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Aims Arrhythmogenic right ventricular cardiomyopathy (ARVC) is associated with a high risk of sudden cardiac death. Three different prediction models for the indication of implanted cardioverter defibrillator (ICD) are now available: the 5 year ARVC risk score, the International Task Force Consensus (ITFC) criteria, and the Heart Rhythm Society (HRS) criteria. We compared these three prediction models in a validation cohort of patients with definite ARVC. Methods and results In a cohort of 140 patients with definite ARVC, the 5 year ARVC risk score and the ITFC and HRS criteria were compared for the prediction of a major combined endpoint of sudden cardiac death, appropriate ICD intervention, resuscitated cardiac arrest, and sustained ventricular tachycardia. During the follow‐up, 65 major events occurred. The 5 year ARVC risk score with a threshold >10%, derived from the maximally selected rank statistic, predicted 62 (95%) events [odds ratio (OR) 9.1, 95% confidence interval (CI) 2.6–32, P = 0.0006], the ITFC criteria 53 (81%, OR 4.8, 95% CI 2.2–10.3, P = 0.0001), and the HRS criteria 29 (45%, OR 4.2, 95% CI 1.9–9.3, P = 0.0003). At the analysis of decision curve for ICD implantation, a 5 year ARVC risk score >10% showed a greater net benefit than the ITFC and HRS criteria over a wide range of threshold probability of events. Finally, at multivariate analysis, the 5 year ARVC risk score >10% was the only independent predictor of major events. Conclusions The 5 year score with a threshold of >10% was more effective for predicting events than the ITFC and HRS criteria.

Funding Acknowledgements Type of funding sources: None. Background. Native T1 values are usually assessed in the end-diastole to minimize motion artifacts while the systolic data acquisition offers the advantage of a thicker myocardium, with reduced partial-volume effects. Higher myocardial T1 values have been detected in diastole at both 1.5T and 3T but the dependence of this difference on myocardial segments or gender has not been fully explored. Aim. We provided a systematic comparison of myocardial native T1 values in diastole and systole, by considering separately myocardial segments and dividing males and females. Methods. Sixty-one healthy subjects (46.0 ± 14.1 years, 32 males) underwent CMR at 1.5T (Signa Artist; GE Healthcare). Three short-axis slices of the left ventricle acquired in diastole and systole using a Modified Look–Locker Inversion Recovery sequence. Image analysis was performed with a commercially available software package. T1 value was assessed in all 16 myocardial segments and global value was the mean. Results. Table 1 shows the comparison between T1 values calculated from maps obtained in diastole and systole. Systolic T1 values were significantly lower in the basal anterolateral segment, in all medium segments except for the medium inferior segment, and in all apical segments. The percentage difference between diastolic and systolic T1 values was considered to compensate for the higher T1 values in females, and a significantly higher value was detected in females for the majority of medium segments, for all apical segments, and for the global value. Conclusion. The diastolic-systolic discrepancy was more pronounced for the females and at the apical level, supporting the hypothesis that, besides the physiologic variations in myocardial blood volume during the cardiac cycle, the partial volume-effect may be a strong additional contributing factor. Native T1 values should be obtained always in the same cardiac phase to avoid a potential bias in the discrimination between healthy and pathologically affected myocardium.

Funding Acknowledgements Type of funding sources: None. Introduction. No data are available in literature about normal ranges for T2 in human myocardium using GE scanners. Aims. Our aims were to obtain myocardial regional and global T2 values as a reference for normality for the first time using a GE scanner and to assess their association with physiological variables. Methods. A stratified approach was adopted for healthy volunteers recruitment, ensuring the presence of 10 participants for both genders in each age decile: 20–30, 30–40, 40-50, 50-60, 60-70 years. Basal, medium, and apical short-axis slices of the left ventricle were acquired by a multi-echo fast-spin-echo (MEFSE) sequence. Image analysis was performed with a commercially available software package. T2 value was assessed in all 16 myocardial segments and global value was the mean. Results. The global T2 value averaged across all subjects was 52.2 ± 2.5 ms (range: 47.0-59.9 ms). Inter-study, intra-observer, and inter-observer reproducibility was good (coefficient of variation The 3.6% of segments was excluded because of artifacts and/or partial-volume effects. Segmental T2 values differed significantly (P Aging was associated with increased segmental and global T2 values. Females showed higher T2 values than males. T2 values were not correlated to heart rate. Mean T2 values, standard deviation, and lower and upper limits of normal for all 16 myocardial segments are shown in Figure 1 for males and in Figure 2 for females, considering separately each age group. Conclusion. The optimized MEFSE sequence allows for robust, reliable, and reproducible quantification of segmental T2 values. T2 values differ among myocardial slices and are influenced by age and gender, making mandatory to define gender- and age-specific segmental reference values for distinguishing between healthy and diseased myocardium. The normal ranges defined in this study on a large cohort of healthy subjects could be used as reference by other sites using the same sequence, allowing them to recruit a smaller population and accelerating the spread of myocardial T2 mapping in the clinical arena.

Funding Acknowledgements Type of funding sources: None. Background Heart disease is a major determinant of prognosis in type 1 myotonic dystrophy (DM1), second only to respiratory complications. Cardiac imaging, possibly including cardiac magnetic resonance (CMR), is recommended in patients with DM1. However, limited information is available on CMR findings and their prognostic significance in DM1. Methods We identified all patients with DM1 evaluated from 2009 to 2020 in a CMR laboratory with an established collaboration with a Neuromuscular Disorder Unit. Results Thirty-four patients were retrieved (21 males, aged 45 ± 12). At the time of CMR examination, 97% had neuromuscular symptoms (mean duration 16 ± 13 years), 12 (35%) presented with atrioventricular block (n = 11 1st degree, n = 1 2nd degree type 1), 15 (44%) with intraventricular conduction disturbances (n = 5 left bundle branch block, n = 5 right bundle branch block, n = 3 left anterior fascicular block, n = 2 other non-specific intraventricular conduction delay), 4 (12%) with atrial fibrillation or flutter. No patient had a device. At CMR, 5 (15%) patients had left ventricular (LV) systolic dysfunction (LV ejection fraction [LVEF] Conclusions Patients with DM1 display several structural and functional cardiac abnormalities, with variable degrees of cardiac muscle hypotrophy, fibrosis and fatty infiltration. The possibility to predict the need for pacemaker implantation, ventricular arrhythmias and all-cause or cardiovascular mortality should be verified in larger cohorts.

Background Left ventricular hypertrophy (LVH) may be due to different causes, ranging from, benign secondary forms (athlete's heart) to severe prognosis cardiomyopathies (i.e. cardiac amyloidosis). Early and accurate differential diagnosis is important to proper patient management. LVH may be detected by echocardiography signs of hypertrophy or other abnormalities often associated to hypertrophic phenotypes. Cardiac magnetic resonance (CMR) is often used to confirm the initial diagnostic suspicion. On the best of our knowledge, there are no study specifically designed to evaluate the final impact of CMR in changing or confirming the initial diagnostic echocardiographic suspicion. Aim To evaluate the clinical prognostic correlates of CMR in patients with echocardiographic or ECG suspicion of LVH (or cardiomyopathies with hypertrophic phenotype). Methods and results We enrolled 275 pts with echocardiographic evidence of LVH. Using current guidelines, the initial echocardiographic diagnostic suspicion was: hypertrophic cardiomyopathy (HCM) in 46.9% of pts; cardiac amyloidosis in 14.5%; hypertensive LVH in 17%; aortic stenosis in 1.5%; athlete's heart in 0.3%; undetermined LVH in 17%. CMR changed the diagnosis in 42% cases: the diagnosis of HCM increased from 44% to 72% of pts; hypertensive and undetermined LVH decreased significantly (respectively to 4% and 5%). Finally, the change in diagnostic suspicion was associated to reclassification of risk of patients: Kaplan-Meier curves demonstrated that HCM and cardiac amyloidosis had worst prognosis than undetermined or hypertensive LVH. Conclusions CMR changed the echocardiographic suspicion in almost half of patients with LVH. This study highlights the indication of CMR in patient with ECG or echocardiographic suspicion of LVH. Kaplan-Meier curves Funding Acknowledgement Type of funding source: None

The use of cocaine constitutes a major health problem. Cocaine use is associated with acute and chronic complications that might involve any system, the most common being the cardiovascular system. The precise incidence of cocaine-induced cardiomyopathy remains mysterious and probably underreported. Cocaine use should be considered in young patients presenting with chest pain or heart failure without other underlying risk factors. Cocaine-related cardiovascular complications can be acute or chronic and include ischemic and non-ischemic events. Frequent cocaine users have a seven-fold higher risk of myocardial infarction. In addition to its ischemic effects, other cardiovascular complications of cocaine use and abuse are hypertensive crises, aortic dissection or aortic rupture, cerebral hemorrhage, arrhythmias and sudden cardiac death, myocarditis, dilated cardiomyopathy, heart failure, and endocarditis. The mechanism of cocaine’s cardiovascular toxicity relates to its sympathomimetic effect, to the block of voltage-dependent K+ and Na2+ channels, and a hypersensitivity reaction to drug or contaminants, such as amphetamine, sugars, or talc. Cardiac magnetic resonance (CMR) can provide a valuable assessment of cocaine-induced myocardial damage both in acute and chronic cardiac complications: it gives prognostic information in clinically relevant settings, and it identifies silent myocardial damage in asymptomatic patients. Indeed, CMR study should be considered in symptomatic cocaine users to assess the extent and evolution of myocardial injury. Furthermore, it was suggested to repeat CMR after 4–8 months of appropriate management to evaluate myocardial response to abstinence and medical therapy.

We sought to evaluate the prognostic role of cardiac magnetic resonance (CMR) in patients with definite, borderline and possible diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC) as defined by the International Task Force (TF) in 2010. CMR was performed in 175 patients: 52 with definite, 50 with borderline and 73 possible ARVC. Abnormal-CMR was defined as the presence of ≥1 CMR abnormalities (including abnormalities of right ventricular and left ventricular wall motion, fat infiltration, late gadolinium enhancement, dilation and dysfunction of either ventricles). During the follow-up time 35 patients had hard cardiac events (sudden cardiac death, appropriate implantable cardioverter defibrillator shock and resuscitated cardiac arrest), and 34 of them occurred in patients with abnormal-CMR (negative predictive value = 96.9%). At the multivariate Cox-regression analysis LV involvement at CMR (fat infiltration and/or late gadolinium enhancement), and episode of nonsustained ventricular tachycardia (NSVT) were independent predictors of cardiac events in both the whole population (LV involvement: HR 3.69, 95% CI 1.57-8.65, p = 0.0002; NSVT: HR 5.8, 95% CI 2.82-11.9, p < 0.0001), and in the group of patients with definite ARVC (LV involvement: HR 3.03, 95% CI 1.15 to 8.02, p = 0.02; NSVT: HR 12.1, 95% CI 4.02-36.5, p < 0.0001). In conclusion, CMR evidence of LV involvement is a strong independent predictor of cardiac events in patients with definite, borderline or possible ARVC diagnosis. Abnormal CMR has very high negative predictive value for hard cardiac events

We thank Dr. Corrado and colleagues for the letter regarding our study ([1][1]). We enrolled patients with definite diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC) based on 2010 Task Force criteria ([1][1]). As previously described, we found that a relevant percentage of patients

Background The variation between rest and peak stress end-systolic pressure-volume relation is an afterload-independent index of left ventricular contractility. This index is easily obtained during routine stress echocardiography but can be derived also during a stress cardiovascular magnetic resonance (CMR) exam, that is the gold standard for the quantification of biventricular volumes. Purpose The aim of this study was to assess for the first time the prognostic value ofdelta rest-stress ESPVR (DESPVR) by dipyridamole stress-CMR in patients with known or suspected coronary artery disease (CAD). Methods One hundred and sixty-six consecutive patients (37 females, main age 61.96 ± 10.05 years) who underwent dipyridamole stress-CMR in a high volume CMR Laboratory were considered. Abnormal wall motion and perfusion at rest and after dipyridamole were analysed. Macroscopic myocardial fibrosis was detected by the late gadolinium enhancement (LGE) technique. The ESPVR was evaluated at rest and peak stress from raw measurement of systolic arterial pressure by cuff sphygmomanometer and end-systolic volume by biplane Simpson method. Results An abnormal stress CMR was found in 39 (23.5%) patients; 24 patients had a reversible stress perfusion defect in at least one myocardial segment and 15 a reversible stress perfusion defect plus worsening of stress wall motion in comparison with rest. Myocardial fibrosis was detected in 69 patients (41.6%). A DESPVR During a median follow up of 55.51 months (IQ range 33.20 months), 54 patients (32.5%) experienced major cardiac events: 5 deaths, 2 ventricular arrhythmias, 18 coronary syndromes, and 29 heart failure hospitalization.Reversible perfusion deficit, DESPVR Conclusions DESPRV assessed by CMR provides a prognostic stratification in patients with known or suspected coronary artery disease, in addition to that supplied by reversible perfusion deficit and diabetes. Abstract 1177 Figure 1

Background: Cardiac magnetic resonance (CMR) is widely used to assess tissue and functional abnormalities in arrhythmogenic right ventricular cardiomyopathy (ARVC). Recently, a ARVC risk score was proposed to predict the 5-year risk of malignant ventricular arrhythmias in patients with ARVC. However, CMR features such as fibrosis, fat infiltration, and left ventricular (LV) involvement were not considered. Objectives: The authors sought to evaluate the prognostic role of CMR phenotype in patients with definite ARVC and to evaluate the effectiveness of the novel 5-year ARVC risk score to predict cardiac events in different CMR presentations. Methods: A total of 140 patients with definite ARVC were enrolled (mean age 42 ± 17 years, 97 males) in this multicenter prospective registry. As per study design, CMR was performed in all the patients at enrollment. The novel 5-year ARVC risk score was retrospectively calculated using the patient's characteristics at the time of enrollment. During a median follow-up of 5 years (2 to 8 years), the combined endpoint of sudden cardiac death, appropriate implantable cardioverter-defibrillator intervention, and aborted cardiac arrest was considered. Results: CMR was completely negative in 14 patients (10%), isolated right ventricular (RV) involvement was found in 58 (41%), biventricular in 52 (37%), and LV dominant in 16 (12%). During the follow-up, 48 patients (34%) had major events, but none occurred in patients with negative CMR. At Kaplan-Meier analysis, patients with LV involvement (LV dominant and biventricular) had a worse prognosis than those with lone RV (p < 0.0001). At multivariate analysis, the LV involvement, a LV-dominant phenotype, and the 5-year ARVC risk score were independent predictors of major events. The estimated 5-year risk was able to predict the observed risk in patients with lone RV but underestimated the risk in those with LV involvement. Conclusions: Different CMR presentations of ARVC are associated with different prognoses. The 5-year ARVC risk score is valid for the estimation of risk in patients with lone-RV presentation but underestimated the risk when LV is involved.

Background The variation between rest and peak stress end-systolic pressure-volume relation (ESPVR; the Suga index) is easily obtained during routine stress echocardiography and has been established as a reasonably load-independent index of myocardial contractile performance that provides prognostic information above and beyond regional wall motion. Purpose This is the first study assessing the delta rest-stress ESPVR (DESPVR) by stress Cardiovascular Magnetic Resonance (CMR). Methods Eighty-five consecutive patients (19 females, main age 62.99±9.26 years) who underwent dipyridamole stress-CMR in a high volume CMR Laboratory were considered. The ESPVR was evaluated at rest and peak stress from raw measurement of systolic arterial pressure by cuff sphygmomanometer and end-systolic volume by biplane Simpson method. Results Mean ESPVR index at rest and peak stress was, respectively, 4.52±2.26 mmHg/mL/m2 and 4.62±2.32 mmHg/mL/m2 and mean DESPVR was 0.11±1.19 mmHg/mL/m2. DESPVR was not associated to age or sex. An inverse relationship between rest left ventricular end-diastolic volume index (LVEDVI) and both rest and peak ESPVR was present (R=-0.805 P An abnormal stress CMR was found in 22 patients and the DESPVR was comparable between patients with normal and abnormal stress exam. During a median follow-up of 60.62 months (IQ range 36.78 months), 27 cardiovascular events occurred: 3 deaths, 1 ventricular arrhythmias, 9 coronary syndromes, 14 heart failure hospitalization. At receiver-operating characteristic (ROC) curve analysis, a DESPVR Conclusions We showed for the first time that dipyridamole stress CMR can be used for the assessment of DESPVR. DESPVR was shown to be independent from chamber size and, as a consequence, can be used for a comparative assessment of patients with different diseases. DESPVR by CMR can provide a prognostic stratification and the optimal cutoff for relevant events was 0.009.