Search

Ischemic cardiomyopathy is a significant cause of mortality and morbidity, with peculiar needs for accurate diagnostic and prognostic characterization. Cardiac magnetic resonance (CMR) can help to satisfy these requirements by allowing a comprehensive evaluation of myocardial function, perfusion and tissue composition, with a demonstrated utility in guiding clinical management of patients with known or suspected ischemic cardiomyopathy. When compared with alternative non-invasive imaging modalities, such as stress echocardiography and nuclear techniques, CMR is able to provide accurate (function and perfusion) or peculiar (tissue characterization) information on cardiac pathophysiology, while avoiding exposition to ionizing radiations and overcoming limitations related to the quality of the imaging window. In particular, stress perfusion CMR showed to be accurate, safe, cost-effective, and clinically valuable as a non-invasive test for detecting severity and distribution of myocardial ischemia. In many circumstances, however, local availability of the technique, together with procedural costs, and scanning and post-processing time duration still limit the use of CMR in clinical routine. In the current review, we focused on clinical applications of CMR in ischemic cardiomyopathy. The consolidated role of the technique is described by illustrating both standard and advanced sequences that constitute the current body of a dedicated CMR examination. Ongoing developments and potential future diagnostic and prognostic applications of CMR when assessing ischemic cardiomyopathy are also discussed, with a focus on artificial intelligence-based implementations proposed for refining the efficiency of CMR analysis and reporting.

Purpose Heart failure (HF) is a primary cause of morbidity and mortality worldwide, with significant impact on life quality and extensive healthcare costs. Assessment of myocardial sympathetic innervation function plays a central role in prognosis assessment in HF patients. The aim of this review is to summarize the most recent evidence regarding the clinical applications of iodine-123 metaiodobenzylguanidine (123I-MIBG) imaging in patients with HF and related comorbidities. Methods A comprehensive literature search was conducted on PubMed and Web of Science databases. Articles describing the impact of 123I-MIBG imaging on HF and related comorbidities were considered eligible for the review. Results We collected several data reporting that 123I-MIBG imaging is a safe and non-invasive tool to evaluate dysfunction of cardiac sympathetic neuronal function and to assess risk stratification in HF patients. HF is frequently associated with comorbidities that may affect cardiac adrenergic innervation. Furthermore, HF is frequently associated with comorbidities and chronic conditions, such as diabetes, obesity, kidney disease and others, that may affect cardiac adrenergic innervation. Conclusion Comorbidities and chronic conditions lead to more severe impairment of sympathetic nervous system in patients with HF, with a negative impact on disease progression and outcome. Cardiac imaging with 123I-MIBG can be a useful tool to reduce morbidity and prevent adverse events in HF patients.

Background In patients affected by heart failure (HF) with reduced ejection fraction (HFrEF), pharmacological treatments have been proven to alleviate symptoms and improve prognosis, while no treatment other than sodium-glucose co-transporter-2 inhibitors have demonstrated significant effects in HF with preserved ejection fraction (HFpEF). Left atrium decompression devices (LADd) have been recently investigated as a new interventional approach in patients with HFpEF. Objectives To assess the efficacy of LADd on soft endpoints in HF patients across the spectrum of ejection fraction. Methods PubMed and Web of Science were searched without restrictions from inception to 28 May 2022 to identify valuable articles. The studies that met the inclusion criteria were analyzed. The prespecified main outcomes were the change from baseline in 6-min walking distance (6MWD), NYHA class and health-related quality of life (HRQoL). Secondary outcomes were reduction in HF hospitalizations, echocardiographic, and hemodynamic parameters. Results Eleven studies, with a total of 547 patients, were included. LADd significantly improved 6MWD by 43.95 m (95% CI 29.64–58.26 m), decreased NYHA class by 0.93 (95% CI 1.20–0.67), and improved HRQoL questionnaire by 20.45 points (95% CI 13.77–27.14) with better results for all outcomes in patients with lower EFs. Conclusion The present meta-analysis suggests that LADd are favorable in improving 6MWD, NYHA class, and HRQoL in HF across a wide spectrum of ejection fraction, with better outcomes in patients with lower EFs. Trial registration CRD42022336077, URL: https://www.crd.york.ac.uk/prospero/display_record.php?RecordID=336077.

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.

A 72-year-old woman who had been diagnosed with pulmonary emphysema and permanent atrial fibrillation was admitted to our emergency department with signs and symptoms of heart failure (NYHA class III). Transthoracic echocardiography (TTE) revealed dilatation of both right and left atria, dilatation and systolic dysfunction of the right ventricle (RV) and severe pulmonary hypertension. Combined severe mitral and tricuspid regurgitation was also noted and, after clinical stabilization, transesophageal echocardiography (TEE) was performed to better assess valvular anatomy and function. TEE revealed clear evidence of a pre-tricuspid left-to-right shunt with signs of significant RV volume overload, but with difficulties in defining the characteristics of a suspected atrial septal defect (ASD). CMR confirmed the presence of a large coronary sinus ASD (Panel A) as responsible for a significant left-to-right shunt (Qp/Qs ∼ 3,5, measured by phase-contrast imaging after correcting for the combined mitral and tricuspid regurgitation; Panel B), as well as severe RV dilatation and dysfunction. Pulmonary venous drainage was normal. Persistent left superior vena cava (SVC; Panel C) draining directly into the left atrium (LA) and a completely unroofed coronary sinus were unveiled. The extremely rare combination of an unroofed coronary sinus with abnormal systemic venous drainage from double SVC circulation (usually as a persistent left SVC) is also known as Raghib syndrome (or Raghib complex). In the ASD with completely unroofed coronary sinus there is no common wall separating the coronary sinus from the LA, making the depiction of the shunt anatomy particularly difficult to assess with echocardiography. When unrecognized in the perinatal age, clinical natural course of Raghib syndrome can vary based on the severity of RV volume overload, and late (or very late) clinical presentations have been rarely reported. CMR, with its large field-of-view capability and multiparametric approach, provides accurate definition of cardiac and great vessels anatomy and function allowing the full non-invasive depiction of most congenital abnormalities. In many cases, multimodality imaging is the key to reach a full depiction of these complex conditions, particularly when adult patients with possible comorbidities need to be evaluated.

A 20-year-old woman with a history of pneumonia, polycystic ovarian syndrome and ovarian teratoma asportation presented with chest pain and worsening dyspnoea. No prodromal or concomitant infective symptoms were reported. On admission, vital parameters were within normal limits, ECG was unremarkable and laboratory tests revealed high white blood cells count and increased hs-cTnI. Transthoracic echocardiography showed diffuse left ventricular (LV) dysfunction, with akinesia of basal segments and a severely reduced ejection fraction (LVEF = 27%). A full heart failure drug treatment was gradually introduced and, after two weeks, a cardiac magnetic resonance (CMR) was performed. CMR showed significant but incomplete recovery of LV systolic function (LVEF = 40%), extensive areas of myocardial oedema with diffuse LV involvement on STIR (short tau inversion recovery) images (Fig. A) and, almost with the same LV distribution, considerable late gadolinium enhancement (LGE) with subepicardial/intramyocardial distribution on post-Gadolinium images (Fig. B-C); all these findings strongly suggested an acute myocarditis with diffuse LV myocardial damage. Definitive diagnosis was made through endo-myocardial biopsy, that revealed significant infiltration of lymphocytes, without additional specific elements. Patient was sent home with an optimized drug therapy for heart failure with reduced ejection fraction and a close clinical follow-up was prescribed. Roughly a month after discharge the patient presented with sustained ventricular tachycardia that required prompt admission and DC shock. Hence, an MR-conditional transvenous ICD was implanted without complications. After 7 months from the acute event, patient underwent a follow-up CMR that, despite artifacts produced by ICD generator and intracavitary leads, still displayed reduced LV systolic function (LVEF = 41%) and extensive LGE with a non-ischemic “ring-like” LV pattern (Fig. D-E). Finally, patient has been put on the waiting list for heart transplantation and, in order to exclude left-dominant arrhythmogenic cardiomyopathy (LDAC), genetic tests have been ordered, but are still pending. The present case emphasizes the importance of CMR for the initial assessment of patients with acute myocarditis: beyond the crucial role in evaluating extension and severity of the inflammatory process at the myocardial level, it can identify specific features (i.e. ring-like or antero-septal patterns of LGE involvement), more frequently reported in high-risk subgroups, such as LDAC, with suggestion for further investigations (including genetic tests for desmosomal mutations). During follow-up, CMR retains a central role to evaluate possible changes in ventricular function and in extension of myocardial tissue involvement, useful to guide the cardiologist to choose for a more tailored management and define a more precise timing for further follow-up. Progressive expansion of CMR indications is helping to understand the pathophysiology of many cardiac conditions (including myocarditis and myocarditis-like entities) and many of the previous limitations to its wider clinical use have progressively been settled. From the present case, it can be appreciated how, even in presence of an implanted cardiac device, precious diagnostic information can still be obtained using CMR. However, to ensure safety and feasibility, MR-conditional devices need to be appropriately checked and reprogrammed before and immediately after the exam, while adapted CMR imaging protocols and adequate patient monitoring need to be applied during the scan.

BACKGROUND: The extent to which health care systems have adapted to the COVID-19 pandemic to provide necessary cardiac diagnostic services is unknown.OBJECTIVES: The aim of this study was to determine the impact of the pandemic on cardiac testing practices, volumes and types of diagnostic services, and perceived psychological stress to health care providers worldwide.METHODS: The International Atomic Energy Agency conducted a worldwide survey assessing alterations from baseline in cardiovascular diagnostic care at the pandemic's onset and 1 year later. Multivariable regression was used to determine factors associated with procedure volume recovery.RESULTS: Surveys were submitted from 669 centers in 107 countries. Worldwide reduction in cardiac procedure volumes of 64% from March 2019 to April 2020 recovered by April 2021 in high- and upper middle-income countries (recovery rates of 108% and 99%) but remained depressed in lower middle- and low-income countries (46% and 30% recovery). Although stress testing was used 12% less frequently in 2021 than in 2019, coronary computed tomographic angiography was used 14% more, a trend also seen for other advanced cardiac imaging modalities (positron emission tomography and magnetic resonance; 22%-25% increases). Pandemic-related psychological stress was estimated to have affected nearly 40% of staff, impacting patient care at 78% of sites. In multivariable regression, only lower-income status and physicians' psychological stress were significant in predicting recovery of cardiac testing.CONCLUSIONS: Cardiac diagnostic testing has yet to recover to prepandemic levels in lower-income countries. Worldwide, the decrease in standard stress testing is offset by greater use of advanced cardiac imaging modalities. Pandemic-related psychological stress among providers is widespread and associated with poor recovery of cardiac testing.

The term arrhythmogenic cardiomyopathy (ACM) describes a large spectrum of myocardial diseases characterized by progressive fibrotic or fibrofatty replacement, which gives the substrate for the occurrence of ventricular tachyarrhythmias and the development of ventricular dysfunction. This condition may exclusively affect the left ventricle, leading to the introduction of the term arrhythmogenic left ventricular cardiomyopathy (ALVC). The clinical features of ALVC are progressive fibrotic replacement with the absence or mild dilation of the LV and the occurrence of ventricular arrhythmias within the left ventricle. In 2019, the diagnostic criteria for the diagnosis of ALVC, based on family history and clinical, electrocardiographic, and imaging features, have been proposed. However, since the significant clinical and imaging overlap with other cardiac diseases, genetic testing with the demonstration of a pathogenic variant in an ACM-related gene is required for diagnostic confirmation. In ALVC, the multimodality imaging approach comprises different imaging techniques, such as echocardiography, cardiac magnetic resonance, and cardiac nuclear imaging. It provides essential information for the diagnosis, differential diagnosis, sudden cardiac death risk stratification, and management purposes. This review aims to elucidate the current role of the different multimodality imaging techniques in patients with ALVC.

Cardiovascular emergencies represent life-threatening conditions requiring a high index of clinical suspicion. In an emergency scenario, a simple stepwise biomarker/imaging diagnostic algorithm may help prompt diagnosis and timely treatment along with related improved outcomes. This article describes several clinical cases of cardiovascular emergencies, such as coronary stent thrombosis-restenosis, takotsubo syndrome, acute myocarditis, massive pulmonary embolism, type A acute aortic dissection, cardiac tamponade, and endocarditis.

Background: Mitral valve prolapse (MVP) is characterized by an abnormal movement of the valvular apparatus which may affect the papillary muscles (PMs) function and structure. Aim of the study was to investigate abnormal PM signal in MVP by using cardiac magnetic resonance imaging (MRI). Methods and Results: We enrolled 47 consecutive patients with MVP evaluated by cardiac MRI. Additional groups included healthy volunteers, patients with moderate-to-severe mitral regurgitation (not caused by MVP) and patients with hypertrophic cardiomyopathy. Visual assessment of the PM signals was carried out and the signal intensity (SI) of both the antero-lateral and postero-medial PMs was normalized by that of the left ventricular (LV) parietal myocardium. Our results show that in the MVP group only, the PM signal intensity was significantly lower compared to the one of the LV parietal myocardium. This sign did not correlate with either LV late gadolinium enhancement or positive anamnesis for significant arrhythmias. Conclusions: In MVP patients only, PM signal is significantly reduced compared to LV parietal myocardium (“darker appearance”). The described findings are not clearly related to evidence of myocardial fibrosis, as assessed by MRI, and to previous occurrence of complex ventricular arrhythmias.

Aims Glucose-lowering, glucagon-like peptide-1 (GLP-1) receptor agonists reduce incidence of major cardiovascular (CV) events in patients with Type 2 diabetes mellitus (DM). However, randomized clinical trials reported inconsistent effects on myocardial infarction (MI) and stroke, and limited data in DM patients without established CV disease (CVD). Very recently, new relevant evidence was available from additional CV outcome trials (CVOTs) that also included large subgroups of patients with DM without established CVD. Thus, the aim of this meta-analysis was to investigate the effects of GLP-1 receptor agonists on major CV events and safety in DM patients with and without established CVD. Methods and results In this trial-level meta-analysis, we analysed data from randomized placebo-controlled CVOTs assessing efficacy and safety of GLP-1 receptor agonists in adult patients with Type 2 DM. We searched PubMed, Embase, Cochrane, ISI Web of Science, SCOPUS, and clinicaltrial.gov databases for eligible trials. Of 360 articles identified and screened for eligibility, seven CVOTs were included, with an overall of 56 004 patients included. The difference in efficacy with respect to the major adverse cardiovascular events (MACE) primary endpoint (including CV mortality, non-fatal MI, and non-fatal stroke) between patients with established CVD and patients with CV risk factors only was not significant [pooled interaction effect, expressed as ratio of hazard ratio (HR) 1.06, 95% confidence interval (CI) 0.85–1.34]. In the analysis of the whole population of DM patients, GLP-1 receptor agonists showed a significant 12% reduction in the hazard of the three-point MACE composite endpoint (HR 0.88, 95% CI 0.80–0.96) and a significant reduction in the risk of CV mortality (HR 0.88, 95% CI 0.79–0.98), all-cause mortality (HR 0.89, 95% CI 0.81–0.97), fatal and non-fatal stroke (HR 0.84, 95% CI 0.76–0.94), and heart failure (HF) hospitalization (HR 0.92, 95% CI 0.86–0.97). No significant effect was observed for fatal and non-fatal MI (HR 0.91, 95% CI 0.82–1.02), although in a sensitivity analysis, based on a less conservative statistical approach, the pooled HR become statistically significant (HR 0.91, 95% CI 0.83–1.00; P = 0.039). No excess of hypoglycaemia, pancreatitis, and pancreatic cancer was observed between GLP-1 receptor agonists and placebo. Conclusion Glucagon-like peptide-1 receptor agonists significantly reduce MACE, CV and total mortality stroke, and hospitalization for HF, with a trend for reduction of MI, in patients with Type 2 DM with and without established CVD.

BACKGROUND The risk of adverse cardiovascular events in patients with acute myocarditis (AM) and desmosomal gene variants (DGV) remains unknown.OBJECTIVES The purpose of this study was to ascertain the risk of death, ventricular arrhythmias, recurrent myocarditis, and heart failure (main endpoint) in patients with AM and pathogenic or likely pathogenetic DGV.METHODS In a retrospective international study from 23 hospitals, 97 patients were included: 36 with AM and DGV (DGV[+]), 25 with AM and negative gene testing (DGV[-]), and 36 with AM without genetics testing. All patients had troponin elevation plus findings consistent with AM on histology or at cardiac magnetic resonance (CMR). In 86 patients, CMR changes in function and structure were re-assessed at follow-up.RESULTS In the DGV(+) AM group (88.9% DSP variants), median age was 24 years, 91.7% presented with chest pain, and median left ventricular ejection fraction (LVEF) was 56% on CMR (P = NS vs the other 2 groups). Kaplan-Meier curves demonstrated a higher risk of the main endpoint in DGV(+) AM compared with DGV(-) and without genetics testing patients (62.3% vs 17.5% vs 5.3% at 5 years, respectively; P < 0.0001), driven by myocarditis recurrence and ventricular arrhythmias. At follow-up CMR, a higher number of late gadolinium enhanced segments was found in DGV(+) AM. CONCLUSIONS Patients with AM and evidence of DGV have a higher incidence of adverse cardiovascular events compared with patients with AM without DGV. Further prospective studies are needed to ascertain if genetic testing might improve risk stratification of patients with AM who are considered at low risk. (J Am Coll Cardiol HF 2022;10:714-727) (c) 2022 by the American College of Cardiology Foundation.

Although in heart failure (HF) there is a strict correlation between heart and kidney, no data are available on the potential relationship in HF between renal dysfunction (RD) and the impaired sympathetic innervation. Aim of the present study was to assess the relationship between RD and cardiac sympathetic innervation in HF patients with reduced ejection fraction. Two hundred and sixty-three patients with mild-to-severe HF underwent iodine-123 meta-iodobenzylguanidine myocardial scintigraphy to assess sympathetic innervation, evaluating early and late heart-to-mediastinum (H/M) ratios and washout rate. In all patients, glomerular filtration rate (eGFR) by Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI) formula was assessed. A direct association was found between EPI-eGFR and late H/M (r = .215; P

Arrhythmogenic cardiomyopathy (ACM) is a genetically determined heart muscle disorder characterized by fibro-fatty replacement of myocardium which may affect the right ventricle (‘dominant right’ variant), the left ventricle (‘dominant left’ variant) or both (‘biventricular disease’ variant). Despite of ACM is one of the main causes of sudden cardiac death (SCD) in young people and athletes as well, the diagnosis of this complex clinical entity still remains a challenge. We report on a case of a 29 years old non-athletic woman with family history of SCD and a long-standing personal history of palpitations leading to frequent accesses to the Accident & Emergency Department. Her echocardiogram was normal while twelve leads ECG revealed negative T waves in the right precordial leads (V1–V3) and post-excitation epsilon waves. ECG Holter monitoring recorded frequent (up to 17 000 in 24 h) polymorphic premature ventricular contractions (PVCs) seldom organized in couplets or triplets. She was followed-up in our clinic and started on Propanolol 40 mg bid with slight relief of symptoms and reduction of PVCs. Cardiac magnetic resonance (CMR) showed regional dyskinesia of both ventricles and RV dilatation. Moreover, tissue characterization findings revealed the presence of diffuse subepicardial late gadolinium enhancement (LGE) of the LV. Genetic analysis was performed and a variant of uncertain significance (VUS) in Desmoplakin was identified. Non sustained monomorphic ventricular tachycardia was induced during electrophysiologic study by programmed ventricular stimulation. In light of these results, we diagnosed biventricular ACM and, after carefully discussion with the patient, a subcutaneous implantable cardioverter defibrillator (ICD) was implanted. The present case should be a persisting reminder that must be considered more than just a single diagnostic tool when dealing with arrhythmic presentations especially in young patients and the importance of contrast CMR along with genetic testing in order to aid clinicians in the demanding selection of the best candidates for ICD implantation.

The left ventricular (LV) ejection fraction (EF) is the preferred parameter applied for the non-invasive evaluation of LV systolic function in clinical practice. It has a well-recognized and extensive role in the clinical management of numerous cardiac conditions. Many imaging modalities are currently available for the non-invasive assessment of LVEF. The aim of this review is to describe their relative advantages and disadvantages, proposing a hierarchical application of the different imaging tests available for LVEF evaluation based on the level of accuracy/reproducibility clinically required.

Antiphospholipid Antibody Syndrome (APS) is a systemic autoimmune disease characterized by acquired hypercoagulability with the possible development of venous, arterial, and microvascular thrombosis. We report a rare case of Libman–Sacks tricuspid valve endocarditis in a 38-year-old pregnant woman at 15 weeks gestation with unknown primary antiphospholipid syndrome. During a routine cardiac examination and echocardiography performed for a previous episode of pleuropericarditis, a large, mobile mass with irregular edges was found at the level of the tricuspid valve. Three main differential diagnoses for intramyocardial mass were examined: tumor, infective endocarditis, and nonbacterial thrombotic endocarditis (NTBE). Cardiac magnetic resonance imaging (CMR) with contrast raised the suspicion of a thrombus. The woman was hospitalized urgently at the Cardiac Intensive Care Unit of the Federico II University Hospital, and anticoagulant and antiplatelet therapy were started. The thrombophilic screening performed and medical history confirmed the diagnosis of primary antibody syndrome (APS). A multidisciplinary consultation with obstetricians, cardiologists, anesthetists, and cardiac surgeons was required. The patient decided not to terminate the pregnancy despite the risk to her health and to undergo cardiac surgery during pregnancy. Histological examination confirmed the presence of nonbacterial thrombotic endocarditis. Weekly obstetric scans were performed after surgery to verify fetal well-being. An emergency cesarean section was performed at the 35th week of gestation due to repeated deceleration and abnormal short-term variability on c-CTG in a pregnancy complicated by fetal growth restriction and gestational hypertension. A newborn weighing 1290 g was born. She was hospitalized in Neonatal Intensive Care and discharged after two months; currently, she enjoys good health. The management of patients with antiphospholipid antibody syndrome has not yet been standardized, but there is a general consensus that patients who do not have thrombocytopenia, thromboembolic phenomena, or pregnancy should not undergo any treatment or should take only low doses of acetylsalicylic acid. In the presence of any of the above conditions, various treatment regimens have been used based on the severity and individuality of the case.

Cardiovascular magnetic resonance is increasingly used in clinical practice, as it has emerged over the years as an invaluable imaging technique for diagnosis and prognosis, with clear-cut applications in managing patients with both ischemic and non-ischemic heart disease. In this review, we focus on the evidence and clinical application of stress CMR in coronary artery disease from diagnosis to prognosis.

Background The most important determinant of long-term survival in patients with idiopathic pulmonary fibrosis is the right ventricular (RV) adaptation to the increased pulmonary vascular resistance. Our aim was to explore RV contractile reserve during stress echocardiography in early-stage IPF. Methods Fifty early-stage patients with IPF and 50 healthy control patients underwent rest and stress echocardiography, including RV two-dimensional speckle tracking echocardiography. At peak exertion, blood gas analysis and spirometry were also assessed. Results At rest, RV diameters were mildly increased in IPF; however, although RV conventional systolic function indexes were similar between the IPF and control groups, RV global longitudinal strain and RV lateral wall longitudinal strain (LWLS) were significantly reduced in the IPF cohort. During physical exercise, patients with IPF showed a reduced exercise tolerance with lower maximal workload (P o 2; P Conclusions RV myocardial dysfunction is already present at rest in early-stage IPF and worsens during exertion as detected by two-dimensional speckle-tracking echocardiography. The RV altered contractile reserve appears to be related to reduced exercise tolerability and impaired pulmonary hemodynamic.

Right heart and pulmonary circulation disorders are generally caused by right ventricle (RV) pressure overload, volume overload, and cardiomyopathy, and they are associated with distinct clinical courses and therapeutic approaches, although they often may coexist. Cardiac magnetic resonance (CMR) provides a noninvasive accurate and reproducible multiplanar anatomic and functional assessment, tissue characterization, and blood flow evaluation of the right heart and pulmonary circulation. This article reviews the current status of the CMR, the most recent techniques, the new parameters and their clinical utility in diagnosis, prognosis, and therapeutic management in the right heart and pulmonary circulation disorders.

This review describes the current role and potential future applications of cardiac magnetic resonance (CMR) for the management of heart failure (HF). CMR allows noninvasive morphologic and functional assessment, tissue characterization, blood flow, and perfusion evaluation. CMR overcomes echocardiography limitations (geometric assumptions, interobserver variability and poor acoustic window) and provides incremental information in relation to cause, prognosis, and treatment monitoring of patients with HF.

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.

Multimodality imaging is a comprehensive strategy to investigate left ventricular hypertrophy (LVH), providing morphologic, functional, and often clinical information to clinicians. Hypertrophic cardiomyopathy (HCM) is defined by an increased LV wall thickness not only explainable by abnormal loading conditions. In the context of HCM, multimodality imaging, by different imaging techniques, such as echocardiography, cardiac magnetic resonance, cardiac computer tomography, and cardiac nuclear imaging, provides essential information for diagnosis, sudden cardiac death stratification, and management. Furthermore, it is essential to uncover the specific cause of HCM, such as Fabry disease and cardiac amyloidosis, which can benefit of specific treatments. This review aims to elucidate the current role of multimodality imaging in adult patients with HCM.

Cardiovascular emergencies represent life-threatening conditions requiring a high index of clinical suspicion. In an emergency scenario, a simple stepwise biomarker/imaging diagnostic algorithm may help prompt diagnosis and timely treatment along with related improved outcomes. This article describes several clinical cases of cardiovascular emergencies, such as coronary stent thrombosis-restenosis, takotsubo syndrome, acute myocarditis, massive pulmonary embolism, type A acute aortic dissection, cardiac tamponade, and endocarditis.

Background and Aim: Echocardiography is a promising technique for the assessment of epicardial adipose tissue (EAT). Increased EAT thickness is associated with different cardiac diseases, including; coronary artery disease (CAD). Since several different echocardiographic approaches have been proposed to measure EAT, the identification of a standardized method is needed. We propose the assessment of EAT maximal thickness at the Rindfleisch fold, the reproducibility of this measurement and its correlation with EAT thickness and volume assessed at cardiac magnetic resonance (CMR). Finally, we will test the predictive role of this measurement on the presence of significant CAD. Methods and Results: In 1061 patients undergoing echocardiography, EAT thickness was measured at the level of the Rindfleisch fold. In 70 patients, we tested the relationship between echo-EAT thickness and EAT thickness and volume assessed at CMR. In 499 patients with suspected CAD, undergoing coronary artery angiography, we tested the predictive value of EAT on the presence of significant CAD. Echo-EAT thickness measurements had an excellent reliability as indicated by the inter-observer (ICC:0.97; 95% C.I. 0.96 to 0.98) and intra-observer (ICC:0.99; 95% C.I. 0.98 to 0.99) reliability rates. Echo-EAT thickness significantly correlated with CMR-EAT thickness and volume (p < 0.001). An EAT thickness value >10 mm discriminated patients with significant CAD at coronary angiography (p < 0.001). At multivariable analysis, including demographic data and cardiovascular risk factors, EAT thickness was an independent predictor of significant CAD and showed an additive predictive value over common atherosclerotic risk factors. Conclusions: Echocardiographic assessment of EAT thickness at the level of the Rindfleisch fold represents a simple and trustworthy method. An increased EAT thickness shows an additive predictive value on CAD over common atherosclerotic risk factors, thus suggesting its potential clinical use for CAD risk stratification.

Introduction The analysis of intracardiac blood flow patterns can significantly contribute to improve the understanding and treatment of cardiovascular disease. In contrast to the substantial literature on the left side of the heart, there is currently a significant lack of knowledge about the fluid mechanics of the right heart – pulmonary circulation unit (RH-PCU), both in healthy and diseased conditions. Purpose It is conjectured that computational modeling can be a key element to enhance current imaging techniques and provide quantitative insights into the unique RH-PCU biomechanics. Here we present a novel methodology that allows personalized numerical simulations of right heart flows, through a proper combination of cardiac magnetic resonance (CMR) with computational fluid dynamics (CFD). Methods and results We developed a patient-specific pipeline from medical images to computational models, as depicted in the figure. First, the RV geometry is reconstructed from time-resolved CMR cine images, comprising short-axis and longitudinal slices of the heart, where feature-tracking techniques are used to extract the motion of the RV endocardium contours. A time-continuous description of the moving geometry is obtained through an image-registration algorithm based on diffeomorphic mappings. The moving model of the RV, including the outflow tract and proximal pulmonary arteries, is finally fed to a dedicated CFD solver. The tool is able to provide a detailed description of the velocity and pressure fields inside the right ventricle and proximal pulmonary arteries during all phases of the cardiac cycle. From these fields, global hemodynamic quantities such as vortex properties, kinetic energy, pressure gradients and hemodynamic forces can be computed. Conclusions CMR-driven computational modeling of intra-ventricular flow enables a promising approach for understanding and evaluating the biomechanical environment of the right heart. This high-fidelity framework can be applied to investigate the RV response and adaptation to abnormal pressure and/or volume load conditions. It can also be used to reproduce the virtual flow that would realize in hypothetical conditions, and therefore adds predictive capabilities to modern flow imaging. The analysis may allow to determine an association between blood flow patterns and disease progression, and ultimately lead to derive and validate imaging biomarkers of clinical significance. Abstract P1449 Figure. Pipeline for patient-specific modeling

The different components of the right heart pulmonary circulation unit can be investigated by MRI and computed tomography. MRI has clear advantages over echocardiography for accurate definition of right heart function and structure and to derive functional information regarding the pulmonary vasculature. Computed tomography is superior for the assessment of parenchymal and vascular pathologies of the lung with indications in the diagnostic work-up of pulmonary hypertension, but with more limited capability to evaluate right ventricular function and in deriving pulmonary hemodynamics. Recent technical developments with these imaging modalities could allow a better evaluation of the right heart pulmonary circulation unit.

The Right Heart International Network is a multicenter international study aiming to prospectively collect exercise Doppler echocardiography tests of the right heart pulmonary circulation unit (RHPCU) in large cohorts of healthy subjects, elite athletes, and individuals at risk of or with overt pulmonary hypertension. It is going to provide standardization of exercise stress echocardiography of RHPCU and explore the full physiopathologic response.

Aims Glucagon-like peptide-1 (GLP-1) agonists improve glycaemic control in type 2 diabetes mellitus (DM). Outcome trials investigating macro and microvascular effects of GLP-1 agonists reported conflicting results. The aim of this study was to assess, in a meta-analysis, the effects of GLP-1 agonists on mortality, major nonfatal cardiovascular (CV) events, renal and retinal events. Data synthesis MEDLINE, Cochrane, ISI Web of Science, SCOPUS and ClinicalTrial.gov databases were searched for articles published until June 2017. Randomized trials enrolling more than 200 patients, comparing GLP-1 versus placebo or active treatments in patients with DM, and assessing outcomes among all-cause death, CV death, MI, stroke, HF, diabetic retinopathy and nephropathy were included. 77 randomized trials enrolling 60,434 patients were included. Compared to control, treatment with GLP-1 significantly reduced the risk of all-cause death (RR: 0.888; CI: 0.804–0.979; p = 0.018) and the risk of CV death (RR: 0.858; CI: 0.757–0.973; p = 0.017). GLP-1 agonists did not affect the risk of MI (RR: 0.917; CI: 0.830–1.014; p = 0.092) as well as the risk of stroke (RR: 0.882; CI: 0.759–1.023; p = 0.097), HF (RR: 0.967; CI: 0.803–1.165; p = 0.725), retinopathy (RR: 1.000; CI: 0.807–1.238; p = 0.997) and nephropathy (RR: 0.866; CI: 0.625–1.199; p = 0.385). Conclusions Treatment with GLP-1 agonists in DM patients is associated with a significant reduction of all cause and CV mortality.

Aim: Anderson-Fabry disease (AFD) is a hereditary disorder caused by a deficiency in the lysosomal enzyme α-galactosidase A which causes dysfunctions in multiple organ systems. Cardiac manifestation includes left ventricular hypertrophy, thickening of the valves, conduction disturbances and in the late phase, extensive areas of myocardial fibrosis with increased risk of sudden cardiac death. Case example: A case of AFD with exclusive cardiac involvement is described. During follow-up, due to the high risk of life-threatening arrhythmic events, implantation of an implantable cardioverter defibrillator is performed. Conclusion: AFD patients with advanced cardiac disease might represent a subgroup of patients who may require an implantable cardioverter defibrillator for primary prevention of sudden cardiac death.

Coarctation of the aorta is a leading cause of morbidity and mortality among adults with congenital heart disease (ACHD). Lifelong surveillance is mandatory to screen for possible long-term cardiovascular events.Left ventricular systolic dysfunction has been reported in association with recoarctation, and association with dilated cardiomyopathy (DCMP) is very rare. Herein, we report the case of a 19-year-old boy with coarctation of the aorta who complained of mild exertional dyspnea. Cardiac magnetic resonance revealed a moderately dilated, hypokinetic left ventricle (LV), with mildly reduced EF (45%), and residual isthmic coarctation was excluded. Genetic tests revealed a heterozygous missense variant in TNNT2 (NM_001001430.2): c.518G>A (p. Arg173Gln). This case highlights the role of careful history taking: a family history of cardiomyopathy should not be overlooked even when the clinical setting seems to suggest a predisposition to hemodynamic factors for LVSD.

Background The prognostic role of cardiac magnetic resonance (CMR) and late gadolinium enhancement (LGE) has not been clarified in acute myocarditis (AM) with preserved left ventricular (LV) ejection fraction (EF). Objectives This study sought to evaluate the role of CMR and LGE in the prognosis of AM with preserved LVEF. Methods This study analyzed data from ITAMY (ITalian multicenter study on Acute MYocarditis) and evaluated CMR results from 386 patients (299 male; mean age 35 ± 15 years) with AM and preserved LVEF. Clinical follow-up was performed for a median of 1,572 days. A clinical combined endpoint of cardiac death, appropriate implantable cardioverter-defibrillator firing, resuscitated cardiac arrest, and hospitalization for heart failure was used. Results Among the 374 patients with suitable images, LGE involved the subepicardial layer inferior and lateral wall in 154 patients (41%; IL group), the midwall layer of the anteroseptal wall in 135 patients (36%; AS [anteroseptal] group), and other segments in 59 patients (16%; other-LGE group), and it was absent in 26 patients (no-LGE group). The AS group had a greater extent of LGE and a higher LV end-diastolic volume index than other groups, but levels of inflammatory markers were lower than in the other groups. Kaplan-Meier curve analysis indicated that the AS group had a worse prognosis than the other groups (p Conclusions In patients with AM and preserved LVEF, LGE in the midwall layer of the AS myocardial segment is associated with a worse prognosis than other patterns of presentation.

Cardiac magnetic resonance (CMR) has emerged as a reliable and accurate diagnostic tool for the evaluation of patients with cardiac disease in several clinical settings and with proven additional diagnostic and prognostic value compared with other imaging modalities. This document has been developed by the working group on the 'application of CMR' of the Italian Society of Cardiology to provide a perspective on the current state of technical advances and clinical applications of CMR and to inform cardiologists on how to implement their clinical and diagnostic pathways with the inclusion of this technique in clinical practice. The writing committee consisted of members of the working group of the Italian Society of Cardiology and two external peer reviewers with acknowledged experience in the field of CMR.

Objectives This study investigated the efficacy and safety of novel oral anticoagulants (NOACs) in patients with atrial fibrillation (AF) and heart failure (HF) by a meta-analysis. Background AF is quite prevalent in patients with HF. Methods Four phase III clinical trials comparing NOACs to warfarin in patients with AF were included. Each patient was defined as affected by HF according to the criteria of the trial in which the patient was enrolled. Pre-specified outcomes were the composite of stroke/systemic embolism (SSE); major, intracranial, and any bleeding; and cardiovascular (CV) and all-cause death. Results A total of 55,011 patients were enrolled, 26,384 (48%) with HF, and 28,627 (52%) without HF; 27,518 receiving NOACs and 27,493 receiving warfarin (median, 70 years of age; 36% females; follow-up: 1.5 to 2.8 years). Rates of SSE (relative risk [RR]: 0.98; 95% confidence interval [CI]: 0.90 to 1.07]; p = 0.68) and major bleeding (RR: 0.95; 95% CI: 0.88 to 1.03; p = 0.21) were comparable in patients with and without HF. HF patients had reduced rates of any (RR: 0.86; 95% CI: 0.81 to 0.91; p interaction > 0.05 for each). Conclusions Patients with AF and HF had increased mortality but reduced rates of intracranial and any bleeding compared with the no-HF patients, with no differences in rates of SSE and major bleeding. NOACs significantly reduced SSE, major bleeding, and intracranial hemorrhage in HF patients. No interactions in efficacy and safety of NOACs were observed between AF patients with and without HF.

The presence of comorbidities significantly influences long-term morbidity and mortality of symptomatic and asymptomatic heart failure (HF) patients. Metabolic syndrome and diabetic cardiomyopathy are two clinical conditions that share multiple pathophysiological mechanisms and that might be both responsible for cardiac dysfunction. However, it is argued whether metabolic syndrome (MS) independently increases HF risk or the association between MS and HF merely reflects the impact of individual risk factors included in its definition on HF development. Similarly, in the context of diabetic cardiomyopathy, many aspects are still challenging starting from the definition up to the therapeutic management. In this clinical review, we focused the attention on molecular pathways, myocyte alterations, and specific patterns of metabolic syndrome and diabetic cardiomyopathy in order to better define the potential diagnostic and therapeutic approaches of these two pathological conditions.

Alterations in myocardial energy metabolism as demonstrated by magnetic resonance spectroscopy (MRS) are critically involved in heart failure development. 1H-MRS allows investigation of the role of myocardial lipid accumulation in the pathophysiology of heart failure. With 31P-MRS, the most useful parameter is the cardiac adenosine triphosphate to phosphocreatine ratio, which is typically reduced in the failing heart and correlates with cardiac functional status. Currently, MRS is mostly limited to the research setting, but recent technical and methodological developments might substantially improve its clinical applicability for initial characterization and successive monitoring of patients with heart failure.

Bicuspid aortic valve (BAV) disease is the most common congenital cardiac malformation associated with an increased lifetime risk and a high rate of surgically-relevant valve deterioration and aortic dilatation. Genomic data revealed that different genes are associated with BAV. A dominant genetic factor for the recent past was the basis to the recommendation for a more extensive aortic intervention. However very recent evidence that hemodynamic stressors and alterations of wall shear stress play an important role independent from the genetic trait led to more conservative treatment recommendations. Therefore, there is a current need to improve the ability to risk stratify BAV patients in order to obtain an early detection of valvulopathy and aortopathy while also to predict valve dysfunction and/or aortic disease development. Imaging studies based on new cutting-edge technologies, such us 4-dimensional (4D) flow magnetic resonance imaging (MRI), two-dimensional (2D) or three-dimensional (3D) speckle-tracking imaging (STI) and computation fluid dynamics, combined with studies demonstrating new gene mutations, specific signal pathways alterations, hemodynamic influences, circulating biomarkers modifications, endothelial progenitor cell impairment and immune/inflammatory response, all detected BAV valvulopathy progression and aortic wall abnormality. Overall, the main purpose of this review article is to merge the evidences of imaging and basic science studies in a coherent hypothesis that underlies and thus projects the development of both BAV during embryogenesis and BAV-associated aortopathy and its complications in the adult life, with the final goal to identifying aneurysm formation/rupture susceptibility to improve diagnosis and management of patients with BAV-related aortopathy.