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Aims To assess the ability of cardiovascular magnetic resonance (CMR) to (i) measure changes in response to chemotherapy; (ii) assess the correlation between haematological response and changes in extracellular volume (ECV); and (iii) assess the association between changes in ECV and prognosis over and above existing predictors. Methods and results In total, 176 patients with cardiac AL amyloidosis were assessed using serial N-terminal pro-B-type natriuretic peptide (NT-proBNP), echocardiography, free light chains and CMR with T1 and ECV mapping at diagnosis and subsequently 6, 12, and 24 months after starting chemotherapy. Haematological response was graded as complete response (CR), very good partial response (VGPR), partial response (PR), or no response (NR). CMR response was graded by changes in ECV as progression (≥0.05 increase), stable ( Conclusions Cardiac amyloid deposits frequently regress following chemotherapy, but only in patients who achieve CR or VGPR. Changes in ECV predict outcome after adjusting for known predictors.

Left ventricular (LV) remodelling (REM) ensuing after ST-elevation myocardial infarction (STEMI), has typically been studied by echocardiography, which has limitations, or cardiac magnetic resonance (CMR) in early phase that may overestimate infarct size (IS) due to tissue edema and stunning. This prospective, multicenter study investigated LV-REM performing CMR in the subacute phase, and 6 months after STEMI.patients with first STEMI undergoing successful primary angioplasty were consecutively enrolled. CMR was done at 30-days and 6-months. Primary endpoint was prevalence at 6 months of LV-REM [≥12% increase in LV end-diastolic volume index (LV-REMIn a contemporary cohort of STEMI patients studied by CMR, prevalence of LV-REM

BackgroundA small but significant reduction in left ventricular (LV) mass after 18 months of migalastat treatment has been reported in Fabry disease (FD). This study aimed to assess the effect of migalastat on FD cardiac involvement, combining LV morphology and tissue characterisation by cardiac magnetic resonance (CMR) with cardiopulmonary exercise testing (CPET).MethodsSixteen treatment-naïve patients with FD (4 women, 46.4±16.2 years) with cardiac involvement (reduced T1 values on CMR and/or LV hypertrophy) underwent ECG, echocardiogram, troponin T and NT-proBNP (N-Terminal prohormone of Brain Natriuretic Peptide) assay, CMR with T1 mapping, and CPET before and after 18 months of migalastat.ResultsNo change in LV mass was detected at 18 months compared to baseline (95.2 g/m2(66.0–184.0) vs 99.0 g/m2(69.0–121.0), p=0.55). Overall, there was an increase in septal T1 of borderline significance (870.0 ms (848–882) vs 860.0 ms (833.0–875.0), p=0.056). Functional capacity showed an increase in oxygen consumption (VO2) at anaerobic threshold (15.50 mL/kg/min (13.70–21.50) vs 14.50 mL/kg/min (11.70–18.95), p=0.02), and a trend towards an increase in percent predicted peak VO2(72.0 (63.0–80.0) vs 69.0 (53.0–77.0), p=0.056) was observed. The subset of patients who showed an increase in T1 value and a reduction in LV mass (n=7, 1 female, age 40.5 (28.6–76.0)) was younger and at an earlier disease stage compared to the others, and also exhibited greater improvement in exercise tolerance.ConclusionIn treatment-naïve FD patients with cardiac involvement, 18-month treatment with migalastat stabilised LV mass and was associated with a trend towards an improvement in exercise tolerance. A tendency to T1 increase was detected by CMR. The subset of patients who had significant benefits from the treatment showed an earlier cardiac disease compared to the others.Trial registration numberNCT03838237.

Cardiac magnetic resonance (CMR) has become an essential tool for the evaluation of patients affected or at risk of developing cardiomyopathies (CMPs). In fact, CMR not only provides precise data on cardiac volumes, wall thickness, mass and systolic function but it also a non-invasive characterization of myocardial tissue, thus helping the early diagnosis and the precise phenotyping of the different CMPs, which is essential for early and individualized treatment of patients. Furthermore, several CMR characteristics, such as the presence of extensive LGE or abnormal mapping values, are emerging as prognostic markers, therefore helping to define patients’ risk. Lastly new experimental CMR techniques are under investigation and might contribute to widen our knowledge in the field of CMPs. In this perspective, CMR appears an essential tool to be systematically applied in the diagnostic and prognostic work-up of CMPs in clinical practice. This review provides a deep overview of clinical applicability of standard and emerging CMR techniques in the management of CMPs.

Objectives To elaborate an ECG-based nomogram estimating the probability to detect cardiac involvement by cardiac magnetic resonance (CMR) in Fabry Disease (FD). Methods 119 FD patients and 26 healthy controls underwent ECG and CMR. Test (n = 88, 60%) and validation cohorts (n = 57, 40%) were randomly derived. Cardiac involvement was defined as the presence of low myocardial T1 value, a CMR-surrogate of myocardial glycosphingolipid storage. ECG changes associated with low T1 value were identified in the test cohort, included in the nomogram and then tested in the validation cohort. Results Sokolow-Lyon index (AUC = 0.769), ratio between P-wave and PR-segment durations (Pwave/PRsegment) (AUC = 0.778), QRS duration (AUC = 0.703), QT (AUC = 0.769) duration were independently associated with the presence of low T1 on CMR at multivariate analysis. An ECG-based nomogram including these four parameters was accurate in identifying patients with CMR evidence of glycosphingolipid storage (c-index of the derived-nomogram = 0.90 in the test group; 0.81 in the validation group). Conclusion We propose a practical ECG-based nomogram accurately estimating the probability to detect low T1 values by CMR in FD patients. The application of this tool in clinical practice could improve early detection of FD cardiac involvement.

Background: Patients with mitral valve prolapse (MVP) may develop adverse outcomes even in the absence of mitral regurgitation or left ventricular (LV) dysfunction. Purpose: To investigate the prognostic value of mitral annulus disjunction (MAD) and myocardial fibrosis at late gadolinium enhancement (LGE) cardiac MRI in patients with MVP without moderate-to-severe mitral regurgitation or LV dysfunction. Materials and Methods: In this longitudinal retrospective study, 118 144 cardiac MRI studies were evaluated between October 2007 and June 2020 at 15 European tertiary medical centers. Follow-up was from the date of cardiac MRI examination to June 2020; the minimum and maximum follow-up intervals were 6 months and 156 months, respectively. Patients were excluded if at least one of the following conditions was present: cardiomyopathy, LV ejection fraction less than 40%, ischemic heart disease, congenital heart disease, inflammatory heart disease, moderate or worse mitral regurgitation, participation in competitive sport, or electrocardiogram suggestive of channelopathies. In the remainder, cardiac MRI studies were reanalyzed, and patients were included if they were aged 18 years or older, MVP was diagnosed at cardiac MRI, and clinical information and electrocardiogram monitoring were available within 3 months from cardiac MRI examination. The end point was a composite of adverse outcomes: sustained ventricular tachycardia (VT), sudden cardiac death (SCD), or unexplained syncope. Multivariable Cox regression analysis was performed. Results: A total of 474 patients (mean age, 47 years ± 16 [SD]; 244 women) were included. Over a median follow-up of 3.3 years, 18 patients (4%) reached the study end point. LGE presence (hazard ratio, 4.2 [95% CI: 1.5, 11.9]; P = .006) and extent (hazard ratio, 1.2 per 1% increase [95% CI: 1.1, 1.4]; P = .006), but not MAD presence (P = .89), were associated with clinical outcome. LGE presence had incremental prognostic value over MVP severity and sustained VT and aborted SCD at baseline (area under the receiver operating characteristic curve, 0.70 vs 0.62; P = .03). Conclusion: In contrast to mitral annulus disjunction, myocardial fibrosis determined according to late gadolinium enhancement at cardiac MRI was associated with adverse outcome in patients with mitral valve prolapse without moderate-to-severe mitral regurgitation or left ventricular dysfunction.

Pericardial cysts are rare mediastinal abnormalities, generally benign, discovered most often incidentally in the course of diagnostic investigations involving the cardiac profile. They have an incidence of 1 case in 100 000 and are mostly located at the right cardiophrenic angle. Spontaneous resolution of cysts is even rarer. We describe an intricate case report in which the cyst was discovered incidentally and was no longer detected at cardiac magnetic resonance performed 7 years after the first diagnosis. The presence of an asymptomatic pericardial cyst should be regularly followed up over time for the potential risks of complications related to its growth but also because over time, although rarely, it may no longer be detected as in the case presented.

Background We present the clinical validation of an AI-based framework for automatic ventricular segmentation and quantification, and automatic wall motion classification from cine CMR. Methods The framework was evaluated using two datasets, the SCMR Consensus dataset and a cohort private dataset composed by 146 patients. The automatic segmentation of left ventricle (LV) of SCMR Consensus dataset was evaluated through Dice Similarity Coefficient (DSC). The ventricular function quantification was assessed by processing both datasets, measuring LV and right ventricle (RV) end-diastolic and end-systolic volumes, ejection fraction (EF), and left ventricle mass (LVM). The validation was based on agreement indices, such as the intra-class correlation coefficients (ICC). Automatic wall motion classification was performed using the cohort dataset, and assessed using confusion matrix metrics. Results The comparison between the proposed framework and experts' manual performances on ventricular segmentation and function quantification processes showed high levels of contouring overlap (DSC=0.72), and agreement (ICC > 0.96 and ICC > 0.81 for volumes and EFs of LV and RV, respectively, and ICC > 0.78 for LVM). The automatic wall motion analysis obtained a classification accuracy of 0.77, with a sensitivity value of 1.00, meaning no false negative.. Conclusions The validation results confirmed the proposed framework robustness and generalization capabilities. Moreover, our proposal demonstrated its potential for automatic wall motion classification in clinical practice.

IMPORTANCE: Low-flow severe aortic stenosis (AS) has higher mortality than severe AS with normal flow. The conventional definition of low-flow AS is an indexed stroke volume (SVi) by echocardiography less than 35 mL/m(2). Cardiovascular magnetic resonance (CMR) is the reference standard for quantifying left ventricular volumes and function from which SVi by CMR can be derived. OBJECTIVE: To determine the association of left ventricular SVi by CMR with myocardial remodeling and survival among patients with severe AS after valve replacement. DESIGN, SETTING, AND PARTICIPANTS: This multicenter longitudinal cohort study was conducted between January 2003 and May 2015 across 6 UK cardiothoracic centers. Patients with severe AS listed for either surgical aortic valve replacement (SAVR) or transcatheter aortic valve replacement (TAVR) were included. Patients underwent preprocedural echocardiography and CMR. Patients were stratified by echocardiography-derived aortic valve mean and/or peak gradient and SVi by CMR into 4 AS endotypes: low-flow, low-gradient AS; low-flow, high-gradient AS; normal-flow, low-gradient AS; and normal-flow, high-gradient AS. Patients were observed for a median of 3.6 years. Data were analyzed from September to November 2021. EXPOSURES: SAVR or TAVR. MAIN OUTCOMES AND MEASURES: All-cause and cardiovascular (CV) mortality after aortic valve intervention. RESULTS: Of 674 included patients, 425 (63.1%) were male, and the median (IQR) age was 75 (66-80) years. The median (IQR) aortic valve area index was 0.4 (0.3-0.4) cm(2)/m(2). Patients with low-flow AS endotypes (low gradient and high gradient) had lower left ventricular ejection fraction, mass, and wall thickness and increased all-cause and CV mortality than patients with normal-flow AS (all-cause mortality: hazard ratio [HR], 2.08; 95% CI, 1.37-3.14; P

Background Time-resolved three-directional velocity-encoded (4D flow) magnetic resonance imaging (MRI) enables the quantification of left ventricular (LV) intracavitary fluid dynamics and energetics, providing mechanistic insight into LV dysfunctions. Before becoming a support to diagnosis and patient stratification, this analysis should prove capable of discriminating between clearly different LV derangements. Purpose To investigate the potential of 4D flow in identifying fluid dynamic and energetics derangements in ischemic and restrictive LV cardiomyopathies. Study Type Prospective observational study. Population Ten patients with post-ischemic cardiomyopathy (ICM), 10 patients with cardiac light-chain cardiac amyloidosis (AL-CA), and 10 healthy controls were included. Field Strength/Sequence 1.5 T/balanced steady-state free precession cine and 4D flow sequences. Assessment Flow was divided into four components: direct flow (DF), retained inflow, delayed ejection flow, and residual volume (RV). Demographics, LV morphology, flow components, global and regional energetics (volume-normalized kinetic energy [KEV] and viscous energy loss [ELV]), and pressure-derived hemodynamic force (HDF) were compared between the three groups. Statistical Tests Intergroup differences in flow components were tested by one-way analysis of variance (ANOVA); differences in energetic variables and peak HDF were tested by two-way ANOVA. AP-value of Results ICM patients exhibited the following statistically significant alterations vs. controls: reduced KEV, mostly in the basal region, in systole (−44%) and in diastole (−37%); altered flow components, with reduced DF (−33%) and increased RV (+26%); and reduced basal–apical HDF component on average by 63% at peak systole. AL-CA patients exhibited the following alterations vs. controls: significantly reduced KEVat the E-wave peak in the basal segment (−34%); albeit nonstatistically significant, increased peaks and altered time-course of the HDF basal–apical component in diastole and slightly reduced HDF components in systole. Data Conclusion The analysis of multiple 4D flow-derived parameters highlighted fluid dynamic alterations associated with systolic and diastolic dysfunctions in ICM and AL-CA patients, respectively.

The use of cardiac magnetic resonance (CMR) for accurate morphological assessment of cardiomyopathies is well described. CMR tissue characterization with late gadolinium enhancement and parametric mapping is highly promising in differentiating key etiologies of left ventricular hypertrophy, diagnosing cardiac involvement in systemic amyloidosis, detecting early disease, and tracking changes over time, as well as providing valuable prognostic information. This review focuses on the typical imaging findings in cardiac amyloidosis by CMR, highlighting its potentials with respect to traditional imaging techniques. Furthermore, the diagnostic and prognostic role of CMR parameters and the future perspectives related to the newest applications are addressed.

Aims Right ventricular systolic dysfunction (RVSD) is an important determinant of outcomes in heart failure (HF) cohorts. While the quantitative assessment of RV function is challenging using 2D-echocardiography, cardiac magnetic resonance (CMR) is the gold standard with its high spatial resolution and precise anatomical definition. We sought to investigate the prognostic value of CMR-derived RV systolic function in a large cohort of HF with reduced ejection fraction (HFrEF). Methods and results Study cohort comprised of patients enrolled in the CarDiac MagnEtic Resonance for Primary Prevention Implantable CardioVerter DefibrillAtor ThErapy registry who had HFrEF and had simultaneous baseline CMR and echocardiography (n = 2449). RVSD was defined as RV ejection fraction (RVEF) Conclusion RV systolic dysfunction is an independent predictor of ACM in HFrEF, with a more pronounced prognostic value in select subgroups, likely reflecting the importance of RVSD in the early stages of HF progression.

Background: Brugada syndrome (BrS) is considered a purely electrical disease with variable electrical substrates. Variable rates of mechanical abnormalities have been also reported. Whether exists a link between electrical and mechanical abnormalities has never been previously explored. This investigational physiopathological study aimed to determine the relationship between the substrate size/location, as exposed by ajmaline provocation, and the severity of mechanical abnormalities, as assessed by cardiac magnetic resonance in patients with BrS. Methods: Twenty-four consecutive high-risk patients with BrS (mean age, 38±11 years, 17 males), presenting with malignant syncope and documented polymorphic ventricular tachycardia/ventricular fibrillation, and candidate to implantable cardioverter defibrillator implantation, underwent cardiac magnetic resonance and electroanatomic maps. During each examination, ajmaline test (1 mg/kg over 5 minutes) was performed. Cardiac magnetic resonance findings were compared with 24 age, sex, and body surface area-matched controls. In patients with BrS, the correlation between the electrical substrate extent and right ventricular regional mechanical abnormalities before/after ajmaline challenge was analyzed. Results: After ajmaline, patients with BrS showed a reduction of right ventricular (RV) ejection fraction ( P P P P P 2 to 14.2±7.3 cm 2 ( P r =−0.830, P Conclusions: BrS is a dynamic RV electromechanical disease, where functional abnormalities correlate with the maximal extent of the substrate size. These findings open new lights on the physiopathology of the disease. Registration: URL: https://clinicaltrial.gov ; Unique identifier: NCT03524079.

A young male with β-thalassemia major was implanted with a single-chamber Implantable cardioverter-defibrillator (ICD) for a cardiac arrest due to ventricular fibrillation. He received multiple inappropriate shocks due to atrioventricular nodal re-entrant tachycardia (AVNRT) treated with radiofrequency catheter ablation and then to high-rate atrial tachycardia refractory to amiodarone and not inducible during electrophysiological study. He refused empirical pulmonary vein isolation. Upgrading to biventricular ICD and performing atrioventricular node ablation avoided further inappropriate shocks.

PLOS ONE 17(1), e0262110 (2022). doi:10.1371/journal.pone.0262110, Published by PLOS, San Francisco, California, US

Funding Acknowledgements Type of funding sources: None. Background Left ventricular (LV) diastolic dysfunction (DD) is a hallmark of hypertrophic cardiomyopathy (HCM) and its phenocopies, such as Fabry disease (FD). Together with left atrial (LA) size, LA function is emerging as a sensitive marker of the adaptive changes to backward transmission of LV cardiac filling pressure, thus implementing DD assessment. Additionally, both HCM and FD are characterized by a primitive atrial myopathy, but LA morpho-functional changes in HCM and FD have never been directly compared. More recently, LA strain by Cardiovascular Magnetic Resonance Feature Tracking (CMR-FT) has been demonstrated to be a feasible and reproducible tool to explore LA function. Purpose To compare LA morpho-functional changes in HCM and FD and to explore their correlation with tissue alterations. Methods 15 HCM and 15 sex-, age- and LV mass index-matched FD patients underwent CMR (Magnetom Aera 1.5T, Siemens) and Doppler Echocardiography for LV diastolic function assessment (E/e’ and DD grading from 0 to 3). LA phasic function was evaluated by CMR-FT strain (Qstrain Medis). The software output included passive (εe, conduit function), active (εa, booster pump function) and total strain (εs, reservoir function), along with LA volumes and ejection fraction (EF). Late gadolinium enhancement (LGE) was quantified as a percentage of LV mass using the standard deviations (SDs) method (≥ 5 SDs). Interstitial fibrosis was assessed by extracellular volume (ECV) quantification in remote myocardium. All patients were in sinus rhythm. Results In the HCM group, the proportion of patients with DD grade 2-3 was only slightly higher than in FD (p 0.26). Accordingly, no significant difference was found in E/e’ value (p 0.78). Compared to FD, HCM patients showed more severe LA morpho-functional changes, including larger LA end-systolic volume (ESV) (113 ± 35 vs 84 ± 23 ml), lower LA EF (37 ± 7 vs 44 ± 9 %) and a greater reduction of εs (-20 ± 5 vs -25 ± 6 %) and εa (-10 ± 4 vs -15 ± 4 %) (all p < 0.05). LV size and function and the burden of fibrosis (LGE quantification and ECV) were comparable between the two groups. Interestingly, in HCM population, unlike in FD, LA morpho-functional measurements significantly correlated with tissue characterization parameters (LA ESV with LGE, r 0.56, p 0.03; εs and εa with ECV, r -0.51, p 0.05 and r -0.59, p 0.02, respectively). Conclusions LA morpho-functional alterations are much more severe in HCM compared to FD with similar degree of LV hypertrophy. A more severe atrial myopathy or different mechanisms of atrial damage in the two cardiomyopathies may explain these findings. LA CMR-FT analysis may represent a sensitive tool to discriminate between HCM and FD, although larger studies are needed to confirm this finding and the possible correlation with the occurrence of atrial arrhythmias and thromboembolic risk.

Funding Acknowledgements Type of funding sources: None. Background Increasing evidence suggests that left atrial (LA) deformation is a sensitive marker of diastolic dysfunction in hypertrophic phenotypes. However, there is little data about the impact of hypertension on LA function; furthermore, LA deformation in hypertensive heart disease (HHD) and hypertrophic cardiomyopathy (HCM) has not been compared yet. Purpose The aim of this study is to compare atrial dimensions and function, evaluated by cardiovascular magnetic resonance feature tracking (CMR-FT) in patients with HHD, HCM and healthy subjects (HS). Methods 67 patients (20 HHD, 27 HCM, 20 HS) underwent CMR and were included in the study. Patients were matched for age, sex and BSA; HHD and HCM were also comparable for LV mass index and ejection fraction (EF). CMR-FT atrial strain analysis was performed using Qstrain, Medis software to obtain i) LA conduit function, ii) LA booster pump function), iii) LA reservoir function, iv) LA volumes and EF. Tissue Doppler echocardiography was used to assess diastolic function, including E/e’. LA stiffness was calculated as the ratio between E/e’ and LA reservoir. Both focal and interstitial myocardial fibrosis were assessed with LGE and extracellular volume (ECV) quantification. Results HHD and HCM showed impaired LA reservoir, conduit function and higher LA volumes vs HS (reservoir: 28 ± 11% and 28 ± 13% vs 41 ± 17%; conduit: 13 ± 7% and 13 ± 7% vs 22 ± 11%; LAESV: 76 ± 21 and 87 ± 22 vs 57 ± 19 ml respectively; all p ≤ 0.03). HHD and HCM were comparable for bi-ventricular morpho-functional parameters and ECV. HHD showed lower E/e’ values (8 ± 2 vs 16 ± 7, p = 0.002) and LA stiffness (0.23 ± 0.3 vs 0.74 ± 0.6, p 0.03), LA dimensions (LA area 13 ± 3 vs 16 ± 3 cm2/m2, p = 0.02 , LAESVi 41 ± 12 vs 48 ± 11 ml/m2, p = 0.05) and LGE extent (1 ± 2% vs 5 ± 5%, p = 0.001) as compared to HCM. Interestingly, HHD revealed a comparable reduced LA reservoir and conduit function (all p = 0.9) vs HCM. In HHD patients LA reservoir function was correlated with E/e’ (r -0.8, p = 0.02), but not in HCM. Conversely, LA reservoir function was correlated with LV mass index in HCM (r -0.5, p Conclusions HHD patients showed a similar and significant impairment of LA function, with lower LA dimensions and E/e’ compared to HCM with similar LV mass index and preserved function. CMR-FT atrial strain analysis could represent a useful tool for HHD management, able to detect diastolic dysfunction (and/or atrial dysfunction) earlier than traditional markers. Further studies are needed to explore the relationship of LA deformation to heart failure symptoms and atrial fibrillation occurrence and potential changes related to response to therapy.

Funding Acknowledgements Type of funding sources: None. OnBehalf Mitral vAlve prolapse and disjunction by cardiac maGnetIC resonance (MA-GIC) registry Backgroung Mitral valve prolapse (MVP) is 2-3% prevalent in the general population with good prognosis. However, some patients develop complex ventricular arrhythmias (CVAs), sudden cardiac death (SCD), or severe mitral regurgitation (MR). Previous studies suggested that bi-leaflet involvement, mitral annulus disjunction (MAD), and myocardial fibrosis (MF) are associated with adverse outcome. Notwithstanding, these findings were limited to autopsic series or single-centre studies involving highly selected patients. Moreover, MF has been scantly investigated as predictor of clinical outcome. Purpose To investigate the prognostic significance of MF in an international multicentre study of MVP patients studied by cardiovascular magnetic resonance (CMR) with late gadolinium enhancement (LGE). Methods From October 2007 to June 2020 patients undergoing LGE-CMR were screened in 14 European centres. Inclusion criteria were: i) age > 18 years; ii) full clinical history and cardiac rhythm monitoring at baseline; iii) MVP (leaflet displacement ≥ 2 mm beyond the annulus). Exclusion criteria were: i) ischemic heart disease; ii) primary cardiomyopathy; iii) inflammatory heart disease; iv) congenital heart diseases; v) moderate-to-severe valvular heart disease. CVAs at the study outset was defined as one of the following: i) ventricular ectopic beats >10000/24h; ii) ≥ 1 episode of non-sustained ventricular tachycardia (VT); iii) sustained VT; iv) aborted SCD. Primary end-point was a composite of SCD, unexplained syncope, and mitral valve repair/replacement. Secondary end-point was a composite of SCD and unexplained syncope. Results Four-hundred-fifty-eight MVP patients were eventually included (46 ± 16 years old, 51% males) of whom 68% had MAD. LGE was detected in 103 (22%) of subjects with mid-wall pattern (46%) in left ventricular (LV) lateral wall (66%) as the most prevalent feature. At baseline, 37% of LGE-positive patients vs. 18% of LGE-negative individuals had CVAs (P Conlusion Myocardial fibrosis by LGE is the strongest independent predictor of clinical outcome in MVP. In contrast, MAD per se does not harbinger worse prognosis.

It is debated whether percutaneous revascularization (PCI) of total coronary chronic occlusion (CTO) is superior to optimal medical therapy (OMT) in improving symptoms, left ventricular (LV) function and major adverse cardiac/cerebrovascular events (MACCE). Furthermore, CTO-PCI is a challenging technique, with lower success rate than in other settings. A systematic analysis of baseline LV function, infarction extent and ischaemic burden to predict response to revascularization has never been performed.To establish a CMR protocol to identify patients (pts) who can benefit most from CTO-PCI. Myocardial viability/ischaemia retains high biological plausibility as predictors of response to revascularization. Therefore, baseline viability (necrotic tissue extent, response to inotropic stimulation) and ischaemia (perfusion defect, wall motion abnormality during stress) will be studied as potential predictors of mechanical LV segmental improvement and ischaemic burden reduction in CTO territory (primary endpoint), LV remodelling and global function, Seattle Angina Questionnaire, and MACCE improvement (secondary endpoints) in the follow-up.Pts with CTO suitable for PCI undergo stress-CMR for viability/ischaemia assessment. Pts with normal LV function undergo adenosine, those with moderately-reduced ejection fraction (EF) and wall motion abnormalities high-dose dobutamine, pts with EF35% low-dose dobutamine. All pts undergo late gadolinium enhancement and repeat the same scan at 12 ± 3 months, regardless of PCI success or decision for OMT.A multi-parameter CMR protocol tailored on pts characteristics to study viability/ischaemia could help in identifying responders in terms of LV function, ischaemic burden and clinical outcome among pts suitable for CTO-PCI, improving selection of best candidates to percutaneous revascularization.

Infiltrative cardiomyopathies are comprised of a variety of inherited or acquired conditions. Myocardial involvement is often part of a complex systemic disorder, but sometimes the disease can manifest predominantly in the heart. Clinical manifestations are heterogeneous, from mild symptoms to early cardiac death. A multimodality imaging approach by means of echocardiography, cardiovascular magnetic resonance (CMR), and molecular imaging is necessary; a comprehensive assessment of morphologic, functional, tissue, and metabolic changes is the key to improve the pathophysiologic understanding of these diseases, favouring early differential diagnosis and risk stratification of patients. Furthermore, quantitative, non-invasive assessment of tissue pathology is now available and plays a crucial role in the follow-up of patients affected by infiltrative cardiomyopathies, improving their management and potentially guiding the development of existing and new therapies.

Funding Acknowledgements Type of funding sources: None. onbehalf The BSCMR Valve Consortium Background Patients with low-flow aortic stenosis (LF-AS) have higher mortality than those with high-flow severe AS. The conventional echocardiographic definition of LF-AS is an indexed stroke volume (SVi) Purpose To determine the association between CMR SVi, myocardial remodelling and survival in severe symptomatic AS. Methods In a multi-centre longitudinal outcome study of patients with severe AS listed for either surgical (SAVR) or transcatheter aortic valve intervention (TAVI) at six cardiothoracic centres, survival was assessed and stratified by SVi. Patients underwent preprocedural echocardiography and CMR between January 2003 and May 2015. Standardised core-lab analyses on pre-procedural CMR for biventricular volumes, function and scar quantification were performed. All-cause and cardiovascular mortality were tracked for a minimum of two years after AVR. Results A total of 674 patients with severe AS (age 75 ± 14years; 63% male, aortic valve area 0.4 ± 0.1 cm2/m2) were included. Patients with low SVi by CMR Conclusion SVi by CMR is an independent predictor of cardiovascular mortality. Mortality hazard increases progressively below a SVi of 50mL/m2. Abstract Figure 1

Background: Alcoholic cardiomyopathy(ACM) is part of the non-ischaemic dilated cardiomyopathy(NI-DCM) spectrum. Little is known about cardiovascular magnetic resonance(CMR) features in ACM patients. The aim of this study is to describe CMR findings and their prognostic impact in ACM patients. Methods: Consecutive ACM patients evaluated in five referral CMR centres from January 2005 to December 2018 were enrolled. CMR findings and their prognostic value were compared to idiopathic NI-DCM(iNI-DCM) patients. The main outcome was a composite of death/heart transplantation/life-threatening arrhythmias. Results: Overall 114 patients (52 with ACM and 62 with iNI-DCM) were included. ACM patients were more often males compared to iNI-DCM (90% vs 64%, respectively, p ≤ 0.001) and were characterized by a more pronounced biventricular adverse remodelling than iNI-DCM, i.e. lower LVEF (31 ± 12% vs 38 ± 11% respectively, p = 0.001) and larger left ventricular end-diastolic volume (116 ± 40 ml/m2 vs 67 ± 20 ml/m2 respectively, p < 0.001). Similarly to iNI-DCM, late gadolinium enhancement (LGE), mainly midwall, was present in more than 40% of ACM patients but, conversely, it was not associated with adverse outcome(p = 0.15). LGE localization was prevalently septal (87%) in ACM vs lateral in iNI-DCM(p < 0.05). Over a median follow-up of 42 months [Interquartile Range 24–68], adverse outcomes were similar in both groups(p = 0.67). Conclusions: ACM represents a specific phenotype of NI-DCM, with severe morpho-functional features at the onset, but similar long-term outcomes compared to iNI-DCM. Despite the presence and pattern of distribution of LGE was comparable, ACM and iNI-DCM showed a different LGE localization, mostly septal in ACM and lateral in iNI-DCM, with different prognostic impact.

Cardiovascular magnetic resonance (MR) is a multiparametric, non-ionizing, non-invasive imaging technique, which represents the imaging gold standard to study cardiac anatomy, function and tissue characterization. Faced with a wide range of clinical application, in this review we aim to provide a comprehensive guide for clinicians about MR safety, contraindications and image quality. Starting from the physical interactions of the static magnetic fields, gradients and radiofrequencies with the human body, we will describe the most common metal and electronic devices which are allowed (MR-safe), allowed under limited conditions (MR-conditional) or contraindicated (MR-unsafe). Moreover, some conditions potentially affecting image quality and patient comfort will be mentioned, including arrhythmias, claustrophobia, and poor breath-hold capacity. Finally, we will discuss the pharmacodynamics and pharmacokinetics of current gadolinium-based contrast agents, their contraindications and their potential acute and chronic adverse effects, as well as the safety issue concerning the use of vasodilating/inotropic agents in stress cardiac MR.

Background Myocardial fibrosis is a hallmark of hypertrophic cardiomyopathy (HCM). Cardiac magnetic resonance (CMR) detects replacement fibrosis (RF) through late gadolinium enhancement (LGE) and interstitial fibrosis (IF) in apparently unscarred myocardium by T1 mapping-derived increased extracellular volume (ECV). Differently from LGE, to date only few small studies have explored the clinical significance of IF in HCM and a correlation between IF and diastolic dysfunction (DD) has been proposed. However, DD detection is challenging in this population since the accuracy of standard echocardiographic parameters is controversial, especially in presence of left ventricular outflow tract obstruction (LVOTO). Left atrial (LA) dysfunction is associated with high left ventricular (LV) filling pressures and may represent an early marker of DD in HCM. Purpose To explore the correlation between IF and LA dysfunction in HCM patients with preserved systolic function. Methods 93 consecutive HCM patients with preserved EF underwent a standard CMR scan. Semi-automatic threshold-based quantification of ventricular volumes, function and mass was performed. LA volumes (LAV) and function were evaluated by CMR feature-tracking (FT) analysis. The three atrial phasic functions were analyzed: (i) passive strain (εe), (ii) active strain (εa) and (iii) total strain (εs). LGE was quantified using the standard deviations (SDs) method (≥4 SDs). IF was assessed by T1 mapping-derived ECV quantification in remote myocardium (r-ECV). A matched group of 15 healthy subjects (HS) served as controls. Results Compared to HS, HCM patients showed increased LAV (LAV max: HS 39±9ml, HCM 59±20 ml; LAV min: HS 16±4 ml, HCM 34±17 ml; p0.05). HCM patients with increased r-ECV showed significantly impaired LA function in terms of all three strain parameters vs. normal r-ECV group (HCM r-ECV Conclusions In HCM patients increased r-ECV correlates with LA dysfunction, hinting towards a possible role for IF in determining altered LV relaxation and DD. LA strain in controls and HCM ECV groups Funding Acknowledgement Type of funding source: None

Background Ischemic cardiomyopathy (ICM) is often associated with negative LV remodelling after myocardial infarction, sometimes resulting in impaired LV function and dilation (iDCM). 4D Flow CMR has been recently exploited to assess intracardiac hemodynamic changes in presence of LV remodelling. Purpose To quantify 4D Flow intracardiac kinetic energy (KE) and viscous energy loss (EL) and investigate their relation with LV dysfunction and remodelling. Methods Patients with prior anterior myocardial infarction underwent a CMR study with 4D Flow sequences acquisition; they were divided into ICM (n=10) and iDCM (n=10, EDV>208 ml and EF Results Both LV volume and EF significantly differ (P Conclusions 4D Flow analyses effectively mapped post-ischemic LV energetic changes, highlighting the disproportionate intraventricular EL relative to produced KE; preliminary good correlation between LV energetic changes and NT-proBNP will deserve further investigation in order to contribute to early detection of heart failure. Funding Acknowledgement Type of funding source: Public grant(s) – National budget only. Main funding source(s): Italian Ministry of Health

Aims Fabry cardiomyopathy is characterized by glycosphingolipid storage and increased myocardial trabeculation has also been demonstrated. This study aimed to explore by cardiac magnetic resonance whether myocardial trabecular complexity, quantified by endocardial border fractal analysis, tracks phenotype evolution in Fabry cardiomyopathy. Methods and results Study population included 20 healthy controls (12 males, age 32±9) and 45 Fabry patients divided into three groups: 15 left ventricular hypertrophy (LVH)-negative patients with normal T1 (5 males, age 28±13; Group 1); 15 LVH-negative patients with low T1 (9 males, age 33±9.6; Group 2); 15 LVH-positive patients (11 males, age 53.5±9.6; Group 3). Trabecular fractal dimensions (Dfs) (total, basal, mid-ventricular, and apical) were evaluated on cine images. Total Df was higher in all Fabry groups compared to controls, gradually increasing from controls to Group 3 (1.27±0.02 controls vs. 1.29±0.02 Group 1 vs. 1.30±0.02 Group 2 vs. 1.34±0.02 Group 3; P Conclusion Fabry cardiomyopathy is characterized by a progressive increase in Df of endocardial trabeculae together with shortening of T1 values. Myocardial trabeculation is increased before the presence of detectable sphingolipid storage, thus representing an early sign of cardiac involvement.

Anderson-Fabry disease (AFD) cardiomyopathy is characterized by glycosphingolipid (Gb3) storage in all cellular components, with consequent left ventricular hypertrophy (LVH). Gb3 accumulation also involves atrial myocytes ([1][1]), ultimately leading to left atrial (LA) enlargement and reduced

Background The right ventricle (RV) is relatively understudied and often not routinely assessed in aortic stenosis (AS). However, there are several potential reasons for its importance. RV function is sensitive to left-sided afterload changes which can result in pulmonary hypertension (PH) in severe AS. PH is also a recognised predictor of poor prognosis in AS, but RV afterload and function can be difficult to assess. Cardiovascular magnetic resonance (CMR) may reveal unrecognised RV dysfunction and simultaneously evaluate other prognostic markers in AS. Purpose To investigate preoperative RV function assessed by CMR in severe AS and its association with mortality after aortic valve replacement (AVR). Methods 674 severe AS patients listed for either surgical or percutaneous AVR at six cardiothoracic centres underwent preoperative CMR (for ventricular function, mass and scar) along with echocardiography for valve severity. Scans were core-lab analysed for LV and RV volumes, function and scar quantification. Eight patients were excluded due to inadequate RV image quality for a total of 666 patients finally included. All-cause mortality was tracked for a minimum of 2 years after AVR. Results 107 (16%) of severe AS undergoing invasive AVR had a RV ejection fraction (RVEF) Cox and Kaplan-Meier for all-cause death Conclusion One out of 6 patients with severe AS undergoing valve replacement manifests a reduction in RV function detectable by CMR. Those with RV dysfunction (RVEF Acknowledgement/Funding British Heart Foundation and National Institute of Health Research

Background Heart involvement represents the main cause of death in Fabry Disease (FD), thus its early detection is important to define the optimal therapeutic strategy. Recently, a disproportionate increase in myocardial trabeculation has been described in FD by cardiac magnetic resonance (CMR), even in early (prehypertrophic) stage of the disease. In addition, CMR with T1 mapping can identity the presence of myocardial sphingolipid storage (causing lowering of native T1 values) in more than 50% of FD patients with no LVH. However, it is not clear whether a relationship exists between trabecular complexity and sphingolipid storage in FD. Aim To explore the association between myocardial trabecular complexity, quantified by endocardial border fractal analysis, and sphingolipid storage, described by CMR T1 mapping, in different stages of Fabry cardiomyopathy. Methods Study population included 60 subjects: 15 FD patients with no detectable signs of cardiac involvement (no LVH, normal T1; 2 M, age 30.6±14; Group 1); 15 FD patients with early sphingolipid storage (no LVH, low T1; 9 M, age 33±9.6; Group 2); 15 FD patients with LVH (11 M, age 53.5±9.6; Group 3); 15 healthy controls (9 M, age 34±10). Patients and controls underwent CMR with T1 mapping; disease severity was quantified using Mainz Severity Score Index (MSSI). Myocardial trabecular fractal dimension was evaluated, blinded to patients'characteristics, on short axis cine images using the Image J dedicated plug-in FracLac, deriving the following parameters: total, basal, mid-ventricular and apical fractal dimensions. Results Total fractal dimension was higher in all Fabry groups compared to controls. Indeed, a gradient of total fractal dimension was observed, with this parameter gradually increasing from healthy controls to Groups 3 (1.27±0.02 in controls vs 1.29±0.02 in Group 1 vs 1.30±0.02 in Group 2 vs 1.34±0.02 in Group 3; p Conclusion Cardiac involvement in FD is characterized by a progressive increase in fractal dimension of endocardial trabeculae (Figure 1C). Both total and basal myocardial trabeculation are increased in Fabry patients even before the presence of detectable sphingolipid storage, thus representing a very early sign of cardiac involvement.

Background: In prehypertrophic Fabry disease, low myocardial T1 values, reflecting sphingolipid storage, are associated with early structural and ECG changes. The correlations between T1 values and functional parameters have not been explored. Furthermore, the potential prognostic role of T1 in predicting disease worsening is still unknown. Methods: ECG, 2D echocardiography, cardiopulmonary test, and cardiac magnetic resonance were performed in 44 Fabry patients without left ventricular hypertrophy (35.7±14.5 years, 68.2% females). After a 12-month follow-up, clinical stability was evaluated using Fabry Stabilization Index. Results: At baseline, T1 values showed a negative correlation with left ventricular mass ( r =−0.79; P r =−0.79; P r =−0.54; P r =−0.49; P r =−0.61; P 20%) at follow-up. Higher left ventricular wall thickness (odds ratio, 2.61; CI, 1.04–6.57; P =0.04), left atrial volume (odds ratio, 1.24; CI, 1.02–1.51; P =0.03), and lower T1 values (odds ratio, 0.98; CI, 0.96–0.99; P =0.03) at baseline were independently associated with clinical worsening at follow-up. Conclusions: In prehypertrophic Fabry disease, low T1 values correlate with early electrocardiographic, morphological cardiac changes, and worsening of global disease severity but are not associated with functional abnormalities. The presence of low T1 values is a risk factor for disease worsening, thus representing a potential new tool in prognostic stratification and therapeutic approach.

Background The hemodynamic definitions of pulmonary hypertension (PH) in left heart disease have recently been refined to better match the characteristics required to reflect the presence of pulmonary vascular disease. Accordingly, we tested the hypothesis that abnormalities in the stiffness of pulmonary circulation would persist after heart transplantation in patients with combined post-capillary and pre-capillary PH (Cpc-PH) in contrast to those with isolated post-capillary PH (Ipc-PH). Methods We retrospectively analyzed right heart hemodynamics in a cohort of 295 consecutive patients with heart failure and advanced left ventricular systolic dysfunction (LVSD) before and 1 year after heart transplantation. Results According to their baseline hemodynamic profile, patients were classified as: 75 Cpc-PH, 111 Ipc-PH, and 98 without PH (no-PH), and 11 pre-capillary PH. One year after heart transplantation, pulmonary artery pressures, pulmonary vascular resistance and cardiac index normalized in all patients regardless of the baseline hemodynamic profile. However, pulmonary arterial compliance remained lower in Cpc-PH patients (from 1.6±1.2 at baseline to 3.7±1.4 ml/mmHg at 1 year) than in Ipc-PH (from 1.2±2.0 to 4.4±2.3 ml/mmHg) and no-PH patients (from 3.7±2.0 to 4.5±1.8 ml/mmHg); (adjusted p = 0.03 Ipc-PH vs. Cpc-PH INT

Contrast-enhanced cardiovascular magnetic resonance plays a pivotal role in diagnostic and prognostic definition of less common pathologies such as Anderson–Fabry disease, cardiac amyloidosis, left ventricular non-compaction, sarcoidosis, and cardiac iron loading. In recent years, new cardiovascular magnetic resonance techniques, such as T1 and T2 mapping, have provided powerful tools for the study of these pathologies, especially those with a hypertrophic phenotype.

Blood is generally modeled as a Newtonian fluid, assuming a standard and constant viscosity; however, this assumption may not hold for the highly pulsatile and recirculating intracavitary flow in the left ventricle (LV), hampering the quantification of fluid dynamic indices of potential clinical relevance. Herein, we investigated the effect of three viscosity models on the patient-specific quantification of LV blood energetics, namely on viscousenergy loss(EL), from 4D Flow magnetic resonance imaging: I) Newtonian with standard viscosity (3.7 cP), II) Newtonian with subject-specific hematocrit-dependent viscosity, III) non-Newtonian accounting for the effect of hematocrit and shear rate. Analyses were performed on 5 controls and 5 patients with cardiac light-chainamyloidosis. In Model II, viscosity ranged between 3.0 (−19%) and 4.3 cP (+16%), mildly deviating from the standard value. In the non-Newtonian model, this effect was emphasized: viscosity ranged from 3.2 to 6.0 cP, deviating maximally from the standard value in low shear rate (i.e., −1) regions. This effect reflected onELquantifications: in particular, as compared to Model I, Model III yielded markedly higherELvalues (up to +40%) or markedly lower (down to −21%) for subjects with hematocrit higher than 39.5% and lower than 30%, respectively. Accounting for non-Newtonian blood behavior on a patient-specific basis may enhance the accuracy of intracardiac energetics assessment by 4D Flow, which may be explored as non-invasive index to discriminate between healthy and pathologic LV.

Objectives Anomalous aortic origin of the coronary artery can be associated with sudden cardiac death and ischemic events. Anatomic static characteristics mainly dictated surgical indications, although adverse events are usually related to dynamic physical effort. We developed a computational model able to simulate anomalous coronary behavior, and we aimed to assess its clinical applicability and to investigate coronary characteristics at increasing loading stress conditions. Methods We selected 5 patients with anomalous aortic origin of the coronary artery and 5 control subjects. For each of them, we construct a 3-dimensional model resembling the aortic root and coronary arteries based on 25 parameters obtained from computed tomography. Structural finite element analysis simulations were run to simulate pressure increasing in the aortic root during exercise (+40 mm Hg, +100 mm Hg with respect baseline condition, assumed at 80 mm Hg) and investigate coronary lumen characteristics. Results The 25 parameters were obtainable in all subjects with a consistent interobserver agreement. In control subjects, the right coronary artery had a more significant lumen expansion at loading conditions compared with anomalous aortic origin of coronary artery (6%-19.2% vs 1.8%-8.1%, P = .008), which also showed an inability to expand within the intramural segment. Conclusions The proposed anomalous aortic origin of coronary artery model is able to represent the pathogenic disease mechanism after being populated with patient-specific data. It can assess the impaired expansion of anomalous right coronary at loading conditions, a process that cannot be quantified in any clinical set-up. This first clinical application showed promising results on quantifying pathological behavior, potentially helping in patient-specific risk stratification.