Percentage Of Late Gadolinium Enhancement Research Articles

Prognostic Utility of Left Atrial Strain From MRI Feature Tracking in Ischemic and Nonischemic Dilated Cardiomyopathy: A Multicenter Study.

BACKGROUND. MRI-based prognostic evaluation in patients with dilated cardiomyopathy (DCM) has historically used markers of late gadolinium enhancement (LGE) and feature tracking (FT)-derived left ventricular global longitudinal strain (LVGLS). Early data indicate that FT-derived left atrial strain (LAS) parameters, including reservoir, conduit, and booster, may also have prognostic roles in such patients. OBJECTIVE. The purpose of our study was to evaluate the prognostic utility of LAS parameters, derived from MRI FT, in patients with ischemic or nonischemic DCM, including in comparison with the traditional parameters of LGE and LVGLS. METHODS. This retrospective study included 811 patients with ischemic or nonischemic DCM (median age, 60 years; 640 men, 171 women) who underwent cardiac MRI at any of five centers. FT-derived LAS parameters and LVGLS were measured using two- and four-chamber cine images. LGE percentage was quantified. Patients were assessed for a composite outcome of all-cause mortality or heart failure hospitalization. Multivariable Cox regression analyses including demographic characteristics, cardiovascular risk factors, medications used, and a wide range of cardiac MRI parameters were performed. Kaplan-Meier analyses with log-rank tests were also performed. RESULTS. A total of 419 patients experienced the composite outcome. Patients who did, versus those who did not, experience the composite outcome had larger LVGLS (-6.7% vs -8.3%, respectively; p < .001) as well as a smaller LAS reservoir (13.3% vs 19.3%, p < .001), LAS conduit (4.7% vs 8.0%, p < .001), and LAS booster (8.1% vs 10.3%, p < .001) but no significant difference in LGE (10.1% vs 11.3%, p = .51). In multivariable Cox regression analyses, significant independent predictors of the composite outcome included LAS reservoir (HR = 0.96, p < .001) and LAS conduit (HR = 0.91, p < .001). LAS booster and LGE were not significant independent predictors in the models. LVGLS was a significant independent predictor only in a model that initially included LAS booster but not the other LAS parameters. In Kaplan-Meier analysis, all three LAS parameters were significantly associated with the composite outcome (p < .001). CONCLUSION. In this multicenter study, LAS reservoir and LAS conduit were significant independent prognostic markers in patients with ischemic or nonischemic DCM, showing greater prognostic utility than the currently applied markers of LVGLS and LGE. CLINICAL IMPACT. FT-derived LAS analysis provides incremental prognostic information in patients with DCM.

Fractal analysis in cardiovascular magnetic resonance: prognostic value of biventricular trabecular complexity in hypertrophic cardiomyopathy.

Trabecular complexity can be quantified by fractal analysis based on cine images of cardiovascular magnetic resonance (CMR), yielding fractal dimension (FD) index. We aimed to investigate the prognostic value of biventricular FD in patients with hypertrophic cardiomyopathy (HCM). This retrospective study included 284 (192 men, median age 53 years) patients with HCM who underwent CMR, with median follow-up of 24 months. Biventricular trabeculae complexity was quantified as FD using short-axis cine images. The primary end point included sudden cardiac death (SCD) events. The secondary end point included both SCD events and rehospitalization due to heart failure. Cox regressions were performed. Prediction models were established by adding ventricular FDs to ESC predictors and late gadolinium enhancement (LGE) percentage and the C indices were calculated. Cox regressions revealed that left ventricular (LV) maximal apical FD (HR range 1.114-1.133; all P<0.05) and right ventricular (RV) global FD (HR range 1.135-1.150; all P<0.05) were significant prognostic factors of both end points after adjustment for the European Society of Cardiology (ESC) predictors (age, maximum LV wall thickness, LV atrial size, peak left ventricular outflow tract (LVOT) gradient, family history of SCD, unexplained syncope, non-sustained ventricular tachycardia), and LGE percentage. The prediction model with the addition of biventricular FDs (C-index: 0.864-0.877) had the best performance. LV maximal apical FD and RV global FD were independent predictors of SCD events and rehospitalization due to heart failure in patients with HCM. The addition of biventricular FDs to the conventional prediction model contributed incremental prognosis value in HCM.

Ventricular arrhythmia outcomes in hypertrophic cardiomyopathy from a high level myectomy centre

Abstract Background The presence of ≥15% late gadolinium enhancement (LGE) on cardiac magnetic resonance imaging (MRI) is used as a prognostic indicator for sudden cardiac death in patients with hypertrophic cardiomyopathy (HCM). [1] According to the European Society of Cardiology clinical practice guidelines (2014), septal myectomy is only indicated for symptomatic relief from left ventricular outflow tract obstruction (LVOTO) that is refractory to medical therapy, provided LVOTO gradient ≥ 50 mmHg. [2] At present, there is scarce data correlating the burden of ventricular arrhythmia and sudden cardiac death with LGE percentage or showing benefit of myectomy in reducing the burden of the same. Purpose We aimed to compare the burden of ventricular arrhythmia burden in patients with HCM who underwent septal myectomy compared with those who did not. Methods Adult patients with HCM followed in the advanced heart failure outpatient clinic at Allegheny General Hospital were included in this study. All included patients had cardiac MRI with LGE quantification and data related to cardiac events through devices placed for various indications (such as evaluation of reported palpitations, established supraventricular tachycardia, implantable cardioverter-defibrillator for primary or secondary prevention). Patients were divided into 2 groups: those undergoing myectomy (post myectomy group) and those that did not undergo myectomy (non-myectomy group). Chi square test was used to calculate incidence of premature ventricular contractions (PVCs), non-sustained ventricular tachycardia (NSVT) and sustained ventricular tachycardia (SVT) in the non-myectomy and post myectomy group. Results 76 patients with HCM (interventricular septal thickness ≥1.5 cm and LGE positive) longitudinally followed in the Allegheny General Hospital advanced heart failure outpatient clinic from 2016 to 2022 were included. 37 patients underwent septal myectomy. Baseline characteristics of post myectomy and non-myectomy group were matched and are listed in table 1. Left ventricular mass index was expectedly higher in patients undergoing myectomy. LGE was quantified on cardiac MRI prior to myectomy (mean 16.4% in myectomy group, 14.6% in non-myectomy group). Burden of ventricular arrhythmia (PVCs, NSVT and SVT) was found to be significantly lower in the post myectomy group (n=13, 44.83%) compared to the non-myectomy group (n=36, 92.31%). None of the patients included were noted to have ventricular fibrillation or sudden cardiac death. Conclusion Septal myectomy may reduce the incidence of ventricular arrhythmia and thus impact outcomes related to sudden cardiac death. This is likely a consequence of removal of arrhythmogenic scar tissue. Larger studies are required to further evaluate the burden and correlation of ventricular arrhythmia with scar tissue volume measured on cardiac MRI.

The predictive value of cardiac MRI strain parameters in hypertrophic cardiomyopathy patients with preserved left ventricular ejection fraction and a low fibrosis burden: a retrospective cohort study.

Prompt interventions prevent adverse events (AE) in hypertrophic cardiomyopathy (HCM). We evaluated the pattern and the predictive role of feature tracking (FT)-cardiac magnetic resonance (CMR) imaging parameters in an HCM population with a normal left ventricular ejection fraction (LVEF) and a low fibrosis burden. The CMR and clinical data of 170 patients, consisting of 142 HCM (45 ± 15.7 years, 62.7% male) and 28 healthy (42.2 ± 11.26 years, 50% male) subjects, who were enrolled from 2015 to 2020, were evaluated. HCM patients had a normal LVEF with a late gadolinium enhancement (LGE) percentage below 15%. Between-group differences were described, and the potent predictors of AE were determined. A P-value below 0.05 was considered significant. LV global longitudinal, circumferential, and radial strains (GLS, GCS, and GRS, respectively) and the LV myocardial mass index (MMI) were different between the healthy and HCM cases (all Ps < 0.05). Strains were significantly impaired in the HCM patients with a normal MMI. A progressive decrease in LVGLS and a distinct fall in LVGCS were noted with a rise in MMI. AE were predicted by LVGLS, LVGCS, and the LGE percentage, and LVGCS was the single robust predictor (HR, 1.144; 95% CI, 1.080-1.212; P = 0.001). An LVGCS below 16.2% predicted AE with 77% specificity and 58% sensitivity. LV strains were impaired in HCM patients with a normal EF and a low fibrosis burden, even in the presence of a normal MMI. CMR parameters, especially FT-CMR values, predicted AE in our HCM patients.

Incidence and Predictors of Relapse After Weaning Immune Suppressive Therapy in Cardiac Sarcoidosis

Cardiac sarcoidosis (CS) is a relapsing-remitting disease, and immune suppression (IS) is the mainstay of therapy. Predictors of relapse for patients with CS in remission are not well characterized. We assessed incidence of relapse in consecutive patients with CS treated with high-dose steroids and/or steroid-sparing agents (SSA) in our center from 2000 to 2020. Remission was defined as reaching maintenance therapy (no IS, SSA, and/or prednisone ≤5mg/d) for ≥1month. Relapse was defined as recurrence of CS syndrome requiring IS intensification: heart failure, ventricular arrhythmia, decrease in left ventricular ejection fraction, or increased disease burden on imaging. Among 68 patients, the mean age was 50.7±9.0years; 25 (37%) were women, and 32 (47%) were Black. In total, 59 patients (87%) reached remission. Over a median follow-up of 39.5months (interquartile range 17.6, 92.5), 28 (48%) relapsed. Greater percentage of late gadolinium enhancement (LGE) on pretreatment magnetic resonance imaging corresponded with increased likelihood of relapse (odds ratio 1.396 per 5% increase [95% confidence interval (CI) 1.04 to 1.88]; p=0.028). LGE ≥11% predicted elevated risk of relapse (adjusted odds ratio 4.998 [1.34 to 18.64]; p=0.017). Shorter time to relapse was observed with isolated CS (adjusted hazard ratio 4.084 [1.44,11.56]; p=0.008) and LGE ≥11% (adjusted hazard ratio 3.007 [1.01, 8.98]; p=0.049). Approximately 1 in 2 patients with CS in remission experienced relapse. Greater burden of LGE on cardiac magnetic resonance imaging and isolated CS are associated with greater risk of relapse. Future work is needed to refine risk stratification for relapse and to optimize surveillance strategies on the basis of the burden of disease.

Correlations between high sensitive troponin I and acute myocarditis extent in cardiac magnetic resonance imaging.

To assess the correlation between high sensitive troponin I (HsTnI) levels and myocardial damage on cardiac magnetic resonance (CMR) represented by late gadolinium enhancement (LGE) percentage in patients diagnosed with myocarditis. Retrospective analysis of consecutive patients who underwent CMR following a suspected diagnosis of acute myocarditis, comparing CMR findings viewed as LGE percentage and HsTnI levels. Between February 2016 and December 2021, 101 patients underwent CMR for suspected myocarditis in Rambam Medical Center. Seventy-six (75.2%) patients with a documented diagnosis of acute myocarditis in the medical records based on clinical history and lab work were included in the final analysis. The median age was 30 years [interquartile range (IQR) 22,42] and 62 patients (81.6%) were male. Thirty-four patients (44.7%) had a history of fever and 26 (34.2%) had upper respiratory tract symptoms. The median maximal HsTnI was 3935 ng/l (1165,10 380) and the median C-reactive protein (CRP) was 7.97 mg/l (2.35,19.28). The median LGE percentage was 4.65% (2.6,8.5) and ventricular ejection fraction 60% (56.00,64.75).Linear association was found between LGE (%) and maximal HsTnI (ng/l) value with r = 0.49 ( P < 0.001). After including only patients in whom the CMR was performed within 5 days of the maximal HsTnI the correlation improved to r = 0.67 ( P < 0.001). HsTnI is an indicator for myocardial damage extent resulting from inflammation in patients with acute myocarditis.

DSP c.6310delA p.(Thr2104Glnfs*12) associates with arrhythmogenic cardiomyopathy, increased trabeculation, curly hair, and palmoplantar keratoderma.

Pathogenic variants in DSP associate with cardiac and cutaneous manifestations including arrhythmogenic right ventricular cardiomyopathy, dilated cardiomyopathy, curly or wavy hair, and palmoplantar keratoderma (PPK). Episodes of myocardial inflammation associated with DSP cardiomyopathy might be confused in clinical work with myocarditis of other etiologies such as viral. Cardiac magnetic resonance imaging (CMR) may help in differential diagnosis. This study comprised 49 Finnish patients: 34 participants from families with suspected DSP cardiomyopathy (9 index patients and 25 family members) and 15 patients with myocarditis. All 34 participants underwent genetic testing and cardiac evaluation, and 29 of them also underwent CMR. Participants with the DSP variant, numbering 22, were dermatologically examined. The 15 patients with myocarditis underwent CMR and were evaluated during their hospitalization.A heterozygous truncating DSP c.6310delA p.(Thr2104Glnfs*12) variant was confirmed in 29 participants. Only participants with the DSP variant had pacemakers and life-threatening ventricular arrhythmias. Of the participants with the DSP variant, 24% fulfilled cardiomyopathy criteria, and the median age at diagnosis was 53. Upon CMR, myocardial edema was found to be more common in patients with myocarditis. Both groups had a substantial percentage of late gadolinium enhancement (LGE). A ring-like LGE and increased trabeculation were observed only in participants with the DSP variant. All the studied participants with the DSP variant had PPK and curly or wavy hair. Hyperkeratosis developed before the age of 20 in most patients. The DSP c.6310delA p.(Thr2104Glnfs*12) variant associates with curly hair, PPK, and arrhythmogenic cardiomyopathy with increased trabeculation. Cutaneous symptoms developing in childhood and adolescence might help recognize these patients at an earlier stage. CMR, together with dermatologic characteristics, may help in diagnosis.

Interpretable machine learning for automated left ventricular scar quantification in hypertrophic cardiomyopathy patients.

Scar quantification on cardiovascular magnetic resonance (CMR) late gadolinium enhancement (LGE) images is important in risk stratifying patients with hypertrophic cardiomyopathy (HCM) due to the importance of scar burden in predicting clinical outcomes. We aimed to develop a machine learning (ML) model that contours left ventricular (LV) endo- and epicardial borders and quantifies CMR LGE images from HCM patients.We retrospectively studied 2557 unprocessed images from 307 HCM patients followed at the University Health Network (Canada) and Tufts Medical Center (USA). LGE images were manually segmented by two experts using two different software packages. Using 6SD LGE intensity cutoff as the gold standard, a 2-dimensional convolutional neural network (CNN) was trained on 80% and tested on the remaining 20% of the data. Model performance was evaluated using the Dice Similarity Coefficient (DSC), Bland-Altman, and Pearson's correlation. The 6SD model DSC scores were good to excellent at 0.91 ± 0.04, 0.83 ± 0.03, and 0.64 ± 0.09 for the LV endocardium, epicardium, and scar segmentation, respectively. The bias and limits of agreement for the percentage of LGE to LV mass were low (-0.53 ± 2.71%), and correlation high (r = 0.92). This fully automated interpretable ML algorithm allows rapid and accurate scar quantification from CMR LGE images. This program does not require manual image pre-processing, and was trained with multiple experts and software, increasing its generalizability.

Role of cardiac magnetic resonance in the differential diagnosis between arrhythmogenic cardiomyopathy with left ventricular involvement and previous infectious myocarditis.

AimsArrhythmogenic cardiomyopathy with left ventricular involvement (ACM-LV), particularly in case of isolated left ventricular involvement (i.e. left dominant arrhythmogenic cardiomyopathy, LDAC) and previous infectious myocarditis (pIM) may have overlapping clinical and cardiac magnetic resonance (CMR) features. To date, there are no validated CMR criteria for the differential diagnosis between these conditions. The present study aimed to identify CMR characteristics to distinguish ACM-LV from pIM. Methods and resultsThis observational, retrospective, single-centre study included 30 pIM patients and 30 ACM-LV patients. In ACM-LV patients CMR was performed at diagnosis; in patients with pIM, CMR was performed six months after acute infection. CMR analysis included quantitative assessment of left ventricle (LV) volumes, systolic function and wall thicknesses, qualitative and quantitative assessment of late gadolinium enhancement (LGE) sequences. Compared with pIM, ACM-LV patients showed slightly larger LV volumes, more frequent regional wall motion anomalies and reduced wall thicknesses. ACM-LV patients had higher amounts of LV LGE and extension. Notably, the LDAC subgroup had the highest amount of LV LGE. LV LGE amount > 15 g and a LV LGE percentage > 30% of LV mass discriminated ACM-LV from pIM with a 100% specificity. LGE segmental distribution was superimposable among the groups, except for septal segments that were more frequently involved in ACM-LV and LDAC patients. ConclusionsA great extension of LV LGE (a cut-off of LGE >15 g and a percentage above 30% of LV LGE in relation to total myocardial mass) discriminates ACM-LV from pIM with extremely high specificity.

ASSOCIATION OF DURATION AND INTENSITY OF EXERCISE WITH SEVERITY OF PHENOTYPIC EXPRESSION IN HYPERTROPHIC CARDIOMYOPATHY

ASSOCIATION OF DURATION AND INTENSITY OF EXERCISE WITH SEVERITY OF PHENOTYPIC EXPRESSION IN HYPERTROPHIC CARDIOMYOPATHY

Adverse events prediction in nonischemic dilated cardiomyopathy by two machine learning models integrating clinical and cardiovascular magnetic resonance data

Abstract Funding Acknowledgements Type of funding sources: None. Background Risk stratification of patients with nonischemic dilated cardiomyopathy (NIDCM) is challenging. Machine learning (ML) models represent novel technologic tools that promise to improve predictive ability of cardiovascular adverse outcomes. Purpose To implement two ML models to predict the risk of adverse outcome (composite endpoint of all-cause death, heart transplantation, implantation of ventricular assistant device), in patients with NIDCM using clinical and radiological data. Methods We retrospective collected data from patients affected by NIDCM referred to our cardiologic unit between 2002 and 2018. Two ML models, an extreme gradient boosting machine (XGBoost) and an artificial neural network (ANN), were developed using cardiovascular magnetic resonance (CMR) and clinical parameters. Both models were trained on 67% of patients and evaluated based on the other 33% as internal validation. To investigate the contribution of different features in our models, we used Shapley additive explanations (SHAP) analysis. Results The final cohort resulted of 161 patients (mean age 48.9±13.6 years, 71% males), with a mean follow up duration of 79.1 months. During the follow-up 29 adverse event were observed (16 deaths, 12 heart transplantation, 1 implantation of ventricular assistant device). ANN model resulted in an accuracy of 76% with an area under the receiver operating characteristic (ROC) curve of 0.66; SHAP analysis identified male sex, percentage of late gadolinium enhancement (LGE) and LGE distribution in the lateral wall of myocardium as the main markers of adverse outcomes. XGBoost model resulted in an accuracy of 78% with a ROC curve of 0.76; SHAP analysis identified NT-proBNP level, age and serum creatinine mainly related with adverse outcomes. About CMR parameters, left ventricular diameter and ejection fraction better related with adverse outcomes. Conclusions Our models achieved a good performance in prediction of adverse events, suggesting that ML could improve our ability of risk stratification of NIDCM. XGBoost better performed than ANN, and SHAP analysis suggested that both CMR parameters and clinical parameters contribute to the definition of risk. ANN performance and SHAP analysisXGBoost performance and SHAP analysis

Effect of Obesity on Left Ventricular Remodeling and Clinical Outcome in Chinese Patients With Hypertrophic Cardiomyopathy: Assessed by Cardiac MRI.

Obesity is highly prevalent in patients with hypertrophic cardiomyopathy (HCM) and believed to influence its phenotype. To explore the effects of obesity on left ventricular (LV) remodeling and long-term clinical course in Chinese patients with HCM. Longitudinal. A total of 247 patients with HCM classified according to body mass index (BMI) (normal weight: BMI=18.0-22.9kg/m2 [N=90]; overweight: BMI=23.0-24.9kg/m2 [N=58]; and obese: BMI ≥ 25 kg/m2 [N=99]). 3.0 T/Balanced steady-state free precession sequence and phase-sensitive inversion recovery late gadolinium enhancement (LGE) sequence. LV function and geometry were measured. LV peak strain analysis was performed. The presence and percentage of LGE in the LV were recorded. The endpoints including heart failure, sudden cardiac death, and overall composite outcome were assessed during a median follow-up of 4.1 years (interquartile range, 3.0-6.2 years). One-way analysis of variance, Kruskal-Wallis test, or chi-square test; Pearson correlation coefficient (r); multivariable linear regression analysis; Kaplan-Meier survival analysis; and Cox proportional hazards model analysis were conducted. A two-tailed P-value < 0.05 was considered statistically significant. Obese patients exhibited a significant progressive increase in LV mass compared with normal-weight patients. The magnitude of all LV strain indices gradually and significantly decreased as BMI increased, whereas LV ejection fraction was not significantly different among BMI groups (P=0.364). Multivariable linear regression analysis showed that obesity had a significant association with impaired strain indices as well as with indexed LV mass. Multivariable Cox model analysis retained obesity as an independent marker for future endpoints, and conveyed a > 3-fold increase in risk compared with patients with normal weight (hazard ratio, 3.04; 95% confidence interval, 1.07-6.57). Obesity is an important environmental modifier that is associated with adverse LV remodeling and is independently associated with future clinical outcomes in Chinese patients with HCM. 3 TECHNICAL EFFICACY: Stage 2.

Layer-specific Strain Analysis with Cardiac MRI Feature Tracking in Acute Myocarditis.

To evaluate the diagnostic performance of layer-specific cardiac MRI feature-tracking (FT) strain analysis in patients with acute myocarditis. Seventy patients (mean age, 43 years ± 19 [SD]; 46 men) with clinically defined acute myocarditis and 42 healthy controls who underwent cardiac MRI from March 2014 to November 2018 were retrospectively analyzed. FT-based left ventricular peak systolic global longitudinal strain (GLS) and global circumferential strain (GCS) were assessed at subendocardial, midmyocardial, and subepicardial layers. The 2018 Lake Louise criteria (LLC) were assessed. Patients with myocarditis were dichotomized into two groups: those with preserved and those with reduced ejection fraction. For statistical analysis, unpaired t test, one-way analysis of variance, Pearson correlation, and receiver operating characteristic analysis were used. GLS and GCS values of all layers (eg, midmyocardial GCS: -21.3% ± 5.5 vs -28.0% ± 4.3; P < .001) were impaired in patients with myocarditis compared with controls. Only subepicardial GLS (-20.0% ± 3.3 vs -17.5% ± 3.3; P < .001) and midmyocardial GCS values (-28.0% ± 4.3 vs -23.1% ± 4.3; P < .001) could differentiate between controls and patients with preserved ejection fraction. Midmyocardial GCS correlated with inflammatory myocardial parameters (eg, late gadolinium enhancement percentage, r = 0.48, P < .001). Midmyocardial GCS (area under the receiver operating characteristic curve [AUC], 0.82) and subepicardial GLS (AUC, 0.77) had the highest diagnostic performance for acute myocarditis diagnosis (P < .05 against all other strain parameters). The diagnostic performance of the 2018 LLC was significantly improved by inclusion of these two strain parameters (AUC, 0.92 vs 0.97; P = .04). Diagnostic performance of cardiac MRI FT strain was different between myocardial layers in acute myocarditis, with midmyocardial GCS and subepicardial GLS providing the highest diagnostic performance.Keywords: MRI, Cardiac, Heart, Left Ventricle, Inflammation, Tissue Characterization, MR-Functional Imaging, Feature-Tracking Strain, Acute Myocarditis Supplemental material is available for this article. © RSNA, 2022.

Global Circumferential Strain by Cardiac Magnetic Resonance Tissue Tracking Associated With Ventricular Arrhythmias in Hypertrophic Cardiomyopathy Patients.

Background: Hypertrophic cardiomyopathy (HCM) is prone to myocardial heterogeneity and fibrosis, which are the substrates of ventricular arrhythmias (VAs). Cardiac magnetic resonance tissue tracking (CMR-TT) can quantitatively reflect global and regional left ventricular strain from different directions. It is uncertain whether the change of myocardial strain detected by CMR-TT is associated with VAs. The aim of the study is to explore the differential diagnostic value of VAs in HCM by CMR-TT.Materials and Methods: We retrospectively included 93 HCM patients (38 with VAs and 55 without VAs) and 30 healthy cases. Left ventricular function, myocardial strain parameters and percentage of late gadolinium enhancement (%LGE) were evaluated.Results: Global circumferential strain (GCS) and %LGE correlated moderately (r = 0.51, P < 0.001). HCM patients with VAs had lower left ventricular ejection fraction (LVEF), global radial strain (GRS), GCS, and global longitudinal strain (GLS), but increased %LGE compared with those without VAs (P < 0.01 for all). %LGE and GCS were indicators of VAs in HCM patients by multivariate logistic regression analysis. HCM patients with %LGE >5.35% (AUC 0.81, 95% CI 0.70–0.91, P < 0.001) or GCS >-14.73% (AUC 0.79, 95% CI 0.70–0.89, P < 0.001) on CMR more frequently had VAs. %LGE + GCS were able to better identify HCM patients with VAs (AUC 0.87, 95% CI 0.79–0.95, P < 0.001).Conclusion: GCS and %LGE were independent risk indicators of VAs in HCM. GCS is expected to be a good potential predictor in identifying HCM patients with VAs, which may provide important values to improve risk stratification in HCM in clinical practice.

Predictors and Mechanisms of Atrial Fibrillation in Patients With Hypertrophic Cardiomyopathy

Atrial fibrillation (AF) in hypertrophic cardiomyopathy (HC) is associated with significant symptomatic deterioration, heart failure, and thromboembolic disease. There is a need for better mechanistic insight and improved identification of at risk patients. We used cardiovascular magnetic resonance (CMR) to assess predictors of AF in HC, in particular the role of myocardial fibrosis. Consecutive patients with HC referred for CMR 2003 to 2013 were prospectively enrolled. CMR parameters including left ventricular volumes, presence and percentage of late gadolinium enhancement in the left ventricle (%LGE) and left atrial volume index (LAVi) were measured. Overall, 377 patients were recruited (age 62 ± 14 years, 73% men). Sixty-two patients (16%) developed new-onset AF during a median follow up of 4.5 (interquartile range 2.9 to 6.0) years. Multivariable analysis revealed %LGE (hazard ratio [HR] 1.3 per 10% (confidence interval: 1.0 to 1.5; p = 0.02), LAVi (HR 1.4 per 10 mL/m2[1.2 to 1.5; p < 0.001]), age at HC diagnosis, nonsustained ventricular tachycardia and diabetes to be independent predictors of AF. We constructed a simple risk prediction score for future AF based on the multivariable model with a Harrell's C-statistic of 0.73. In conclusion, the extent of ventricular fibrosis and LA volume independently predicted AF in patients with HC. This finding suggests a mechanistic relation between fibrosis and future AF in HC. CMR with quantification of fibrosis has incremental value over LV and LA measurements in risk stratification for AF. A risk prediction score may be used to identify patients at high risk of future AF who may benefit from more intensive rhythm monitoring and a lower threshold for oral anticoagulation.

P340Inflammatory cardiomyopathy in Fabry disease

Abstract Background Fabry disease (FD) is an X-linked lysosomal storage disorder caused by mutations in α-galactosidase A. Cardiovascular magnetic resonance (CMR) has helped unveil the pathogenesis of Fabry cardiomyopathy: sphingolipid storage (low T1 mapping values), left ventricular hypertrophy (LVH) and myocardial fibrosis with late gadolinium enhancement (LGE) characteristically present in the basal inferolateral (BIFL) wall. Recent evidence has suggested that the LGE may be inflammation and oedema as part of this pathogenic process. Purpose To assess the presence of inflammation in patients with FD using T2 mapping (for oedema/inflammation) supported by blood troponin levels (showing myocyte death and by inference inflammation). Methods A multi-centre international study in gene positive FD patients using CMR and blood biomarkers. All participants underwent CMR at 1.5 T. Native T1 and T2 mapping were performed. The T1 mapping sequence was MOLLI with sampling scheme in seconds. LGE used a phase sensitive inversion recovery sequence. Global longitudinal 2D strain (GLS) values were obtained using feature tracking analysis. Blood high-sensitivity troponin T (hsTnT) was measured on the same day. Results 100 FD patients (age 43.8±1.3 years, 42% male) were included. 45% had LVH, 35% LGE. Low T1 mapping (normal &lt;943ms) was found in 49% and 33% had high hsTnT values (normal &lt;15ng/L). Mean T2 mapping values were 52.6±0.6ms in the BIFL wall and 49.5±0.3ms in the remote myocardium/septum (p&lt;0.001, normal &lt;53ms). T2 values in the BIFL wall were significantly higher among patients with LGE (58.2±6.1ms vs 49.2±3.1ms, p&lt;0.001, Figure 1). In a per-segment analysis of 1600 segments, higher T2 values correlated positively with percentage of LGE per segment (r=0,262, p&lt;0.001), T1 values (r=0,205, p&lt;0.001), maximum wall thickness (r=0,253, p&lt;0.001) and GLS values (r=0,212, p&lt;0.001). HsTnT values were higher among patients with LGE (median of 31 vs 3ng/L in patients without LGE, p&lt;0.001). There was a strong positive correlation between T2 values in the BIFL wall and ln(hsTnT) (r=0.776, p&lt;0.001, Figure 2). The strongest predictor of increased hsTnT in multivariate analysis (age, sex, LVH, septum T1, T2 in the BIFL, GLS, LGE) was T2 in the BIFL wall (β=0.4, p=0.001). Conclusions Cardiac involvement in FD goes beyond storage (low T1 values). When LGE is present, this is almost always associated with a high T2 and troponin elevation supporting FD as a chronic inflammatory cardiomyopathy. Initial reports of LGE being fibrosis are too simplistic – LGE in FD appears to have a significant chronic inflammation/oedema component.

P5554Follow the light - The prognostic value of late gadolinium enhancement in hypertrophic cardiomyopathy

Abstract Background and aim Hypertrophic cardiomyopathy (HCM) is a genetic based cardiomyopathy with heterogeneous phenotypic expression. Since it is one of the most common cause of sudden cardiac death (SCD) in the young different risk score have been proposed to properly identify the patients that would benefit from a primary prevention with an implantable cardioverter-defibrillator (ICD). ESC guidelines on HCM suggest to estimate the risk of SCD considering clinical and echocardiographic parameters and mention the use of cardiac magnetic resonance (CMR) only in the case of poor echo windows. The aim of the present study-level meta-analysis was to explore the prognostic value of late gadolinium enhancement (LGE) cardiac magnetic resonance (CMR) for adverse fatal events. Methods We searched PubMed and EMBASE for studies that investigated the prognostic value of LGE in patients with HCM. The outcomes of interest were SCD or aborted SCD, all-cause mortality and cardiovascular (CV) mortality. Random-effects Odds Ratios (ORs) were estimated using a DerSimonian-Laird method with a person-year approach. Moreover, an univariate meta-regression was performed to assess the moderator effect of mean age, LGE % of left ventricle (LV) and gender (expressed as male percentage). Results A total of 7 studies (n=3351) were included in the analysis. Mean follow-up was 3±0.63 years. Mean age was 47.7±14.6 years and 56.9% were male. LGE was detected in 1845 (55%) patients with a mean LGE percentage of LV of 7%. The presence of LGE was associated with an increased incidence of SCD or aborted SCD (OR 3.44; 95% CI 2.02–5.86; p&lt;0.001- Figure), all-cause mortality (OR 1.92; 95% CI 1.31–2.81; p&lt;0.001) and CV mortality (OR 3.16; 95% CI 1.77–5.64; p&lt;0.001) compared with the absence of LGE at CMR. The LGE percentage of LV, mean age and gender did not have any moderator effect on the outcomes of interest. However, LGE % of LV was reported only in 4 studies and the absence of any moderator effect of this parameter could be due to a type II error. Prognostic value of LGE for SCD Conclusions The presence of LGE at CMR in patients with HCM exhibited a substantial prognostic value in fatal events and, in particular, in the prediction of SCD. LGE assessment is an effective tool to stratify the arrhythmic risk in HCM. Therefore, it should be considered, especially in borderline cases, to improve the identification of HCM patients who could benefit from ICD implantation.

Predictive Values of Apelin for Myocardial Fibrosis in Hypertrophic Cardiomyopathy.

Apelin was proved to attenuate cardiac interstitial fibrosis. However, the association between apelin level and myocardial fibrosis in patients with hypertrophic cardiomyopathy (HCM) is still unclear.This study aims to determine whether apelin is associated with myocardial fibrosis in HCM and investigate the predictive values of apelin for myocardial fibrosis in HCM.One hundred sixteen patients with HCM were enrolled in this study. Plasma apelin-13 and high-sensitivity cardiac troponin I (cTNI) were measured. The cardiac systolic and diastolic functions were evaluated by echocardiography, and the presence and extent of cardiac fibrosis were assessed by cardiac magnetic resonance. All statistical data were analyzed by SPSS version 21.0.The percentage of late gadolinium enhancement (LGE) was negatively correlated with apelin and positively correlated with cTNI, maximum wall thickness (MWT), and left ventricular mass index in the overall patients with HCM and LGE. Apelin, cTNI, MWT, and left ventricular ejection fraction were independent predictors of the presence of LGE. The cutoff values of apelin, cTNI, and MWT were 1.24 pg/mL, 0.031 ng/mL, and 19 mm, respectively, for the prediction of LGE. The combined measurements of MWT ≥ 19 mm and/or apelin ≤ 1.24 pg/mL, as well as the combined measurements of MWT ≥ 19 mm and/or cTNI ≥ 0.031 ng/mL, obtained higher specificity and higher sensitivity, thus, indicating the presence of LGE.Plasma apelin and cTNI are independent predictors of myocardial fibrosis. The combined measurements of serum apelin and MWT, as well as cTNI and MWT, showed higher predictive values for predicting myocardial fibrosis in patients with HCM.

Galectin-3 predicts response and outcomes after cardiac resynchronization therapy

BackgroundCardiac resynchronization therapy (CRT) reduces symptoms, morbidity and mortality in chronic heart failure patients with wide QRS complexes. However, approximately one third of CRT patients are non-responders. Myocardial fibrosis is known to be associated with absence of response. We sought to see whether galectin-3, a promising biomarker involved in fibrosis processes, could predict response and outcomes after CRT.MethodsConsecutive patients eligible for implantation of a CRT device with a typical left bundle branch block ≥ 120 ms were prospectively included. Serum Gal-3 level, Selvester ECG scoring, and cardiac magnetic resonance with analysis of late gadolinium enhancement (LGE) were ascertained. Response to CRT was defined by a composite endpoint at 6 months: no death, nor hospitalization for major cardiovascular event, and a significant decrease in left ventricular end-systolic volume of 15% or more.ResultsSixty-one patients were included (age 61 ± 5 years, ejection fraction 27 ± 5%), 59% with non-ischemic cardiomyopathy. At 6 months, 49 patients (80%) were considered responders. Responders had a lower percentage of LGE (8 ± 13% vs 22 ± 16%, p = 0.006), and a trend towards lower rates of galectin-3 (16 ± 6 ng/mL vs 19 ± 8 ng/mL, p = 0.13). LGE ≥ 14% and Gal-3 ≥ 22 ng/mL independently predicted response to CRT (OR = 0.17 [0.03–0.62], p = 0.007, and OR = 0.11 [0.02–0.04], p < 0.001, respectively). At 48 months of follow-up, 12 patients had been hospitalized for a major cardiovascular event or had died. Galectin-3 level predicted long-term outcomes (HR = 3.31 [1.00–11.34], p = 0.05).ConclusionsGal-3 serum level predicts the response to CRT at 6 months and long-term outcomes in chronic heart failure patients.

Predictors of late gadolinium enhancement in hypertrophic cardiomyopathy by electrocardiogram and echocardiography

Objective To explore the predictive value of routine echocardiographic and electrocardiographic parameters in late gadolinium enhancement (LGE) in hypertrophic cardiomyopathy (HCM). Methods The study population consisted of a consecutive series of 95 HCM patients.According to the presence of LGE on cardiac magnetic resonance (CMR), these patients were divided into two groups: HCM patients with LGE (n=71) and HCM patients without LGE (n=24). The parameters of routine echocardiography and electrocardiography were compared between the two groups. ROC and Logistic analysis were made to find the predictors of LGE. Results ①As compared to those without LGE, HCM patients with LGE had higher prevalence of chest pain (P=0.027), β-blocker treatment (P=0.024), increased maximal left ventricular wall thickness (MLVWT) (P<0.000 1), non-sustained ventricular tachycardia (P=0.034), prolonged the rate-corrected cardiac QT interval (QTc) (P=0.011), T-wave inversion (TWI) (P=0.009), but reduced early diastolic mitral annular velocity (e′) (P=0.001). ②Univariate predictors of LGE on CMR were: increased MLVWT, reduced e′, prolonged QTc and more TWI. Only MLVWT (OR=1.23, 95%CI=1.05-1.44, P=0.013) and e′(OR=1.23, 95%CI=0.52-0.96, P=0.028) remained independent after multivariable analysis. Furthermore, the ROC analysis showed that these two parameters had discriminative ability to identify those with LGE. To be specific, HCM patients with MLVWT≥21.5 mm or e′≤5.55 cm/s were more likely to present with LGE. ③The leads number of TWI was positively correlated with percentage of LGE in left ventricular mass (LGE%) (r=0.220, P=0.044), but there was no correlation between location of TWI on ECG and territory of LGE on CMR. Conclusions In HCM patients, MLVWT and e′ are independent predictors of LGE on CMR. Furthermore, although the leads number of TWI is correlated with LGE%, no correlation has been found between location of TWI on ECG and territory of LGE on CMR. Key words: Echocardiography; Cardiomyopathy, hypertrophic; Electrocardiography; Late gadolinium enhancement