With Recurrent Pericarditis

BackgroundRecurrent pericarditis (RP) is a complex condition associated with significant morbidity. Prior studies have evaluated which variables are associated with clinical remission. However, there is currently no established risk-stratification model for predicting outcomes in these patients. ObjectivesWe developed a risk stratification model that can predict long-term outcomes in patients with RP and enable identification of patients with characteristics that portend poor outcomes. MethodsWe retrospectively studied a total of 365 consecutive patients with RP from 2012 to 2019. The primary outcome was clinical remission (CR), defined as cessation of all anti-inflammatory therapy with complete resolution of symptoms. Five machine learning survival models were used to calculate the likelihood of CR within 5 years and stratify patients into high-risk, intermediate-risk, and low-risk groups. ResultsAmong the cohort, the mean age was 46 ± 15 years, and 205 (56%) were women. CR was achieved in 118 (32%) patients. The final model included steroid dependency, total number of recurrences, pericardial late gadolinium enhancement, age, etiology, sex, ejection fraction, and heart rate as the most important parameters. The model predicted the outcome with a C-index of 0.800 on the test set and exhibited a significant ability in stratification of patients into low-risk, intermediate-risk, and high-risk groups (log-rank test; P < 0.0001). ConclusionsWe developed a novel risk-stratification model for predicting CR in RP. Our model can also aid in stratifying patients, with high discriminative ability. The use of an explainable machine learning model can aid physicians in making individualized treatment decision in RP patients.

Additive effect of metabolic dysfunction-associated fatty liver disease on left ventricular function and global strain in type 2 diabetes mellitus patients: a 3.0 T cardiac magnetic resonance feature tracking study

BackgroundType 2 diabetes mellitus (T2DM) and metabolic-associated fatty liver disease (MAFLD) are both metabolic disorders that negatively impact the cardiovascular system. This study comprehensively analyzed the additive effect of MAFLD on left ventricular function and global strain in T2DM patients by cardiac magnetic resonance (CMR).MethodsData of 261 T2DM patients, including 109 with and 152 without MAFLD, as well as 73 matched normal controls from our medical center between June 2015 and March 2022 were retrospectively analyzed. CMR-derived parameters, including LV function and global strain parameters, were compared among different groups. Univariate and multivariate linear regression analyses were conducted to investigate the impact of various factors on LV function and global strain.ResultsOur investigation revealed a progressive deterioration in LV functional parameters across three groups: control subjects, T2DM patients without MAFLD, and T2DM patients with MAFLD. Statistically significant increases in left ventricular end-diastolic volume index (LVEDVI), left ventricular end-systolic volume index (LVESVI), left ventricular mass index (LVMI) were observed, along with decreases in left ventricular ejection fraction (LVEF) and left ventricular global function index (LVGFI). Among these three groups, significant reductions were also noted in the absolute values of LV global radial, circumferential, and longitudinal peak strains (GRPS, GCPS, and GLPS), as well as in peak systolic (PSSR) and peak diastolic strain rates (PDSR). MAFLD was identified as an independent predictor of LVEF, LVMI, LVGFI, GRPS, GCPS, and GLPS in multivariate linear analysis. Besides, the incidence of late gadolinium enhancement was higher in MAFLD patients than in non-MAFLD patients (50/109 [45.9%] vs. 42/152 [27.6%], p = 0.003). Furthermore, escalating MAFLD severity was associated with a numerical deterioration in both LV function parameters and global strain values.ConclusionsThis study thoroughly compared CMR parameters in T2DM patients with and without MAFLD, uncovering MAFLD’s adverse impact on LV function and deformation in T2DM patients. These findings highlight the critical need for early detection and comprehensive management of cardiac function in T2DM patients with MAFLD.

Patterns of Late Gadolinium Enhancement in Myosin-binding Protein C3 (MYBPC3)-related Dilated Cardiomyopathy: Imitating the Imitator.

Patterns of Late Gadolinium Enhancement in Myosin-binding Protein C3 (MYBPC3)-related Dilated Cardiomyopathy: Imitating the Imitator.

Myocardial Fibrosis Quantification Methods by Cardiovascular Magnetic Resonance Imaging in Patients with Fabry Disease.

Background/Objectives: The presence of late gadolinium enhancement (LGE) on cardiac magnetic resonance (CMR) in patients with Fabry disease (FD) is a predictor of adverse cardiac events. The aim of this study was to establish the most reliable and reproducible technique for quantifying LGE in patients with FD. Methods: Twenty FD patients with LGE who underwent CMR on the same scanner and LGE sequence were included. LGE quantifications were done using gray-scale thresholds of 2, 3, 4, 5 and 6 standard deviations (SD) above the mean signal intensity of the remote myocardium, the full width at half maximum method (FWHM), visual assessment with threshold (VAT) and the fully manual method (MM). Results: The mean amount of fibrosis varied between quantification techniques from 36 ± 19 at 2SD to 2 ± 2 g using the FWHM (p < 0.0001). Intraobserver reliability was excellent for most methods, except for the FWHM which was good (ICC 0.84; all p < 0.05). Interobserver reliability was excellent for VAT (ICC 0.94) and good for other techniques (all p < 0.05). Intraobserver reproducibility showed the lowest coefficient of variation (CV, 6%) at 5SD and at 2SD and VAT (35% and 38%) for interobserver reproducibility. The FWHM revealed the highest CV (63% and 94%) for both intra- and interobserver reproducibility. Conclusions: The available methods for LGE quantification demonstrate good to excellent intra- and interobserver reproducibility in patients with FD. The most reliable and reproducible techniques were VAT and 5SD, whereas the FWHM was the least reliable in the setting of our study. The total amount of LGE varies strongly with the quantification technique used.

Factor analysis of hepcidin on cardiac iron overload and fibrosis among thalassemia major children

BackgroundCardiac iron overload is a common cause of death in thalassemia major and is associated with hepcidin, which is primary iron homeostasis. Therapeutics that could increase hepcidin levels or act as hepcidin agonists might help treat the abnormal iron absorption in individuals with β-thalassemia and related disorders. We aimed to verify the structure and study the relationship of release variables, hepcidin, cardiac iron overload, or fibrosis.MethodsA cross-sectional study was conducted among thalassemia majors, aged 6–18 years at Rumah Sakit Anak dan Bunda Harapan Kita Indonesia, between January 2019 and May 2020. Clinical data, hepcidin-25, interacting variables laboratory test, MRIT2* used to assess cardiac iron overload, late gadolinium enhancement (LGE), and soluble suppression of tumoregenicity2 (ST2) to assess fibrosis were studied. The correlation test was performed with SPSS version 20, Amos 22 was used to assess confirmatory factor analysis (CFA), and squared multiple correlation (SMC) was used to determine the proportion of total variation explained by the model.ResultsWe recruited 80 patients, of those 8 (10%) were cardiac iron overload, 5 (5.25%) were fibrosis, and 3 (3,75%) were ST2 > 35 mg/dL. CFA showed variables that interact with hepcidin release were Hb, reticulocyte-He (Re-He), HIF-1α, Immature granulocyte (IG), hs-CRP, IL-6, ferritin, and transferrin saturation. There was no direct hepcidin role in cardiac iron overload and fibrosis. No interacting variable role on hepcidin. SMC of hepcidin on cardiac iron overload was 20%.ConclusionFactor analysis of hepcidin was Hb, Ret-He, HIF-1α, IG, hs-CRP, IL-6, ferritin, and transferrin saturation. No direct hepcidin role in cardiac iron overload and fibrosis. Hepcidin explains approximately 20% of the total variation in cardiac iron overload.

Cardiac magnetic resonance for ventricular arrhythmias: a systematic review and meta-analysis

BackgroundCardiac magnetic resonance (CMR) allows comprehensive myocardial tissue characterisation, revealing areas of myocardial inflammation or fibrosis that may predispose to ventricular arrhythmias (VAs). With this study, we aimed to estimate...

The Role of Cardiac Magnetic Resonance in mRNA COVID-19 Vaccine-Related Myopericarditis: An Evolutive Case Series.

Numerous cases of myocarditis related to mRNA vaccines for COVID-19 have recently been described, usually in young men. Long-term evolutive cardiac magnetic resonance imaging (CMR) data are lacking. We describe four consecutive cases of COVID-19 vaccine-induced myocarditis. The pathological findings of cardiac magnetic resonance confirmed the diagnosis in the acute phase, showing edema, as well as pericardial enhancement, with light pericardial effusion and late gadolinium enhancement (LGE), predominantly in the inferolateral wall. These cases highlight the unique value of cardiac magnetic resonance in patients with suspected myocarditis induced by COVID-19 RNAm vaccines as a tool to confirm the diagnosis, avoiding other invasive techniques, as well as for the long-term follow-up of patients. Our iterative CMR imaging demonstrated frequent long-term LGE persistence.

Enhanced detection of damaged myocardium and risk stratification in hypertrophic cardiomyopathy using integrated [68Ga]Ga-FAPI-04 PET/CMR imaging.

This study aims to explore the correlation between PET and CMR in integrated [68Ga]Ga-FAPI-04 PET/CMR multimodal imaging and its value in the diagnosis and risk assessment of hypertrophic cardiomyopathy (HCM). This study included 20 HCM patients and 11 age- and gender-matched controls. PET analysis evaluated left ventricular (LV) [68Ga]Ga-FAPI-04 uptake, including SUVmax, TBR, cardiac fibroblast activity (CFA) and volume (CFV), and total SUV of the 16 segments. CMR tissue characterization parameters included cardiac function, myocardial thickness, late gadolinium enhancement (LGE), relaxation time, extracellular volume (ECV), and peak strain parameters. The 5-year sudden cardiac death (SCD) risk score and the 2-year and 5-year atrial fibrillation (AF) risk scores were calculated for each patient. The study analyzed differences between HCM patients and controls, the correlation between [68Ga]Ga-FAPI-04 PET and concurrent CMR imaging results, and the predictive value of PET/CMR. The FAPI uptake, myocardial mass, myocardial thickness, and T1/T2 mapping values were significantly higher in HCM patients compared to controls. Twenty HCM patients and their 320 myocardial segments were discussed. Increased [68Ga]Ga-FAPI-04 uptake in the left ventricular wall was observed in 95% (19/20) of the patients, covering 48.8% (156/320) of the segments. On concurrent CMR, 80% (16/20) of the patients showed LGE, including 95 (29.7%) segments. The FAPI(+)LGE(+) segments exhibited the highest myocardial PET uptake, greatest thickness, longest T1/T2 native values, largest ECV value and the greatest loss of myocardial strain capacity (P < 0.05). There was a significant correlation between FAPI uptake and CMR parameters (P < 0.05). Higher [68Ga]Ga-FAPI-04 uptake showed a positive correlation with SCD and AF risk scores (P < 0.05). The number of LGE(+) segments, mapping parameters, and ECV values in CMR also had prognostic significance. Combining PET with CMR aided in further risk stratification of HCM. [68Ga]Ga-FAPI-04 PET/CMR multimodal imaging has potential value in the detection of damaged myocardial lesions and risk assessment of HCM patients. [68Ga]Ga-FAPI-04 PET can detect more affected myocardium compared to CMR, and segments with abnormalities in both PET and CMR show more severe myocardial damage.

Prognostic value of electroanatomic-guided endomyocardial biopsy in patients with myocarditis, arrhythmogenic cardiomyopathy and non dilated left ventricular cardiomyopathy

A wide variety of non-invasive and invasive techniques for SCD risk stratification in non ischemic cardiomyopathy (NICM) have been proposed, including left ventricular (LV) ejection fraction, QRS duration, late gadolinium enhancement (LGE) on cardiac magnetic resonance (CMR) and invasive electrophysiologic study with or without three-dimensional electroanatomic mapping (3D-EAM), to identify and characterize the arrhythmogenic substrate. There is still no clear consensus on the risk stratification in this clinical setting.The aim of our study is to characterize the 3D-EAM substrate in patients with the same clinical presentation of unexplained complex VAs and NICM using CMR, three-dimensional electranatomic mapping (3D-EAM) in association with endomyocardial biopsy (EMB) and genetic screening, as a more precise and early diagnostic assessment may provide important subsequent prognostic impact. The study was designed as a prospective multi-center observational evaluation and the patient follow-up was scheduled at 6 months interval. We enrolled 125 patients distinct into four different group by complete diagnostic work-up: myocarditis, non-dilated left ventricular cardiomyopathy, arrhythmogenic cardiomyopathy and control group. The four groups were compared in terms of clinical, imaging and 3D-EAM data. At multivariate analysis sustained VT/VF on admission [HR: 3.64 (1.79–7.4), p < 0.001], total bipolar scar area of left and right ventricle detected by 3D-EAM [HR: 2.24 (1.13–4.49), p = 0.02], histological diagnosis of myocarditis by 3D-EAM guided endomyocardial biopsy (EBM) [HR: 2.79 (1.04–7.44), p = 0.01] were independent predictors of complex VAs or death at follow-up. 3D-EAM guided EMB represent not only a valid diagnostic tool to identify the arrhythmogenic substrate in patients with NICM and ventricular arrhythmic phenotype but also an important predictor of complex Vas at long term follow-up.

Myocardial Injury by COVID-19 Infection Assessed by Cardiovascular Magnetic Resonance Imaging - A Prospective Multicenter Study.

This prospective multicenter study assessed the prevalence of myocardial injury in patients with COVID-19 using cardiac magnetic resonance imaging (CMR). We prospectively screened 505 patients with moderate to severe COVID-19 disease from 7 hospitals in Japan. Of these patients, 31 (mean [±SD] age 63.5±10.4 years, 23 [74%] male) suspected of myocardial injury, based on elevated serum troponin or B-type natriuretic peptide concentrations either upon admission or 3 months after discharge, underwent CMR 3 months after discharge. The primary endpoint was the presence of myocardial injury, defined by any of the following: (1) contrast enhancement in the left or right ventricle myocardium on late gadolinium enhancement CMR; (2) left or right ventricular dysfunction (defined as <50% and <45%, respectively); and (3) pericardial thickening on contrast enhancement. The mean (±SD) duration between diagnosis and CMR was 117±16 days. The primary endpoint was observed in 13 of 31 individuals (42%), with 8 (26%) satisfying the modified Lake Louise Criteria for the diagnosis of acute myocarditis. This study revealed a high incidence of myocardial injury identified by CMR in patients with moderate to severe COVID-19 and abnormal findings for cardiac biomarkers.

Longitudinal cardiac magnetic resonance imaging following clinical response to rilonacept and prior to recurrence upon treatment suspension: a RHAPSODY subgroup analysis.

In the phase 3 trial, RHAPSODY, rilonacept effectively resolved active pericarditis recurrences, and long-term treatment led to sustained pericarditis recurrence risk reduction. Prior analysis suggested association between higher late gadolinium enhancement (LGE) at baseline and more rapid recurrence upon rilonacept suspension after 12 weeks of treatment. This subgroup analysis assessed the utility of longitudinal serial cardiac magnetic resonance (CMR) imaging for tracking clinical improvement and predicting post-treatment-cessation outcomes to help guide clinical decision making. At an 18-month decision milestone (18MDM) in the RHAPSODY long-term extension, investigators decided if patients would continue rilonacept, suspend rilonacept for off-treatment observation, or discontinue the study. Pericardial thickness, pericardial edema (T2-STIR), and LGE were determined at baseline and 18MDM by an imaging core lab blinded to clinical data, and pericarditis recurrence was investigator-assessed. CMR results in patients with data at both baseline and 18MDM (n=13) showed that pericardial thickness, T2-STIR, and LGE were reduced during rilonacept treatment. Among patients with CMR data who suspended rilonacept at the 18MDM (n=7), 5 (71%) had a pericarditis recurrence within 1-4 months of rilonacept suspension, despite all having had none/trace LGE (n=7) and negative T2-STIR (n=7) at the 18MDM and 2 having received prophylactic colchicine. Continued clinical improvement during prolonged rilonacept treatment corresponded with improvement on CMR, including reduced pericardial thickness, resolution of pericardial edema, and resolution of LGE. However, none/trace LGE at 18MDM while on treatment did not predict absence of pericarditis recurrence upon subsequent rilonacept suspension in this size-limited subgroup.

Challenges in the approach to a patient with aortic stenosis and cardiac amyloidosis with ATTR mutation associated with negative scintigraphy - A case report

IntroductionCardiac amyloidosis (CA) poses significant diagnostic and therapeutic challenges. In this case report, we detail a patient with CA due to a rare transthyretin (CA-TTR) mutation, manifesting with negative myocardial scintigraphy and requiring genetic testing for diagnosis. The patient also had severe aortic stenosis (AS), necessitating discussion with a heart team to determine the optimal treatment strategy. Case reportA 70-year-old male with a family history of sudden death was previously diagnosed with third-degree atrioventricular block and treated with a pacemaker. He presented with worsening exertional dyspnoea, and examination revealed a third heart sound, a systolic murmur indicative of AS and bilateral muscular atrophy in the thenar region. Transthoracic echocardiography indicated severe AS and moderate left ventricular dysfunction, with images suggesting infiltrative disease. Pyrophosphate scintigraphy revealed no abnormal cardiac tracer uptake. Cardiac magnetic resonance imaging (MRI) revealed extensive, heterogeneous, subendocardial late gadolinium enhancement in both the atria and ventricles, which was consistent with CA. Genetic testing identified the Phe84Leu mutation in the TTR gene. Following heart team discussions, the patient underwent successful transcatheter aortic valve implantation (TAVI) and remained asymptomatic in follow-up, being monitored at an outpatient clinic specializing in CA and using tafamidis. DiscussionCA-TTR can be an autosomal dominant disease with variable penetrance involving abnormal amyloid protein deposition in tissues and can often be diagnosed noninvasively via myocardial scintigraphy. However, some TTR mutations do not affect scintigraphy results, necessitating genetic testing when clinical suspicion is high, potentially avoiding endomyocardial biopsy. Moreover, AS occurs in up to 16 % of TTR amyloidosis patients, with the conditions mutually exacerbating each other. Recent consensus suggests that TAVI reduces mortality in patients with severe AS and amyloidosis. ConclusionsVarious diagnostic algorithms emphasize the use of myocardial scintigraphy for suspected CA-TTR. Genetic testing is crucial when scintigraphy results are negative, but clinical suspicion remains high, potentially circumventing invasive procedures. Compared with medical management alone, TAVI has been shown to improve quality of life and survival in patients with concurrent severe AS and CA.