Novel Insights into Non-Invasive Diagnostic Techniques for Cardiac Amyloidosis: A Critical Review.

Cardiac amyloidosis (CA) is a cardiac storage disease caused by the progressive extracellular deposition of misfolded proteins in the myocardium. Despite the increasing interest in this pathology, it remains an underdiagnosed condition. Non-invasive diagnostic techniques play a central role in the suspicion and detection of CA, also thanks to the continuous scientific and technological advances in these tools. The 12-lead electrocardiography is an inexpensive and reproducible test with a diagnostic accuracy that, in some cases, exceeds that of imaging techniques, as recent studies have shown. Echocardiography is the first-line imaging modality, although none of its parameters are pathognomonic. According to the 2023 ESC Guidelines, a left ventricular wall thickness ≥ 12 mm is mandatory for the suspicion of CA, making this technique crucial. Cardiac magnetic resonance provides high-resolution images associated with tissue characterization. The use of contrast and non-contrast sequences enhances the diagnostic power of this imaging modality. Nuclear imaging techniques, including bone scintigraphy and positron emission tomography, allow the detection of amyloid deposition in the heart, and their role is also central in assessing the prognosis and response to therapy. The role of computed tomography was recently evaluated by several studies, above in population affected by aortic stenosis undergoing transcatheter aortic valve replacement, with promising results. Finally, machine learning and artificial intelligence-derived algorithms are gaining ground in this scenario and provide the basis for future research. Understanding the new insights into non-invasive diagnostic techniques is critical to better diagnose and manage patients with CA and improve their survival.

Diagnostic value of late gadolinium enhancement at cardiovascular magnetic resonance to distinguish arrhythmogenic right ventricular cardiomyopathy from differentials.

BACKGROUND: While late gadolinium enhancement (LGE) is proposed as a diagnostic criterion for arrhythmogenic right ventricular cardiomyopathy (ARVC), the potential of LGE to distinguish ARVC from differentials remains unknown. We aimed to assess the diagnostic value of LGE for ARVC diagnosis. METHODS: We included 132 subjects (60% male, 47 ± 11 years) who had undergone cardiac magnetic resonance imaging with LGE assessment for ARVC or ARVC differentials. ARVC was diagnosed as per 2010 Task Force Criteria (n = 55). ARVC differentials consisted of familial/genetic dilated cardiomyopathy (n = 25), myocarditis (n = 13), sarcoidosis (n = 20), and amyloidosis (n = 19). The diagnosis of all differentials was based on the most current standard of reference. The presence of LGE was evaluated using a 7-segment right ventricle (RV) and 17-segment left ventricle (LV) model. Subsequently, we assessed LGE patterns for every patient individually for fulfilling LV- and/or RV-LGE per Padua criteria, independent of their clinical diagnosis (i.e. phenotype). Diagnostic values were analyzed using sensitivity and specificity for any RV-LGE, any LV-LGE, RV-LGE per Padua criteria, and prevalence graphs for LV-LGE per Padua criteria. The optimal integration of LGE for ARVC diagnosis was determined using classification and regression tree analysis. RESULTS: One-third (38%) of ARVC patients had RV-LGE, while half (51%) had LV-LGE. RV-LGE was less frequently observed in ARVC vs non-ARVC patients (38% vs 58%, p = 0.034) leading to a poor discriminatory potential (any RV-LGE: sensitivity 38%, specificity 42%; RV-LGE per Padua criteria: sensitivity 36%, specificity 44%). Compared to ARVC patients, non-ARVC patients more often had LV-LGE (91% vs 51%, p < 0.001) which was also more globally distributed (median 9 [interquartile range (IQR): 3-13] vs 0 [IQR: 0-3] segments, p < 0.001). The absence of anteroseptal and absence of extensive (≥5 segments) mid-myocardial LV-LGE, and absence of moderate (≥2 segments) mid-myocardial LV-LGE predicted ARVC with good diagnostic performance (sensitivity 93%, specificity 78%). CONCLUSION: LGE is often present in ARVC differentials and may lead to false positive diagnoses when used without knowledge of LGE patterns. Moderate RV-LGE without anteroseptal and mid-myocardial LV-LGE is typically observed in ARVC.

Cardiac Magnetic Resonance to Predict Cardiac Mass Malignancy: The CMR Mass Score.

BACKGROUND: Multimodality imaging is currently suggested for the noninvasive diagnosis of cardiac masses. The identification of cardiac masses' malignant nature is essential to guide proper treatment. We aimed to develop a cardiac magnetic resonance (CMR)-derived model including mass localization, morphology, and tissue characterization to predict malignancy (with histology as gold standard), to compare its accuracy versus the diagnostic echocardiographic mass score, and to evaluate its prognostic ability. METHODS: Observational cohort study of 167 consecutive patients undergoing comprehensive echocardiogram and CMR within 1-month time interval for suspected cardiac mass. A definitive diagnosis was achieved by histological examination or, in the case of cardiac thrombi, by histology or radiological resolution after adequate anticoagulation treatment. Logistic regression was performed to assess CMR-derived independent predictors of malignancy, which were included in a predictive model to derive the CMR mass score. Kaplan-Meier curves and Cox regression were used to investigate the prognostic ability of predictors. RESULTS: In CMR, mass morphological features (non-left localization, sessile, polylobate, inhomogeneity, infiltration, and pericardial effusion) and mass tissue characterization features (first-pass perfusion and heterogeneity enhancement) were independent predictors of malignancy. The CMR mass score (range, 0-8 and cutoff, ≥5), including sessile appearance, polylobate shape, infiltration, pericardial effusion, first-pass contrast perfusion, and heterogeneity enhancement, showed excellent accuracy in predicting malignancy (areas under the curve, 0.976 [95% CI, 0.96-0.99]), significantly higher than diagnostic echocardiographic mass score (areas under the curve, 0.932; P=0.040). The agreement between the diagnostic echocardiographic mass and CMR mass scores was good (κ=0.66). A CMR mass score of ≥5 predicted a higher risk of all-cause death (P<0.001; hazard ratio, 5.70) at follow-up. CONCLUSIONS: A CMR-derived model, including mass morphology and tissue characterization, showed excellent accuracy, superior to echocardiography, in predicting cardiac masses malignancy, with prognostic implications.

Adding Surgical Edge-to-Edge Mitral Valve Repair to Myectomy in Hypertrophic Obstructive Cardiomyopathy: Long-Term Functional and Echocardiographic Outcome.

This study evaluates the early and long-term clinical and echocardiographic outcome of edge-to-edge (E2E) mitral valve repair (MVr) concomitant to septal myectomy (SM) in patients with symptomatic hypertrophic obstructive cardiomyopathy (HOCM). A retrospective single-center analysis was performed of patients who underwent isolated SM or SM with E2E MVr from 2011 to 2022. Exclusion criteria were primary mitral valve (MV) disease or concomitant valve surgery. Early and long-term safety, functional and echocardiographic outcomes were compared between groups. Between January 2011 and April 2022, 76 consecutive patients underwent SM for HOCM: 42 patients (55%) underwent SM without additional E2E MVr (Group 1) and 34 patients (45%) underwent SM with additional E2E MVr (Group 2). At latest follow-up, 87% of patients were in New York Heart Association (NYHA) class I-II with no significant differences in NYHA class between groups. Incidence of safety events was comparable between groups. Echocardiographic relief of left ventricular outflow tract (LVOT) obstruction was comparable at early follow-up (P = 0.68), with a significant but small difference in maximum LVOT pressure gradient at latest follow-up in favor of E2E MVr (P = 0.04). Furthermore, patients who underwent SM with E2E MVr showed less residual systolic anterior motion at early and latest follow-up (P = 0.020; P = 0.178). Reintervention on the MV was absent in both groups at 1 year and equally low at follow-up (P = 0.27). This study demonstrates that adding E2E MVr to septal myectomy is as safe as isolated myectomy for the treatment of HOCM. Moreover, the addition of E2E MVr is associated with similar excellent functional improvement and freedom from MV reintervention.

Does the AVNeo valve reduce wall stress on the aortic wall? A cardiac magnetic resonance analysis with 4D-flow for the evaluation of aortic valve replacement with the Ozaki technique.

OBJECTIVES: Aortic valve neocuspidalization aims to replace the 3 aortic cusps with autologous pericardium pre-treated with glutaraldehyde, and it is a surgical alternative to the classical aortic valve replacement (AVR). Image-based patient-specific computational fluid dynamics allows the derivation of shear stress on the aortic wall [wall shear stress (WSS)]. Previous studies support a potential link between increased WSS and histological alterations of the aortic wall. The aim of this study is to compare the WSS of the ascending aorta in patients undergoing aortic valve neocuspidalization versus AVR with biological prostheses. METHODS: This is a prospective nonrandomized clinical trial. Each patient underwent a 4D-flow cardiac magnetic resonance scan after surgery, which informed patient-specific computational fluid dynamics models to evaluate WSS at the ascending aortic wall. The adjusted variables were calculated by summing the residuals obtained from a multivariate linear model (with ejection fraction and left ventricle outflow tract-aorta angle as covariates) to the mean of the variables. RESULTS: Ten patients treated with aortic valve neocuspidalization were enrolled and compared with 10 AVR patients. The aortic valve neocuspidalization group showed a significantly lower WSS in the outer curvature segments of the proximal and distal ascending aorta as compared to AVR patients (P = 0.0179 and 0.0412, respectively). WSS levels remained significantly lower along the outer curvature of the proximal aorta in the aortic valve neocuspidalization population, even after adjusting the WSS for the ejection fraction and the left ventricle outflow tract-aorta angle [2.44 Pa (2.17-3.01) vs 1.94 Pa (1.72-2.01), P = 0.02]. CONCLUSIONS: Aortic valve neocuspidalization hemodynamical features are potentially associated with a lower WSS in the ascending aorta as compared to commercially available bioprosthetic valves.

Quantification of extracellular volume with cardiac computed tomography in patients with dilated cardiomyopathy.

BACKGROUND: Cardiac computed tomography (CCT) was recently validated to measure extracellular volume (ECV) in the setting of cardiac amyloidosis, showing good agreement with cardiovascular magnetic resonance (CMR). However, no evidence is available with a whole-heart single source, single energy CT scanner in the clinical context of newly diagnosed left ventricular dysfunction. Therefore, the aim of this study was to test the diagnostic accuracy of ECVCCT in patients with a recent diagnosis of dilated cardiomyopathy, having ECVCMR as the reference technique. METHODS: 39 consecutive patients with newly diagnosed dilated cardiomyopathy (LVEF <50%) scheduled for clinically indicated CMR were prospectively enrolled. Myocardial segment evaluability assessment with each technique, agreement between ECVCMR and ECVCCT, regression analysis, Bland-Altman analysis and interclass correlation coefficient (ICC) were performed. RESULTS: Mean age of enrolled patients was 62 â€‹± â€‹11 years, and mean LVEF at CMR was 35.4 â€‹± â€‹10.7%. Overall radiation exposure for ECV estimation was 2.1 â€‹± â€‹1.1 â€‹mSv. Out of 624 myocardial segments available for analysis, 624 (100%) segments were assessable by CCT while 608 (97.4%) were evaluable at CMR. ECVCCT demonstrated slightly lower values compared to ECVCMR (all segments, 31.8 â€‹± â€‹6.5% vs 33.9 â€‹± â€‹8.0%, p â€‹< â€‹0.001). At regression analysis, strong correlations were described (all segments, r â€‹= â€‹0.819, 95% CI: 0.791 to 0.844). On Bland-Altman analysis, bias between ECVCMR and ECVCCT for global analysis was 2.1 (95% CI: -6.8 to 11.1). ICC analysis showed both high intra-observer and inter-observer agreement for ECVCCT calculation (0.986, 95%CI: 0.983 to 0.988 and 0.966, 95%CI: 0.960 to 0.971, respectively). CONCLUSIONS: ECV estimation with a whole-heart single source, single energy CT scanner is feasible and accurate. Integration of ECV measurement in a comprehensive CCT evaluation of patients with newly diagnosed dilated cardiomyopathy can be performed with a small increase in overall radiation exposure.

Successful sequential liver and hematopoietic stem cell transplantation in a patient with Fanconi anemia.

BACKGROUND: In Fanconi anemia bone marrow failure is the major cause of morbidity and mortality and hematopoietic stem cell transplantation represents the only curative treatment. Liver disease, in terms of elevated liver function tests, as well as benign and malignant liver tumors, occurs especially in case of androgen treatment. We report a unique case of a child with Fanconi anemia with FANCD2 mutation who developed neonatal cryptogenic liver cirrhosis and bone marrow failure. The child successfully underwent sequential liver transplantation and hematopoietic stem cell transplantation in the first 2 years of life. Nineteen months after hematopoietic stem cell transplantation and 30 months after liver transplantation, the patient is clinically well with normal hematopoietic function and excellent liver function. CONCLUSION: This is the first FA patient who successfully received sequential LT and HSCT highlighting that successful sequential transplantation is feasible in Fanconi anemia patients.

What's behind your eosinophilic myocarditis? A case of Churg-Strauss syndrome diagnosed during acute heart failure.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare multisystem disorder; cardiac involvement may include eosinophilic myocarditis. A 67-year-old woman presented with 1-week history of dyspnoea and orthopnoea. She had a history of adult-onset asthma and peripheral eosinophilia. The investigations showed T-wave inversion on lateral leads, peripheral eosinophilia, elevated troponin and BNP values, and severe biventricular systolic dysfunction with diffuse hypokinesia and apical akinesia. Computed tomography excluded coronary disease and showed bilateral basal ground-glass opacities, air-space consolidation, and bilateral reticular-nodular pattern. Cardiac magnetic resonance findings were compatible with active myocardial inflammation. An endomyocardial biopsy (EMB) confirmed the diagnosis of eosinophilic myocarditis, and a therapy with oral corticosteroids and heart failure medications was started.

The Role of Cardiovascular Magnetic Resonance in Patients with Mitral Regurgitation.

The 2019 Global Burden of Disease (GBD) study estimated that there were approximately 24.2 million people affected worldwide by degenerative mitral regurgitation (MR), resulting in 34,200 deaths. After aortic stenosis, MR is the most prevalent VHD in Europe and the second-most common VHD to pose indications for surgery in western countries. Current ESC and AHA/ACC guidelines for the management of VHD emphasize the importance of an integrative approach for the assessment of MR severity, which is of paramount importance in dictating the timing for surgery. Transthoracic echocardiography (TTE) and transesophageal echocardiography (TEE) are the first-line imaging modalities; however, despite the technological advancement, sometimes, the final diagnosis on the degree of the disease may still be challenging. In the last 20 years, CMR has emerged as a robust technique in the assessment of patients with cardiac disease, and, recently, its role is gaining more and more importance in the field of VHD. In fact, CMR is the gold standard in the assessment of cardiac volumes, and it is possible to accurately evaluate the regurgitant volume. The purpose of this review is to outline the current state-of-the-art management of MR by using Cardiac Magnetic Resonance (CMR).

Multimodality Imaging of the Neglected Valve: Role of Echocardiography, Cardiac Magnetic Resonance and Cardiac Computed Tomography in Pulmonary Stenosis and Regurgitation.

The pulmonary valve (PV) is the least imaged among the heart valves. However, pulmonary regurgitation (PR) and pulmonary stenosis (PS) can occur in a variety of patients ranging from fetuses, newborns (e.g., tetralogy of Fallot) to adults (e.g., endocarditis, carcinoid syndrome, complications of operated tetralogy of Fallot). Due to their complexity, PR and PS are studied using multimodality imaging to assess their mechanism, severity, and hemodynamic consequences. Multimodality imaging is crucial to plan the correct management and to follow up patients with pulmonary valvulopathy. Echocardiography remains the first line methodology to assess patients with PR and PS, but the information obtained with this technique are often integrated with cardiac magnetic resonance (CMR) and computed tomography (CT). This state-of-the-art review aims to provide an updated overview of the usefulness, strengths, and limits of multimodality imaging in patients with PR and PS.

The Role of Cardiac Magnetic Resonance in Aortic Stenosis and Regurgitation.

Cardiac magnetic resonance (CMR) imaging is a well-set diagnostic technique for assessment of valvular heart diseases and is gaining ground in current clinical practice. It provides high-quality images without the administration of ionizing radiation and occasionally without the need of contrast agents. It offers the unique possibility of a comprehensive stand-alone assessment of the heart including biventricular function, left ventricle remodeling, myocardial fibrosis, and associated valvulopathies. CMR is the recognized reference for the quantification of ventricular volumes, mass, and function. A particular strength is the ability to quantify flow, especially with new techniques which allow accurate measurement of stenosis and regurgitation. Furthermore, tissue mapping enables the visualization and quantification of structural changes in the myocardium. In this way, CMR has the potential to yield important prognostic information predicting those patients who will progress to surgery and impact outcomes. In this review, the fundamentals of CMR in assessment of aortic valve diseases (AVD) are described, together with its strengths and weaknesses. This state-of-the-art review provides an updated overview of CMR potentials in all AVD issues, including valve anatomy, flow quantification, ventricular volumes and function, and tissue characterization.

Appropriateness criteria for the use of cardiac computed tomography, SIC-SIRM part 2: acute chest pain evaluation; stent and coronary artery bypass graft patency evaluation; planning of coronary revascularization and transcatheter valve procedures; cardiomyopathies, electrophysiological applications, cardiac masses, cardio-oncology and pericardial diseases evaluation.

In the past 20 years, cardiac computed tomography (CCT) has become a pivotal technique for the noninvasive diagnostic workup of coronary and cardiac diseases. Continuous technical and methodological improvements, combined with fast growing scientific evidence, have progressively expanded the clinical role of CCT. Randomized clinical trials documented the value of CCT in increasing the cost-effectiveness of the management of patients with acute chest pain presenting in the emergency department, also during the pandemic. Beyond the evaluation of stents and surgical graft patency, the anatomical and functional coronary imaging have the potential to guide treatment decision-making and planning for complex left main and three-vessel coronary disease. Furthermore, there has been an increasing demand to use CCT for preinterventional planning in minimally invasive procedures, such as transcatheter valve implantation and mitral valve repair. Yet, the use of CCT as a roadmap for tailored electrophysiological procedures has gained increasing importance to assure maximum success. In the meantime, innovations and advanced postprocessing tools have generated new potential applications of CCT from the simple coronary anatomy to the complete assessment of structural, functional and pathophysiological biomarkers of cardiac disease. In this complex and revolutionary scenario, it is urgently needed to provide an updated guide for the appropriate use of CCT in different clinical settings. This manuscript, endorsed by the Italian Society of Cardiology (SIC) and the Italian Society of Medical and Interventional Radiology (SIRM), represents the second of two consensus documents collecting the expert opinion of cardiologists and radiologists about current appropriate use of CCT.

Cardiac MRI after first episode of acute pericarditis: A pilot study for better identification of high risk patients.

BACKGROUND: Cardiac magnetic resonance (CMR) was proposed as an accurate non-invasive tool to evaluate pericardial inflammation. Aim of the present study was to evaluate the role of CMR early in the course of the first episode of acute pericarditis. MATERIAL AND METHODS: A clinical registry of consecutive patients who underwent clinical indicated CMR due to pericardial disease from January 2014 to January 2020 was screened. We analyzed patients with the clinical diagnosis of first episode of acute pericarditis needing hospitalization less than 7 days before CMR. Outcome measures were obtained using a single combined end-point, defined as pericardial event, including all the following: recurrent pericarditis, chronic constrictive pericarditis, surgery for pericardial disease. RESULTS: Twenty-six patients meet the study criteria and were enrolled. A mean follow-up of 34 ± 7 months was obtained and a second episode of pericardial event were recorded in 9 patients. At multivariate analysis adjusted for propensity score, based on clinical significative variable (younger age and higher CRP) the association between pericardial inflammation identified by CMR (positive late gadolinium enhancement on pericardium) and recurrence of pericardial events was confirmed [OR (95%CI) 8.94 (1.74-45.80), p = 0.008]. CONCLUSION: Pericardial inflammation identified by CMR, with LGE images, has a prognostic value independently from clinical and bio-humoral variables.

Advances in Multimodality Cardiovascular Imaging in the Diagnosis of Heart Failure With Preserved Ejection Fraction.

Heart failure with preserved ejection fraction (HFpEF) is a syndrome defined by the presence of heart failure symptoms and increased levels of circulating natriuretic peptide (NP) in patients with preserved left ventricular ejection fraction and various degrees of diastolic dysfunction (DD). HFpEF is a complex condition that encompasses a wide range of different etiologies. Cardiovascular imaging plays a pivotal role in diagnosing HFpEF, in identifying specific underlying etiologies, in prognostic stratification, and in therapeutic individualization. Echocardiography is the first line imaging modality with its wide availability; it has high spatial and temporal resolution and can reliably assess systolic and diastolic function. Cardiovascular magnetic resonance (CMR) is the gold standard for cardiac morphology and function assessment, and has superior contrast resolution to look in depth into tissue changes and help to identify specific HFpEF etiologies. Differently, the most important role of nuclear imaging [i.e., planar scintigraphy and/or single photon emission CT (SPECT)] consists in the screening and diagnosis of cardiac transthyretin amyloidosis (ATTR) in patients with HFpEF. Cardiac CT can accurately evaluate coronary artery disease both from an anatomical and functional point of view, but tissue characterization methods have also been developed. The aim of this review is to critically summarize the current uses and future perspectives of echocardiography, nuclear imaging, CT, and CMR in patients with HFpEF.

Preoperative Ozaki technique measures on tridimensional engineered root.

BACKGROUND: The aortic valve neocuspidalization (AVNeo) is an innovative surgical technique aiming at the reconstruction of the aortic valve using autologous pericardium. One of the main criticisms to AVNeo is the longer duration of the aortic clamping time (ACT) as compared to standard aortic valve replacement due to the sizing of the valve neocusps. METHODS: We retrospectively enrolled 30 consecutives patients underwent AVNeo. For each patient we developed a 3D aortic root model (ARM) based on CT-scan datasets. We retrospectively compared the leaflets measurements performed during surgery with those obtained on the corresponding ARMs. RESULTS: In 100% of cases no difference between the in vitro and in vivo measurements exceeded the acceptable error limit of 2 â€‹mm. The correlation of each single in vitro versus in vivo measurements demonstrates a strong coincidence between the two different methods of sizing (r â€‹> â€‹0,9, p â€‹< â€‹.0001). By analyzing the data considering the annulus perimeter and not the single cusp size, the perfect coincidence was to be found in 89.9% with a slight acceptable discrepancy (2 â€‹mm on total) in the remaining 10.1%. CONCLUSIONS: 3D-ARMs, printed from CT-scan, represent a reproducible process to obtain overlapping cusp sizes compared to those measured in-vivo, possibly reducing the ACT.

Prevalence and prognosis of pericardial effusion in patients affected by pectus excavatum: A case-control study.

BACKGROUND: The presence of pectus excavatum(PEX) has been occasionally associated with pericardial effusion. Aim of the present study was to compare incidence and prognosis of pericardial effusion in a group of unselected patients with PEX vs a control group. METHODS: From a prospective registry of consecutive patients who underwent chest CT for cardiovascular disease, subjects with a radiological diagnosis of PEX were retrospectively identified (cases); from the same registry patients (controls) without rib cage abnormalities were randomly selected, until a 1:2 ratio was reached. The presence of pericardial effusion at CT was quantified. Follow-up was obtained for a composite end-point: cardiac tamponade, need for pericardiocentesis, need for cardiac surgery for relapsing pericardial effusion. RESULTS: A total of 43 patients with PEX (20 females) and a control group of 86 cases (31 females) without rib cage abnormalities were identified. Pericardial effusion evaluated at CT was significatively more prevalent in patients with PEX vs control group, 37.2% vs 13.9% (p < 0.001), respectively; four patients with PEX (9.3%) had at least moderate pericardial effusion vs no subjects among the controls (p = 0.004). PEX diagnosis was significantly associated to pericardial effusion at multi-variate analysis (OR95%CI 10.91[3.47-34.29], p < 0.001). At a mean follow-up of 6.5 ± 3.4 years no pericardial events were recorded. CONCLUSION: Our findings support the higher prevalence of pericardial effusion in patients with PEX when compared to a control group. The absence of adverse pericardial events at follow-up suggest the good prognosis of these effusions, that in the appropriate clinical setting might not be considered "idiopathic".

Additional diagnostic value of cardiac magnetic resonance feature tracking in patients with biopsy-proven arrhythmogenic cardiomyopathy.

BACKGROUND: We aim to evaluate the value of Cardiac magnetic resonance (CMR) feature tracking (CMR-FT) in addition to Task Force Criteria(TFC) in patients with (arrhythmogenic cardiomyopathy) AC biopsy-proved. METHODS: Thirty-five patients with AC histologically proven who performed CMR with late gadolinium enhancement (LGE) acquisition were enrolled. The study population was divided in Group1 (negative CMR TFC and LV ejection fraction≥55%) and Group2 (positive CMR TFC and/or LVEF<55%) and compared to an age and gender-matched control group. CMR datasets of all patients were analyzed to calculate LV indexed end-diastolic (LVEDi) and end-systolic (LVESi) volumes and RV indexed end-diastolic (RVEDi) and end-systolic (RVESi) volumes, both LV ejection fraction (LVEF) and RV ejection fraction (RVEF). Moreover, LV and RV global longitudinal (GLS), circumferential (GCS) and radial (GRS) strain were measured. RESULTS: The AC patients showed both higher LVEDi (p:0.002) and RVEDi (p:0.017) and lower LVEF (p: 0.016) as compared to control patients. Moreover, AC patients showed impaired LV-GLS (p < 0.001), LV-GRS (p < 0.001), LV-GCS (p < 0.001) and RV-GRS (p:0.026) as compared to control subjects. Group1 patients showed a significant reduction of LV-GRS (p < 0.05) and LV-GCS p < 0.01) as compared to control subjects. At univariate analysis LV-GCS was the most discriminatory parameter between Group1 vs heathy subjects with an optimal cut-off of -15.8 (Sensitivity: 74%; Specificity: 10%). CONCLUSIONS: In patients with AC biopsy-proven, CMR-FT could improve the diagnostic yield in the subset of patients who results negative for imaging TFC criteria resulting as useful gatekeeper for indication of myocardial biopsy in case of equivocal clinical and imaging presentation.