Late Gadolinium Enhancement Pattern Research Articles

Myocardial strain analysis by cardiac magnetic resonance 3D feature-tracking identifies subclinical abnormalities in patients with neuromuscular disease and no overt cardiac involvement.

Cardiovascular magnetic resonance (CMR) is valuable for the detection of cardiac involvement in neuromuscular diseases (NMDs). We explored the value of 2D- and 3D-left ventricular (LV) myocardial strain analysis using feature-tracking (FT)-CMR to detect subclinical cardiac involvement in NMD. The study included retrospective analysis of 111 patients with NMD; mitochondrial cytopathies (n = 14), Friedreich's ataxia (FA, n = 27), myotonic dystrophy (n = 27), Becker/Duchenne's muscular dystrophy (BMD/DMD, n = 15), Duchenne's carriers (n = 6), or other (n = 22) and 57 age- and sex-matched healthy volunteers. Biventricular volumes, myocardial late gadolinium enhancement (LGE), and LV myocardial deformation were assessed by FT-CMR, including 2D and 3D global circumferential strain (GCS), global radial strain (GRS), global longitudinal strain (GLS), and torsion. Compared with the healthy volunteers, patients with NMD had impaired 2D-GCS (P < 0.001) and 2D-GRS (in the short-axis, P < 0.001), but no significant differences in 2D-GRS long-axis (P = 0.101), 2D-GLS (P = 0.069), or torsion (P = 0.122). 3D-GRS, 3D-GCS, and 3D-GLS values were all significantly different to the control group (P < 0.0001 for all). Especially, even NMD patients without overt cardiac involvement (i.e. LV dilation/hypertrophy, reduced LVEF, or LGE presence) had significantly impaired 3D-GRS, GCS, and GLS vs. the control group (P < 0.0001). 3D-GRS and GCS values were significantly associated with the LGE presence and pattern, being most impaired in patients with transmural LGE. 3D-FT CMR detects subclinical cardiac muscle disease in patients with NMD even before the development of replacement fibrosis or ventricular remodelling which may be a useful imaging biomarker for early detection of cardiac involvement.

3.0T cardiac magnetic resonance quantification of native T1 and myocardial extracellular volume for the diagnosis of late gadolinium enhancement-negative cardiac amyloidosis.

BackgroundLate gadolinium enhancement (LGE) by cardiac magnetic resonance (CMR) is useful for the detection of cardiac amyloidosis (CA), but characteristic LGE patterns do not always occur or they appear late in the disease. Native T1 and extracellular volume (ECV) by T1 mapping may improve disease detection and quantify myocardial amyloid load.MethodsThirty patients with definite CA, 10 patients with possible CA, 20 patients with hypertrophic cardiomyopathy (HCM) and 40 healthy volunteers were performed 3.0-T CMR including cine, pre- and postcontrast T1 mapping and LGE. Receiver-operating characteristic (ROC) curves were constructed to assess the diagnostic ability of native T1 and ECV for CA. Correlation analysis between native T1 or ECV and cardiac biomarkers, structure, and function indexes were assessed using Pearson or Spearman correlation, as appropriate.ResultsNative T1 values were 1,429±93, 1,290±49, 1,304±42, and 1,225±21 ms, in definite CA, possible CA, HCM, and healthy controls, respectively. ECV values were 44%±9%, 34%±5%, 33%±4%, and 24%±3%, in definite CA, possible CA, HCM, and healthy controls, respectively. Native T1 [area under curve (AUC) =0.89, 95% confidence interval (CI): 0.75–1.00, P<0.001] and ECV (AUC =0.99, 95% CI: 0.98–1.00, P<0.001) showed good ability to differentiate LGE-negative patients with possible CA from healthy controls, especially ECV. Positive correlations were found between native T1 or ECV and New York Heart Association (NYHA) functional class (r=0.673 and r=0.594, respectively; P<0.001), NT-proBNP (r=0.668 and r=0.603, respectively; P<0.001), troponin T (r=0.724 and r=0.591, respectively; P<0.001), left ventricular (LV) mass index (r=0.668 and r=0.579, respectively; P<0.001), and global LV wall thickness (r=0.765 and r=0.629, respectively; P<0.001). Negative correlations were found between native T1 or ECV and left ventricular ejection fraction (LVEF) (r=−0.761 and r=−0.668, respectively; P<0.001) and left ventricular stroke volume (LVSV) (r=−0.777 and r=−0.729, respectively; P<0.001).ConclusionsNative T1 and ECV, which are able to reflect cardiac biochemistry, structure, and function, have high diagnostic accuracy for detecting CA, especially in LGE-negative patients, and thus could be used for early diagnosis of CA.

Potential clinical relevance of cardiac magnetic resonance to diagnose cardiac light chain amyloidosis.

While patients with cardiac transthyretin amyloidosis are easily diagnosed with bone scintigraphy, the detection of cardiac light chain (AL) amyloidosis is challenging. Cardiac magnetic resonance (CMR) analyses play an essential role in the differential diagnosis of cardiomyopathies; however, limited data are available from cardiac AL-Amyloidosis. Hence, the purpose of the present study was to analyze the potential role of CMR in the detection of cardiac AL-amyloidosis. We included 35 patients with proved cardiac AL-amyloidosis and two control groups constituted by 330 patients with hypertrophic cardiomyopathy (HCM) and 70 patients with arterial hypertension (HT), who underwent CMR examination. The phenotype and degree of left ventricular (LV) hypertrophy and the amount and pattern of late gadolinium enhancement (LGE) were evaluated. In addition, global and regional LV strain parameters were also analyzed using feature-tracking techniques. Sensitivity and specificity of several CMR parameters were analyzed in diagnosing cardiac AL-amyloidosis. The sensitivity and specificity of diffuse septal subendocardial LGE in diagnosing cardiac AL-amyloidosis was 88% and 100%, respectively. Likewise, the sensitivity and specificity of septal myocardial nulling prior to blood pool was 71% and 100%, respectively. In addition, a LV end-diastolic septal wall thickness ≥ 15 mm had an optimal diagnostic performance to differentiate cardiac AL-amyloidosis from HT (sensitivity 91%, specificity 89%). On the other hand, a reduced global LV longitudinal strain (< 15%) plus apical sparing (apex-to-base longitudinal strain > 2) had a very low sensitivity (6%) in detecting AL-Amyloidosis, but with very high specificity (100%). The findings from this study suggest that CMR could have an optimal diagnostic performance in the diagnosis of cardiac AL-amyloidosis. Hence, further larger studies are warranted to validate the findings from this study.

Cardiac MRI Findings in Male Patients with Acute Myocarditis in the Presence or Absence of COVID-19 Vaccination.

By comparing phenotypic clinical characteristics and cardiovascular magnetic resonance (CMR) findings in 14 patients with COVID-19 mRNA vaccine-associated myocarditis to 14 patients with acute myocarditis from other causes, we found that patients with COVID-19 vaccination- associated acute myocarditis have higher left ventricular ejection fraction, higher left ventricular global circumferential and radial strain, and less involvement of late gadolinium enhancement in the septal segments with less involvement of midmyocardial pattern of late gadolinium enhancement, compared to patients with acute myocarditis from other causes.

The prevalence of ischemic pattern of heart damage in patients with acute myocardial infarction depending on the presence of obstructive coronary artery disease: Magnetic resonance study

Currently, there are practically no works that are devoted to the analysis of different types of damage to ischemic and nonischemic patterns in patients with acute myocardial infarction (AMI), depending on the nature of atherosclerotic lesions of the coronary arteries.In this regard, the aim of this work was to evaluate the frequency of ischemic pattern of heart damage in patients with AMI depending on the presence of obstructive coronary artery disease.Material and Methods. The study retrospectively included patients with a working diagnosis of acute coronary syndrome (ACS) who underwent contrast-enhanced cardiac magnetic resonance (MR) imaging (CMR) during the first week after hospitalization. A 1.5 T CMR was performed using a standard protocol. T2-WI, T1-WI, and IR-images were visually assessed for edema and scar/necrosis, respectively. The pattern of acute ischemic damage to the left ventricular (LV) myocardium was an increase in the signal intensity on T2-WI as a sign of myocardial edema and late gadolinium enhancement (LGE) with a typical ischemic nature of contrast enhancement: subendocardial/subtransmural/transmural. The non-ischemic nature of myocardial injury was defined as intramyocardial/subepicardial LGE.Results. Based on invasive coronary angiography (ICA) data, patients were divided into two groups: group of myocardial infarction (MI) and coronary artery disease (MICAD group) and group of MI and non-obstructive coronary arteries (MINOCA group). It was found that the frequencies of occurrence of subendocardial pattern of cardiac LGE in early CMR did not differ in the groups of AMI patients against the background of obstructive and non-obstructive coronary artery disease (CAD). The most characteristic MR-pattern in MICAD patients was transmural LGE, including that in combination with microvascular obstruction (MVO). The most characteristic MR-pattern in MINOCA patients was mid-wall/insertion point LGE. Subepicardial LGE occurred with equal frequency in MICAD and MINOCA patients.Conclusion. The most pathognomonic MR-pattern of myocardial damage in AMI against the background of obstructive CAD was the transmural type of LGE with or without the phenomenon of MVO. Identification of the subendocardial type of LGE in patients with MINOCA allowed to stratify these patients into a higher risk group with a further change in treatment tactics.

T1 Mapping and Extracellular Volume in Cardiomyopathy Showing Left Ventricular Hypertrophy: Differentiation Between Hypertrophic Cardiomyopathy and Hypertensive Heart Disease.

BackgroundThe aim of this study was to evaluate the capability of different magnetic resonance imaging (MRI) parameters for distinguishing between hypertrophic cardiomyopathy (HCM) and hypertensive heart disease (HHD).MethodsThirty-eight patients with HCM, 35 patients with HHD, and 29 healthy controls subjects were enrolled in this study. All subjects underwent cardiac MRI to measure T1 values and extracellular volume (ECV), as well as the extent and patterns of late gadolinium enhancement (LGE). Myocardial segments were categorized as non-hypertrophic, mild-hypertrophic, moderate-hypertrophic, and severe-hypertrophic based on end-diastolic wall thickness (EDWT). The differences in native T1 values between all four groups were evaluated.ResultsNative T1 values were significantly higher in patients with HCM than in patients with HHD and in healthy controls (both P < 0.001). Moreover, significantly increased ECV was shown in patients with HCM than in patients with HHD and in healthy controls (both P = 0.001). Native T1 values in the basal slice and apex slice were significantly higher in patients with HCM than in patients with HHD (P < 0.01). In patients with HCM, the non-hypertrophic myocardial segments demonstrated significantly elevated T1 values compared with patients with HHD (both P < 0.001). Using a cut-off value of 28.8% for ECV, it could differentiate between HCM and HHD with 85% sensitivity, 62.07% specificity, and an area under the curve of 0.772.ConclusionIn patients with HCM, T1 tissue remodeling occurs in the normal-appearing myocardial segments, but not in patients with HHD. Both native T1 values and ECV can support clinically relevant discrimination between HCM and HHD, although ECV had better diagnostic efficacy.

Severe Isolated Cardiomyopathy Requiring Heart Transplant Following COVID-19 Infection and Subsequent Vaccination

IntroductionCardiomyopathy is a known complication associated with COVID-19, and 7% of all COVID-19 related deaths are thought to be due to myocarditis. There is also growing evidence for COVID-19 vaccine-related myocarditis. We present the first case of a patient with severe isolated cardiomyopathy requiring heart transplant after both COVID-19 infection and vaccination.Case ReportA 58-year-old male with no prior medical history was diagnosed with COVID-19 infection in December 2020 without hospitalization. He experienced declining symptoms over the next 7 months and completed COVID-19 vaccination in July. On 8/10/2021, he presented to the emergency room with worsening exertional dyspnea and orthopnea. Initial labs revealed elevated NT-proBNP (1,701 pg/mL) and high-sensitivity cardiac troponin-T (45 ng/L). Transthoracic echocardiography revealed reduced left ventricular (LV) ejection fraction at 15% and LV end-diastolic diameter at 5.6 cm. Coronaries were clear. Cardiac MRI (cMR) is depicted in Figure 1. He was managed with an Impella device and dobutamine but failed weaning from this cardiac support. He was listed for transplant as UNOS Status 2 and underwent successful OHT on 9/19/2021.SummaryAcute myocardial injury is known to be a frequent complication during the COVID-19 course, but it is not known to require advanced therapies. There is only one other case from France that describes a patient bridged with temporary mechanical support to OHT following COVID-19 induced cardiomyopathy. In both cases, cMR revealed similar late gadolinium enhancement (LGE) patterns. The cases differ as our patient experienced Long COVID symptoms over 8 months after diagnosis and was vaccinated while the French report describes a patient who was diagnosed with COVID-19, experienced end-stage heart failure and underwent transplant all within 11 days. This case contributes to the lacunae in data in the era of COVID-19 and its vaccines.

Diagnosis of Cardiac Amyloidosis Using a Radiomics Approach Applied to Late Gadolinium-Enhanced Cardiac Magnetic Resonance Images: A Retrospective, Multicohort, Diagnostic Study.

ObjectivesTo assess the potential of a radiomics approach of late gadolinium enhancement (LGE) cardiac magnetic resonance (CMR) in the diagnosis of cardiac amyloidosis (CA).Materials and MethodsThis retrospective study included 200 patients with biopsy-proven light-chain (AL) amyloidosis. CA was diagnosed on the basis of systemic amyloidosis confirmed with evidence of cardiac involvement by imaging and clinical biomarkers. A total of 139 patients [54 ± 8 years, 75 (54%) men] in our institution were divided into training cohort [n = 97, mean age of 53 ± 8 years, 54 (56%) men] and internal validation cohort [n = 42, mean age: 56 ± 8 years, 21 (50%) men] with a ratio of 7:3, while 61 patients [mean age: 60 ± 9 years, 42 (69%) men] from the other two institutions were enrolled for external validation. Radiomics features were extracted from global (all short-axis images from base-to-apex) left ventricular (LV) myocardium and three different segments (basal, midventricular, and apex) on short-axis LGE images using the phase-sensitive reconstruction (PSIR) sequence. The Boruta algorithm was used to select the radiomics features. This model was built using the XGBoost algorithm. The two readers performed qualitative and semiquantitative assessment of the LGE images based on the visual LGE patterns, while the quantitative assessment was measured using a dedicated semi-automatic CMR software. The diagnostic performance of the radiomics and other qualitative and quantitative parameters were compared by a receiver operating characteristic (ROC) curve analysis. A correlation between radiomics and the degree of myocardial involvement by amyloidosis was tested.ResultsA total of 1,906 radiomics features were extracted for each LV section. No statistical significance was indicated between any two slices for diagnosing CA, and the highest area under the curve (AUC) was found in basal section {0.92 [95% confidence interval (CI), 0.86–0.97] in the LGE images in the training set, 0.89 (95% CI, 0.79–1.00) in the internal validation set, and 0.92 (95% CI, 0.85–0.99) in the external validation set}, which was superior to the visual assessment and quantitative LGE parameters. Moderate correlations between global or basal radiomics scores (Rad-scores) and Mayo stage in all patients were reported (Spearman’s Rho = 0.61, 0.62; all p < 0.01).ConclusionA radiomics analysis of the LGE images provides incremental information compared with the visual assessment and quantitative parameters on CMR to diagnose CA. Radiomics was moderately correlated with the severity of CA. Further studies are needed to assess the prognostic significance of radiomics in patients with CA.

Diagnostic Value of 11C-PIB PET/MR in Cardiac Amyloidosis.

BackgroundThe thioflavin T derivative, 11C-Pittsburgh-B (PIB), is used for Alzheimer's disease imaging because it specifically binds to β-amyloid protein deposits in the brain. The aim of this study was to estimate the diagnostic value of combined 11C-PIB positron emission tomography/magnetic resonance (PET/MR) in cardiac amyloidosis (CA).MethodsWe enrolled 23 heart failure patients with suspected CA based on echocardiographic and electrocardiograph findings. All patients underwent cardiac 11C-PIB PET/MR and non-cardiac biopsy within one week. We also enrolled eight healthy volunteers that underwent cardiac 11C-PIB PET/MR as a control group. The cardiac magnetic resonance (CMR) protocol included cine imaging, late gadolinium enhancement (LGE), and native and post-contrast T1 mapping. Extracellular volume (ECV) was measured using pre- and post-contrast T1 mapping images. LVEF, IVSD, LVPW, LVmass, LVESV, LVEDV, native T1 value, ECV, and maximum uptake of myocardial tissue-to-blood background ratio (TBR) values were obtained from PET/MR images in all patients and healthy subjects.ResultsThirteen out of twenty-three heart failure patients were clinically diagnosed with CA. The remaining 10 patients were CA-negative (non-CA patient group). Twelve of the thirteen CA patients showed diffuse transmural LGE patterns, whereas LGE was either absent or patchy in the non-CA patients. The diagnostic sensitivity and specificity of TBRmax were 92.3 and 100%, respectively, at a cut-off value of 1.09. Several CMR imaging parameters (LVEF, IVSD, LVmass, LVEDV, LVESV, LVPW, native T1 value and ECV) and TBR showed significant differences between CA patients, non-CA patients, and healthy controls (P < 0.05). Native T1 mapping values positively correlated with TBRmax values in CA and non-CA patients (r = 0.38, P = 0.0004).Conclusions11C-PIB PET/MRI is a valuable tool for the accurate and non-invasive diagnosis of CA because it distinguishes CA patients from non-CA patients and healthy subjects with high specificity and sensitivity. Moreover, native T1 mapping values positively correlated with TBRmax values in CA and non-CA patients. In the future, larger cohort studies are necessary to confirm our findings.

Predictores de muerte súbita en miocardiopatía dilatada: más allá de la presencia de realce tardío de gadolinio

Introduction and objectivesSudden cardiac death (SCD) risk stratification needs to be improved in patients with dilated cardiomyopathy (DCM). The aim of our study was to identify clinical and cardiac magnetic resonance predictors that could identify patients with DCM at higher risk of SCD. MethodsMulticenter, retrospective study. We included 386 patients with DCM and left ventricular ejection fraction <50%. The presence of late gadolinium enhancement (LGE) was evaluated and then characterized according to its pattern and location. Primary event was defined as a composite of SCD and aborted SCD. Events were assigned through clinical records review. ResultsAfter a 4.9-year follow-up (P25-75: 3.1-7.1) 27 primary events (6.98%) were registered. In multivariate models the following variables were associated with higher risk of the primary event: History of syncope, time since diagnosis of DCM> 1 year, LGE presence, subepicardial pattern of LGE, multiple coexisiting LGE patterns, LGE in multiple locations and biventricular ejection fraction <35%. Multivariate competing risk models showed C-index of 0.90 to 0.91 (95%CI, 0.84-0.96), with good calibration. ConclusionsIn patients with DCM, beyond LGE presence, clinical parameters (syncope and DCM evolution time> 1 year), characterization of LGE and biventricular ejection fraction <35% identified patients with DCM at higher risk of SCD/aborted SCD. Our SCD predictive competing risks models showed excellent accuracy. Further studies will be needed to confirm these results.

A Risk Score to Diagnose Cardiac Involvement and Provide Prognosis Information in Patients at Risk of Cardiac Light-Chain Amyloidosis

BackgroundCardiac light-chain amyloidosis (AL CA) portends poor prognosis. Contrast cardiac magnetic resonance (CMR) with late gadolinium enhancement (LGE) imaging is an important tool in recognizing AL CA. But contraindications to contrast CMR would significantly restrict its clinical application value. Our study aims to construct a convenient risk score to help identify cardiac involvement in patients at risk of AL CA. Moreover, we also investigate whether this risk score could provide prognosis information.Materials and MethodsSixty-three patients at risk of AL CA were retrospectively included in our study. Basic clinical characters, lab results, 12-lead electrocardiogram data, and cardiac magnetic resonance image data were collected. AL CA was diagnosed according to typical CA LGE pattern. Logistic analysis was used to figure out predictive parameters of AL CA and their β coefficients, further constructing the risk score. Receiver operating characteristics (ROC) curve was used to find the cut-off point best distinguishing AL CA+ from AL CA–patients. Bootstrapping was used for internal validation. All patients were divided into high-risk and low-risk group according to the diagnostic cut-off point, and followed up for survival information. Kaplan-Meier plots and log-rank test were performed to analyze if this score had prognostic value.ResultsThe risk score finally consisted of 4 parameters: pericardial effusion (PE) (1 point), low electrocardiographic QRS voltages (LQRSV) (1 point), CMR-derived impaired global radial strain (GRS) (<15.14%) (1 point) and increased left ventricular maximum wall thickness (LVMWT) (>13 mm) (2 points). Total score ranged from 0 to 5 points. A cut-off point of 1.5 showed highest accuracy in diagnosing AL CA with an AUC of 0.961 (95% CI: 0.924–0.997, sensitivity: 90.6%, specificity: 83.9%). Kaplan-Meier plots and log-rank test showed that the high-risk group had significantly poor overall survival rates.ConclusionIn patients at risk of AL CA, a risk score incorporating the presence of PE, LQRSV, and CMR-derived impaired GRS and increased LVMWT is predictive of a diagnosis of AL CA by LGE criteria. This risk score may be helpful especially when contrast CMR is not available or contraindicated, and further studies should be considered to validate this score.

Cardiovascular magnetic resonance for suspected cardiac amyloidosis: where are we now?

Cardiac amyloidosis (CA) is an underdiagnosed form of restrictive cardiomyopathy leading to a rapid progression into heart failure. Evaluation of CA requires a multimodality approach making use of echocardiography, cardiac magnetic imaging (CMR), and nuclear imaging. With superior tissue characterization, high-resolution imaging, and precise cardiac assessment, CMR has emerged as a versatile tool in the workup of cardiac amyloidosis with a wide array of parameters both visual and quantitative. This includes late gadolinium enhancement patterns, T1/T2 mapping, and extracellular volume (ECV) measurement providing robust diagnostic accuracies, patient stratification, and prognostication. Recent advancements have introduced new measures able to identify early disease, track disease progression, and response to therapy positioning CMR as an instrumental imaging modality in the era of rising interest in CA screening and emerging effective therapies.

Non-rigid motion-corrected free-breathing 3D myocardial Dixon LGE imaging in a clinical setting

ObjectivesTo investigate the efficacy of an in-line non-rigid motion-compensated reconstruction (NRC) in an image-navigated high-resolution three-dimensional late gadolinium enhancement (LGE) sequence with Dixon water–fat separation, in a clinical setting.MethodsForty-seven consecutive patients were enrolled prospectively and examined with 1.5 T MRI. NRC reconstructions were compared to translational motion-compensated reconstructions (TC) of the same datasets in overall and different sub-category image quality scores, diagnostic confidence, contrast ratios, LGE pattern, and semiautomatic LGE quantification.ResultsNRC outperformed TC in all image quality scores (p < 0.001 to 0.016; e.g., overall image quality 5/5 points vs. 4/5). Overall image quality was downgraded in only 23% of NRC datasets vs. 53% of TC datasets due to residual respiratory motion. In both reconstructions, LGE was rated as ischemic in 11 patients and non-ischemic in 10 patients, while it was absent in 26 patients. NRC delivered significantly higher LGE-to-myocardium and blood-to-myocardium contrast ratios (median 6.33 vs. 5.96, p < 0.001 and 4.88 vs. 4.66, p < 0.001, respectively). Automatically detected LGE mass was significantly lower in the NRC reconstruction (p < 0.001). Diagnostic confidence was identical in all cases, with high confidence in 89% and probable in 11% datasets for both reconstructions. No case was rated as inconclusive.ConclusionsThe in-line implementation of a non-rigid motion-compensated reconstruction framework improved image quality in image-navigated free-breathing, isotropic high-resolution 3D LGE imaging with undersampled spiral-like Cartesian sampling and Dixon water–fat separation compared to translational motion correction of the same datasets. The sharper depictions of LGE may lead to more accurate measures of LGE mass.Key Points• 3D LGE imaging provides high-resolution detection of myocardial scarring.• Non-rigid motion correction provides better image quality in cardiac MRI.• Non-rigid motion correction may lead to more accurate measures of LGE mass.

Hybrid CMR- and FDG-PET-Imaging Gives New Insights Into the Relationship of Myocardial Metabolic Activity and Fibrosis in Patients With Becker Muscular Dystrophy.

BackgroundCardiac involvement in patients with Becker muscular dystrophy (BMD) is an important predictor of mortality. The cardiac phenotype of BMD patients is characterized by slowly progressive myocardial fibrosis that starts in the left ventricular (LV) free wall segments and extends into the septal wall during the disease course.PurposeSince the reason for this characteristic cardiac phenotype is unknown and comprehensive approaches using e.g. hybrid imaging combining cardiovascular magnetic resonance (CMR) with 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET) are limited, the present study addressed this issue by a comprehensive non-invasive imaging approach.MethodsHybrid CMR- and FDG-PET-imaging was performed in N = 14 patients with BMD on a whole-body Biograph mMR system (Siemens, Erlangen, Germany). The CMR protocol comprised cine- and late-gadolinium-enhancement (LGE)-imaging. Metabolism was assessed with FDG-PET after oral glucose loading to effect myocardial carbohydrate uptake. PET was acquired for 65 min starting with tracer injection. Uptake values from 60 to 65 min p.i. were divided by the area under the blood activity curve and reported as percentages relative to the segment with maximal myocardial FDG uptake.ResultsA characteristic pattern of LGE in the LV lateral wall was observed in 13/14 patients whereas an additional septal LGE pattern was documented in 6/14 patients only. There was one patient without any LGE. Segmental FDG uptake was 88 ± 6% in the LV lateral wall vs. 77 ± 10% in the septal wall (p < 0.001). There was an inverse relationship between segmental FDG activity compared to segmental LGE extent (r = −0.33, p = 0.089). There were N = 6 LGE-positive patients with a segmental difference in FDG uptake of >15% in the LV lateral wall compared to the septal wall = ΔFDG-high group (lateral FDG = 91±3% vs. septal FDG = 69±8%; p < 0.001) while the remaining N = 7 LGE-positive patients showed a segmental difference in FDG uptake of ≤ 15% = ΔFDG-low group (lateral FDG = 85±7% vs. septal FDG = 83 ± 5%; p = 0.37). Patients in the ΔFDG-high group showed only a minor difference in the LGE extent between the LV lateral wall vs. septal wall (p = 0.09) whereas large differences were observed in the ΔFDG-low group (p < 0.004).ConclusionsSegmental FDG uptake—reflecting myocardial metabolic activity—is higher in the LV free wall of BMD patients—possibly due to a higher segmental work load. However, segmental metabolic activity seems to be dependent on and limited by the respective segmental extent of myocardial fibrosis as depicted by LGE-imaging.

Characteristics of COVID-19 Myocarditis With and Without Multisystem Inflammatory Syndrome

Multisystem inflammatory syndrome (MIS) is a severe complication described in a minority of patients with COVID-19. Myocarditis has been reported in patients with COVID-19, including MIS. In this study, we compared the clinical characteristics and cardiac magnetic resonance (CMR) findings of COVID-19 myocarditis in patients with and without MIS. In the 330 patients with COVID-19 who were referred for CMR at our institution between July 24, 2020, to March 31, 2021, 40 patients were identified as having myocarditis, MIS myocarditis (n = 21) and non-MIS myocarditis (n = 19). MIS myocarditis was characterized by global myocardial inflammation/edema with significantly elevated native T1, whereas only regional inflammation, and edema were noted in the non-MIS group. Distinct late gadolinium enhancement (LGE) patterns—inferior myocardial involvement in non-MIS myocarditis and septal involvement in MIS myocarditis—were identified. The LGE burden was comparable between the 2 groups (5.9% vs 6.6%, MIS vs non-MIS group, p = 0.83). Myocarditis was diagnosed more frequently by CMR in the MIS group (70% vs 6.3%, MIS vs non-MIS, p <0.001). In the 20 patients with a sequential CMR study at a median 102-day follow-up, 25% had persistent myocardial edema. The LGE burden improved over time, from a median of 5.0% (interquartile range 3.4% to 7.3%) to 3.2% (interquartile range 2.0% to 3.8%; p <0.001). In conclusion, MIS and non-MIS myocarditis exhibit distinct characteristics by CMR. Persistent LGE and edema were common at follow-up CMR examination in both groups.

Prevalence and prognostic significance of ischemic late gadolinium enhancement pattern in non-ischemic dilated cardiomyopathy

Typical late gadolinium enhancement (LGE) patterns in dilated cardiomyopathy (DCM) include intramyocardial and subepicardial distribution. However, the ischemic pattern of LGE (subendocardial and transmural) has also been reported in DCM without coronary artery disease (CAD), but its correlates and prognostic significance are still not known. On these bases, this study sought to describe the prevalence and prognostic significance of the ischemic LGE pattern in DCM. A total of 611 DCM patients with available cardiac magnetic resonance were retrospectively analyzed. A composite of all-cause-death, major ventricular arrhythmias (MVAs), heart transplantation (HTx) or ventricular assist device (VAD) implantation was the primary outcome of the study. Secondary outcomes were a composite of sudden cardiac death or MVAs and a composite of death for refractory heart failure, HTx or VAD implantation. Ischemic LGE was found in 7% of DCM patients without significant CAD or history of myocardial infarction, most commonly inferior/inferolateral/anterolateral. Compared to patients with non-ischemic LGE, those with ischemic LGE had higher prevalence of hypertension and atrial fibrillation or flutter. Ischemic LGE was associated with worse long-term outcomes compared to non-ischemic LGE (36% vs 23% risk of primary outcome events at 5 years respectively, P=.006), and remained an independent predictor of primary outcome after adjustment for clinically and statistically significant variables (adjusted hazard ratio 2.059 [1.055-4.015], P=.034 with respect to non-ischemic LGE). The ischemic pattern of LGE is not uncommon among DCM patients without CAD and is independently associated with worse long-term outcomes.

Role of magnetic resonance in the detection of cardiac involvement in patients with newly diagnosed extracardiac sarcoidosis: Single center experience.

Sarcoidosis is a systemic inflammatory disease of unknown etiology, which can affect almost any organ. Cardiac involvement determines the prognosis of the affected individuals. Its prevalence in patients with extracardiac sarcoidosis with the absence of cardiac symptoms remains unclear. Cardiac magnetic resonance (CMR) provides excellent diagnostic accuracy in the detection of heart involvement by sarcoidosis. We sought to determine the prevalence of cardiac sarcoidosis in asymptomatic individuals with newly diagnosed extracardiac sarcoidosis using CMR. We prospectively evaluated 55 consecutive patients including 23 women with newly diagnosed extracardiac sarcoidosis who underwent contrast-enhanced CMR and had no symptoms of heart disease. The mean (standard deviation) age of patients was 43 (11) years. The presence of myocardial late gadolinium enhancement (LGE) of non-ischemic etiology on CMR examination was considered diagnostic for cardiac sarcoidosis. In 3 (6%) patients, the LGE pattern consistent with cardiac sarcoidosis was detected. In all patients, preserved left ventricular systolic regional and global function was present, and in none of them, the elevation of blood biomarkers of myocardial injury or overload was found. Our study suggests that the prevalence of cardiac involvement in patients with newly diagnosed extracardiac sarcoidosis and no symptoms of heart disease is very low as assessed by CMR. However, CMR may be considered as part of routine evaluation of patients with extracardiac sarcoidosis due to its higher diagnostic yield in comparison with echocardiography and electrocardiography, respectively.

73 Prevalence and prognostic significance of ischaemic late gadolinium enhancement pattern in non-ischaemic dilated cardiomyopathy

Abstract Aims Typical late gadolinium enhancement (LGE) patterns in dilated cardiomyopathy (DCM) include intramyocardial and subepicardial distribution. However, the ischaemic pattern of LGE (subendocardial and transmural) has also been reported in DCM without coronary artery disease (CAD), but its correlates and prognostic significance are still not known. On these bases, this study sought to describe the prevalence and prognostic significance of the ischaemic LGE pattern in DCM. Methods and results 611 DCM patients with available cardiac magnetic resonance were retrospectively analysed. A composite of all-cause-death, major ventricular arrhythmias (MVAs), heart transplantation (HTx) or ventricular assist device (VAD) implantation was the primary outcome of the study. Secondary outcomes were a composite of sudden cardiac death or MVAs and a composite of death for refractory heart failure, HTx or VAD implantation. Ischaemic LGE was found in 7% of DCM patients without significant CAD or history of myocardial infarction, most commonly inferior/inferolateral/anterolateral. Compared to patients with non-ischaemic LGE, those with ischaemic LGE had higher prevalence of hypertension and atrial fibrillation or flutter. Ischaemic LGE was associated with worse long-term outcomes compared to non-ischaemic LGE (36% vs. 23% risk of primary outcome events at 5 years, respectively, P = 0.006), and remained an independent predictor of primary outcome after adjustment for clinically and statistically significant variables [adjusted hazard ratio 2.059 (1.055–4.015), P = 0.034 with respect to non-ischaemic LGE]. Conclusions The ischaemic pattern of LGE is not uncommon among DCM patients without CAD and is independently associated with worse long-term outcomes.

99mTechnetium‐pyrophosphate scintigraphy: a practical guide for early diagnosis of transthyretin amyloid cardiomyopathy

Transthyretin amyloid cardiomyopathy (ATTR‐CM) is caused by the cardiac deposition of insoluble amyloid fibrils formed by misfolded transthyretin proteins and is associated with various cardiac symptoms, such as progressive heart failure, conduction disturbance, and arrhythmia. The implementation of 99mtechnetium (99mTc)‐labelled bone radiotracer scintigraphy for diagnosing ATTR‐CM has enabled accurate diagnosis of the disease with high sensitivity and specificity and positioned this diagnostic modality as an integral part of disease diagnostic algorithms. In 2020, 99mTc‐pyrophosphate scintigraphy received exceptional approval for Japanese national health insurance reimbursement as a diagnostic method of ATTR‐CM. Nevertheless, the utility of 99mTc‐labelled bone radiotracer scintigraphy and the importance of an early diagnosis of suspected ATTR‐CM using this technique have yet to be internalized as common practice by general cardiologists, and guidance on daily clinical scenarios to consider this technique for a diagnosis of suspected ATTR‐CM is warranted. In this review, we discuss the utility of 99mTc‐labelled bone radiotracer scintigraphy for the early diagnosis of ATTR‐CM based on published literature and the outcomes of an advisory board meeting. This review also discusses clinical scenarios that could support early diagnosis of suspected ATTR‐CM as well as common pitfalls, correct implementation, and future perspectives of 99mTc‐labelled bone radiotracer scintigraphy in daily clinical practice. The clinical scenarios to consider 99mTc‐labelled bone radiotracer scintigraphy in daily practice may include, but are not limited to, patients with a family history of the hereditary type of disease; elderly patients (aged ≥60 years) with unexplained cardiac findings (e.g. cardiac hypertrophy associated with abnormalities on an electrocardiogram, heart failure with preserved ejection fraction associated with unexplained left ventricular hypertrophy, and heart failure with reduced ejection fraction associated with atrial fibrillation and left ventricular hypertrophy); and patients with cardiac hypertrophy associated with diastolic dysfunction, right ventricular/interatrial septum/valve thickness, left ventricular sparkling, or apical sparing. Cardiac hypertrophy and persistent elevation in cardiac troponin in elderly patients are also suggestive of ATTR‐CM. 99mTc‐labelled bone radiotracer scintigraphy is also recommended in patients with characteristic cardiac magnetic resonance findings (e.g. diffuse subendocardial late gadolinium enhancement patterns, native T1 increase, and increase in extracellular volume) or patients with cardiac hypertrophy and bilateral carpal tunnel syndrome.

Abstract 12851: Prognostic Value Of Late Gadolinium Enhancement In Patients With Idiopathic Dilated Cardiomyopathy

Introduction: The aim of this study was to investigate the association between the extent and pattern of late gadolinium enhancement (LGE) and outcomes in a large idiopathic dilated cardiomyopathy (DCM) cohort Hypothesis: LGE is associated with worse outcomes in patient with idiopathic dilated cardiomyopathy Methods: In this study, we retrospectively identified all patients with idiopathic DCM who underwent cardiac MRI (CMR) from 2015 to 2020 at a single large academic center. The association between the pattern of LGE and event free survival from transplantation and mechanical circulatory support was analyzed using Kaplan Meier analysis and the log-rank test. Results: Of a total of 286 patients (64% male, 62% Caucasian) with idiopathic DCM who underwent CMR, LGE was detected in 59 (21%) patients. Of the patients with LGE, 7 (3%) patients had free-wall LGE, 40 (14%) had septal LGE and 12 (4%) had both septal and free wall LGE. All patients had linear mid-wall LGE and two patients had both linear-mid wall and sub-epicardial involvement. The presence of septal LGE was associated with older age (58 ± 12 vs 47 ± 16 years, p&lt;0.001), a lower LV ejection fraction (EF) (30% ± 12 vs 37% ± 12, p&lt;0.001), higher LV end-diastolic volume index (146 ± 47 vs 126 ± 36 ml/m 2 , p&lt;0.001), lower right ventricular EF (37% ± 13 vs 42% ± 13, p=0.013), higher LV mass index (82 ± 25 vs 73 ± 24 g/m 2 , p=0.013), higher incidence of LBBB (29% ± 16 vs 16% ± 29) and atrial fibrillation (31% vs 17%, p=0.021). Over a mean follow-up time of 26 ± 19 months, patients with septal LGE were less likely to experience event free survival from heart transplant or durable mechanical circulatory support (Figure 1, p=0.009). Conclusions: The presence of septal LGE in this large idiopathic DCM cohort is associated with worse outcomes. Additional research is needed to determine whether these findings will aid clinical management of these patients.