Presence Of Late Gadolinium Enhancement Research Articles

Serial Cardiac Magnetic Resonance Imaging in Patients with Mitral Valve Prolapse-A Single-Center Retrospective Registry.

Background: Mitral valve prolapse (MVP) and mitral annular disjunction (MAD) are common valvular abnormalities that have been associated with ventricular arrhythmias (VA). Cardiac magnetic resonance imaging (CMR) has a key role in risk stratification of VA, including assessment of late gadolinium enhancement (LGE). Methods: Single-center retrospective analysis of patients with MVP or MAD who had >1 CMR and >1 24 h Holter registration available. Data are presented in detail, including evolution of VA and presence of LGE over time. Results: A total of twelve patients had repeated CMR and Holter registrations available, of which in four (33%) patients, it was conducted before and after minimal invasive mitral valve repair (MVR). After a median of 4.7 years, four out of eight (50%) patients without surgical intervention had new areas of LGE. New LGE was observed in the papillary muscles and the mid to basal inferolateral wall. In four patients, presenting with syncope or high-risk non-sustained ventricular tachycardia (VT), programmed ventricular stimulation was performed and in two (50%), sustained monomorphic VT was easily inducible. In two patients who underwent MVR, new LGE was observed in the basal inferolateral wall of which one presented with an increased burden of VA. Conclusions: In patients with MVP and MAD, repeat CMR may show new LGE in a small subset of patients, even shortly after MVR. A subgroup of patients who presented with an increase in VA burden showed new LGE upon repeat CMR. VA in patients with MVP and MAD are part of a heterogeneous spectrum that requires further investigation to establish risk stratification strategies.

The role of cardiac magnetic resonance in sports cardiology: results from a large cohort of athletes.

Cardiac magnetic resonance (CMR) provides information on morpho-functional abnormalities and myocardial tissue characterisation. Appropriate indications for CMR in athletes are uncertain. To analyse the CMR performed at our Institute to evaluate variables associated with pathologic findings in a large cohort of athletes presenting with different clinical conditions. All the CMR performed at our Institute in athletes aged > 14years were recruited. CMR indications were investigated. CMR was categorised as "positive" or "negative" based on the presence of morphological and/or functional abnormalities and/or the presence of late gadolinium enhancement (excluding the right ventricular insertion point), fat infiltration, or oedema. Variables associated with "positive" CMR were explored. A total of 503 CMR were included in the analysis. "Negative" and "positive" CMR were 61% and 39%, respectively. Uncommon ventricular arrhythmias (VAs) were the most frequent indications for CMR, but the proportion of positive results was low (37%), and only polymorphic ventricular patterns were associated with positive CMR (p = 0.006). T-wave inversion at 12-lead ECG, particularly on lateral and inferolateral leads, was associated with positive CMR in 34% of athletes (p = 0.05). Echocardiography abnormalities resulted in a large proportion (58%) of positive CMR, mostly cardiomyopathies. CMR is more efficient in identifying a pathologic cardiac substrate in athletes in case of VAs (i.e., polymorphic beats), abnormal ECG repolarisation (negative T-waves in inferolateral leads), and borderline echocardiographic findings (LV hypertrophy, mildly depressed LV function). On the other hand, CMR is associated with a large proportion of negative results. Therefore, a careful clinical selection is needed to indicate CMR in athletes appropriately.

Biventricular Impairment and Ventricular Interdependence in Patients With Alcoholic Cardiomyopathy: Insights Through Cardiac Magnetic Resonance Imaging.

Alcoholic cardiomyopathy (ACM) can lead to progressive cardiac dysfunction and heart failure, but little is known about biventricular impairment and ventricular interdependence (VI) in ACM patients. To use cardiac MRI to investigate biventricular impairment and VI in ACM patients. Retrospective. Forty-one male patients with ACM and 45 sex- and age-matched controls. 3.0 T/balanced steady-state free precession sequence, inversion recovery prepared echo-planar imaging sequence and phase-sensitive inversion recovery sequence. Biventricular structure, function, and global strain (encompassing peak strain [PS], peak systolic, and diastolic strain rate), PS of interventricular septal (IVS), microvascular perfusion (including upslope and time to maximum signal intensity [TTM]), late gadolinium enhancement (LGE), and baseline characteristics were compared between the controls and ACM patients. Student's t-test, Mann-Whitney U test, Pearson's correlation, and multivariable linear regression models with a stepwise selection procedure. A two-tailed P value <0.05 was deemed as statistically significant. Compared to control subjects, ACM patients showed significantly biventricular adverse remodeling, reduced left ventricle (LV) global upslope and prolonged global TTM, and the presence of LGE. ACM patients were characterized by a significant decline in all global strain within the LV, right ventricle (RV), and IVS compared with the controls. RV global PS was significantly associated with LV global PS and IVS PS in radial, circumferential, and longitudinal directions. Multivariable analyses demonstrated the longitudinal PS of IVS was significantly correlated with RV global radial PS (β = 0.614) and circumferential PS (β = 0.545). Additionally, RV global longitudinal PS (GLPS) was significantly associated with radial PS of IVS (β = -0.631) and LV GLPS (β = 1.096). ACM patients exhibited biventricular adverse structural alterations and impaired systolic and diastolic function. This cohort also showed reduced LV microvascular perfusion, the presence of LGE, and unfavorable VI. 3 TECHNICAL EFFICACY: Stage 3.

Evaluation the relationship between myocardial fibrosis and left ventricular torsion measured by cardiac magnetic resonance feature-tracking in hypertrophic cardiomyopathy patients with preserved ejection fraction.

The relationship between left ventricular (LV) torsion and myocardial fibrosis (MF) in hypertrophic cardiomyopathy (HCM) patients with preserved ejection fraction was still not well understood. New developments in cardiac magnetic resonance (CMR) enable a much fuller assessment of cardiac characteristics. This study sought to assess the impact of HCM on myocardial function as assessed by LV torsion and its relationship with MF. HCM (n = 79) and healthy controls (n = 40) underwent CMR. According to whether there was late gadolinium enhancement (LGE), patients were divided into LGE+ group and LGE- group. LV torsion and torsion rate were measured by CMR feature-tracking (CMR-FT). MF was quantitatively evaluated through LGE imaging. LGE was present in 44 patients (56%). Compared with healthy controls, torsion increased in the LGE- group (P < 0.001). Compared with LGE+ group, torsion was higher in the LGE- group (P < 0.001). There was no significant difference in torsion between LGE+ group and healthy controls. Correlation analysis showed that torsion was correlated with LGE% (r= -0.443) and LGE mass (r= -0.435) respectively. On multivariable logistic regression analysis, LV torsion was the only feature that was independently associated with the presence of LGE (OR 0.130; 95% CI 0.040 to 0.420, P = 0.01). The best torsion value associated with MF was 1.91 (sensitivity 60.0%, specificity 77.3%, AUC = 0.733). In HCM patients with preserved ejection fraction, CMR-FT derivedLV torsion analysis holds promise for myocardial fibrosis detection.

Prevalence and clinical significance of late gadolinium enhancement in children and adolescents with hypertrophic cardiomyopathy: a systematic review and meta-analysis.

Hypertrophic cardiomyopathy is the leading cause of sudden cardiac death among the paediatric population. The aim of this study is to investigate the prevalence and clinical significance of late gadolinium enhancement, as assessed by cardiac MRI, in paediatric hypertrophic cardiomyopathy. A systematic literature search was conducted in PubMed, SCOPUS, and Ovid SP to identify relevant studies. Pooled estimates with a 95% confidence interval were calculated using the random-effects generic inverse variance model. Statistical analysis was performed using Review Manager v5.4 and R programming. Seventeen studies were included in this meta-analysis, encompassing a total of 778 patients. Late gadolinium enhancement was highly prevalent in paediatric hypertrophic cardiomyopathy, with a pooled prevalence of 51% (95% confidence interval, 40-62%). The estimated extent of focal fibrosis expressed as a percentage of left ventricular mass was 4.70% (95% confidence interval, 2.11-7.30%). The presence of late gadolinium enhancement was associated with an increased risk of adverse cardiac events (pooled odds ratio 3.49, 95% confidence interval 1.10-11.09). The left ventricular mass index of late gadolinium enhancement-positive group was higher than the negative group, with a standardised mean difference of 0.91 (95% confidence interval, 0.42-1.41). This meta-analysis demonstrates that prevalence of late gadolinium enhancement in paediatric hypertrophic cardiomyopathy is similar to that in the adult population. The presence and extent of late gadolinium enhancement are independent predictors of adverse cardiac events, underscoring their prognostic significance among the paediatric population.

Prognostic value of visual and quantitative CMR regional myocardial function in patients with suspected myocarditis

According to updated Lake-Louise Criteria, impaired regional myocardial function serves as a supportive criterion in diagnosing myocarditis. This study aimed to assess visual regional wall motional abnormalities (RWMA) and novel quantitative regional longitudinal peak strain (RLS) for risk stratification in the clinical setting of myocarditis. In patients undergoing CMR and meeting clinical criteria for suspected myocarditis global longitudinal strain (GLS), late gadolinium enhancement (LGE), RWMA and RLS were assessed in the anterior, septal, inferior, and lateral regions and correlated to the occurrence of major adverse cardiac events (MACE), including heart failure hospitalization, sustained ventricular tachycardia, recurrent myocarditis, and all-cause death. In 690 consecutive patients (age: 48.0 ± 16.0 years; 37.7% female) with suspected myocarditis impaired RLS was correlated with RWMA and LV-GLS but not with the presence of LGE. At median follow up of 3.8 years, MACE occurred in 116 (16.8%) patients. Both, RWMA and RLS in anterior-, septal-, inferior-, and lateral- locations were univariately associated with outcomes (all p < 0.001), but not after adjusting for clinical characteristics and LV-GLS. In the subgroup of patients with normal LV function, RWMA were not predictive of outcomes, whereas septal RLS had incremental and independent prognostic value over clinical characteristics (HRadjusted = 1.132, 95% CI 1.020–1.256; p = 0.020). RWMA and RLS can be used to assess regional impairment of myocardial function in myocarditis but are of limited prognostic value in the overall population. However, in the subgroup of patients with normal LV function, septal RLS represents a distinctive marker of regional LV dysfunction, offering potential for risk-stratification.Graphical abstractCI: confidence interval, CMR: cardiac magnetic resonance imaging, HR: hazard ratio, MACE major adverse cardiovascular events

The usefulness of speckle tracking echocardiography for the prediction of cardiac involvement in patients with biopsy-proven sarcoidosis.

Cardiac sarcoidosis (CS) is commonly diagnosed based on clinical criteria and abnormalities in noninvasive imaging reported in patients with biopsy-proven extracardiac sarcoidosis. Electrocardiogram and two-dimensional echocardiography have a low sensitivity for CS detection. Cardiovascular magnetic resonance imaging (CMR) and positron emission tomography (PET) have limitations in terms of cost and availability. This study aimed to assess the usefulness of left ventricular longitudinal strain, measured using two-dimensional speckle tracking echocardiography (STE), for the prediction of late gadolinium enhancement (LGE) presence in CMR in patients with biopsy-proven sarcoidosis. A total of 119 patients with biopsy-proven extracardiac sarcoidosis were divided, according to the clinical criteria proposed by the 2014 Heart Rhythm Society expert consensus statement (HRS 2014), into two groups: 43 individuals with "probable cardiac sarcoidosis", CS(+) and 76 individuals without cardiac sarcoidosis, CS (-). Data from echocardiography, CMR, 12-lead ECG and 24h Holter monitoring were analyzed. Left ventricular global longitudinal strain (LV-GLS) was slightly reduced in the entire sarcoidosis group (-18.61±2.96), no difference between the CS (+) and CS (-) subgroups was found (-18.0%±3.2% and -18.9%±2.8%, respectively; p=.223). No cut-off value for LV-GLS was identified that could predict the presence of LGE. Segmental longitudinal strain impairment partially correlated with the presence of LGE on CMR. In our cohort of sarcoidosis patients, segmental longitudinal strain proved more helpful in the diagnostic process than LV-GLS. The ultimate role of STE in the diagnosis of CS remains to be established.

Assessment of myocardial strain patterns in patients with left bundle branch block using cardiac magnetic resonance.

Recently, a classification with four types of septal longitudinal strain patterns was described using echocardiography, suggesting a pathophysiological continuum of left bundle branch block (LBBB)-induced left ventricle (LV) remodeling. The aim of this study was to assess the feasibility of classifying these strain patterns using cardiovascular magnetic resonance (CMR), and to evaluate their association with LV remodeling and myocardial scar. Single center registry included LBBB patients with septal flash (SF) referred to CMR to assess the cause of LV systolic dysfunction. Semi-automated feature-tracking cardiac resonance (FT-CMR) was used to quantify myocardial strain and detect the four strain patterns. A total of 115 patients were studied (age 66 ± 11years, 57% men, 28% with ischemic heart disease). In longitudinal strain analysis, 23 patients (20%) were classified in stage LBBB-1, 37 (32.1%) in LBBB-2, 25 (21.7%) in LBBB-3, and 30 (26%) in LBBB-4. Patients at higher stages had more prominent septal flash, higher LV volumes, lower LV ejection fraction, and lower absolute strain values (p < 0.05 for all). Late gadolinium enhancement (LGE) was found in 55% of the patients (n = 63). No differences were found between the strain patterns regarding the presence, distribution or location of LGE. Among patients with LBBB, there was a good association between strain patterns assessed by FT-CMR analysis and the degree of LV remodeling and LV dysfunction. This association seems to be independent from the presence and distribution of LGE.

Prognostic value of native T1 and extracellular volume in patients with immunoglubin light-chain amyloidosis

BackgroundCardiac involvement in patients with immunoglubin light-chain amyloidosis (AL) is a major determinant of treatment choice and prognosis, and early identification of high-risk patients can initiate intensive treatment strategies to achieve better survival. This study aimed to investigate the prognostic value of native T1 and ECV in patients with AL-cardiac amyloidosis (CA).MethodsA total of 38 patients (mean age 59 ± 11 years) with AL diagnosed histopathologically from July 2017 to October 2021 were collected consecutively. All patients were performed 3.0-T cardiac magnetic resonance (CMR) including cine, T1 mapping, and late gadolinium enhancement (LGE). Pre- and post-contrast T1 mapping images were transferred to a dedicated research software package (CVI42 v5.11.3) to create parametric T1 and ECV values. In addition, clinical and laboratory data of all patients were collected, and patients or their family members were regularly followed up by telephone every 3 months. The starting point of follow-up was the time of definitive pathological diagnosis, and the main endpoint was all-cause death. Kaplan-Meier analysis and Cox proportional risk model were used to evaluate the association between native T1 and ECV and death in patients with CA.ResultsAfter a median follow-up of 27 (16, 37) months, 12 patients with CA died. Kaplan-Meier analysis showed that elevated native T1 and ECV were closely associated with poor prognosis in patients with CA. The survival rate of patients with ECV > 44% and native T1 > 1389ms were significantly lower than that of patients with ECV ≤ 44% and native T1 ≤ 1389ms (Log-rank P < 0.001), and was not associated with the presence of LGE. After adjusting for clinical risk factors and CMR measurements in a stepwise multivariate Cox regression model, ECV [risk ratio (HR):1.37, 95%CI: 1.09–1.73, P = 0.008] and native T1 (HR:1.01, 95%CI: 1.00-1.02, P = 0.037) remained independent predictors of all-cause mortality in patients with CA.ConclusionsBoth native T1 and ECV were independently prognostic for mortality in patients with CA, and can be used as important indicators for clinical prognosis assessment of AL.

Cardiovascular magnetic resonance in muscular dystrophies: looking ahead.

Cardiac magnetic resonance (CMR) is an established tool for risk stratification in several cardiomyopathies, and its role in muscular dystrophies (MuD) looks promising. We sought to assess how CMR performs in predicting cardiac events in a real cohort of MuD patients. A prospective single-center study with the enrollment of consecutive adult MuD patients referred to cardiac screening from 2012 to 2018 with the collection of clinical and CMR data. During follow-up (FUP), major adverse cardiac events were considered a composite of device implantation, ventricular tachycardia (VT), hospitalization due to heart failure, and death. Sixty-five patients were included (mean age of 32±16, 51% female); the majority had myotonic dystrophy (34; 52.3%); most were asymptomatic (60; 92.3%) and at sinus rhythm (64; 98.5%). CMR was abnormal in 23 (43.3%) patients: left ventricle ejection fraction (LVEF) <55% was found in 7 patients, and late gadolinium enhancement (LGE) was present in 23 patients, mainly intra-myocardial or subepicardial (10 and 8 patients, respectively). During a median FUP of 77 months (interquartile range: 33), there were 7 deaths, 8 implanted devices, and one sustained VT. LVEF<55% and the presence of LGE were associated with the occurrence of all events (log rank test, p=0.002 and p=0.045, respectively). LVEF<55% was associated with a 6-fold higher risk of events (crude hazard ratio of 6.15; 95% confidence interval of 1.65-22.93), that remained significant after adjusting for LGE presence (adjusted hazard ratio of 4.81, 95% confidence interval of 1.07-15.9). In our cohort, CMR LVEF<55% and the presence of LGE were significantly associated with adverse events during follow-up, reinforcing the role of this technique on risk stratification of MuD populations.

Evaluating a novel accelerated free-breathing late gadolinium enhancement imaging sequence for assessment of myocardial injury

IntroductionCardiac magnetic resonance imaging (MRI), including late gadolinium enhancement (LGE), plays an important role in the diagnosis and prognostication of ischemic and non-ischemic myocardial injury. Conventional LGE sequences require patients to perform multiple breath-holds and require long acquisition times. In this study, we compare image quality and assessment of myocardial LGE using an accelerated free-breathing sequence to the conventional standard-of-care sequence. MethodsIn this prospective cohort study, a total of 41 patients post Coronavirus 2019 (COVID-19) infection were included. Studies were performed on a 1.5 Tesla scanner with LGE imaging acquired using a conventional inversion recovery rapid gradient echo (conventional LGE) sequence followed by the novel accelerated free-breathing (FB-LGE) sequence. Image quality was visually scored (ordinal scale from 1 to 5) and compared between conventional and free-breathing sequences using the Wilcoxon rank sum test. Presence of per-segment LGE was identified according to the American Heart Association 16-segment myocardial model and compared across both conventional LGE and FB-LGE sequences using a two-sided chi-square test. The perpatient LGE extent was also evaluated using both sequences and compared using the Wilcoxon rank sum test. Interobserver variability in detection of per-segment LGE and per-patient LGE extent was evaluated using Cohen's kappa statistic and interclass correlation (ICC), respectively. ResultsThe mean acquisition time for the FB-LGE sequence was 17 s compared to 413 s for the conventional LGE sequence (P < 0.001). Assessment of image quality was similar between both sequences (P = 0.19). There were no statistically significant differences in LGE assessed using the FB-LGE versus conventional LGE on a per-segment (P = 0.42) and per-patient (P = 0.06) basis. Interobserver variability in LGE assessment for FB-LGE was good for per-segment (= 0.71) and per-patient extent (ICC = 0.92) analyses. ConclusionsThe accelerated FB-LGE sequence performed comparably to the conventional standard-of-care LGE sequence in a cohort of patients post COVID-19 infection in a fraction of the time and without the need for breath-holding. Such a sequence could impact clinical practice by increasing cardiac MRI throughput and accessibility for frail or acutely ill patients unable to perform breath-holding.

Discrimination models with radiomics features derived from cardiovascular magnetic resonance images for distinguishing hypertensive heart disease from hypertrophic cardiomyopathy.

Discriminating hypertrophic cardiomyopathy (HCM) and hypertensive heart disease (HHD) is challenging, because both are characterized by left ventricular hypertrophy (LVH). Radiomics might be effective to differentiate HHD from HCM. Therefore, this study aimed to investigate discriminators and build discrimination models between HHD and HCM using multiparametric cardiac magnetic resonance (CMR) findings and radiomics score (radscore) derived from late gadolinium enhancement (LGE) and cine images. In this single center, retrospective study, 421 HCM patients [median and interquartile range (IQR), 50.0 (38.0-59.0) years; male, 70.5%] from January 2017 to September 2021 and 200 HHD patients [median and IQR, 44.5 (35.0-57.0) years; male, 88.5%] from September 2015 to July 2022 were consecutively included and randomly stratified into a training group and a validation group at a ratio of 6:4. Multiparametric CMR findings were obtained using cvi42 software and radiomics features using Python software. After dimensional reduction, the radscore was calculated by summing the remaining radiomics features weighted by their coefficients. Multiparametric CMR findings and radscore that were statistically significant in univariate logistic regression were used to build combined discrimination models via multivariate logistic regression. After multivariate logistic regression, the maximal left ventricular end diastolic wall thickness (LVEDWT), left ventricular ejection fraction (LVEF), presence of LGE, cine radscore and LGE radscore were identified as significant characteristics and used to build a combined discrimination model. This model achieved an area under the receiver operator characteristic curve (AUC) of 0.979 (0.968-0.990) in the training group and 0.981 (0.967-0.995) in the validation group, significantly better than the model using multiparametric CMR findings alone (P<0.001). Radiomics features derived from cardiac cine and LGE images can effectively discriminate HHD from HCM.

Diabetes mellitus is associated to high-risk late gadolinium enhancement and worse outcomes in patients with nonischemic dilated cardiomyopathy

BackgroundDiabetes mellitus (DM) is associated with a worse prognosis in patients with heart failure. Our aim was to analyze the clinical and imaging features of patients with DM and their association with outcomes in comparison to nondiabetic patients in a cohort of patients with nonischemic dilated cardiomyopathy (DCM).MethodsThis is a prospective cohort study of patients with DCM evaluated in a tertiary care center from 2018 to 2021. Transthoracic echocardiography and cardiac magnetic resonance findings were assessed. A high-risk late gadolinium enhancement (LGE) pattern was defined as epicardial, transmural, or septal plus free-wall. The primary outcome was a composite of heart failure hospitalizations and all-cause mortality. Multivariable analyses were performed to evaluate the impact of DM on outcomes.ResultsWe studied 192 patients, of which 51 (26.6%) had DM. The median left ventricular ejection fraction was 30%, and 106 (55.2%) had LGE. No significant differences were found in systolic function parameters between patients with and without DM. E/e values were higher (15 vs. 11.9, p = 0.025), and both LGE (68.6% vs. 50.4%; p = 0.025) and a high-risk LGE pattern (31.4% vs. 18.5%; p = 0.047) were more frequently found in patients with DM. The primary outcome occurred more frequently in diabetic patients (41.2% vs. 23.6%, p = 0.017). DM was an independent predictor of outcomes (OR 2.01; p = 0.049) and of LGE presence (OR 2.15; p = 0.048) in the multivariable analysis. Patients with both DM and LGE had the highest risk of events (HR 3.1; p = 0.003).ConclusionDM is related to a higher presence of LGE in DCM patients and is an independent predictor of outcomes. Patients with DM and LGE had a threefold risk of events. A multimodality imaging approach allows better risk stratification of these patients and may influence therapeutic options.

MRI Assessment of Myocardial Deformation for Risk Stratification of Major Arrhythmic Events in Patients With Non-Ischemic Cardiomyopathy Eligible for Primary Prevention Implantable Cardioverter Defibrillators.

Implantable cardioverter-defibrillator (ICD) intervention is an established prophylactic measure. Identifying high-benefit patients poses challenges. To assess the prognostic value of cardiac magnetic resonance imaging (MRI) parameters including myocardial deformation for risk stratification of ICD intervention in non-ischemic cardiomyopathy (NICM) while accounting for competing mortality risk. Retrospective and prospective. One hundred and fifty-nine NICM patients eligible for primary ICD (117 male, 54 ± 13 years) and 49 control subjects (38 male, 53 ± 5 years). Balanced steady state free precession (bSSFP) and three-dimensional phase-sensitive inversion-recovery late gadolinium enhancement (LGE) sequences at 1.5 T or 3 T. Patients underwent MRI before ICD implantation and were followed up. Functional parameters, left ventricular global radial, circumferential and longitudinal strain, right ventricular free wall longitudinal strain (RV FWLS) and left atrial strain were measured (Circle, cvi42). LGE presence was assessed visually. The primary endpoint was appropriate ICD intervention. Models were developed to determine outcome, with and without accounting for competing risk (non-sudden cardiac death), and compared to a baseline model including LGE and clinical features. Wilcoxon non-parametric test, Cox's proportional hazards regression, Fine-Gray competing risk model, and cumulative incidence functions. Harrell's c statistic was used for model selection. A P value <0.05 was considered statistically significant. Follow-up duration was 1176 ± 960 days (median: 896). Twenty-six patients (16%) met the primary endpoint. RV FWLS demonstrated a significant difference between patients with and without events (-12.5% ± 5 vs. -16.4% ± 5.5). Univariable analyses showed LGE and RV FWLS were significantly associated with outcome (LGE: hazard ratio [HR] = 3.69, 95% CI = 1.28-10.62; RV FWLS: HR = 2.04, 95% CI = 1.30-3.22). RV FWLS significantly improved the prognostic value of baseline model and remained significant in multivariable analysis, accounting for competing risk (HR = 1.73, 95% CI = 1.12-2.66). In NICM, RV FWLS may provide additional predictive value for predicting appropriate ICD intervention. 2 TECHNICAL EFFICACY: Stage 5.

A Predictive Model for Cardiomyopathy Progression in a Longitudinal Cohort of DMD Patients

Background: Cardiomyopathy (CM) is currently the leading cause of mortality in patients with Duchenne muscular dystrophy (DMD). DMD CM is asymptomatic during the early stages of disease and age of onset and clinical progression vary. We previously showed that 4D kinematic analysis of cardiovascular magnetic resonance (CMR) imaging is a robust method to visualize strain and function changes in DMD patients. Our objective, using 4D CMR image analysis, is to build a predictive model to quantify disease progression, and predict functional decline as measured by left ventricular ejection fraction (LVEF) in a longitudinal cohort of DMD patients. &#x0D; &#x0D; Methods: We obtained 4D CMR images from 40 DMD patients imaged every 12 months for three years. Localized surface area strain (Ea) values were derived from compiled 4D CMR images. Patient demographics, presence or absence (+/-) of late gadolinium enhancement (LGE) and LVEF were obtained by a trained cardiologist. Principal component analysis was used to identify prominent variables contributing most to variability. These variables, along with clinically relevant variables, were selected to build the predictive model. We built a linear mixed effects model using visit, age, basal Ea, and LGE presence. Each patient was considered a random effect to account for variability in initial CM severity. &#x0D; &#x0D; Results: Study patients were grouped into stages of heart failure according to the American Heart Association: Stage A, LVEF&gt;55%, LGE(-); Stage B, LVEF&gt;55%, LGE(+); Stage C, 40%&lt;LVEF&lt;55%, LGE(+); Stage D, LVEF&lt;40%, LGE(+). Linear mixed effects model showed a good prediction of LVEF on year 3 with a root mean squared error of 3.6% and an R2 value of 0.75 (p&lt;0.0001.). &#x0D; &#x0D; Conclusion: Early detection is crucial for appropriate clinical intervention. This novel predictive model was developed to determine if Ea derived from 4D imaging, LVEF, and (+/-) LGE can be used to predict functional outcomes in DMD CM.

Liver T1 Mapping Derived From Cardiac Magnetic Resonance Imaging: A Potential Prognostic Marker in Idiopathic Dilated Cardiomyopathy.

Hepatic alterations are common aftereffects of heart failure (HF) and ventricular dysfunction. The prognostic value of liver injury markers derived from cardiac MRI studies in nonischemic dilated cardiomyopathy (DCM) patients is unclear. Evaluate the prognostic performance of liver injury markers derived from cardiac MRI studies in DCM patients. Prospective. Three hundred fifty-six consecutive DCM patients diagnosed according to ESC guidelines (age 48.7 ± 14.2 years, males 72.6%). Steady-state free precession, modified Look-Locker inversion recovery T1 mapping and phase sensitive inversion recovery late gadolinium enhancement (LGE) sequences at 3 T. Clinical characteristics, conventional MRI parameters (ventricular volumes, function, mass), native myocardial and liver T1, liver extracellular volume (ECV), and myocardial LGE presence were assessed. Patients were followed up for a median duration of 48.3 months (interquartile range 42.0-69.9 months). Primary endpoints included HF death, sudden cardiac death, heart transplantation, and HF readmission; secondary endpoints included HF death, sudden cardiac death, and heart transplantation. Models were developed to predict endpoints and the incremental value of including liver parameters assessed. Optimal cut-off value was determined using receiver operating characteristic curve and Youden method. Survival analysis was performed using Kaplan-Meier and Cox proportional hazard. Discriminative power of models was compared using net reclassification improvement and integrated discriminatory index. P value <0.05 was considered statistically significant. 47.2% patients reached primary endpoints; 25.8% patients reached secondary endpoints. Patients with elevated liver ECV (cut-off 34.4%) had significantly higher risk reaching primary and secondary endpoints. Cox regression showed liver ECV was an independent prognostic predictor, and showed independent prognostic value for primary endpoints and long-term HF readmission compared to conventional clinical and cardiac MRI parameters. Liver ECV is an independent prognostic predictor and may serve as an innovative approach for risk stratification for DCM. 1 TECHNICAL EFFICACY: Stage 2.

Prognostic Value of Left Ventricular Trabeculae Fractal Analysis in Patients with Dilated Cardiomyopathy.

The prognostic value of left ventricular (LV) myocardial trabecular complexity on cardiac magnetic resonance (CMR) in dilated cardiomyopathy (DCM) remains unknown. This study aimed to evaluate the prognostic value of LV myocardial trabecular complexity using fractal analysis in patients with DCM. Consecutive patients with DCM who underwent CMR between March 2017 and November 2021 at two hospitals were prospectively enrolled. The primary endpoints were defined as the combination of all-cause death and heart failure hospitalization. The events of cardiac death alone were defined as the secondary endpoints.LV trabeculae complexity was quantified by measuring the fractal dimension (FD) of the endocardial border based on fractal geometry on CMR. Cox proportional hazards regression and Kaplan-Meier survival analysis were used to examine the association between variables and outcomes. The incremental prognostic value of FD was assessed in nested models. A total of 403 patients with DCM (49.31±14.68 years, 69% male) were recruited. After a median follow-up of 43 months (interquartile range, 28-55 months), 87 and 24 patients reached the primary and secondary endpoints, respectively. Age, heart rate, New York Heart Association (NYHA) functional class >II, N-terminal pro-B-type natriuretic peptide (NT-proBNP), LV ejection fraction (LVEF), left ventricular end-diastolic volume index (LVEDVi), left ventricular end-systolic volume index (LVESVi), LV mass index (LVmassi), presence of late gadolinium enhancement (LGE), global FD, LV mean apical FD, and LV maximal apical FD were univariably associated with the outcomes (all P<0.05). After multivariate adjustment, LV maximal apical FD remained a significant independent predictor of outcome [Hazard ratio = 1.179 (CI 1.116, 1.246), P < 0.001]. The addition of LV maximal apical FD in the nested models added incremental prognostic value to other common clinical and imaging risk factors (all <0.001; C-statistic: 0.84 to 0.88, P< 0.001). LV maximal apical FD was an independent predictor of the adverse clinical outcomes in patients with DCM and provided incremental prognostic value over conventional clinical and imaging risk factors.

Clinical and Echocardiographic Predictors for the Presence of Late Gadolinium Enhancement on Cardiac Magnetic Resonance Imaging in Patients with Carbon Monoxide Poisoning.

Late gadolinium enhancement (LGE) on cardiac magnetic resonance imaging (CMRI) reflects the burden of myocardial damage in carbon monoxide (CO) poisoning. This study aimed to identify the clinical and echocardiographic parameters that can predict myocardial LGE on CMRI in CO poisoning. This prospective observational study included patients who presented with acute CO poisoning and elevated troponin I and underwent echocardiography and CMRI to identify myocardial damage at a tertiary university hospital between August 2017 and May 2019 and August 2020 and July 2022. Based on the CMRI findings, participants were categorized into LGE and non-LGE groups. The median age of the 155 patients was 51.0 years, and 98 (63.2%) were males. Median times from emergency department arrival to either CMRI or echocardiography were 3.0 days each. The LGE group included 99 (63.9%) patients with LGE positivity on CMRIs. Time from rescue to hyperbaric oxygen therapy >4 h (odds ratio (OR): 3.31, 95% confidence interval (CI): 1.28-8.56, p = 0.01); serum lactate levels >2 mmol/L (OR: 2.62, 95% CI: 1.20-5.73, p = 0.02); and left ventricular global longitudinal strain >-16% (OR: 2.95, 95% CI: 1.35-6.47, p = 0.007) were significant predictors of LGE positivity. The area under the curve of these predictors was 0.711. Our prediction model, which combines the clinical parameters with left ventricular global longitudinal strain, may be helpful in the early detection of LGE positivity.

Association between late gadolinium enhancement and outcome in dilated cardiomyopathy: A meta-analysis.

The prognostic value of late gadolinium enhancement (LGE) derived from cardiovascular magnetic resonance (CMR) is well studied, and several new metrics of LGE have emerged. However, some controversies remain; therefore, further discussion is needed, and more precise risk stratification should be explored. To investigate the associations between the positivity, extent, location, and pattern of LGE and multiple outcomes in dilated cardiomyopathy (DCM). PubMed, Ovid MEDLINE, and Cochrane Library were searched for studies that investigated the prognostic value of LGE in patients with DCM. Pooled hazard ratios (HRs) and 95% confidence intervals were calculated to assess the role of LGE in the risk stratification of DCM. Nineteen studies involving 7330 patients with DCM were included in this meta-analysis and covered a wide spectrum of DCM, with a mean left ventricular ejection fraction between 21% and 50%. The meta-analysis revealed that the presence of LGE was associated with an increased risk of multiple adverse outcomes (all-cause mortality, HR: 2.14; arrhythmic events, HR: 5.12; and composite endpoints, HR: 2.38; all P < 0.001). Furthermore, every 1% increment in the extent of LGE was associated with an increased risk of all-cause mortality. Analysis of a subgroup revealed that the prognostic value varied based on different location and pattern of LGE. Additionally, we found that LGE was a stronger predictor of arrhythmic events in patients with greater left ventricular ejection fraction. LGE by CMR in patients with DCM exhibited a substantial value in predicting adverse outcomes, and the extent, location, and pattern of LGE could provide additional information for risk stratification.

Diagnosis and Outcome of Patients with Idiopathic Hypereosinophilic Syndrome and Cardiac Involvement

Introduction: Diagnostic criteria of idiopathic hypereosinophilic syndrome (iHES) comprise persisting reactive/non-clonal eosinophilia ≥1.5 G/l and associated organ infiltration/dysfunction. The pattern and extent of organ involvement is most relevant for symptoms, quality of life, complications, response to treatment and prognosis. Of note, the potentially life-threatening cardiac involvement (CI) is frequently underrecognized. Patients and Methods: Based on the ´German Registry on Disorders of Eosinophils and Mast Cells (GREM)', we sought to investigate CI in 62 patients (pts) with iHES and predominantly multifocal organ involvement (sinus [n=26], lungs [n=36, including asthma, n=23], abdominal organs [n=15], skin [n=15] and lymph nodes [n=9]) by cardiac magnetic resonance imaging (MRI, 62/62 pts), echocardiography (30/62 pts), myocardial biopsy (10/62 pts) and the cardiac biomarkers troponin I (TNI, 49/62 pts) and N-terminal prohormone B-type natriuretic peptide (NT-proBNP, 38/62 pts). Clonal eosinophilia, e.g. FIP1L1::PDGFRA, alternative tyrosine kinase fusion genes and point mutations, e.g. KIT D816V, was excluded by the combination of cytogenetic/molecular analyses, bone marrow histology and immunohistochemistry. Myeloid peroxidase (MPO)-ANCA antibodies as characteristic marker of eosinophilic granulomatosis with polyangiitis (EGPA) were absent in all 62/62 (100%) pts and no evaluable patient had histologic evidence of vasculitis. Results: Cardiac MRI was pathologic in 34/62 (55%) pts with overlapping presence of late gadolinium enhancement (LGE, 31/34, 91%), pericardial effusion (11/34, 32%), involvement of heart valves/conduction system (9/34, 26%), impaired left-ventricular ejection fraction (LVEF &amp;lt;50%, 9/34, 26%) and presence of intracavitary thrombus (6/34, 18%). Concomitant echocardiography revealed pathological findings only in 10/24 (42%) pts with a pathologic MRI. Myocardial biopsy unveiled fibrosis (4/10, 40%) or eosinophilic infiltration (2/10, 20%) but was also non-informative (4/10, 40%). CI was rather associated with involvement of lungs (24/34, 71%) and sinus (23/34, 68%) than of abdominal organs (8/34, 24%) and skin (5/34, 15%). The combination of CI and asthma, potentially suggestive of ANCA-negative EGPA, was observed in 16/34 (47%) pts, but an indistinguishable pattern of multifocal organ involvement was also present in 18/34 (53%) pts with CI and absence of asthma. All 10/49 (20%) pts with an elevated vs. 0/49 (0%) pts with a normal troponin I (&amp;lt;0.045µg/l) and 20/38 (53%) pts with an elevated vs. 7/38 (18%) pts with a normal NT-proBNP (&amp;lt;125ng/l) revealed a pathologic cardiac MRI (Table). During a median follow-up of 108 months (range 3-470), the occurrence of cardiac events (n=15; death, n=3; cerebral embolism, n=4; worsening of LVEF, n=2; arrhythmias, n=2; valve surgery, n=2; pericardial effusion, n=1; coronary artery aneurysm, n=1) was strongly associated with a pathologic cardiac MRI in 15/15 (100%) pts (prior to MRI, n=6; after MRI, n=9) and an elevated troponin I and/or elevated NT-proBNP in 14/15 (93%) or 12/15 (80%) pts, respectively. During a median follow-up of 7.6 years (range 0.3-29.2), glucocorticoid-based immunosuppressive treatment resulted in improved MRI findings in 9/20 (45%) pts (improved LGE, n=7; regression of thrombus, n=1; improved LVEF, n=1) and a median NT-proBNP decrease of 48%. In pts with a pathologic MRI and elevated biomarkers, 9/13 (69%) and 5/19 (26%) pts, respectively, had a normalization of troponin I or NT-proBNP. Conclusions: In pts with iHES, i) the incidence of CI is high, particularly in pts with multifocal organ involvement, ii) it is questionable whether absence or presence of asthma in pts with multiple organ involvement is qualified for the challenging differentiation between iHES and ANCA-negative EGPA, iii) CI should be assessed by troponin I/NT-proBNP and cardiac MRI as they are superior to echocardiography, iv) the combination of elevated troponin I and to a lesser extent also elevated NT-proBNP with a pathologic cardiac MRI is associated with retrospective and subsequent, potentially life threatening cardiac events, and v) cardiac biomarkers and cardiac MRI are useful for monitoring response to treatment.