Left Ventricular Late Gadolinium Enhancement Research Articles

Left and right atrioventricular coupling index in patients with beta-thalassemia major.

The aim of this cross-sectional study was to investigate the relationship of left atrioventricular coupling index (LACI) and right atrioventricular coupling index (RACI) with demographics, clinical data, cardiovascular magnetic resonance findings, and cardiac complications (heart failure, arrhythmias, and pulmonary hypertension) in a cohort of patients with beta-thalassemia major (β-TM). We evaluated 292 β-TM patients (151 females, 36.72 ± 11.76years) consecutively enrolled in the Extension-Myocardial Iron Overload in Thalassemia (E-MIOT) project. Moreover, we assessed 32 sex- and age-matched healthy controls (12 females, mean age 40.78 ± 14.35years). LACI was determined by calculating the ratio of the left atrium end-diastolic volume to the left ventricle end-diastolic volume, while RACI was defined by calculating the ratio of the right atrium end-diastolic volume to the right ventricle end-diastolic volume. Compared to healthy control, β-TM demonstrated increased LACI (22.99 ± 13.58% vs. 16.05 ± 5.28%; p < 0.0001) and RACI (27.84 ± 10.30% vs. 17.06 ± 5.03%; p < 0.0001). Aging, diabetes, splenectomy, and the presence of late gadolinium enhancement (LGE) showed a significant positive association with both LACI and RACI. In stepwise regression analysis, the presence of LGE was found to be an independent predictor of both impaired LACI and RACI (β coefficient = 0.244, p < 0.0001 and β coefficient = 0.218, p = 0.003; respectively). LACI and RACI were not correlated with myocardial iron overload. Patients with cardiac complications had significantly higher LACI and RACI than patients without cardiac complications. In patients with β-TM, LACI and RACI were significantly associated with the presence of LV LGE. In addition, patients with cardiac complications had impaired LACI and RACI.

Asymmetric Myocardial Involvement as an Early Indicator of Cardiac Dysfunction in Pediatric Dystrophinopathies: A Study on Cardiac Magnetic Resonance (CMR) Parametric Mappings.

Dystrophinopathies, such as Duchenne and Becker muscular dystrophy, frequently lead to cardiomyopathy, being its primary cause of mortality. Detecting cardiac dysfunction early is crucial, but current imaging methods lack insight into microstructural remodeling. This study aims to assess the potential of cardiac magnetic resonance (CMR) parametric mappings for early detection of myocardial involvement in dystrophinopathies and explores whether distinct involvement patterns may indicate impending dysfunction. In this prospective study, 23 dystrophinopathy patients underwent CMR with tissue mappings. To establish a basis for comparison, a control group of 173 subjects was analyzed. CMR protocols included SSFP, T2-weighted and T1-weighted sequences pre and post gadolinium, and tissue mappings for native T1 (nT1), extracellular volume (ECV), and T2 relaxation times. The difference between the left ventricular posterior wall and the interventricular septum was calculated to reveal asymmetric myocardial involvement. Significant differences in LV ejection fraction (LVEF), myocardial mass, and late gadolinium enhancement confirmed abnormalities in patients. Tissue mappings: nT1 (p < 0.001) and ECV (p = 0.002), but not T2, displayed substantial variations, suggesting sensitivity to myocardial involvement. Asymmetric myocardial involvement in nT1 (p = 0.01) and ECV (p = 0.012) between septal and LV posterior wall regions was significant. While higher mapping values didn't correlate with dysfunction, asymmetric involvement in nT1 (ρ=-0.472, p = 0.023) and ECV (ρ=-0.460, p = 0.049) exhibited a significant negative correlation with LVEF. CMR mappings show promise in early myocardial damage detection in dystrophinopathies. Although mapping values may not directly correspond to dysfunction, the negative correlation between asymmetric involvement in nT1 and ECV with LVEF suggests their potential as early biomarkers. Larger, longitudinal studies are needed for a comprehensive understanding and improved risk stratification in dystrophinopathies.

Time to Recovery from Systolic Dysfunction Correlates with Left Ventricular Fibrosis in Arrhythmia-Induced Cardiomyopathy.

Arrhythmia-induced cardiomyopathy (AIC) is characterized by the reversibility of left ventricular (LV) systolic dysfunction (LVSD) after rhythm restoration. This study is a cardiac magnetic resonance tomography substudy of our AIC trial with the purpose to investigate whether left ventricular fibrosis affects the time to recovery (TTR) in patients with AIC. Patients with newly diagnosed and otherwise unexplainable LVSD and tachyarrhythmia were prospectively recruited. LV ejection fraction (LVEF) was measured by echocardiography at baseline and 2, 4, and 6 months after rhythm control, and stress markers were assessed. After initial rhythm control, LV fibrosis was assessed through late gadolinium enhancement (LGE). Patients were diagnosed with AIC if their LVEF improved by ≥15% (or ≥10% when LVEF reached ≥50%). Non-responders served as controls (non-AIC). The LGE analysis included 39 patients, 31 of whom recovered (AIC). LV end-systolic diameters decreased and LVEF increased during follow-up. LV LGE content correlated positively with TTR (r = 0.63, p = 0.003), with less LGE favoring faster recovery, and negatively with ΔLVEF (i.e., LVEF at month 2 compared to baseline) as a marker of fast recovery (r = -0.55, p = 0.012), suggesting that LV fibrosis affects the speed of recovery. LV fibrosis correlated positively with the time to recovery in patients with AIC. This correlation may help in the estimation of the recovery period and in the optimization of diagnostic and therapeutic strategies for patients with AIC.

Cardiac magnetic resonance imaging in heart transplant recipients with biopsy-negative graft dysfunction.

Graft dysfunction (GD) after heart transplantation (HTx) can develop without evidence of cell- or antibody-mediated rejection. Cardiac magnetic resonance imaging (CMR) has an evolving role in detecting rejection; however, its role in biopsy-negative GD has not been described. This study examines CMR findings, evaluates outcomes based on CMR results, and seeks to identify the possibility of rejection missed through endomyocardial biopsy by using CMR in HTx recipients with biopsy-negative GD. HTx recipients with GD [defined as a decrease in left ventricular ejection fraction (LVEF) by >5% and LVEF<50%] in the absence of rejection by biopsy or allograft vasculopathy and who underwent CMR were included in the study. The primary outcome was a composite of all-cause mortality, re-transplantation, or persistent LVEF<50%. Overall, 34 HTx recipients developed biopsy-negative GD and underwent CMR. Left ventricular late gadolinium enhancement (LGE) on CMR was observed in 16 patients with two distinct patterns: diffuse epicardial (n=13) and patchy (n=3) patterns. Patients with LGE developed GD later after HTx [4 (1.4-6.8) vs. 0.8 (0.3-1.2) years, P<0.001], were more often symptomatic (88% vs. 56%, P=0.06), and had greater haemodynamic derangement (pulmonary capillary wedge pressure: 19±7 vs. 13±3mmHg, P=0.002) as compared with those without LGE. No significant difference was observed in the primary composite outcome between patients with LGE and those without LGE (50% vs. 38% of patients with events, P=0.515). During a median follow-up of 3.8years, mean LVEF improved similarly in the LGE-negative (37-55%) and LGE-positive groups (32-55%) (P=0.16). Biopsy-negative GD occurs with and without LGE when assessed by CMR, indicative of possible rejection/inflammation occurring only in a subset of patients. Irrespective of LGE, LVEF improvement occurs in most GD patients, suggesting that other neurohormonal or immunomodulatory mechanisms may also contribute to GD development.

Prognostic Value of Cardiac Magnetic Resonance Feature Tracking Strain in Aortic Stenosis.

Recent data have suggested that global longitudinal strain (GLS) could be useful for risk stratification of patients with severe aortic stenosis (AS). In this study, we aimed to investigate the prognostic role of GLS in patients with AS and also its incremental value in relation to left ventricular ejection fraction (LVEF) and late gadolinium enhancement (LGE). We analysed all consecutive patients with AS and LGE-CMR in our institution. Survival data were obtained from office of national statistics, a national body where all deaths in England are registered by law. Death certificates were obtained from the general register office. Some 194 consecutive patients with aortic stenosis were investigated with CMR at baseline and followed up for 7.3 ± 4 years. On multivariate Cox regression analysis, only increasing age remained significant for both all-cause and cardiac mortality, while LGE (any pattern) retained significance for all-cause mortality and had a trend to significance for cardiac mortality. Kaplan-Meier survival analysis demonstrated that patients in the best and middle GLS tertiles had significantly better mortality compared to patients in the worst GLS tertiles. Importantly though, sequential Cox proportional-hazard analysis demonstrated that GLS did not have significant incremental prognostic value for all-cause mortality or cardiac mortality in addition to LVEF and LGE. Our study has demonstrated that age and LGE but not GLS are significant poor prognostic indicators in patients with moderate and severe AS.

Cardiac magnetic resonance-based layer-specific strain in immune checkpoint inhibitor-associated myocarditis.

To assess the different imaging characteristics between corticosteroid-sensitive (CS) and corticosteroid-refractory (CR) immune checkpoint inhibitor-associated myocarditis (ICIaM) with cardiac magnetic resonance (CMR) and the potential CMR parameters in the early detection of CR ICIaM. Thirty-five patients diagnosed with ICIaM and 30 age and gender-matched cancer patients without a history of ICI treatment were enrolled. CMR with contrast was performed within 2days of clinical suspicion. Left ventricular ejection fraction (LVEF) and late gadolinium enhancement (LGE) were assessed by CMR. LV sub-endocardial (GLSendo) and sub-epicardial (GLSepi) global longitudinal strains were quantified by offline feature tracking analysis. CS and CR ICIaM were defined based on the trend of Troponin I and clinical course during corticosteroid treatment. All 35 patients presented with non-fulminant symptoms upon initial assessment. Twenty patients (57.14%) were sensitive, and 15 (42.86%) were refractory to corticosteroids. Compared with controls, 22 patients (62.86%) with ICIaM developed LGE. LVEF decreased in CR ICIaM compared with the CS group and controls. GLSendo (-14.61±2.67 vs. -18.50±2.53, P<0.001) and GLSepi (-14.75±2.53 vs. -16.68±2.05, P<0.001) significantly increased in patients with CR ICIaM compared with the CS ICIaM. In patients with CS ICIaM, although GLSepi (-16.68±2.05 vs. -19.31±1.80, P<0.001) was impaired compared with the controls, GLSendo was preserved. There was no difference in CMR parameters between LGE-positive and negative groups. LVEF, GLSendo, and GLSepi were predictors of CR ICIaM. When LVEF, GLSendo, and GLSepi were included in multivariate analysis, only GLSendo remained an independent predictor of CR ICIaM (OR: 2.170, 95% CI: 1.189-3.962, P=0.012). A GLSendo of ≥-17.10% (sensitivity, 86.7%; specificity, 80.0%; AUC, 0.860; P<0.001) could predict CR ICIaM in the ICIaM cohort. Kaplan-Meier analysis showed that in patients with impaired GLSendo of ≥-17.10%, cardiovascular adverse events (CAEs) occurred much earlier than in patients with preserved GLSendo of <-17.10% (Log-rank test P=0.017). CR and CS ICIaM demonstrated different functional and morphological characteristics in different myocardial layers. An impaired GLSendo could be a helpful parameter in early identifying corticosteroid-refractory individuals in the ICIaM population.

The predictive value of coronary microvascular dysfunction for left ventricular reverse remodelling in dilated cardiomyopathy.

To evaluate the degree of coronary microvascular dysfunction (CMD) in dilated cardiomyopathy (DCM) patients by cardiac magnetic resonance (CMR) first-pass perfusion parameters and to examine the correlation between myocardial perfusion and left ventricle reverse remodelling (LVRR). In this study, 94 DCM patients and 35 healthy controls matched for age and sex were included. Myocardial perfusion parameters, including upslope, time to maximum signal intensity (Timemax), maximum signal intensity (SImax), baseline signal intensity (SIbaseline), and the difference between maximum and baseline signal intensity (SImax-baseline) were measured. Additionally, left ventricular (LV) structure, function parameters, and late gadolinium enhancement (LGE) were also recorded. The parameters were compared between healthy controls and DCM patients. Univariable and multivariable logistic regression analyses were used to determine the predictors of LVRR. With a median follow-up period of 12 months [interquartile range (IQR), 8-13], 41 DCM patients (44%) achieved LVRR. Compared with healthy controls, DCM patients presented CMD with reduced upslope, SIbaseline, and increased Timemax (all p < 0.01). Timemax, SImax, and SImax-baseline were further decreased in LVRR than non-LVRR group (Timemax: 60.35 [IQR, 51.46-74.71] vs. 72.41 [IQR, 59.68-97.70], p = 0.017; SImax: 723.52 [IQR, 209.76-909.27] vs. 810.92 [IQR, 581.30-996.89], p = 0.049; SImax-baseline: 462.99 [IQR, 152.25-580.43] vs. 551.13 [IQR, 402.57-675.36], p = 0.038). In the analysis of multivariate logistic regression, Timemax [odds ratio (OR) 0.98; 95% confidence interval (CI) 0.95-1.00; p = 0.032)], heart rate (OR 1.04; 95% CI 1.01-1.08; p = 0.029), LV remodelling index (OR 1.73; 95% CI 1.06-3.00; p = 0.038) and LGE extent (OR 0.85; 95% CI 0.73-0.96; p = 0.021) were independent predictors of LVRR. CMD could be found in DCM patients and was more impaired in patients with non-LVRR than LVRR patients. Timemax at baseline was an independent predictor of LVRR in DCM.

Cardiac MRI and Clinical Outcomes in TMEM43 Arrhythmogenic Cardiomyopathy.

Arrhythmogenic cardiomyopathy is an inherited cardiomyopathy that can involve both ventricles. Several genes have been identified as pathogenic in arrhythmogenic cardiomyopathy, including TMEM43. However, there are limited data on cardiac MRI findings in patients with TMEM43 variants to date. In this case series, cardiac MRI findings and clinical outcomes are described in 14 patients with TMEM43 variants, including eight (57%) with the pathogenic p.Ser358Leu variant (six female patients; mean age, 33 years ± 15 [SD]) and six (43%) with a TMEM43 variant of unknown significance (three female patients; mean age, 38 years ± 11). MRI findings demonstrated left ventricular systolic dysfunction in eight (57%) patients and right ventricular dysfunction in four (29%) patients. Among the nine patients with late gadolinium enhancement imaging, left ventricular late gadolinium enhancement was present in seven (78%; all subepicardial) patients. In summary, TMEM43 variants are associated with high prevalence of subepicardial late gadolinium enhancement and left ventricular dysfunction. Keywords: Arrhythmogenic Cardiomyopathy, Arrhythmogenic Right Ventricular Cardiomyopathy, TMEM43, Cardiac MRI, Genetic Variants Supplemental material is available for this article.

Left ventricular fibrosis influences the time to recovery in patients with arrhythmia-induced cardiomyopathy

Abstract Background The hallmark of arrhythmia-induced cardiomyopathy (AIC) is reversibility of left ventricular systolic dysfunction (LVSD) following rhythm restoration. Atrial fibrillation and LVSD very frequently co-exist and hence AIC is likely to be under recognized in daily practice. We tested the hypothesis, that left ventricular fibrosis has an influence on the time to recovery (TTR) in patients with AIC. Method We prospectively screened patients hospitalized for newly diagnosed and otherwise unexplainable LVSD (i.e. left ventricular ejection fraction (LVEF) &amp;lt;50%) and coexisting tachyarrhythmia (atrial fibrillation/flutter + heart rate (HR) &amp;gt;100/min). Causes other than tachyarrhythmic for LVSD had to be excluded (e.g. valvular, ischaemic, inflammatory). Left ventricular function was assessed by echocardiography at presentation and at follow-up visits 2, 4, and 6 months after rhythm control. Left ventricular (LV) late gadolinium enhancement (LGE) was measured via magnetic resonance imaging after rhythm restoration. Patients were classified AIC, if LVEF significantly recovered after 6 months (increase by ≥15% or above 50% with an increase of ≥10%) and then correlated with LGE as an indicator for fibrosis. Results 39 patients were included in the LGE analysis, 31 of them recovered and were classified AIC. Although we found no difference in LGE content in AIC vs. non-AIC patients (mean ± SEM 7.7 ± 0.9% vs. 6.5 ± 1.4%; p = 0.542) and contingency analysis of AIC/non-AIC vs. LGE-positive/LGE-negative patients revealed no difference between the groups (p = 0.682), there was a negative correlation of ΔLVEF (month 2 minus baseline) with LGE (Fig. 1) and a positive correlation of the TTR with LGE (Fig. 2) in LGE-positive patients, suggesting an influence of LV fibrosis on the speed of recovery. Conclusion Reliable criteria for recovery of LVEF in patients with newly diagnosed LVSD and coexisting arrhythmia are lacking. We found, that LV LGE correlated with the time to recovery of LVEF in patients with AIC. These results may help to estimate the recovery period and thereby optimize the diagnostic and therapeutic strategy for patients with AIC.Figures 1 and 2.

Cardiac magnetic resonance for prophylactic implantable-cardioVerter defibrillator therapy international registry in patients with ischemic cardiomyopathy

Abstract Background Sudden cardiac death (SCD) in patients with ischemic cardiomyopathy (ICM) remains a leading cause of death. Current guidelines provide a class I recommendation for implantable cardioverter–defibrillator (ICD) implantation in ICM patients with transthoracic echocardiographic (TTE) left ventricular ejection fraction (LVEF) ≤35%. and NYHA functional class II or III. The current paradigm is suboptimal in identifying patients who are likely to benefit. In fact, only a relatively small proportion of ICD recipients for SCD primary prevention receive appropriate device therapy and instead there is a not negligible percentage of patients who experienced SCD event despite not fulfilling criteria for implantation. Purpose The DERIVATE-ICM registry sought to evaluate the additional prognostic value for the indication of ICD implantation using cardiac magnetic resonance (CMR) as compared to standard of care based on TTE in a large cohort of ICM patients. Methods We enrolled 861 ICM patients (86% male, mean age 65±11 years) with chronic heart failure and TTE-LVEF&amp;lt;50%. Major adverse arrhythmic cardiac events (MAACE), defined as the combination of SCD, aborted SCD event, and sustained ventricular tachycardia. were the primary endpoint. Independent predictors were used to calculate a CMR risk score for each patient. Risk levels were defined as low (quantile 1 [Q1]), medium (Q2 and Q3) and high (Q4). Results During a median follow-up of 1054 days, MAACE occurred in 88 (10.2%). Left ventricular end-diastolic volume index (HR:1.007, 95%CI:1.000-1.011, p=0.05), CMR-LVEF (HR:0.972, 95%CI: 0.945-0.999, p=0.045) and late gadolinium enhancement (LGE) mass (HR:1.010, 95%CI: 1.002–1.018, p=0.015) were independent predictors of MAACE. Based on the multivariate analysis, CMR weighted risk score was developed according to the following equation [0.005 * EDV/BSA (mL/m2) – 0.029 * LVEF (%) + 0.010 * LGE ischemic mass (g)]. A multiparametric CMR weighted predictive derived score effectively identifies subjects at high risk for MAACE as compared to TTE-LVEF cut-off of 35% with a Continuous NRI: 31.7% (95%CI: 8.6%-54.8%, p=0.007) (Figure 1, Figure 2). Conclusions In this large multicenter registry, a multi-parametric CMR predictive model provides additional prognostic stratification for MAACE predictions in a large cohort of ICM patients as compared to standard of care. This model identifies a large number of patients with TTE-LVEF&amp;lt;35% at low risk of SCD event and on the other side it identifies a subset of patients who are at high risk of MAACE despite TTE-LVEF≥35% (i.e. the population currently not fulfilling the guideline-based criteria for ICD implantation). Further randomized trials to test a CMR guided strategy for ICD implantation versus standard of care are now needed.Kaplan-Meier curvesEvent rate redistribution

Shared and distinguishing phenotypic characteristics of filamin c and desmoplakin cardiomyopathy

Abstract Background Rare truncating variants in the genes Filamin C (FLNC) and Desmoplakin (DSP) are increasingly recognised causes of Dilated Cardiomyopathy and Arrhythmogenic Cardiomyopathy (1,2). A direct comparison of both genotypes is needed to aid in subclassification and genotype-specific risk modelling. Purpose We sought to identify shared and distinguishing clinical characteristics and imaging phenotypes between FLNC and DSP cardiomyopathy. Methods 41 patients with ‘pathogenic’ or ‘likely pathogenic’ FLNC or DSP variants by American College of Medical Genetics (ACMG) criteria were identified from a tertiary centre cardiovascular genetics laboratory. Clinical, genetic and imaging data were compiled. Continuous variables are presented as median [IQR] or mean±SD and analysed by nonparametric Mann Whitney U or t-test. Categorical variables were analysed by Chi-Squared or Fisher’s exact test. Results FLNC and DSP cohorts included a similar proportion of patients with truncating variants (91% vs 100%) and female sex (12/21, 57% vs 13/20, 65%). FLNC patients were younger (median age 30 [13-51] vs 57 [41-65] years, p&amp;lt;0.0008) and more likely to present with sustained ventricular arrhythmia (5/21 24% vs 3/20, 15%) (Table 1). Acute myocarditis episodes were exclusively linked to DSP cardiomyopathy (4/20, 20%) with no association to FLNC (0/21, 0%, p&amp;lt;0.05). FLNC cardiomyopathy patients had normal left ventricular (LV) systolic function (LVEF 57±9% SD vs 46±13% DSP, p&amp;lt;0.03) and normal left ventricular cavity side (mean indexed LV end diastolic volume (EDV) 85±12 vs 112±26ml/m2 DSP, p&amp;lt;0.02) (Table 2). Both FLNC and DSP cause exclusive left ventricular or biventricular late gadolinium enhancement (75% Vs 82%) on cardiac magnetic resonance imaging (MRI). Fibrosis was predominantly in a subepicardial distribution in both cohorts (66% FLNC vs 57% DSP). In FLNC, circumferential fibrosis was the most frequent pattern of distribution (5/9, 55%), whereas in DSP inferior and lateral wall fibrosis was the predominant feature (8/14, 57%). There were no cases of isolated RV involvement and no cases fulfilled ‘definite’ ARVC taskforce diagnostic criteria. Over a follow up of median 45months, there was no significant difference in the proportion of FLNC and DSP patients experiencing a composite end point of LVAD implantation, heart transplant, aborted sudden cardiac death or mortality (7/21, 33% FLNC Vs 7/20, 29% DSP p&amp;gt;0.99). Conclusion Normal systolic function with marked circumferential fibrosis is the predominant feature of FLNC cardiomyopathy whereas mild LV dilatation, mild systolic impairment and inferolateral fibrosis are more frequent in DSP cardiomyopathy. Myocarditis episodes are a distinguishing feature of DSP cardiomyopathy, suggesting a different underlying molecular mechanism driven by inflammation. The overall clinical trajectory of FLNC and DSP cardiomyopathy patients are comparable.

Abstract 18920: Left Atrial Function is Associated With Left Ventricular Mass in Women With Preserved Left Ventricular Ejection Fraction and Late Gadolinium Enhancement on Cardiac MRI

Introduction: Myocardial fibrosis detected by late gadolinium enhancement (LGE) on cardiac MRI may be related to conditions with increased left ventricular (LV) mass and diastolic dysfunction. Women have an increased incidence of LV diastolic dysfunction and poorer clinical outcomes compared to men. Aim: To examine gender-based associations between LV mass and left atrial total emptying fraction (LATEF), a surrogate for diastolic function, in patients with LGE on cardiac MRI. Methods: We analyzed 134 consecutive patients undergoing cardiac MRI and LGE sequences on a 1.5T GE scanner. LV mass and LV/LA volumes (indexed by BSA) were measured offline from cine SSFP images using Precession software. Presence of LGE was reported. Nonparametric analyses were performed. Results: 40 patients with more than mild mitral regurgitation, pericardial disease, or LVEF &lt;50% were excluded. 94 patients studied (49% women) had a median age of 51 years [IQR 38-67] with no gender-based difference. Median LV mass index (LVMi) for all patients was 69.5 g/m2 [IQR 57-82] and was higher in LGE vs no-LGE patients (p&lt;0.001) and higher in men vs women (p&lt;0.05). Median LATEF for all patients was 0.50 [IQR 0.41-0.54] and was lower in LGE vs no-LGE patients (p&lt;0.001) with no gender-based difference. LVMi had a strong inverse correlation with LATEF for all patients and for the LGE group (r=-0.58, p&lt;0.001 and r=-0.51, p&lt;0.001 respectively). LVMi had a very strong inverse correlation with LATEF (r=-0.74, p&lt;&lt;0.001) in women with LGE and moderate inverse correlation in women with no-LGE (r=-0.45, p=0.02). LVMi had a moderate inverse correlation with LATEF in men with LGE (r=-0.48, p. &lt;0.01) and no correlation in men with no-LGE (Figure). Conclusions: LA function inversely correlates with indexed LV mass, most strongly in women with LV LGE. Further exploration of these parameters may allow earlier detection of LV diastolic dysfunction and improved risk stratification.

Abstract 15164: The Different Prognostic Values of T1 Mapping in Patients With Hypertrophic Cardiomyopathy

Objectives: This study aimed to determine the impact of T1 mapping on cardiovascular magnetic resonance (CMR) on long-term adverse outcomes in patients with hypertrophic cardiomyopathy (HCM). Background: In patients with HCM, the prognostic value of native T1 and extracellular volume fraction (ECV) for sudden cardiac death (SCD) has not been well defined. Methods: Consecutive 663 participants with HCM who underwent CMR were recruited. The follow-up endpoints included heart failure (HF)-related death and SCD or aborted SCD. Results: During the median follow-up time of 44 months (interquartile range [IQR]: 23 to 65.5 months), 55 (8.3%) participants reached the endpoint including 37 (5.6%) with SCD or aborted SCD and 18 (2.7%) with HF-related death. On Cox proportional hazards regression multivariable analyses, native T1 but not ECV was independently associated with CD (hazard ratio [HR]: 1.00; 95% confidence interval[CI] : 1.00 to 1.01; P = 0.02), while native T1 (HR: 1.01; 95%CI: 1.00 to 1.01; P = 0.008) and ECV (HR:1.15 for every 1% increase; 95%CI: 1.05 to 1.26; P = 0.003) were both independently associated with HF-related death after the adjustment for left ventricular ejection fraction (LVEF) and LV late gadolinium enhancement (LGE) extent. Conversely, native T1(HR: 1.00; 95% CI: 1.00 to 1.01; P = 0.40) and ECV (HR: 1.01; 95% CI: 0.94 to 1.08; P = 0.81) were not associated with SCD after the adjustment for non-sustained ventricular tachycardia, history of syncope and LV LGE extent. Conclusions: In this study of patients with HCM, T1 mapping had distinct prognostic associations; native T1 and ECV were both independently associated with HF-related death, whereas T1 mapping was not independently associated with SCD or aborted SCD. These findings may indicate distinct implications for the management of left ventricular fibrosis in HCM.

Abstract 15484: Heart Transplantation in a Light-Chain Amyloidosis Patient at Mayo Stage IIIa After Anti-Plasma Cell Chemotherapy With Complete Remission

A 42-year-old female patient was admitted for lower limb edema. Laboratory tests showed nephrotic proteinuria (2.76g/24h). Serum-free λ light chains were 84.9 mg/L, with a κ/λ ratio of 0.12. Bone marrow examination detected 6% immature clonal plasma cells. Amyloid deposition was identified in a renal biopsy. Cardiac magnetic resonance (CMR) revealed normal left ventricular ejection fraction (LVEF), left ventricular circumferential late gadolinium enhancement (LGE), and elevated myocardial T1 value (1459ms) and extracellular volume (47.4%). She was diagnosed with AL amyloidosis and classed as IIIa by the Mayo Stage 2004. A hematologic complete response (CR) was achieved and sustained after treatment with sequencing cyclophosphamide, bortezomib, dexamethasone (CyBorD) and daratumumab regimen. The follow-up CMRs were underwent at 6, 12, and 18 months after starting chemotherapy, which monitored cardiac changes in reponse to CR. Unfortunately, there was no alleviation of cardiac involvement. CMR re-examination (at 42 months) revealed severe cardiac function impairment (LVEF 28%), and dramatically increased extracellular volume (66.2%). Then, she underwent heart transplantation (HTx) evaluated by the heart transplantation multidisciplinary team. Three months later, she was doing well, CMR demonstrated stable cardiac allograft function. Repeated endomyocardial biopsies showed no amyloid deposits. This case illustrates that cardiac amyloid deposition might be a dynamic process in AL amyloidosis and highlights the value of CMR in long-term monitoring and identifying the patients reached CR who need HTx.

Abstract 16341: A Rare Case of Dual Cardiomyopathy: Acute Decompensated Heart Failure Secondary to Eosinophilic Myocarditis and Ischemic Cardiomyopathy

Introduction: Dual cardiomyopathy, characterized by the coexistence of two distinct forms of myocardial dysfunction, is a rare phenomenon with complex diagnostic and therapeutic challenges. We present a unique case of a patient with combined eosinophilic myocarditis and ischemic cardiomyopathy. Case Presentation: A 65-year-old female with history of peripheral eosinophilia presented with worsening dyspnea and abdominal pain. CT abdomen was notable for right renal artery thrombosis. Troponin I was elevated at 0.057 ng/ml and 0.056 ng/ml. Notably, her absolute eosinophil count was elevated to 20.34 K/mcL. EKG with LVH and repolarization abnormalities. TTE showed a severely reduced LVEF of 18% and severe global hypokinesis. Decision Making: Given her newly diagnosed cardiomyopathy, a cardiac MRI was obtained that demonstrated presence of two moderate-sized cylindrical thrombi in the LV and subendocardial late gadolinium enhancement in the basal to mid antero/inferoseptum and inferolateral wall, characteristic of both myocardial infarction and eosinophilic myocarditis (Figure 1). She underwent a coronary angiogram that revealed severe mid LAD and OM1 artery stenosis that required PCI. She was initiated on appropriate medical therapy including steroids with normalization of her peripheral eosinophilia and improvement in her clinical symptoms. Discussion: This case highlights the intricate interplay between eosinophilic myocarditis and underlying ischemic cardiomyopathy. The timely initiation of appropriate therapies, including corticosteroids for eosinophilic myocarditis and revascularization for ischemic cardiomyopathy, can lead to favorable outcomes in these complex cases. Figure 1: Cardiac MRI showing inferolateral LGE and Cylindrical LV thrombi.

Aortic stiffness effectively risk stratifies diabetic patients with suspected myocardial ischemia undergoing vasodilatory stress perfusion cardiac magnetic resonance

Background and aimsCardiovascular magnetic resonance (CMR) comprehensively assesses aortic stiffness and myocardial ischemia in a single examination. Aortic stiffness represents a subclinical marker of cardiovascular risk in the general population, including patients with diabetes mellitus. However, there is no prognostic data regarding aortic stiffness in patients with diabetes mellitus undergoing stress perfusion CMR.MethodsConsecutive patients with diabetes mellitus with suspected myocardial ischemia referred for adenosine stress perfusion CMR with aortic pulse wave velocity (PWV) during 2010–2013 were studied. The primary outcome was major adverse cardiovascular events (MACE), defined as the composite of cardiac mortality, nonfatal myocardial infarction (MI), hospitalization for heart failure, coronary revascularization (> 90 days post-CMR), and ischemic stroke. The secondary outcome was hard cardiac events, defined as the composite of cardiac mortality and nonfatal MI.ResultsA total of 424 patients (median follow-up 7.2 years) were included. The mean PWV was 12.16 ± 6.28 m/s. MACE and hard cardiac events occurred in 26.8% and 9.4% of patients, respectively. Patients with elevated PWV (> 12.16 m/s) had a significantly higher incidence of MACE (HR 2.14 [95%CI 1.48, 3.09], p < 0.001) and hard cardiac events (HR 2.69 [95%CI 1.42, 5.10], p = 0.002) compared to those with non-elevated PWV. Multivariable analysis demonstrated that PWV independently predicts MACE (p = 0.003) and hard cardiac events (p = 0.01). Addition of PWV provided incremental prognostic value beyond clinical data, left ventricular mass index, myocardial ischemia, and late gadolinium enhancement in predicting MACE (incremental χ² 7.54, p = 0.006) and hard cardiac events (incremental χ² 5.99, p = 0.01).ConclusionsAortic stiffness measured by CMR independently predicts MACE and hard cardiac events and confers significant incremental prognostic value in patients with diabetes mellitus with suspected myocardial ischemia. Aortic stiffness measurement could potentially be considered as part of a stress perfusion CMR protocol to enhance risk prediction in patients with diabetes mellitus.

Entropy as a novel predictor of cardiovascular events in patients with left ventricular noncompaction

BackgroundThe risk stratification of left ventricular noncompaction (LVNC) remains ambiguous. LV entropy derived from late gadolinium enhancement (LGE) in cardiac magnetic resonance (CMR) as a novel measurement of myocardial heterogeneity may serve as the substrate of major adverse cardiovascular events (MACEs). This retrospective study aimed to investigate the value of LV entropy for predicting MACEs in LVNC patients. MethodsConsecutive patients who underwent CMR and met the diagnosis criteria of LVNC were included. All patients were follow-up for MACEs (cardiac death, ventricular arrhythmia requiring therapy or heart failure hospitalization), and their LV entropy derived from the distribution of pixel signal intensities in the LGE of the LV myocardium was analyzed. ResultsOne hundred and forty-three patients (mean age 40 years, 64.3% male) were followed for a median of 3.2 years, and forty-two (29.4%) experienced MACEs. Presenting of symptoms, left ventricular end-diastolic diameter (LVEDD), LV end-diastolic volume (LVEDV) index, LV end-systolic volume (LVESV) index, LV ejection fraction (LVEF), LGE extent, and LV entropy showed association with MACEs. LV entropy maintained independent association with MACEs (HR: 4.76, 95%CI 3.68–5.15, p < 0.001) in multivariable analysis. Entropy was also strong independent predictor of MACEs in patients with and without LGE (HR: 5.89, 95% CI4.18–7.73, p < 0.001; HR: 3.06, 95% CI:1.53–4.80, p = 0.013, respectively). ConclusionsLV entropy can predict MACEs in LVNC patients and provide incremental prognostic value on top of LVEF and LGE. Also, LV entropy may help risk stratification in LGE-negative LVNC patients.

Phenotyping of giant cell myocarditis versus cardiac sarcoidosis using cardiovascular magnetic resonance

BackgroundGiant cell myocarditis (GCM) and cardiac sarcoidosis (CS) are rare inflammatory diseases of the myocardium with poor prognosis. Little is known about the cardiovascular magnetic resonance (CMR) appearance of GCM and the methods ability to distinguish the two rare entities from one another. MethodsWe assessed a total of 40 patients with endomyocardial biopsy-proven GCM (n = 14) and CS (n = 26) concerning their clinical and CMR appearance in a blinded manner. ResultsPatients with GCM and CS were of similar median age (55 vs 56 years), and a male predominance was observed in both groups. In GCM, median levels of troponin T (313 vs 31 ng/L, p < 0.001), and natriuretic peptides (6560 vs 676 pg/mL, p < 0.001) were higher than in CS, and the clinical outcome worse (p = 0.04). On CMR imaging, the observed alterations of left and right ventricular (LV/RV) dimensions and function were similar. GCM showed multifocal LV late gadolinium enhancement (LGE) with a similar longitudinal, circumferential, and radial distribution as in CS, including suggested signature imaging biomarkers of CS like the “hook sign” (71% vs 77%, p = 0.702). The median LV LGE enhanced volume was 17% and 22% in GCM and CS (p = 0.150), respectively. The number of RV segments with pathologically increased T2 signal and/or LGE were most extensive in GCM. ConclusionsThe CMR appearance of both GCM and CS is highly similar, making the differentiation between the two rare entities solely based on CMR challenging. This stands in contrast to the clinical appearance, which seems to be more severe in GCM.

Cardiac magnetic resonance for prophylactic implantable-cardioverter defibrillator therapy international study in ischemic cardiomyopathy: international DERIVATE-ICM Registry

Abstract Funding Acknowledgements Type of funding sources: None. Background Implantable cardioverter–defibrillator (ICD) therapy is the most effective sudden cardiac death (SCD) event prophylactic-strategy in ischemic cardiomyopathy (ICM) patients with transthoracic echocardiographic (TTE) left ventricular ejection fraction (LVEF) ≤35%. This approach has been recently questioned due to the low rate of ICD interventions in patients who received implantation and the not negligible percentage of patients who experienced SCD event despite not fulfilling criteria for implantation. Purpose The DERIVATE-ICM registry (RCT#NCT03352648) is an international, multicenter and multivendor study to assess the net reclassification improvement (NRI) for the indication of ICD implantation by the use of cardiac magnetic resonance (CMR) as compared to standard of care based on TTE in ICM patients. Methods We enrolled 861 ICM patients (mean age 65±11 years, 86% male) with chronic heart failure and TTE-LVEF&amp;lt;50%. Major adverse arrhythmic cardiac events (MAACE), defined as the combination of SCD, aborted SCD event, and sustained ventricular tachycardia were the primary endpoint. Independent predictors were used to calculate a CMR risk score for each patient. Risk levels were defined as low (quantile 1 [Q1]), medium (Q2 and Q3) and high (Q4). Results During a median follow-up of 1054 days, MAACE occurred in 88 (10.2%). Left ventricular end-diastolic volume index (HR: 1.007, 95%CI:1.000–1.011, p = 0.050), CMR-LVEF (HR: 0.972, 95%CI: 0.945–0.999, p = 0.045) and late gadolinium enhancement (LGE) mass (HR: 1.010, 95%CI: 1.002–1.018, p = 0.015) were independent predictors of MAACE. Based on the multivariable analysis, CMR weighted risk score was developed according to the following equation [0.005 * EDV/BSA (mL/m²) – 0.029 * LVEF (%) + 0.010 * LGE ischemic mass (g)]. Figure 1 (top) shows the Kaplan-Meier curves according to the guidelines-based TTE-LVEF model and the CMR risk score. Moreover, the table of reclassification of the CMR versus TTE predictive model is reported in Figure 1 (bottom). This multiparametric CMR weighted predictive derived score effectively identifies subjects at high risk for MAACE as compared to TTE-LVEF cut-off of 35% with a NRI of 31.7% (p = 0.007). The distribution of CMR predictive score in patients with TTE-LVEF≤35% (panel A) and with TTE LVEF&amp;gt;35% (panel C) is represented in Figure 2, together with the event rate redistribution according to the CMR risk score (panel B and panel D). Conclusions The DERIVATE-ICM study is the first large multicenter registry testing the additional value of CMR to stratify the risk for MAACE in a large cohort of ICM patients as compared to standard of care. This model identifies a large number of patients with TTE-LVEF&amp;lt;35% at low risk of SCD event and on the other side it identifies a subset of patients who are at high risk of MAACE despite TTE-LVEF≥35%. Further randomized trials to test a CMR guided strategy for ICD implantation versus standard of care are now needed.

A distinct septal pattern of late gadolinium enhancement specific for COVID-induced myocarditis: A multicenter cardiovascular magnetic resonance study.

Coronavirus disease-19 (COVID-19) is a great medical challenge provoking acute respiratory distress, pulmonary manifestations, and cardiovascular (CV) consequences. This study compares cardiac injury in COVID-19 myocarditis patients with non-COVID myocarditis patients. Patients who recovered from COVID-19 were scheduled for cardiovascular magnetic resonance (CMR) owing to clinical myocarditis suspicion. The retrospective non-COVID-19 myocarditis (2018-2019) group was enrolled (n=221 patients). All patients underwent a contrast-enhanced CMR, conventional myocarditis protocol, and late gadolinium enhancement (LGE). The COVID study group included 552 patients with a mean (standard deviation [SD]) age 45.9 (12.6) years old. CMR assessment confirmed a myocarditis-like LGE in 46% of the cases (68.5% of the segments with LGE <25% transmural extent), left ventricular (LV) dilatation in 10%, and systolic dysfunction in 16%. The COVID-myocarditis group showed a smaller median (interquartile range [IQR]) LV LGE (4.4% [2.9%-8.1%] vs. 5.9% [4.4%-11.8%]; P <0.001), lower LVEDV (144.6 [125.5-178] ml vs. 162.8 [136.6-194] ml; P <0.001), limited functional consequence (LVEF, 59% [54.1%-65%] vs. 58% [52%-63%]; P = 0.01), and a higher rate of pericarditis (13.6% vs. 6%; P = 0.03) compared to non-COVID myocarditis. The COVID-induced injury was more frequent in septal segments (2, 3, 14), and non-COVID myocarditis showed higher affinity to lateral wall segments (P <0.01). Neither obesity nor age was associated with LV injury or remodeling in subjects with COVID-myocarditis. COVID-19-induced myocarditis is associated with minor LV injury with a significantly more frequent septal pattern and a higher pericarditis rate than non-COVID-19 myocarditis.