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Objective: Cardiac magnetic resonance (CMR) is a diagnostic tool that provides precise and reproducible information about cardiac structure, function, and tissue characterization, aiding in the monitoring of chemotherapy response in patients with light-chain cardiac amyloidosis (AL-CA). This study aimed to evaluate the feasibility of CMR in monitoring responses to chemotherapy in patients with AL-CA., Materials and Methods: In this prospective study, we enrolled 111 patients with AL-CA (50.5% male; median age, 54 [interquartile range, 49-63] years). Patients underwent longitudinal monitoring using biomarkers and CMR imaging. At follow-up after chemotherapy, patients were categorized into superior and inferior response groups based on their hematological and cardiac laboratory responses to chemotherapy. Changes in CMR findings across therapies and differences between response groups were analyzed., Results: Following chemotherapy (before vs. after), there were significant increases in myocardial T2 (43.6 ± 3.5 ms vs. 44.6 ± 4.1 ms; P = 0.008), recovery in right ventricular (RV) longitudinal strain (median of -9.6% vs. -11.7%; P = 0.031), and decrease in RV extracellular volume fraction (ECV) (median of 53.9% vs. 51.6%; P = 0.048). These changes were more pronounced in the superior-response group. Patients with superior cardiac laboratory response showed significantly greater reductions in RV ECV (-2.9% [interquartile range, -8.7%-1.1%] vs. 1.7% [-5.5%-7.1%]; P = 0.017) and left ventricular ECV (-2.0% [-6.0%-1.3%] vs. 2.0% [-3.0%-5.0%]; P = 0.01) compared with those with inferior response., Conclusion: Cardiac amyloid deposition can regress following chemotherapy in patients with AL-CA, particularly showing more prominent regression, possibly earlier, in the RV. CMR emerges as an effective tool for monitoring associated tissue characteristics and ventricular functional recovery in patients with AL-CA undergoing chemotherapy, thereby supporting its utility in treatment response assessment., Competing Interests: The authors have no potential conflicts of interest to disclose., (Copyright © 2024 The Korean Society of Radiology.)

Objective: Myocardial fibrosis occurs in the early subclinical stage of cardiac involvement in idiopathic inflammatory myopathies (IIMs). Soluble suppression of tumorigenicity 2 (sST2) is known to have an immunomodulatory impact during autoimmune disease development. The current study investigated the diagnostic value of sST2 for myocardial fibrosis during early stage of cardiac involvement in IIM., Methods: A total of 44 IIM patients with normal heart function and 32 age- and gender-matched healthy controls (HCs) were enrolled. Serum sST2 levels were measured by ELISA and cardiac magnetic resonance (CMR) parameters for myocardial fibrosis [native T1, extracellular volume (ECV), late-gadolinium enhancement (LGE)] and oedema (T2 values) were analysed., Results: IIM patients had significantly higher sST2 levels than HCs [67.5 ng/ml (s.d. 30.4)] vs 14.4 (5.5), P < 0.001] and levels correlated positively with diffuse myocardial fibrosis parameters, native T1 (r = 0.531, P = 0.000), ECV (r = 0.371, P = 0.013) and focal myocardial fibrosis index and LGE (r = 0.339, P = 0.024) by Spearman's correlation analysis. sST2 was an independent predictive factor for diffuse and focal myocardial fibrosis after adjustment for age, gender, BMI and ESR. Risk increased ≈15.4% for diffuse [odds ratio (OR) 1.154 (95% CI 1.021, 1.305), P = 0.022] and 3.8% for focal [OR 1.038 (95% CI 1.006, 1.072), P = 0.020] myocardial fibrosis per unit increase of sST2. Cut-off values for diagnosing diffuse and focal myocardial fibrosis were sST2 ≥51.3 ng/ml [area under the curve (AUC) = 0.942, sensitivity = 85.7%, specificity = 98.9%, P < 0.001] and 53.3 ng/ml (AUC = 0.753, sensitivity = 87.5%, specificity = 58.3%, P < 0.01), respectively., Conclusion: sST2 showed a marked elevation during the subclinical stage of cardiac involvement in IIM and has potential as a biomarker for predicting diffuse and focal myocardial fibrosis in IIM., (© The Author(s) 2023. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Competing Interests: Competing interests: None declared.

Background: Left ventricular abnormalities in cardiac sarcoidosis (CS) are associated with adverse cardiovascular events, whereas the prognostic value of right ventricular (RV) involvement found on cardiac magnetic resonance is unclear., Objectives: This study aimed to systematically assess the prognostic value of right ventricular ejection fraction (RVEF) and RV late gadolinium enhancement (LGE) in known or suspected CS., Methods: This study was prospectively registered in PROSPERO (CRD42022302579). PubMed, Embase, and Web of Science were searched to identify studies that evaluated the association between RVEF or RV LGE on clinical outcomes in CS. A composite endpoint of all-cause death, cardiovascular events, or sudden cardiac death (SCD) was used. A meta-analysis was performed to determine the pooled risk ratio (RR) for these adverse events. The calculated sensitivity, specificity, and area under the curve with 95% CIs were weighted and summarized., Results: Eight studies including a total of 899 patients with a mean follow-up duration of 3.2 ± 0.7 years were included. The pooled RR of RV systolic dysfunction was 3.1 (95% CI: 1.7-5.5; P < 0.01) for composite events and 3.0 (95% CI: 1.3-7.0; P < 0.01) for SCD events. In addition, CS patients with RV LGE had a significant risk for composite events (RR: 4.8 [95% CI: 2.4-9.6]; P < 0.01) and a higher risk for SCD (RR: 9.5 [95% CI: 4.4-20.5]; P < 0.01) than patients without RV LGE. Furthermore, the pooled area under the curve, sensitivity, and specificity of RV LGE for identifying patients with CS who were at highest SCD risk were 0.8 (95% CI: 0.8-0.9), 69% (95% CI: 50%-84%), and 90% (95% CI: 70%-97%), respectively., Conclusions: In patients with known or suspected CS, RVEF and RV LGE were both associated with adverse events. Furthermore, RV LGE shows good discrimination in identifying CS patients at high risk of SCD., Competing Interests: Funding Support and Author Disclosures This work was supported by a grant from the 1.3.5 Project for Disciplines of Excellence, West China Hospital, Sichuan University (grant number ZYJC18003). Dr Hosadurg was funded by the National Institutes of Health T32 grant (grant number T32EB003841), and Dr Wang was funded by the National Natural Science Foundation of China (grant number 82202248). All other authors have reported that they have no relationships relevant to the contents of this paper to disclose, (Copyright © 2023 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: Myocardial fibrosis is a common pathophysiological process involved in many cardiovascular diseases. However, limited prior studies suggested no association between focal myocardial fibrosis detected by cardiovascular magnetic resonance (CMR) late gadolinium enhancement (LGE) and disease severity in Eisenmenger syndrome (ES). This study aimed to explore potential associations between myocardial fibrosis evaluated by the CMR LGE and T1 mapping and risk stratification profiles including exercise tolerance, serum biomarkers, hemodynamics, and right ventricular (RV) function in these patients., Methods: Forty-five adults with ES and 30 healthy subjects were included. All subjects underwent a contrast-enhanced 3T CMR. Focal replacement fibrosis was visualized on LGE images. The locations of LGE were recorded. After excluding LGE in ventricular insertion point (VIP), ES patients were divided into myocardial LGE-positive (LGE + ) and LGE-negative (LGE - ) subgroups. Regions of interest in the septal myocardium were manually contoured in the T1 mapping images to determine the diffuse myocardial fibrosis. The relationships between myocardial fibrosis and 6-min walk test (6MWT), N-terminal pro-brain natriuretic peptide (NT-pro BNP), hematocrit, mean pulmonary arterial pressure (mPAP), pulmonary vascular resistance index (PVRI), RV/left ventricular end-systolic volume (RV/LV ESV), RV ejection fraction (RVEF), and risk stratification were analyzed., Results: Myocardial LGE (excluding VIP) was common in ES (16/45, 35.6%), and often located in the septum (12/45, 26.7%). The clinical characteristics, hemodynamics, CMR morphology and function, and extracellular volume fraction (ECV) were similar in the LGE + and LGE - groups (all P > 0.05). ECV was significantly higher in ES patients (28.6 ± 5.9% vs. 25.6 ± 2.2%, P < 0.05) and those with LGE - ES (28.3 ± 5.9% vs. 25.6 ± 2.2%, P < 0.05) than healthy controls. We found significant correlations between ECV and log NT-pro BNP, hematocrit, mPAP, PVRI, RV/LV ESV, and RVEF (all P < 0.05), and correlations trends between ECV and 6MWT (P = 0.06) in ES patients. An ECV threshold of 29.0% performed well in differentiating patients with high-risk ES from those with intermediate or low risk (area under curve 0.857, P < 0.001)., Conclusions: Myocardial fibrosis is a common feature of ES. ECV may serve as an important imaging marker for ES disease severity., (© 2022. The Author(s).)

Background: Congenital shunt location is related to Eisenmenger syndrome (ES) survival. Moreover, right ventricular (RV) remodeling is associated with poor survival in pulmonary hypertension., Purpose: To investigate RV remodeling using comprehensive magnetic resonance imaging (MRI) techniques and identify its relationship with prognosis in ES subgroups classified by shunt location., Study Type: Prospective observational study., Population: Fifty-four adults with ES (16 with pre-tricuspid shunt and 38 with post-tricuspid shunt)., Field Strength/sequence: 3.0 T/cine MRI with balanced steady-state free precession sequence, late gadolinium enhancement with inversion recovery segmented gradient echo sequence and phase-sensitive reconstruction, and T1 mapping with modified Look-Locker inversion recovery sequence., Assessment: Demographics, clinical characteristics, hemodynamics, RV remodeling features (morphology, systolic function, RV-pulmonary artery (PA) coupling and myocardial fibrosis), and prognosis were compared between ES subgroups. The adverse endpoint was all-cause mortality or readmission for heart failure., Statistical Tests: The independent samples t-test, Fisher's exact test or Chi-squared test, and the Kaplan-Meier method were used. P < 0.05 was considered significant., Results: Compared to patients with post-tricuspid shunt, patients with pre-tricuspid shunt were significantly older and had higher N-terminal pro-B-type natriuretic peptide concentrations and poorer exercise tolerance. Pre-tricuspid shunt showed significantly larger RV dimensions (end-diastolic volume index: 185.81 ± 37.49 vs. 98.20 ± 36.26 mL/m 2 ), worse RV ejection fraction (23.54% ± 12.35% vs. 40.82% ± 10.77%), and RV-PA decoupling (0.35 ± 0.31 vs. 0.72 ± 0.29). Biventricular myocardial fibrosis was significantly more severe in pre-tricuspid shunt than post-tricuspid shunt (extracellular volume, left ventricle: 35.85% ± 2.58% vs. 29.10% ± 5.20%; RV free wall: 30.93% ± 5.65% vs. 26.75% ± 5.15%). In addition, pre-tricuspid shunt demonstrated a significantly increased risk of adverse endpoint (hazard ratio: 2.938, 95% confidence interval: 1.204-7.172)., Data Conclusion: ES with pre-tricuspid shunt might be a unique subtype with worse clinically decompensated RV remodeling and poor prognosis., Level of Evidence: 2 Technical Efficacy Stage: 5., (© 2021 International Society for Magnetic Resonance in Medicine.)

Danon disease (DD) is a rare X-chromosome-linked dominant lysosomal glycogen storage disease. Its features have seldom been reported by using cardiac MRI. This case series aimed to evaluate cardiac features of DD on the basis of MRI observations from five centers in China. From January 2010 to May 2019, 16 patients with DD (13 male patients [81%]; median age, 19 years; age range, 14-44 years) underwent MRI. The most frequent DD cardiomyopathy manifestation was symmetric hypertrophy cardiomyopathy (HCM) phenotype (nine of 16; 56%), followed by asymmetric HCM phenotype (six of 16; 38%) and dilated cardiomyopathy phenotype (one of 16; 6%). The characteristic late gadolinium enhancement features included midbasal septum sparing (14 of 16; 88%) and apex involvement (16 of 16; 100%) with a base-to-apex increasing tendency, free wall involvement (15 of 16; 94%), and extensive subendocardium involvement (14 of 16; 88%). Abnormal T2 signal (seven of 16; 44%) and resting perfusion defect (14 of 16; 88%) were not uncommon in patients with DD. Furthermore, the cardiac MRI features of DD cohort in this study were compared with those of DD in previous literature and with genetically confirmed sarcomeric HCM., (© RSNA, 2021 See also the editorial by Hanneman in this issue.)

Introduction: Patients with cardiac amyloidosis light chain (AL) present with negative Tc-99m pyrophosphate (PYP) scintigraphy (absent or mild heart uptake). On the contrary, patients with cardiac amyloidosis transthyretin (ATTR) present with positive Tc-99m PYP scanning (intensive heart uptake). We present a false positive Tc-99m PYP scintigraphy (grade 2, the heart-to-contralateral ratio is 1.65) in a patient with AL., Patient Concerns: A 42-year-old Chinese man complained of effort intolerance, chest discomfort, and short of breath progressively over 1 year. New York Heart Association Class III. Physical examination showed legs swelling. Laboratory revealed elevated brain natriuretic peptide of 23,031 ng/mL (0-88) and Troponin-T of 273.4 ng/mL (0-14)., Diagnosis: Cardiac amyloidosis light chain. Evidences: free light chains (FLCs): decreased serum free kappa/lambda ratio of 0.043 (0.31-1.56). Immunofixation electrophoresis: a positive lambda light chain monoclonal protein. Cardiac biopsy: HE: Ambiguity Congo red strain. Myocardial immunofluorescence: positive lambda light chain. Myocardial immunohistochemistry: positive lambda light chain, negative kappa light chain, and TTR., Interventions: Furosemide 40 mg qd, torasemide 20 mg qd, spirolactone 20 mg qd, potassium chloride 10 mL per 500 mL urine, atorvastatin calcium tablet 20 mg qd, aspirin enteric-coated tablets 100 mg qd during the 2-weeks in-hospital., Outcomes: The patient died 2 months later after discharge., Conclusion: False positive Tc-99m PYP scintigraphy may rarely presented in patients with cardiac amyloidosis light chain. So, the clonal plasma cell process based on the FLCs and immunofixation is a base to rule out AL cardiac amyloidosis when we interpret a positive Tc-99m PYP scintigraphy., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.)

Background: Idiopathic inflammatory myopathy (IIM) manifest as systematic muscle involvement. Multiparametric cardiovascular magnetic resonance (CMR) could be a useful technique to detect systemic involvement and disease progression in IIM patients. This study aimed to describe the tissue characteristics and dynamic changes in myocardial and skeletal muscles after treatment in IIM patients., Methods: Forty-four consecutively recruited IIM patients (49.0 ± 12.0 years; 22 males) underwent 3 T CMR at first diagnosis, and 28 patients underwent follow-up scan after receiving standard treatment for more than 1 year. Thirty age- and sex-matched healthy subjects served as controls. The CMR protocol included: cines, T2-weighted (T2w), late gadolinium enhancement (LGE), T1 and T2 mapping, and extracellular volume (ECV) evaluated for the myocardium, and T1 and T2 mapping and ECV evaluated for skeletal muscles. Correlations between laboratory biomarkers and myocardial and skeletal tissue characteristics were analyzed. Comparisons between baseline and follow-up scans were performed using paired t-tests., Results: At baseline, IIM patients showed significantly decreased hematocrit, higher left ventricular (LV) mass index, right ventricular (RV) volume index, myocardial and skeletal native T1, T2 mapping, and ECV than healthy controls. Significant correlations were found among myocardial native T1, T2 mapping, and ECV values and N-terminal pro b-type natriuretic peptide (NT-proBNP) levels, and significant correlations between skeletal T2 mapping and inflammatory biomarkers in IIM patients. During the follow-up, 28 patients underwent repeated CMR scan (median interval, 14.5 months, interquartile range: 13.2-15.5 months). Significant relief from clinical symptoms and decreased inflammatory biomarkers levels were observed. Significant reduction in myocardial native T1, T2, ECV, and skeletal native T1, T2, and ECV were observed during the follow-up assessment., Conclusions: Both myocardial and skeletal muscles in newly diagnosed IIM patients show distinct characteristics on multiparametric CMR. In addition, significant changes were observed in patients showing clinical remission after effective treatment, which suggests that quantitative T1, T2, and ECV techniques may have potential clinical value in IIM patients.

Cardiovascular magnetic resonance imaging (CMR) is a comprehensive and versatile diagnostic and prognostic imaging modality that plays an increasingly important role in management of patients with cardiovascular disease. In this review, we discuss CMR applications in nonischemic cardiomyopathy, ischemic heart disease, arrhythmias, right ventricular diseases, and valvular heart disease. We emphasize the quantitative nature of CMR in current practice, from volumes, function, myocardial strain analysis, and late gadolinium enhancement to parametric mapping, including T1, T2, and T2 * relaxation times and extracellular volume fraction assessment.

Background: Segmental myocardial strain using feature tracking (FT) cardiac MRI is not acceptable due to poor reproducibility., Purpose: To assess the reproducibility of left ventricle (LV) segmental myocardial strain measured by deformation registration algorithm (DRA)., Study Type: Prospective clinical trial., Subjects: Sixteen healthy volunteers and 28 hypertrophic cardiomyopathy (HCM) patients., Field Strength/sequence: Retrospective ECG gating cardiac MRI imaging was performed at 3.0T with a steady-state free precession (SSFP) sequence., Assessment: LV global and segmental myocardial strains were analyzed by DRA, FT, and speckle tracking echocardiography (STE) by two experienced observers and the reproducibility of global and segmental strains were compared., Statistical Tests: Reproducibility was tested by coefficient of variation (COV) and intraclass correlation coefficient (ICC). Receiver operator curves as well as comparison of areas under the curve (AUC) were analyzed., Results: DRA showed the best observer agreement on segmental strain evaluated by ICC, LS (longitudinal strain): intraobserver variability range (0.98,1.00), interobserver variability range (0.83,0.92), CS (circumferential strain): intraobserver variability range (0.90,0.99), interobserver variability range (0.80,0.97), RS (radial strain): intraobserver variability range (0.84,0.99), interobserver variability range (0.85,0.99). Segmental LS, CS, and RS agreements evaluated by COV for FT and STE were poor. LV global myocardial strain of HCM was significantly lower than controls for all applied techniques, but global CS by DRA had better accuracy compared to FT or STE for distinguishing HCM from healthy subjects: AUC 0.880 (DRA) vs. 0.577 (FT) or 0.736 (STE), P < 0.05., Data Conclusions: DRA is a reliable and robust analysis tool for segmental myocardial strain. Global CS by DRA allows discrimination between HCM and normal controls with better accuracy compared with FT and STE., Level of Evidence: 1 Technical Efficacy: Stage 2 J. MAGN. RESON. IMAGING 2018;48:404-414., (© 2017 International Society for Magnetic Resonance in Medicine.)

Objectives: This study aimed to test the characteristics of the myocardial extracellular volume fraction (ECV) derived from pre- and post-contrast T1 measurements among healthy volunteers., Background: Cardiac magnetic resonance (CMR) T1 measurements of myocardium and blood before and after contrast allow quantification of the ECV, a tissue parameter that has been shown to change in proportion to the connective tissue fraction., Methods: Healthy volunteers underwent standard CMR imaging with administration of gadolinium. T1 measurements were performed with a Look-Locker sequence followed by gradient-echo acquisition. We tested the segmental, interslice, inter-, intra-, and test-retest characteristics of the ECV, as well as the association of the ECV with other variables. Juvenile and aged mice underwent a similar protocol, and cardiac sections were harvested for measurement of fibrosis., Results: In healthy volunteers (N = 32, 56% female; age 21 to 72 years), the ECV averaged 0.28 ± 0.03 (range 0.23 to 0.33). The intraclass coefficients for the intraobserver, interobserver, and test-retest absolute agreements of the ECV were 0.94 (95% confidence interval: 0.84 to 0.98), 0.93 (95% confidence interval: 0.80 to 0.98), and 0.95 (95% confidence interval: 0.52 to 0.99), respectively. In volunteers, the ECV was associated with age (r = 0.74, p < 0.001), maximal left atrial volume index (r = 0.67, p < 0.001), and indexed left ventricular mass. There were no differences in the ECV between segments in a slice or between slices. In mice (N = 12), the myocardial ECV ranged from 0.20 to 0.32 and increased with age (0.22 ± 0.02 vs. 0.30 ± 0.02, juvenile vs. aged mice, p < 0.001). In mice, the ECV correlated with the extent of myocardial fibrosis (r = 0.94, p < 0.001)., Conclusions: In healthy volunteers, the myocardial ECV ranges from 0.23 to 0.33, has acceptable test characteristics, and is associated with age, left atrial volume, and left ventricular mass. In mice, the ECV also increases with age and strongly correlates with the extent of myocardial fibrosis., (Copyright © 2013 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Aims To explore the prognosis of myocardial oedema measured by T2 mapping in hypertrophic cardiomyopathy (HCM). Methods and results A total of 674 patients with HCM (age: 50 ± 15 years, 60.5% males) who underwent cardiovascular magnetic resonance were prospectively enrolled from 2011 to 2020. One hundred healthy controls (age: 48 ± 19 years, 58.0% males) were included for comparison. Myocardial oedema was quantitatively measured by T2 mapping in both global and segmental myocardium. The endpoints were defined as a combination of cardiovascular death and appropriate implantable cardioverter defibrillator discharge. During a median follow-up of 36 months (interquartile range: 24–60 months), 55 patients (8.2%) had cardiovascular events. Patients with cardiovascular events had a higher T2 max, T2 min, and T2 global values (all P < 0.001) than patients who remained event free. Survival analysis demonstrated that patients with HCM with late gadolinium enhancement [LGE(+)] and T2 max ≥44.9 ms had a higher risk of developing cardiovascular events (P < 0.001). A multivariate Cox regression analysis showed that T2 max, T2 min, and T2 global provided significant prognostic value to predict cardiovascular events (all P < 0.001). According to the C-index (0.825, 0.814), net reclassification index (0.612, 0.536, both P < 0.001), and integrative discrimination index (0.029, 0.029, both P < 0.05), T2 max or T2 min significantly increased the predictive performance of established risk factors, including extensive LGE. Conclusion Patients with HCM with LGE(+) and higher T2 had worse prognosis than those with LGE(+) and lower T2. [ABSTRACT FROM AUTHOR]

Objectives: Hypertrophic cardiomyopathy (HCM) patients are closely related to LA functional impairment. Left atrial (LA) strain provides more insight into LA function. The study aimed to investigate the left atrial dysfunction of HCM patients by rapid semi-automatic method and determine the predictive value of left atrial long-axis strain (LAS) for atrial fibrillation (AF). Methods: We enrolled 372 HCM patients and 100 healthy participants to assess the LA functional parameters. LAS was obtained by semi-automated tracking of the distance between the mid-posterior point of LA wall which is defined as the intersection of the LA long axis and the posterior wall, and the origins of the mitral valve. The inferior and anterior mitral valve annular insertion points on the 2-chamber view and the lateral and septal insertion points on the 4-chamber view were chosen as the origins of the mitral valve. The clinical outcome was defined as detecting the onset of AF. Results: The LA strain values were analyzed as 20.8 ± 7.48% for εs, 9.81 ± 5.09% for εe, and 10.91 ± 4.24% for εa in HCM patients, which decreased significantly compared with normal controls. Significant correlations were detected between LV functional parameters and LA strain. During a median follow-up of 61 months, 44 (11.8%) patients developed AF. In the Cox regression analysis, LA strain was identified as a significant predictor of the onset of AF. Conclusions: HCM patients had impairment of LA strain before LA enlargement and reduced EF. LA-LAS can be used as a predictive value for predicting the occurrence of AF in HCM patients. Key points: • Fast semi-automated long-axis strain analysis by CMR is feasible and effective for evaluating the LA longitudinal function. • Hypertrophic cardiomyopathy patients show significant impairment of left atrial strain before LA enlargement. • The left atrial long-axis strain was an independent predictor of atrial fibrillation in hypertrophic cardiomyopathy. [ABSTRACT FROM AUTHOR]

This article discusses a case study of a 35-year-old man with FLNC cardiomyopathy, a rare type of cardiomyopathy with a high risk of arrhythmia. The patient initially presented with symptoms of fatigue and exertional dyspnea, and cardiac imaging revealed significant enlargement of the left ventricle and reduced ejection fraction. After 1.5 years of medical treatment, the patient showed improvement in left ventricular ejection fraction and thickening of the myocardium. However, there was also progression of myocardial fibrosis, indicating that the arrhythmogenic risk associated with FLNC cardiomyopathy may not be changed by functional recovery. Further investigation is needed to understand the relationship between cardiac function and myocardial tissue characteristics in this patient. [Extracted from the article]

Objective: To evaluate the prognostic value of late gadolinium enhancement (LGE) in myocarditis and clinically suspected myocarditis.Methods: The study was registered in PROSPERO (CRD42019144976). A systematic search of PubMed, Ovid Medline, Embase, Web of Science and the Cochrane Central Register of Controlled Trials was completed. Major adverse cardiac event (MACE) was defined as the combination of all-cause mortality or cardiovascular death, resuscitated cardiac arrest, heart transplantation, appropriate implantable cardioverter-defibrillator shock, rehospitalisation following a cardiac event and recurrent acute myocarditis. Combined outcome was defined as the combination of all adverse events. Pooled odds ratios (ORs) and 95% confidence intervals (CIs) were calculated to evaluate the prognostic value of LGE.Results: Eight articles including 1319 patients (mean age, 38.8 ± 12.9 years) were included in the meta-analysis. The study showed that positive LGE was strongly associated with an increased risk of combined outcome (pooled OR, 5.85; 95% CI, 2.88 to 11.86; p < 0.001) and of MACE (pooled OR, 4.57; 95% CI, 2.18 to 9.59; p < 0.001). Additionally, in a subgroup analysis with mean ejection fraction (EF) point of 50%, the pooled ORs for the combined outcome were 6.46 for left ventricular EF (LVEF) > 50% and 7.90 for LVEF ≤ 50%, and the pooled ORs for MACE were 9.03 and 3.45, respectively. After 3 years of follow-up, the worse outcomes occurred mainly in patients with positive LGE.Conclusion: Positive LGE is a powerful prognosticator of adverse outcome in myocarditis and clinically suspected myocarditis, irrespective of LVEF.Key Points: • Forty-four percent to 100% of myocarditis patients have positive late gadolinium enhancement. • Positive LGE was a powerful prognosticator of adverse outcome in myocarditis and clinically suspected myocarditis, irrespective of LVEF. • LGE-CMR is important tool for risk stratification in myocarditis and clinically suspected myocarditis. [ABSTRACT FROM AUTHOR]

Background: The cardiac Magnetic Resonance Imaging (MRI) characteristics of rare diseases with the hypertrophic cardiomyopathy (HCM) phenotype are not well defined. Methods: Seventy-three sequential patients and 34 of their relatives, who have the HCM phenotype, were included. All subjects underwent cardiac MRI and genetic testing. Results: Of these 107 patients with phenotypic HCM, seven rare diseases were identified: four cases with LAMP2, one case with PRKAG2, one case with TTR mutation, and one case with senile systemic amyloidosis. Subjects with rare diseases had diffuse LGE, and the percentage of those with LGE was significantly higher than that of other HCM (median: 18.9%, interquartile range (IQR): 14.05 to 28.2% versus 7.8%, IQR: 4.41 to 14.56%; p = 0.003). Additionally, global T1 and ECV were significantly higher in subjects with rare diseases (global T1: 1423.1 ± 93.3 ms versus 1296.2 ± 66.6 ms; global ECV: 44.3 ± 11.5% versus 29.9 ± 4.5%; all p < 0.001). Conclusions: Cardiac MRI suggests the existence of distinct imaging characteristics, including via LGE and T1 mapping, among rare diseases that mimic HCM and HCM itself. [ABSTRACT FROM AUTHOR]

An abstract of the conference paper "Quantitative of myocardial extracellular volume fraction improves characterization of fibrotic burden in patients with apical hypertrophic cardiomyopathy beyond visual assessment with late gadolinium enhancement," by Ravi Shah and colleagues is presented.