Amount Of Late Gadolinium Enhancement Research Articles

Myocardial Fibrosis Quantification Methods by Cardiovascular Magnetic Resonance Imaging in Patients with Fabry Disease.

Background/Objectives: The presence of late gadolinium enhancement (LGE) on cardiac magnetic resonance (CMR) in patients with Fabry disease (FD) is a predictor of adverse cardiac events. The aim of this study was to establish the most reliable and reproducible technique for quantifying LGE in patients with FD. Methods: Twenty FD patients with LGE who underwent CMR on the same scanner and LGE sequence were included. LGE quantifications were done using gray-scale thresholds of 2, 3, 4, 5 and 6 standard deviations (SD) above the mean signal intensity of the remote myocardium, the full width at half maximum method (FWHM), visual assessment with threshold (VAT) and the fully manual method (MM). Results: The mean amount of fibrosis varied between quantification techniques from 36 ± 19 at 2SD to 2 ± 2 g using the FWHM (p < 0.0001). Intraobserver reliability was excellent for most methods, except for the FWHM which was good (ICC 0.84; all p < 0.05). Interobserver reliability was excellent for VAT (ICC 0.94) and good for other techniques (all p < 0.05). Intraobserver reproducibility showed the lowest coefficient of variation (CV, 6%) at 5SD and at 2SD and VAT (35% and 38%) for interobserver reproducibility. The FWHM revealed the highest CV (63% and 94%) for both intra- and interobserver reproducibility. Conclusions: The available methods for LGE quantification demonstrate good to excellent intra- and interobserver reproducibility in patients with FD. The most reliable and reproducible techniques were VAT and 5SD, whereas the FWHM was the least reliable in the setting of our study. The total amount of LGE varies strongly with the quantification technique used.

THE DIAGNOSTIC VALUE OF CARDIAC MRI IN DETERMINING HYPERTROPHIC CARDIOMYOPATHY PHENOTYPES Using T1 and T2 mapping and late gadolinium enhancement to accurately diagnose hypertrophic cardiomyopathy

Objective: Hypertrophic cardiomyopathy (HCM) is a genetic disorder characterized by thickening of the heart muscle and may have serious consequences. Diversity in the distribution and degree of hypertrophy has led to the classification of HCM types. Cardiac MR (CMR) imaging is a non-invasive tool that maintains accurate and detailed data about cardiac functions and structure. The aim of the study was to observe and compare the CMR characteristics of the three HCM morphologic types (asymmetric, concentric, and apical). Materials and Methods: 56 patients (female/male: 29/58, mean age 46.31±15.12, body surface area (BSA) 1.88±0.19 m2) who were referred for HCM diagnosis or follow-up between January 2020- January 2023 were included in this single-center retrospective study. The imaging procedure was conducted using a 1.5 T device (Aera, Siemens Medical Systems, Erlangen, Germany). Cardiac volume and functions were determined quantitatively. The location and amount of late gadolinium enhancement (LGE) was evaluated qualitatively. The statistical analyses were performed using SPSS version 25.0. A P value under 0.05 was considered statistically significant. Results: LGE revealed myocardial fibrosis in apical HCM (ApHCM), a higher prevalence than in asymmetric and concentric HCM. ApHCM and asymmetric HCM patients widely presented midmyocardial fibrosis, where most concentric HCM patients had patchy involvement. The mean native myocardial T1 values for individuals with concentric HCM was found to be higher compared to those with asymmetric and ApHCM and the healthy participants. Conclusion: CMR imaging enables the multiparametric evaluation of HCM through the utilization of native T1 and T2 mapping techniques, as well as LGE. Both T1 and T2 values have the potential to be used in conjunction with LGE to identify regions of myocardial fibrosis.

Abstract 18098: Prolonged Filtered QRS Duration on Signal Averaged Electrocardiogram is Associated With Structural Remodeling in Hypertrophic Cardiomyopathy

Background: Hypertrophic cardiomyopathy (HCM) is characterized by myocyte hypertrophy, electrical remodeling and fibrosis that lead to inhomogeneous ventricular depolarization and arrhythmias. Signal averaged ECG (SAECG) is useful for non-invasive risk stratification in ischemic cardiomyopathy. However, the association between SAECG and structural remodeling in HCM has not been investigated. Methods: We retrospectively studied 594 HCM patients from the Johns Hopkins HCM Registry, who underwent echocardiography and cardiac magnetic resonance (CMR) imaging within 12 months of SAECG or 12-lead ECG. Seventy-three patients underwent SAECG and were divided into 2 groups (Normal-SAECG, Abnormal-SAECG) based on filtered QRS duration and/or presence of late potentials . Results: Abnormal SAECG was seen in 50% of HCM patients (37/73). Of the 37 HCM patients with abnormal SAECG, 29 patients only had prolonged filtered QRS duration ( fQRS&gt; 114ms ), and 8 patients had prolonged fQRS duration as well as late potentials ( LAS40 &gt;38ms; RMS40 &lt;20μV). Mean fQRS and LAS40 were significantly higher in the Abnormal-SAECG group ( fQRS : 125.7 vs 107msec, p&lt;0.0001; LAS40 : 31.2 vs 22.9msec; p=0.002; RMS40 : 40 vs 54μV; p=0.07). HCM patients with abnormal SAECG also had 1) larger left atrial size (p=0.03), lower global LV peak systolic strain (p=0.04), systolic strain rate (p=0.008), early diastolic strain rate (p=0.01) by ECHO; 2) greater LV mass index (p=0.004) 3) Higher prevalence and amount of late gadolinium enhancement (LGE) reflecting LV-scar (p=0.045 and 0.01) by CMR; 4) higher prevalence of repolarization abnormalities on 12-lead ECG (p=0.02). Adverse outcomes (sustained-VT/VF, heart failure, death) were similar in the 2 groups. Filtered QRS duration (SAECG) was positively correlated with LV mass index (r=0.43, p=0.0002), QRS duration on 12-lead ECG (r=0.67, p=0.000), and negatively correlated with global LV early diastolic strain rate (r= -0.34, p=0.009) in our HCM cohort. Conclusion: In HCM, prolonged filtered QRS on SAECG reflects a greater degree of structural remodeling characterized by more LV hypertrophy, larger LA size, worse cardiac mechanics, and higher prevalence and amount of fibrosis.

Clinical and CMR characteristics associated with cardiac events in patients with Fabry disease

BackgroundThe assessment of late gadolinium enhancement (LGE) and left ventricular hypertrophy (LVH) by cardiac magnetic resonance (CMR) as diagnostic and prognostic maker in Fabry disease is advancing. We aimed to investigate the impact of clinical characteristics and CMR findings on cardiac outcome in patients with FD. MethodsIn this study 55 patients with genetically confirmed FD and available CMR imaging were included. The primary endpoint was defined as a composite of cardiac events including cardiac death, new occurrence of atrial fibrillation, heart failure, ventricular tachycardia and bradycardia requiring device insertion. ResultsDuring a median follow-up of 4.9 years (IQR 3.7–5.9), 9 patients (16.3%) reached the primary cardiac end point. The global amount of LGE was associated with an increased risk for primary endpoint in the univariate analysis (HR 1.4 per 10% increase in LGE, p = 0.002). However maximal wall thickness (MWT) was the sole independent predictor of the primary endpoint in a stepwise logistic regression model (HR 9.8 per mm increase in MWT, p < 0.0001). Kaplan-Meier analysis revealed significant difference in event free survival rate between patients with and without LVH (Long-rank p = 0.006) and in patients with and without LGE (Long-rank p < 0.001). Patients without LVH and LGE were free of adverse cardiac events. ConclusionLVH and LGE detected by CMR were associated with adverse cardiac events in FD. In particular maximal wall thickness can be useful in cardiac risk stratification of FD patients.

Abstract 13312: Can the Extent of Late Gadolinium Enhancement on Cardiac Magnetic Resonance Be Predicted by the 12-Lead Electrocardiogram?

Background: In hypertrophic cardiomyopathy (HCM), late gadolinium enhancement (LGE) on cardiac magnetic resonance imaging (CMR) is an in vivo marker of replacement fibrosis with a continuous relationship between amount of LGE and both risk for sudden death and development of endstage disease. Cost and accessibility can limit routine utilization of CMR. In contrast, electrocardiogram (ECG) is an inexpensive readily available test and identifies patients with myocardial scarring in ischemic heart disease. Its ability to identify presence and extent of LGE in HCM is unknown. Methods: 1983 consecutive HCM patients were included; 62% male, average wall thickness 18 ± 3mm, and 36% with resting LV outflow tract obstruction ≥ 30mmHg. ECGs were analyzed for LV hypertrophy, T wave abnormalities, ST-segment depression or elevations, abnormal Q waves, conduction disease, atrial enlargement and QTc prolongation. Extent of LGE was quantified and expressed as a proportion of total LV myocardium. Results: 822 (41%) had no LGE, while 1161 (59%) had LGE, including 687 (35%) with &lt;5%, 299 (15%) with 5-9%, 92 (5%) with 10-14%, and 83 (4%) with ≥ 15%. Compared to no LGE, Patients with LGE were more likely to have pathologic T wave inversions (55% vs. 36%, p&lt;0.01), positive T waves in aVR (34% vs. 20%, p&lt;0.01), and ST-depressions (29% vs. 21%, p&lt;0.01), with no difference in LV hypertrophy, bundle branch block, pathologic Q waves, first degree AV block, QTc prolongation ≥ 480ms, or ST-elevations (p&gt;0.05). Notably, as extent of LGE increased there was a corresponding progressive increase in the prevalence of both positive T waves in aVR (20% in no LGE to 42% in ≥ 15% LGE, p&lt;0.01) and pathologic T wave inversions (36% in no LGE to 61% in ≥ 15% LGE, p&lt;0.01) but with no incremental differences in ST depressions (p&gt;0.05). In contrast, 43% of patients with LGE did not have either positive T waves in aVR or pathologic T wave inversions, including 34% with ≥ 15% LGE. Conclusion: Most ECG findings including pathologic Q waves have no relation to presence or extent of LGE. While positive T waves in aVR and pathologic T wave inversions are associated with LGE, they have limited sensitivity. These finding support that routinely evaluated ECG abnormalities do not predict presence or extent of scarring in HCM.

Myocardial scar detection in free-breathing Dixon-based fat- and water-separated 3D inversion recovery late-gadolinium enhancement whole heart MRI

The aim of this study was to investigate the diagnostic accuracy and reader confidence for late-gadolinium enhancement (LGE) detection of a novel free-breathing, image-based navigated 3D whole-heart LGE sequence with fat–water separation, compared to a free-breathing motion-corrected 2D LGE sequence in patients with ischemic and non-ischemic cardiomyopathy. Cardiac MRI patients including the respective sequences were retrospectively included. Two independent, blinded readers rated image quality, depiction of segmental LGE and documented acquisition time, SNR, CNR and amount of LGE. Results were compared using the Friedman or the Kruskal–Wallis test. For LGE rating, a jackknife free-response receiver operating characteristic analysis was performed with a figure of merit (FOM) calculation. Forty-two patients were included, thirty-two were examined with a 1.5 T-scanner and ten patients with a 3 T-scanner. The mean acquisition time of the 2D sequence was significantly shorter compared to the 3D sequence (07:12 min vs. 09:24 min; p < 0.001). The 3D scan time was significantly shorter when performed at 3 T compared to 1.5 T (07:47 min vs. 09:50 min; p < 0.001). There were no differences regarding SNR, CNR or amount of LGE. 3D imaging had a significantly higher FOM (0.89 vs. 0.78; p < 0.001). Overall image quality ratings were similar, but 3D sequence ratings were higher for fine anatomical structures. Free-breathing motion-corrected 3D LGE with high isotropic resolution results in enhanced LGE-detection with higher confidence and better delineation of fine structures. The acquisition time for 3D imaging was longer, but may be reduced by performing on a 3 T-scanner.

Follow-up cardiac magnetic resonance in children with vaccine-associated myocarditis.

Myocarditis is a rare complication of the COVID-19 mRNA vaccine. We previously reported a case series of 15 adolescents with vaccine-associated myocarditis, 87% of whom had abnormalities on initial cardiac magnetic resonance (CMR), including late gadolinium enhancement (LGE) in 80%. We performed follow-up CMRs to determine the trajectory of myocardial recovery and better understand the natural history of vaccine-associated myocarditis. Case series of patients age < 19 years admitted to Boston Children’s Hospital with acute vaccine-associated myocarditis following the BNT162b2 vaccine who had abnormal CMR at the time of initial presentation, and underwent follow-up testing. CMR assessment included left ventricular (LV) ejection fraction, T2-weighted myocardial imaging, LV global native T1, LV global T2, extracellular volume (ECV), and late gadolinium enhancement (LGE). Ten patients (9 male, median age 15 years) with vaccine-associated myocarditis underwent follow-up CMR at a median of 92 days (range 76–119) after hospital discharge. LGE was persistent in 80% of patients, though improved from prior in all cases. Two patients (20%) had abnormal LV global T1 at presentation, which normalized on follow-up. ECV decreased between acute presentation and follow-up in 6/10 patients; it remained elevated at follow-up in 1 patient and borderline in 3 patients.Conclusion: CMR performed ~3 months after admission for COVID-19 vaccine-associated myocarditis showed improvement of LGE in all patients, but persistent in the majority. Follow-up CMR 6–12 months after acute episode should be considered to better understand the long-term cardiac risks. What is Known:• Myocarditis is a rare side effect of COVID-19 mRNA vaccine.•Late gadolinium enhancement is present on most cardiac magnetic resonance at the time of acute presentation.What is New:•Late gadolinium enhancement improved on all repeat cardiac magnetic resonance at 3-month follow-up.•Most patients still had a small amount of late gadolinium enhancement, the clinical significance of which is yet to be determined.Supplementary informationThe online version contains supplementary material available at 10.1007/s00431-022-04482-z.

The role of feature-tracking strain analysis in the differentiation of cardiac amyloidosis from hypertrophic cardiomyopathy

Abstract Cardiac magnetic resonance (CMR) examinations have an essential role in the differentiation of cardiac involvement of amyloidosis (CA) from hypertrophic cardiomyopathy (HCM). The CMR diagnosis is traditionally based on morphologic features and the pattern of late gadolinium enhancement (LGE). However, in patients contraindicated for contrast agent administration, the diagnosis can be challenging. Novel CMR techniques, including strain analysis, can help in the differentiation of these patients. The aim of our study was to investigate the differential diagnostic and prognostic importance of feature-tracking strain analysis in patients with left ventricular hypertrophy caused by CA or HCM. We investigated 89 HCM patients (48 males; 50±18 years) and 46 CA patients (29 males; 64±10 years) who underwent CMR examination. The left ventricular ejection fraction (LVEF), volumes (end-diastolic volume: LVEDV, end-systolic volume: LVESV, stroke volume: LVSV), mass (LVM), and the amount of LGE were quantified. Global longitudinal (GLS), circumferential (GCS) and radial (GRS) LV strain parameters, and basal, midventricular, and apical LS and CS were measured. The apex-to-base regional LS and CS ratios were calculated. The all-cause mortality of the patients was analyzed. The characteristics of groups were compared with an independent t-test or Mann-Whitney test, as appropriate. ROC curve analysis was performed to analyze the diagnostic accuracy of a parameter and to identify optimal cutoff values. The prognostic value of CMR parameters was assessed with Cox proportional hazard regression analyses. Survival probability was analyzed with Kaplan-Meier curves and compared by the log-rank test. CA patients had significantly lower LVEF (51±11 vs. 63±8%; p&amp;lt;0.0001), lower LVSVi (43±12 vs. 54±12 ml/m2; p&amp;lt;0.0001), higher LVMi (94±24 vs. 79±24 g/m2; p&amp;lt;0.001), higher amount of LGE (29±15 vs. 8±8%; p&amp;lt;0.0001), and more impaired global and regional strain values (GRS: 55±22 vs. 93±29%; GCS: −33±10 vs. −42±8%; GLS: −18±4 vs. −25±6%; basal CS: −26±9 vs. −39±7%; basal LS: −15±4 vs. −22±6%; p&amp;lt;0.0001) than HCM patients. The apex-to-base CS and LS ratios were higher in CA patients, suggestive of apical sparing (1.71±0.68 vs. 1.29±0.33; p&amp;lt;0.0001; 1.88±0.76 vs. 1.57±0.62; p&amp;lt;0.05; respectively). In the differentiation of CA and HCM, LGE, basal CS, basal LS, GRS, and GLS had the highest diagnostic accuracies (AUCs: 0.911, 0.866, 0.848, 0.859, 0.849). During the mean 2.1±2.0 years of follow-up, three HCM patients (3.4%) and 29 CA patients (63%) died (p&amp;lt;0.0001). The significant independent predictors of mortality were a diagnosis of CA, LVSVi and basal LS. Our results show that CMR-based strain analysis might be a useful method for differentiating cardiac involvement of amyloidosis from HCM. Furthermore, this technique provides additional information for the assessment of prognosis in this patient population. Funding Acknowledgement Type of funding sources: Public Institution(s). Main funding source(s): National Research, Development and Innovation Office of Hungary; Ministry for Innovation and Technology in Hungary, within the framework of the Therapeutic Development and Bioimaging programmes of the Semmelweis University

Myocardial fibrosis quantification methods by cardiovascular magnetic resonance imaging with contrast-enhancement in patients with Fabry disease

Abstract Funding Acknowledgements Type of funding sources: Public grant(s) – EU funding. Main funding source(s): National Agency for Academic Exchange (NAWA). Background. Presence and extent of late gadolinium enhancement (LGE) in patients with Fabry disease (FD) is a predictor of adverse cardiac events. However there is no gold standard method to quantify the amount of myocardial fibrosis in cardiac magnetic resonance imaging (CMR). Purpose. The aim of this study was to establish the most reliable and reproducible technique for quantifying LGE in patients with FD. Methods. 68 patients with FD (40% male, 40 ± 16 years old) treated in our outpatient clinic, who underwent CMR with administration of 0.2 mmol of gadobutrol per kilogram of body weight between December 2012 and March 2019 were enrolled into the study. Presence of LGE was described in 25 patients (37%). Twenty patients underwent CMR with the same LGE sequence (Philips 1.5 Tesla, two-dimensional multi-breath-hold inversion recovery sequence) and were enrolled into further analysis. LGE quantifications were performed using gray-scale thresholds with 2, 3, 4, 5 and 6 standard deviations (SD) above the mean signal intensity for the remote myocardial tissue, full width at half maximum method (FWHM), visual assessment with threshold (VAT) and fully manual method (MM; Philips IntelliSpace Portal 10). LGE quantifications were done twice by the same observer in different time periods and once by another observer. Intraclass correlation coefficient (ICC), Bland- Altman analysis and coefficient of variation (CV) were used to assess intra- and interobserver reliability and reproducibility. Results. The mean quantity of fibrosis mass (in gram) in all studied patients was: 35.5 ± 18.7 at 2SD, 21.0 ± 12.8 at 3SD, 12.7 ± 8.5 at 4SD, 8.0 ± 5.7 at 5SD, 5.3 ± 4.1 at 6SD, 1.9 ± 1.8 at FWHM, 8.6 ± 7.4 at VAT and 9.1 ± 6.1 at MM. Intra-observer reliability of almost all studied LGE quantification methods was excellent, with a range of ICCs from 0.90 for 6SD to 0.95 for VAT, with one exception for FWHM, which had good intraobserver reliability (ICC 0.84; all P &amp;lt; 0.05). Interobserver reliability was excellent for VAT (ICC 0.94) and good for all other LGE quantifications methods (range of ICCs from 0.76 for MM to 0.87 for 5SD, all P &amp;lt; 0.05). 5SD had the lowest CV (6%) for intraobserver reproducibility and 2SD and VAT for interobserver reproducibility (35% and 38%). FWHM had the highest CV for both intra- and interobserver reproducibility (63% and 94%, accordingly). Conclusions 1. All studied methods of LGE quantification in patients with FD and presence of myocardial fibrosis have good to excellent intra- and interobserver reliability. 2. The total amount of LGE differs in studied LGE quantifications methods. Therefore in clinical practice it is important to report which technique of LGE quantification was used and choose the same for CMR-follow up. 3. FWHM might be avoided for LGE quantification in patients with FD due to the highest intra- and interobserver variability in comparison to other available techniques.

Long-term risk of sudden cardiac death in hypertrophic cardiomyopathy - a cardiac magnetic resonance outcome study

Abstract Funding Acknowledgements Type of funding sources: Foundation. Main funding source(s): Robert Bosch Stiftung; Deutsche Forschungsgemeinschaft Background Sudden cardiac death (SCD) is an appalling complication of hypertrophic cardiomyopathy (HCM). There is an ongoing discussion about the optimal SCD risk stratification strategy in HCM since established SCD risk models have suboptimal discriminative power. Objective To evaluate the prognostic value of late gadolinium enhancement (LGE) cardiac magnetic resonance (CMR) for SCD risk stratification compared to the ESC SCD risk score and traditional SCD risk factors in an &amp;gt;10-year follow-up study. Methods 220 consecutive patients with HCM and LGE-CMR were enrolled. Follow-up data was available in 203 patients (median age 58 years, 61% male) after a median follow-up period of 10.4 years. Results LGE was present in 70% of patients with a median LGE amount of 1.6%, the median ESC 5-year SCD risk score was 1.84. In the overall cohort, SCD rates were 2.3% at 5 years, 4.8% at 10 years, and 15.7% at 15 years, independent from established risk models. A LGE amount of &amp;gt;5% (LV mass) portends the highest risk for SCD with SCD prevalences of 5.5% at 5 years, 13.0% at 10 years and 33.3% at 15 years. Conversely, patients with no or ≤5% LGE amount (of LV mass) have favorable prognosis. Conclusions LGE-CMR in HCM patients allows effective 10-year SCD risk stratification beyond established risk factors. LGE amount might be added to established risk models to improve its discriminatory power. Specifically, patients with &amp;gt;5% amount of LGE should be carefully monitored and might be adequate candidates for primary prevention ICD during the clinical long-term course. Abstract Figure.

Contribution of sarcomere gene mutations to left atrial function in patients with hypertrophic cardiomyopathy

BackgroundLeft atrial (LA) enlargement and dysfunction are related to clinical course in patients with hypertrophic cardiomyopathy (HCM). We aimed to investigate genetic contribution to LA structural and functional remodeling.MethodsTwo hundred twelve patients were consecutively enrolled, and echocardiography and extensive genetic analysis were performed. Cardiac magnetic resonance (CMR) was performed in 135 patients. Echocardiography was also performed in controls (n = 30).ResultsPatients with HCM had lower late-diastolic mitral annular velocity (a’) and higher LA volume index (LAVI) than controls. Patients with pathogenic or likely pathogenic sarcomere gene mutations (PSM, n = 67, 32%) had higher LAVI and lower CMR-derived LA total emptying fraction (37.0 ± 18.5 vs. 44.2 ± 12.4%, p = 0.025). In patients without AF (n = 187), the PSM had lower a’ (6.9 ± 2.0 vs. 7.8 ± 1.9 cm/s, p = 0.004) than others. The PSM had higher prevalence and amount of late gadolinium enhancement (LGE) in the left ventricle (LV). In multivariate analysis, PSM was significantly related to lower a’ independent of E/e’, LV mass index, and LAVI. However, the relation significantly attenuated after adjustment for the extent of LGE in the LV, suggesting common myopathy in the LV and LA. In addition, PSM was significantly related to lower LA total emptying fraction independent of age, E/e’, s’, LV ejection fraction, LV myocardial global longitudinal strain and %LGE mass.ConclusionsPSM was related to LA dysfunction independent of LV filling pressure and LAVI, suggesting its contribution to atrial myopathy in HCM.

321 Differentiating Myocarditis from Myocardial Infarction in Infarct-Like Presentations Using Troponin and Cardiac MRI

Infarct-like presentations are usually well differentiated on cardiac MRI by the pattern of late gadolinium enhancement (LGE), but transmural LGE can occur. The aim of this study is to determine if there is a difference in the relationship between the peak troponin and the amount of LGE in acute myocarditis and acute myocardial infarction in infarct-like presentations. If so, we aim to define a ratio that can be used as an objective differentiator. 111 patients across two sites were included as they had an infarct-like presentation, troponin rise and cardiac MRI between 2010 and 2018. CVI42 was used for MRI tissue characterisation. Of the 51 patients with myocarditis, 36 (59%) were male and the mean peak troponin I was 10872 ng/L. Of the 61 patients with a myocardial infarction, 47 (77%) were male and the mean peak troponin I was 74133 ng/L. Preliminary results from the analysis of 26 patients showed the LGE mass was significantly greater in the infarct group compared to the myocarditis group (23.8 vs 9.8 grams, p=0.02). There was also a significant greater ratio of peak troponin to LGE mass in the infarct group compared with the myocarditis group (mean ratio 4306:1 vs 557:1, p=0.01). Preliminary results suggest there is a significantly greater peak troponin for the same amount of LGE in infarction compared with myocarditis. Further analysis is underway to confirm these findings over the larger sample size, and validate an objective cut off to differentiate myocarditis from infarction.

P1832 T1 mapping and echo-global longitudinal strain are early markers of cardiac involvement in patients with fabry disease

Abstract OnBehalf Constantin Gatterer Introduction Fabry disease (FD) is one of the most common lysosomal storage diseases caused by deficiency of Alpha-Galactosidase A and accumulation of glycosphingolipids in all cells containing lysosomes. Cardiac involvement is characterized by left ventricular hypertrophy, conduction abnormalities and myocardial fibrosis with consecutive cardiomyopathy in late stages. Purpose As the detection of early signs of cardiac involvement is crucial for the initiation of enzyme replacement therapy (ERT) or Chaperon therapy, we intended to find early imaging markers. Methods Cardiac MRI (CMR) including Late Gadolinium Enhancement (LGE), representing fibrosis, and T1-mapping as well as echocardiography with measurement of global longitudinal strain (GLS) were done as part of the regular check-ups for patients with FD. Results The study cohort of 30 FD patients (20-69 years, 53% women) in different stages of disease showed low GLS values already at the time of baseline echocardiography (mean: -17,22%), correlating with the amount of LGE (r = 0,73; p = 0,003) and ejection fraction measured by CMR (CMR-EF; r=-0,74; p = 0,002). After an average follow-up of 39 months (STD 18), GLS values were significantly declined (-15,51 %; p = 0,009) and correlated with T1 times (r = 0,7; p = 0,04), while LGE and CMR-EF did not significantly change compared to baseline. Baseline T1 times correlated negatively with the reduction of GLS (r=-0,7; p = 0,003), while LGE and CMR-EF showed no correlation with the course of GLS. ERT appeared to have no influence on the extend of GLS reduction. Conclusion Low native CMR T1 times, indicating sphingolipid accumulation in the myocardium, seem to represent the beginning of cardiac involvement in FD and might predict the course of GLS. GLS is a sensitive parameter for early detection of cardiac manifestation and disease progression, while LGE and CMR-EF could not recognize slight deterioration of left ventricular function.

P1772Diagnostic accuracy of a novel electrocardiographic criterion for the diagnosis of left ventricular hypertrophy in hypertrophic cardiomyopathy

Abstract Introduction The 12-lead electrocardiogram (ECG) is a fundamental initial diagnostic modality for the early evaluation of a patient suspected of having hypertrophic cardiomyopathy (HCM). ECG criteria for the diagnosis of left ventricular hypertrophy (LVH) typically have low sensitivity and high specificity. Recently, a novel ECG criterion (Peguero-Lo Presti, PLP) with higher sensitivity (62%) and similar specificity (90%) was developed in a cohort of hypertensive patients, but its accuracy in patients with HCM has not been tested. We hypothesized that Peguero-Lo Presti criterion would improve upon the sensitivity of other criteria, while maintaining high specificity, for the diagnosis of LVH in patients for with HCM. Methods We retrospectively analyzed 215 consecutive patients who underwent cardiac magnetic resonance (CMR) between 2010 and 2018 for suspected HCM. All patients aged 18 years or older, who had CMR-confirmed HCM and an ECG without confounders (complete left or right bundle brunch block or paced ventricular rhythm) were included for analysis (n=88). Left ventricular mass (LVM) index and maximum wall thickness were derived from CMR analysis. The PLP criteria was defined as the sum of the deepest S wave (SD) in any lead and the S wave amplitude of lead V4 (SV4). Cornell voltage (CL) and Sokolow-Lyon (SL) were used for comparison. We randomly selected 88 gender-matched patients who performed an ECG and CMR for other clinical reasons and who had no structural heart disease or LVH for use as controls. The DeLong and McNemar's test were used to compare ROC area under the curve (AUC) and sensitivity and specificity, respectively, between the three criteria. Results 88 patients with HCM (63% male, mean age 56.7±15 years) were analyzed. The mean maximum wall thickness was 19.9±4.4mm and mean indexed LVM was 89.7±27g/m2. 34 patients (38.6%) had increased indexed LVM and 77 (87.5%) had at least one segment with late gadolinium enhancement (LGE). Discrimination by AUC was highest for PLP (0.85 [95% CI 0.8–0.9]), compared to CL (0.79, p=0.03) and SL (0.73, p=0.02). Using literature cut-offs, the sensitivity of PLP (60% [95% CI 50–70%]) was significantly higher compared to CL (40% [95% CI 30–50%, p&lt;0,001) and SL (41%, [95% CI 31–51%], p=0.01), whilst maintaining high specificity (PLP 96%; CL 98%; SL 94%). After adjusting for LVM, the amount of LGE had a positive correlation with PLP amplitude (Spearman's rho=0.6, coef=2.4, p=0.01), but not Cornell or Sokolow. The sensitivity of PLP was significantly higher than CL and Sokolow in patients with LGE (61% vs 44% vs 43%, p&lt;0.05). Conclusion The Peguero-Lo Presti criteria demonstrated higher sensitivity and similar specificity when compared to the Cornell and Sokolow-Lyon criteria for the diagnosis of LVH in a cohort of patients with hypertrophic cardiomyopathy. Therefore, they could become the standard ECG diagnostic criteria in patients suspected of having LVH and HCM.

Agreement between gadolinium-enhanced cardiac magnetic resonance and electro-anatomical maps in patients with non-ischaemic dilated cardiomyopathy and ventricular arrhythmias.

We sought to investigate the overlap between late gadolinium enhancement (LGE) in cardiac magnetic resonance (CMR) and electro-anatomical maps (EAM) of patients with non-ischaemic dilated cardiomyopathy (NIDCM) and how it relates with the outcomes after catheter ablation of ventricular arrhythmias (VA). We identified 50 patients with NIDCM who received CMR and ablation for VA. Late gadolinium enhancement was detected in 16 (32%) patients, mostly in those presenting with sustained ventricular tachycardia (VT): 15 patients. Low-voltage areas (<1.5 mV) were observed in 23 (46%) cases; in 7 (14%) cases without evidence of LGE. Using a threshold of 1.5 mV, a good and partially good agreement between the bipolar EAM and LGE-CMR was observed in only 4 (8%) and 9 (18%) patients, respectively. With further adjustments of EAM to match the LGE, we defined new cut-off limits of median 1.5 and 5 mV for bipolar and unipolar maps, respectively. Most VT exits (12 out of 16 patients) were found in areas with LGE. VT exits were found in segments without LGE in two patients with VT recurrence as well as in two patients without recurrence, P = 0.77. In patients with VT recurrence, the LGE volume was significantly larger than in those without recurrence: 12% ± 5.8% vs. 6.9% ± 3.4%; P = 0.049. In NIDCM, the agreement between LGE and bipolar EAM was fairly poor but can be improved with adjustment of the thresholds for EAM according to the amount of LGE. The outcomes were related to the volume of LGE.

Comparison on CMR characteristics and clinical prognosis between hypertrophic cardiomyopathy patients with and without left ventricular apical aneurysms

Objective: To compare the imaging characteristics and long-term prognosis in hypertrophic cardiomyopathy(HCM) patients with or without left ventricular apical aneurysm(LVAA). Methods: Retrospectively analyzed the clinical data from 18 patients diagnosed as HCM complicating with LVAA(HCM-LVAA group), hospitalized and underwent cardiac magentic resonance (CMR) examination in Fuwai Hospital between December 2012 and December 2016. Eighteen age and gender matched patients with HCM diagnosed by CMR served as control(HCM group). Outpatient and in-hospital clinical data as well as follow up results were compared. The major adverse cardiovascular events were defined as malignant arrhythmia events (including sudden cardiac death, ventricular flutter/ventricular fibrillation) and heart failure events (including heart transplantation, progressive heart failure). Results: Compared with HCM group, patients in HCM-LVAA group had a more positive family history of HCM(P=0.04), higher incidence of ST-T segment changes and abnormal Q wave in electrocardiograms (both P<0.01), the CMR derived left ventricular end-diastolic transverse diameter and end-diastolic volume index were also significantly higher (both P<0.05), and delayed enhancement was more significant ((25.26±10.60)% vs. (15.78±7.33)%, t=3.12, P=0.004) in HCM-LVAA group. Moreover, the left ventricular ejection fraction ((54.4±10.6)% vs. (67.5±7.6)%, t=-4.28, P<0.000 1) and the thickness of the apical wall ((3.11±1.05) mm vs. (5.46±1.94) mm, t=-4.49, P<0.000 1) were significantly lower in HCM-LVAA group than in HCM group. The mean follow-up duration was (3.46±1.64) years, 4 patients in HCM-LVAA group (22.2%) developed 4 cardiovascular events, including 1 sudden cardiac death, 3 progressive heart failures. One patient in HCM group developed progressive heart failure. Conclusion: The prognosis of the HCM complicating with LVAA patients is worse than that of HCM patients without LVAA, and the amount of late gadolinium enhancement is higher than that of HCM patients without LVAA.

PHASE ANALYSIS USING LOW DOSE 99M TC GATED-SPECT DETECTS SIGNIFICANT LATE GADOLINIUM ENHANCEMENT IN PATIENTS WITH NON-ISCHEMIC DILATED CARDIOMYOPATHY

Previous reports demonstrated that higher amount of late gadolinium enhancement (LGE), especially over 5%, of cardiac magnetic resonance (CMR) causes poor clinical outcomes for non-ischemic dilated cardiomyopathy (NIDCM); however patients with renal failure or with pacemaker device are

The amount of late gadolinium enhancement outperforms current guideline-recommended criteria in the identification of patients with hypertrophic cardiomyopathy at risk of sudden cardiac death

BackgroundIdentifying the patients with hypertrophic cardiomyopathy (HCM) in whom the risk of sudden cardiac death (SCD) justifies the implantation of a cardioverter-defibrillator (ICD) in primary prevention remains challenging. Different risk stratification and criteria are used by the European and American guidelines in this setting. We sought to evaluate the role of cardiovascular magnetic resonance (CMR) late gadolinium enhancement (LGE) in improving these risk stratification strategies.MethodsWe conducted a multicentric retrospective analysis of HCM patients who underwent CMR for diagnostic confirmation and/or risk stratification. Eligibility for ICD was assessed according to the HCM Risk-SCD score and the American College of Cardiology Foundation/American Heart Association (ACCF/AHA) algorithm. The amount of LGE was quantified (LGE%) and categorized as 0%, 0.1–10%, 10.1–19.9% and ≥ 20%. The primary endpoint was a composite of SCD, aborted SCD, sustained ventricular tachycardia (VT), or appropriate ICD discharge.ResultsA total of 493 patients were available for analysis (58% male, median age 46 years). LGE was present in 79% of patients, with a median LGE% of 2.9% (IQR 0.4–8.4%). The concordance between risk assessment by the HCM Risk-SCD, ACCF/AHA and LGE was relatively weak. During a median follow-up of 3.4 years (IQR 1.5–6.8 years), 23 patients experienced an event (12 SCDs, 6 appropriate ICD discharges and 5 sustained VTs). The amount of LGE was the only independent predictor of outcome (adjusted HR: 1.08; 95% CI: 1.04–1.12; p < 0.001) after adjustment for the HCM Risk-SCD and ACCF/AHA criteria. The amount of LGE showed greater discriminative power (C-statistic 0.84; 95% CI: 0.76–0.91) than the ACCF/AHA (C-statistic 0.61; 95% CI: 0.49–0.72; p for comparison < 0.001) and the HCM Risk-SCD (C-statistic 0.68; 95% CI: 0.59–0.78; p for comparison = 0.006). LGE was able to increase the discriminative power of the ACCF/AHA and HCM Risk-SCD criteria, with net reclassification improvements of 0.36 (p = 0.021) and 0.43 (p = 0.011), respectively.ConclusionsThe amount of LGE seems to outperform the HCM Risk-SCD score and the ACCF/AHA algorithm in the identification of HCM patients at increased risk of SCD and reclassifies a relevant proportion of patients.

Late gadolinium enhancement and adverse outcomes in a contemporary cohort of adult survivors of tetralogy of Fallot.

Myocardial fibrosis has been associated with poorer outcomes in tetralogy of Fallot, however only a handful of studies have assessed its significance in the current era. Our aim was to quantify the amount of late gadolinium enhancement in both the LV and RV in a contemporary cohort of adults with surgically repaired tetralogy of Fallot, and assess the relationship with adverse clinical outcomes. Single centre cohort study SETTING: National tertiary referral center Patients: One hundred fourteen patients with surgically repaired tetralogy of Fallot with median age 29.5 years (range 17.5-64.2). Prospective follow-up for mean 2.4 years (SD 1.29). Cardiovascular magnetic resonance was performed, and late gadolinium enhancement mass was estimated for the LV using the 5-SD remote myocardium method, and for the RV using a segmental scoring system. Cohort characterization was determined through the use of a computerized database. Survival analysis from time of scan to first adverse event, defined as an episode of atrial arrhythmia, sustained ventricular arrhythmia, hospitalization with heart failure, or implantable cardioverter-defibrillator insertion. Eleven patients experienced an adverse outcome in the follow-up period, although there were no deaths. LV late gadolinium enhancement was associated with adverse outcomes in a univariate model (P = .027). However, when adjusted for age at scan the significant variables included NYHA class (P = .006), peak oxygen uptake (P = .028), number of prior sternotomies (P = .044), and higher indexed RV and LV end diastolic volumes (P = .002 and P < .001), but not RV or LV late gadolinium enhancement. Formal quantification of late gadolinium enhancement is not currently as helpful in ascertaining prognosis compared to other, more easily assessed parameters in a contemporary cohort of tetralogy of Fallot survivors, however assessment particularly of the LV holds promise for the future.

Detection of the mutation may guide treatment of heart and muscle in Duchenne muscular dystrophy.

We read with great interest the article, by Kono et al, about a 32-year-old male with Duchenne muscular dystrophy (DMD), who was admitted for dilated cardiomyopathy manifesting as heart failure, left bundle branch block, Mobitz-II block, bradycardia, and arterial hypotension. He profited from implantation of a cardiac resynchroniza-tion therapy-D system with a defibrillator and beta-blocker treatment. 1 We have the following comments and concerns. Diagnosing DMD only by muscle biopsy is insufficient. The diagnosis needs to be confirmed by genetic studies. 2 Was the patient investigated for deletions, duplications , or point mutations in the DMD gene? Which were the results of the Western blot for dystrophin? Was there complete absence of dystrophin or were there indications for a truncated protein, compatible with the diagnosis of Becker muscular dystrophy (BMD)? Since the patient was quite old for DMD, it has to be convincingly shown that BMD was definitively excluded. Diagnosing DMD on a molecular genetic level is important as genetic therapy in form of exon skipping is available for a certain type of mutations. 3 It has been shown that patients with DMD profit from the administration of ste-roids, usually deflazacort. 4 There are indications that steroids not only improve the performance of the skeletal muscle but also improve cardiac functions. 5 Furthermore, steroids reduce the myocardial fibrosis burden in these patients as has been shown by cardiac magnetic resonance imaging (MRI). 6 Did the presented patient receive steroids in addition to heart failure therapy? Did he profit from steroids with regard to cardiac and muscle function? Which side effects did he develop from steroids? Dilated cardiomyopathy in DMD is characterized by replacement of the myocar-dium with connective tissue. 6 Myocardial fibrosis in DMD can be best documented by application of cardiac MRI and administration of gadolinium to assess the amount of late gadolinium enhancement (LGE). 6 Did the patient undergo cardiac MRI and was gadolinium administered to assess the amount and location of myocardial fibrosis? Assessment of LGE in DMD is important since the amount of LGE strongly correlates with left ventricular systolic dysfunction. 6 Approximately two-thirds of the DMD patients inherit the mutation from their mothers. DMD carriers may manifest clinically or biochemically. Was the mother of the described patient investigated for her carrier status? Did she manifest clinically or were there other indications for being a DMD carrier? Did she undergo electrocardiography