Accurate differentiation between Arterial Pulmonary Hypertension (PAH) and Chronic Thromboembolic Pulmonary Hypertension (CTEPH), two subtypes of Pulmonary Hypertension (PH), from CT Pulmonary Angiography (CTPA) scans may facilitate earlier diagnosis and improve patient prognosis. In this work, we reimplemented an existing framework originally developed for the identification of chronic thrombi in CTPA scans and adapted it to the differentiation between PAH and CTEPH. The original framework was based on generating Maximum Intensity Projections (MIP) of the CTPA scans from different spatial positions, which were then embedded and classified using a neural network architecture. Furthermore, we introduce a variant of the approach that allows ranking MIP projections and generating relevance maps in the original CT image space, thereby improving explainability. The two methods exhibited comparable classification performance. However, a preliminary radiologist assessment of the relevance maps generated by the proposed variant suggests an ability to focus on regions of abnormal pulmonary perfusion that are characteristic of thromboembolic disease.

Background Stereotactic arrhythmia radioablation (STAR) may represent an alternative to standard catheter ablation (CA) for treatment of refractory ventricular arrhythmias (VA). Most STAR treatments performed to date have used photon radiotherapy. However, protons have advantageous ballistic properties suitable for this application, potentially improving sparing of organs at risk (OARs). Methods ECG-gated CT scans and electroanatomical mapping were performed to reconstruct a 3D bipolar voltage map and identify ablation targets in a cohort of patients with VA candidates for CA. Three radiation treatment modalities were investigated/compared using the breath-hold technique: photon volumetric modulated arc therapy (VMAT) and proton active pencil beam scanning delivered with/without cardiac gating. When cardiac gating was applied, the diastolic phase—longer and more stable than the systolic one—was selected as the reference. Results Twenty-three patients were enrolled, and three presented multifocal ventricular arrhythmias, resulting in a total of 27 treatment targets. Both photon and proton plans achieved adequate target coverage. Compared with photon plans, proton therapy showed a statistically significant reduction in the mean maximum dose to several OARs and to important non-target cardiac structures (coronary arteries, valvular apparatus and right atrium) while maintaining comparable target coverage. These findings were consistent for proton plans with and without cardiac gating. In a subset of 14 patients, cardiac gating provided additional reductions in dose to healthy cardiac tissue. Conclusions This in-silico study suggests that STAR using proton beams may significantly reduce radiation exposure to non-target cardiac substructures and surrounding extracardiac OARs, regardless of the cardiac motion management strategy adopted.

Background: Cardiac computed tomography (CCT) is recommended as a complementary exam and for coronary artery disease evaluation in patients with aortic valve IE (AVIE). We aimed to evaluate the performance of CCT in visualizing coronary arteries in patients with AVIE and its concordance with transoesophageal findings. Methods: We included patients admitted for AVIE who underwent CCT from 2016 to 2023. Coronary arteries were divided into 17 segments according to the American Heart Association and compared using generalized linear models extended to the binomial family. Results were presented as risk differences (RDs) and 95% confidence intervals (CIs). For those undergoing cardiac surgery, we evaluated the concordance among CCT, TOE, and surgical inspection using Cohen's kappa (κ) test. Results: During the study period, 39 patients with AVIE performed CCT, of whom 28 underwent cardiac surgery. Visibility of coronary arteries was lower in median segments (RD -0.23; 95% CI -0.32 to -0.14) and in distal segments (RD -0.25; 95% CI -0.36 to -0.15) compared to proximal ones. Visibility of left anterior descending artery 2 was higher compared to right coronary artery 2 (RD 0.33; 95% CI 0.07 to 0.45) and obtuse marginal (RD 0.33; 95% CI 0.52 to 0.15). Concordance between CCT and TOE was globally low for vegetations (kappa 0.14), pseudoaneurysms (kappa 0.46), and paravalvular leakage (kappa 0.36). Conclusions: This study highlights the complementary role of CCT in diagnosing IE, given its low concordance with TOE for specific IE lesions. Furthermore, while CCT offers good visibility of the proximal coronary arteries in patients with AVIE, the visibility of the median and distal segments may be suboptimal.

INTRODUCTION:The left atrioventricular coupling index (LACI) has emerged as a potential prognostic marker in several clinical settings. This study evaluated the prognostic value of cardiac magnetic resonance (CMR)-derived LACI in patients with heart failure (HF) and reduced left ventricular ejection fraction (LVEF). METHODS:Patients from the multicentre DERIVATE registry with LVEF <50% who underwent CMR were included. LACI was calculated as the ratio between left atrial and left ventricular end-diastolic volumes. Univariable and multivariable Cox regression models estimated hazard ratios (HR) with 95% confidence intervals (CI) for predicting all-cause mortality (ACM), ACM or HF, and HF alone (competing-risk analysis). Time-dependent receiver operating characteristic analysis identified optimal cut-offs for 3-year outcomes. RESULTS:A total of 2170 patients were included (mean age 59.8 ± 13.9 years; 24.7% women; mean LVEF 31.6 ± 11.3%). Median follow-up was 1016 days (580-1609). Median LACI was 19.4% (13.3-28.8). During follow-up, ACM occurred in 191 patients (8.8%), ACM or HF in 565 (26.0%), and HF in 442 (20.4%). After adjustment for clinical and CMR parameters, including LVEF and late gadolinium enhancement (LGE), each 5% increase in LACI was associated with higher risk of ACM (HR 1.06, 95% CI 1.01-1.11; P = .016), ACM or HF (HR 1.09, 95% CI 1.06-1.12; P < .001), and HF (HR 1.09, 95% CI 1.05-1.12; P < .001). The optimal cut-off for ACM was LACI ≥21% (AUC 0.617, 95% CI 0.561-0.673), identifying patients at higher risk of ACM, ACM or HF, and HF (log-rank P < .001 for all). CONCLUSION:CMR-derived LACI independently predicts ACM and HF in patients with reduced LVEF and provides incremental prognostic value beyond LVEF and LGE. A cut-off of ≥21% identifies higher-risk patients and may support clinical risk stratification.

AIMS:Implantable cardioverter-defibrillator (ICD) therapy is the most effective prophylactic strategy of sudden cardiac death (SCD) in patients with ischemic cardiomyopathy (ICM). The aim of current analysis is to evaluate the prognostic impact of late gadolinium enhancement-papillary muscles (LGE-PMs) at cardiovascular magnetic resonance (CMR) and specifically its capability to re-stratify the arrhythmic risk on top to the DERIVATE-ICM Risk Score previously published. METHODS:Eighty-hundred-thirty-nine patients (mean age 65 ± 11 years; males:721[86%]) with ICM and TTE-LVEF <50% were enrolled from the DERIVATE-ICM registry (CarDiac MagnEtic Resonance for Primary Prevention Implantable CardioVerter DebrillAtor ThErapy- Ischemic Cardiomyopathy). Major adverse arrhythmic cardiac events (MAACE) were the primary endpoints. RESULTS:During a median follow-up of 1054 days, MAACE occurred in 86 (9.7%). DERIVATE-ICM Risk Score quartiles Q2-Q3 (HR:2.124 [95% CI:1.084-4.162]; p = 0.028), Q4 (HR: 3.865 [95% CI: 1.875-7.970]; p < 0.001) and the involvement of isolated posteromedial (P)PM (HR:1.985 [95% CI:1.073-3.673]; p = 0.029) were independent predictors of MAACE. The Kaplan-Meier survival curves showed a higher event-free rate in absence of LGE-PPM in patients categorized in the DERIVATE-ICM Risk Score quartiles Q2-Q3 (p = 0.018). Finally, adding LGE-PPM involvement on top of the model included TTE LVEF<35% plus DERIVATE-ICM Risk Score quartiles Q2-Q3 provided a significant improvement of prognostic stratification (p = 0.044). CONCLUSION:This study suggests that, in a wide population of ICM patients, LGE-PPM is independently associated with the occurrence of MAACE. In the intermediate quartiles of the DERIVATE-ICM Risk Score, the absence of LGE-PPM, when added to the Score, may contribute to downward re-stratification of arrhythmic risk. CLINICAL TRIAL REGISTRATION:RCT#NCT03352648.

BACKGROUND:Early detection of cardiac involvement in light-chain (AL) amyloidosis remains challenging despite its prognostic importance. [18F]-florbetaben positron emission tomography/computed-tomography (F-PET) shows promise for amyloid detection. OBJECTIVES:The authors report results from the prospective MoRBiDA (Molecular, magnetic resonance, and echocardiographic imaging combined with biomarkers of cardiac and clonal disease to predict survival and assess response to therapy in cardiac AL amyloidosis) study integrating multimodality imaging in treatment-naive AL amyloidosis patients to evaluate F-PET diagnostic sensitivity and correlations with established markers. METHODS:Patients with suspected cardiac involvement underwent baseline echocardiography, cardiac magnetic resonance (CMR), and F-PET. Cardiac involvement was defined by consensus criteria. F-PET included 50-minute dynamic cardiac acquisition (static reconstructions at ∼15 and 50-min postinjection), delayed static scan at 110 min, and myocardial tracer retention. Positive uptake was defined by visual myocardial retention exceeding blood-pool/background plus quantitative mean and maximum standardized uptake value. RESULTS:Of the 28 enrolled patients, 25 had cardiac involvement (3 patients without cardiac involvement had no myocardial-uptake). Early and late F-PET uptake (15-50 min) was present in all 25. Delayed uptake (110 min) was absent in 4/25 (84% sensitivity); these showed retention up to 50-min with typical CMR findings. F-PET parameters correlated moderately with N-terminal pro-B-type natriuretic peptide (standardized uptake value rho = 0.40-0.54, P < 0.05) and echocardiographic global longitudinal strain (myocardial tracer retention rho = 0.70, P < 0.001) but not with CMR-derived extracellular volume. CONCLUSIONS:Early-to-late F-PET demonstrates excellent sensitivity for cardiac AL amyloidosis. Delayed (110-min) retention is not universal, indicating variable washout kinetics that limits its discriminatory value for amyloid typing vs late imaging or multiorgan analysis. Extracellular volume may not fully capture amyloid burden. These findings complement recent multicentric data and support further studies on optimal timing and volumetric metrics.

AIMS:Selection of the patients for implantable cardioverter defibrillator primary prevention therapy in non-ischaemic cardiomyopathy (NICM) needs to be improved. To evaluate the additional prognostic value of a new cardiac magnetic resonance (CMR) score based on late gadolinium enhancement (LGE) pattern distribution (DERIVATE Risk Score 2.0) when compared with previously published DERIVATE Risk Score 1.0, which is based solely on quantitative parameters, in a cohort of NICM patients enrolled in the DERIVATE registry. METHODS AND RESULTS:One thousand three hundred and eighty-four NICM patients with chronic heart failure and left ventricular ejection fraction (LVEF) < 50% were evaluated for primary sudden cardiac death prevention therapy. Major adverse arrhythmic cardiac events (MAACEs) were the primary endpoint. During a median follow-up of 959 days, MAACE occurred in 128 (9.2%) patients. In the multivariate analyses, male gender [hazard ratio (HR): 1.605 (95% confidence interval, CI: 1.051-2.451); P = 0.028], LVEF per point % [HR: 0.977 (95% CI: 0.961-0.993); P = 0.005] and presence and location of midwall LGE [weighted HR: 1.066 (95% CI: 1.045-1.086), P < 0.001] were independent predictors of MAACE. A multi-parametric CMR-weighted predictive-derived score (DERIVATE Risk Score 2.0) provided a higher additional prognostic value vs. transthoracic echocardiography-LVEF cut-off of 35% when compared with the previous published DERIVATE Risk Score 1.0 with a net reclassification improvement of 54.52% (95% CI: 36.52-72.52%; P < 0.001). These findings were confirmed in the validation cohort. CONCLUSION:The presence of midwall LGE, but also the location of scar, confers an added and independent MAACE risk to a large NICM population influencing the choice of treatment.

AIMS:Accurate risk stratification for patients with non-dilated left ventricular cardiomyopathy (NDLVC) remains challenging due to lack of dedicated clinical trials. This post hoc analysis aims to delineate the arrhythmic risk and assess the incremental value of cardiac magnetic resonance (CMR) imaging in the CarDiac magnEtic Resonance for prophylactic Implantable-cardioVerter defibrillAtor ThErapy (DERIVATE) study cohort meeting the NDLVC diagnostic criteria. METHODS AND RESULTS:Patients with NDLVC from the DERIVATE registry were identified in the absence of left ventricular (LV) dilatation and in the presence of non-ischaemic LV scarring ('fibrotic NDLVC') or isolated LV systolic dysfunction (LV ejection fraction < 50%) without fibrosis ('hypokinetic NDLVC'). The primary endpoint was all-cause mortality. Major adverse arrhythmic cardiac events (MAACE) were the secondary endpoint and included sudden cardiac death (SCD) and aborted SCD. One hundred and ninety-seven NDLVC patients were identified from the cohort of the DERIVATE study (mean age: 59 ± 14 years; male: 135). Over a median follow-up of 2.7 years, 15 (8%) patients died and 8 (4%) experienced MAACE. Patients with 'hypokinetic' NDLVC had significantly lower rates of MAACE than non-ischaemic dilated cardiomyopathy (NIDCM) (P = 0.001), while patients with 'fibrotic' NDLVC had same rate of both primary (P = 0.48) and secondary endpoints (P = 0.616) compared with NIDCM patients. Multivariable analysis identified late gadolinium enhancement (LGE) with midwall distribution as an independent predictor of MAACE in NDLVC patients (hazard ratio 6.7, 95% confidence interval: 1.33-33.67; P = 0.021). CONCLUSION:NDLVC patients exhibit a heterogeneous risk profile for arrhythmic events. The presence of midwall LGE, similarly to NIDCM, is a significant predictor of MAACE, highlighting the importance of CMR imaging for risk stratification.

INTRODUCTION:Regular screening for pulmonary hypertension (PH) is recommended in patients with SSc for the early detection and treatment of pulmonary arterial hypertension. Whether Doppler echocardiography may predict subsequent development of PH is still unknown. In this context, there is growing awareness of the potential importance of right atrial function in reflecting an initial overload of the right heart due to the hypertensive state in the pulmonary circulation is a matter of considerable interest. AIM:We tested the hypothesis that right atrial reservoir strain (RARs) might be a sensitive parameter to reflect an initial overload of the right heart and predict the development of PH in SSc patients. METHODS:We enrolled 113 SSc patients followed at our Scleroderma Unit from May 2010 to April 2022, who underwent a complete echocardiographic examination which included the estimate of systolic pulmonary artery systolic pressure, the measurement of tricuspid annular plane systolic excursion (TAPSE), its ratio with systolic pulmonary artery pressure (TAPSE/PAPs) and RARs. RESULTS:During a subsequent median follow-up period of 43 months, 11 patients underwent right heart catheterization because of suspect PH, which was confirmed in 10 patients. At multivariable analysis, RARs was the only echocardiographic parameter with a statistically significant, independent predictive accuracy for PH (hazard ratio 0.85, 95% CI 0.75-0.96, P = 0.01). At receiver operating characteristic curves, the optimal baseline cut-off value of RARs to predict PH development was 39.6 (area under the curve 0.7, P = 0.04, sensitivity of 70% and specificity of 60%). CONCLUSION:RARs may be a sensitive echocardiographic parameter to predict subsequent development of PH in patients with SSc.

BACKGROUND:Stereotactic arrhythmia radioablation (STAR) is an emerging, non-invasive treatment for refractory ventricular arrhythmias. The technology requires target motion management. PURPOSE:We studied the integration of a novel ultrasound probe and holder for heart motion management into proton-beam STAR treatment plans. METHODS:Data were collected in eight of the 23 patients enrolled to-date, in an ongoing prospective, multicenter, observational in silico study comparing treatment planning with photons versus proton radiotherapy in patients affected by Ventricular Tachycardia with indication for catheter ablation procedure. In such subgroup of patients hands-free transthoracic echocardiography was performed in apical and parasternal positions, followed by planning Computed Tomography with ultrasound probe positions marked on the chest. A total of eleven targets were contoured and, for the proton therapy part, pencil beam scanning intensity-modulated proton therapy plans were optimized, assuming cardio-respiratory (dual) gated delivery. RESULTS:In all eleven cases, it was possible to avoid beams intercepting the probe and the dosimetric constraints were fulfilled. In four cases, the position of the probe did not interfere with the beam angles defined a-priori. In four cases, beam angles had to be modified to avoid intercepting the probe but the modified plan was equivalent to the a-priori plan. In three cases, the modified plans included beams with longer penetration depth compared to the a-priori plan, but all planning constraints were fulfilled. CONCLUSIONS:These results support the feasibility of using the novel ultrasound probe and holder for heart motion management. The technology would facilitate dedicated patient setup, monitoring and cardiorespiratory gating for STAR using proton beams.

Purpose Lung cancer screening (LCS) by low-dose computed tomography (LDCT) demonstrated a 20–40% reduction in lung cancer mortality. National stakeholders and international scientific societies are increasingly endorsing LCS programs, but translating their benefits into practice is rather challenging. The “Model for Optimized Implementation of Early Lung Cancer Detection: Prospective Evaluation Of Preventive Lung HEalth” (PEOPLHE) is an Italian multicentric LCS program aiming at testing LCS feasibility and implementation within the national healthcare system. PEOPLHE is intended to assess (i) strategies to optimize LCS workflow, (ii) radiological quality assurance, and (iii) the need for dedicated resources, including smoking cessation facilities. Methods PEOPLHE aims to recruit 1.500 high-risk individuals across three tertiary general hospitals in three different Italian regions that provide comprehensive services to large populations to explore geographic, demographic, and socioeconomic diversities. Screening by LDCT will target current or former (quitting < 10 years) smokers (> 15 cigarettes/day for > 25 years, or > 10 cigarettes/day for > 30 years) aged 50–75 years. Lung nodules will be volumetric measured and classified by a modified PEOPLHE Lung-RADS 1.1 system. Current smokers will be offered smoking cessation support. Conclusion The PEOPLHE program will provide information on strategies for screening enrollment and smoking cessation interventions; administrative, organizational, and radiological needs for performing a state-of-the-art LCS; collateral and incidental findings (both pulmonary and extrapulmonary), contributing to the LCS implementation within national healthcare systems.

Abstract Background Stereotactic arrhythmia radio-ablation (STAR) has proven to be a valid alternative treatment for refractory ventricular tachycardia (VT) in patients who are unsuitable to undergo standard catheter ablation (CA). It consists in the application of external beam radiotherapy in a single dose of 25 Gy to the target areas. There is increased research activity on better understanding the complex and fast motion of the heart and on reducing radiation toxicity. Purpose In the context of an in silico study to evaluate the feasibility of STAR with protons in patients with VT, the initial findings related to cardiac motion on the first 10 patients are here presented. Methods Prior to CA, each patient underwent ECG gated CT scans in expiratory breath-hold, with and without contrast and reconstructed at 30% (systole) and 80% (diastole) of the cardiac R-R cycle. Contouring of the ablation target as a clinical target volume (CTV) for proton treatment planning was performed based on a standard electrophysiological study with 3D eletroanatomical mapping. Two different treatment plans were created: the first with only the diastolic CTV as target (gated treatment), the second including both diastolic and systolic CTVs to create an Internal Target Volume (ITV) in the hypothesis of non-gated treatment. Robust optimization was used to create the Planning Target Volume (PTV). Results The target volumes used for treatment planning are given in Table 1. The median CTV was 10.90 (2.93-36.94) cm3 for diastole and 14.26 (1.73-36.31) cm3 for systole. These volumes are somewhat smaller than those reported in patients treated previously with STAR: this difference may be due to the fact that no respiratory motion was included in the target contour and that the study population includes many patients referred for ventricular ectopic beats without structural heart disease, where the ablation target is expected to be smaller as compared to patients with VT in structural heart disease. Despite a small median difference between the diastolic and systolic CTV of 0.53 (0.03-9.61) cm3, the ITV approach resulted in a median increase in volume compared to the largest of the two CTVs of 29 % (1%-44%). When considering the proton range uncertainty and potential errors in patient positioning (PTV), the target is this time enlarged by a factor 2.9 (1.9-3.9). This data indicates that both cardiac motion and uncertainties in patient positioning before treatment will have a large impact on the treatment volume thus affecting the amount of surrounding tissue exposed to radiation. Conclusion The ablation target contour at systole and diastole are of similar magnitude but their non-overlap results in a large increase in treated volume when radiation delivery is not gated for cardiac motion. Further analysis shall investigate on a case-by-case basis the potential reduction in risk of healthy tissue toxicity with cardiac-gated proton delivery.Table 1

To verify whether the new hemodynamic definition of pulmonary hypertension (PH) has any implication in treatment of Chronic Thrombo-Embolic Pulmonary Disease (CTEPD) patients without PH, we retrospectively analysed the clinical and functional changes determined by pulmonary endarterectomy (PEA) in 63 CTEPD patients without PH who underwent surgery at our center, comparing those in whom the hemodynamic diagnosis of PH met recent guideline recommendations versus those in whom the diagnosis only met previous hemodynamic thresholds. The results show that the vast majority of CTEPD patients without PH operated at our center would now be defined as chronic thromboembolic pulmonary hypertension (CTEPH) patients. PEA did not result in any improvement in exercise capacity nor in right ventricular function or lung function test in patients with mean pulmonary artery pressure (mPAP) <= 20 mm Hg and pulmonary vascular resistance (PVR) <= 2 WU; on the contrary, hemodynamic parameters, exercise capacity, right ventricular function and lung function significantly improved in patients with mPAP between 21 and 24 mm Hg. J Heart Lung Transplant 2024;43:681-685 (c) 2024 International Society for Heart and Lung Transplantation. All rights reserved.

AIMS:While factors associated with adverse events are well elucidated in setting of isolated left ventricular dysfunction, clinical and imaging-based prognosticators of adverse outcomes are lacking in context of biventricular dysfunction. The purpose of this study was to establish role of clinical variables in prognosis of biventricular heart failure (HF), as assessed by cardiac magnetic resonance imaging. METHODS:Study cohort consisted of 840 patients enrolled in DERIVATE registry with coexisting CMR-derived right ventricular (RV) and left ventricular (LV) dysfunction, as defined by RV and LV ejection fractions ≤45 % and ≤ 50 %, respectively. The primary objective was to identify factors associated with adverse long-term outcomes, defined as composite of all-cause death and HF hospitalizations (DHFH). Kaplan-Meir curves were plotted for survival analysis. Cox proportional hazard models were constructed to estimate adjusted hazard ratios (aHRs) and associated 95 % confidence intervals for clinical variables and their correlation with adverse events. RESULTS:Mean age was 61.0 years; 83.1 % were male, 26.6 % had diabetes mellitus (DM), and 45.9 % had non-ischemic cardiomyopathy. At median follow-up of 2 years, DHFH occurred in 32.5 % of the cohort. Kaplan-Meir analysis showed higher rate of DHFH in patients with DM (35.2 % vs. 22.6 %, p < 0.001). Multivariate Cox regression analysis showed that DM was independently associated with DHFH (aHR 1.61 [95 % CI: 1.15-2.25]; p = 0.003). Importantly, ACE-inhibitor/ARB usage in patients with DM was associated with significant reduction in DHFH (aHR 0.53 [95 % CI: 0.31-0.90]; p = 0.02). CONCLUSION:In patients with biventricular HF, DM was a strong predictor of DHFH, with ACE-inhibitor/ARB usage having cardioprotective effect.

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

AIMS:The aim of this registry was to evaluate the additional prognostic value of a composite cardiac magnetic resonance (CMR)-based risk score over standard-of-care (SOC) evaluation in a large cohort of consecutive unselected non-ischaemic cardiomyopathy (NICM) patients. METHODS AND RESULTS:In the DERIVATE registry (www.clinicaltrials.gov/registration: RCT#NCT03352648), 1000 (derivation cohort) and 508 (validation cohort) NICM patients with chronic heart failure (HF) and left ventricular ejection fraction <50% were included. All-cause mortality and major adverse arrhythmic cardiac events (MAACE) were the primary and secondary endpoints, respectively. During a median follow-up of 959 days, all-cause mortality and MAACE occurred in 72 (7%) and 93 (9%) patients, respectively. Age and >3 segments with midwall fibrosis on late gadolinium enhancement (LGE) were the only independent predictors of all-cause mortality (HR: 1.036, 95% CI: 1.0117-1.056, P < 0.001 and HR: 2.077, 95% CI: 1.211-3.562, P = 0.008, respectively). For MAACE, the independent predictors were male gender, left ventricular end-diastolic volume index by CMR (CMR-LVEDVi), and >3 segments with midwall fibrosis on LGE (HR: 2.131, 95% CI: 1.231-3.690, P = 0.007; HR: 3.161, 95% CI: 1.750-5.709, P < 0.001; and HR: 1.693, 95% CI: 1.084-2.644, P = 0.021, respectively). A composite clinical and CMR-based risk score provided a net reclassification improvement of 63.7% (P < 0.001) for MAACE occurrence when added to the model based on SOC evaluation. These findings were confirmed in the validation cohort. CONCLUSION:In a large multicentre, multivendor cohort registry reflecting daily clinical practice in NICM work-up, a composite clinical and CMR-based risk score provides incremental prognostic value beyond SOC evaluation, which may have impact on the indication of implantable cardioverter-defibrillator implantation.