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Background. Hypertrophic cardiomyopathy (HCM) primarily affects the left ventricle (LV) sparing the right ventricle (RV) in vast majority of cases. However, several studies employing CMR have revealed that myocardial hypertrophy may also involve the RV.Aim of the study. To assess RV size and function in a large prospectively cohort of HCM patients and to evaluate whether these parameters in association with other MR findings can predict cardiac eventsMethods. Two participating centers prospectively included patients with known or suspected HCM between 2011 and 2017. CMR studies were performed with three different scanners. Outcome measures were a composite of ventricular arrhythmias, hospitalization for HF and cardiac death.Results. Of 607 consecutive patients with known or suspected HCM, 315 had complete follow-up information (mean 65 ± 20 months). Among them, 115 patients developed major cardiac events (MACE) during follow-up. At CMR evaluation, patients with events had higher left atrium (LA) diameter (41.5 ± 8 mm vs. 37.17 ± 7.6 mm, p Conclusions Anatomic and functional RV anomalies detected and characterized with CMR may have may have a major role in predicting the prognosis of HCM patients.

Systemic sclerosis (SSc) is a severe connective tissue disease characterized by diffuse vascular damage and aberrant activation of immune system, resulting in inflammation and fibrosis of skin and internal organs, including the heart. Cardiac involvement is frequent in SSc, even though often unrecognized due to the occult nature at early stages and to the lack of a defined diagnostic algorithm. Once clinically evident, heart involvement is associated with a poor prognosis, representing the leading cause of death in about one third of SSc patients. Thus, its early recognition and monitoring are of crucial importance to allow a prompt therapeutic intervention and to improve patients' outcomes. Cardiac Magnetic Resonance (CMR) is a non-invasive, non-radiating imaging technique of great importance for the assessment of cardiovascular system, and represents the modality of choice for the morpho-functional and structural characterization of the heart. In SSc, CMR allows a precise definition of biventricular and biatrial size and function, and a detailed tissue characterization. CMR has been therefore extensively proposed in SSc as a non-invasive diagnostic tool to characterize heart involvement, particularly myocardial involvement. In this review, we summarize the most recent evidences to support the use of CMR in SSc as an important tool to recognize and characterize scleroderma heart disease. Furthermore, the unmet needs and the future perspectives of a CMR-based approach for the early detection of SSc heart involvement are discussed.

Background: Hospital mortality and admission to the Intensive Care Unit (ICU) are markers of disease severity in COVID-19 patients. Cardiovascular co-morbidities are one of the Academic Editor: Atsushi Tanaka Received: 17 June 2022 Accepted: 12 July 2022 Published: 15 July 2022 Publisher’s Note: MDPI stays neutral with regard to jurisdictional claims in published maps and institutional affiliations. Copyright: © 2022 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https:// creativecommons.org/licenses/by/ 4.0/). main determinants of negative outcomes. In this study we investigated the impact of cardiovascular co-morbidities on mortality and admission to the ICU in first-wave COVID-19 patients. Methods: Amulticenter, retrospective, cohort study. A total of 1077 patients were analyzed for mortality and ICU admission. Cardiovascular risk factors were explored as determinants of the outcomes after correction for other confounders. Results: In the multivariable model, after correction for age, only a history of heart failure remained independently associated (p = 0.0013) with mortality (hazard ratio 2.22, 95% confidence interval 1.37 to 3.62). Age showed a mortality risk increase of 8% per year (hazard ratio 1.08, 95% confidence interval 1.05 to 1.10, p = 0.001). The transition from ward to the ICU had, as a single determinant, the age, but in a reversed fashion (hazard ratio 0.96, 95% confidence interval 0.94 to 0.98, p = 0.0002). Conclusions: Once adjusted for the main determinant of mortality (age) heart failure only remained independently associated with mortality. Admission to the ICU was less likely for elderly patients. This may reflect the catastrophic impact of the first wave of COVID-19 pandemic in terms of ICU bed availability in Lombardy, leading to a selection process for ICU admission.

Aims Cardiovascular sequelae may occur in patients recovered from COVID-19. Recent studies have detected a considerable incidence of subclinical myocardial dysfunction – assessed with speckle-tracking echocardiography – and of long-COVID symptoms in these patients. This study aimed to define the long-term prognostic role of subclinical myocardial dysfunction and long-COVID condition in patients recovered from COVID-19 pneumonia. Methods and Results We prospectively followed-up 110 patients hospitalized at our Institution due to COVID-19 pneumonia in April 2020 and then recovered from SARS-CoV-2 infection. A 6-month clinical and echocardiographic evaluation was performed, followed by a 21-month clinical follow-up. The primary outcome was major adverse cardiovascular events (MACE), a composite of myocardial infarction, stroke, heart failure hospitalization and all-cause mortality. A subclinical myocardial dysfunction – defined as an impairment of left ventricular global longitudinal strain (≥ -18%) - was identified at 6-month follow-up in 37 patients (34%), was associated with an increased risk of long-term MACE with a good discriminative power (AUC: 0.73) and resulted a strong independent predictor of extended MACE in a multivariate regression analysis (OR 9.29, 95%CI 2.20–39.3, p=0.002). Long-COVID condition was not associated with a worse long-term prognosis, instead. Conclusion In patients recovered from COVID-19 pneumonia, a subclinical myocardial dysfunction is present in one third of the whole population at 6-month follow-up and is associated with a higher risk of MACE at long-term follow-up. Speckle-tracking echocardiography is a promising tool to optimise the risk-stratification in patients recovered from COVID-19 pneumonia, while the definition of a long-COVID condition has not prognostic relevance.

Given the highly variable clinical phenotype of Coronavirus disease 2019 (COVID-19), a deeper analysis of the host genetic contribution to severe COVID-19 is important to improve our understanding of underlying disease mechanisms. Here, we describe an extended GWAS meta-analysis of a well-characterized cohort of 3,260 COVID-19 patients with respiratory failure and 12,483 population controls from Italy, Spain, Norway and Germany/Austria, including stratified analyses based on age, sex and disease severity, as well as targeted analyses of chromosome Y haplotypes, the human leukocyte antigen (HLA) region and the SARS-CoV-2 peptidome. By inversion imputation, we traced a reported association at 17q21.31 to a highly pleiotropic ∼0.9-Mb inversion polymorphism and characterized the potential effects of the inversion in detail. Our data, together with the 5th release of summary statistics from the COVID-19 Host Genetics Initiative, also identified a new locus at 19q13.33, including NAPSA, a gene which is expressed primarily in alveolar cells responsible for gas exchange in the lung., Andre Franke and David Ellinghaus were supported by a grant from the German Federal Ministry of Education and Research (01KI20197), Andre Franke, David Ellinghaus and Frauke Degenhardt were supported by the Deutsche Forschungsgemeinschaft Cluster of Excellence “Precision Medicine in Chronic Inflammation” (EXC2167). David Ellinghaus was supported by the German Federal Ministry of Education and Research (BMBF) within the framework of the Computational Life Sciences funding concept (CompLS grant 031L0165). David Ellinghaus, Karina Banasik and Søren Brunak acknowledge the Novo Nordisk Foundation (grant NNF14CC0001 and NNF17OC0027594). Tobias L. Lenz, Ana Teles and Onur Özer were funded by the Deutsche Forschungsgemeinschaft (DFG, German Research Foundation), project numbers 279645989; 433116033; 437857095. Mareike Wendorff and Hesham ElAbd are supported by the German Research Foundation (DFG) through the Research Training Group 1743, "Genes, Environment and Inflammation". This project was supported by a Covid-19 grant from the German Federal Ministry of Education and Research (BMBF; ID: 01KI20197). Luca Valenti received funding from: Ricerca Finalizzata Ministero della Salute RF2016-02364358, Italian Ministry of Health ""CV PREVITAL – strategie di prevenzione primaria cardiovascolare primaria nella popolazione italiana; The European Union (EU) Programme Horizon 2020 (under grant agreement No. 777377) for the project LITMUS- and for the project ""REVEAL""; Fondazione IRCCS Ca' Granda ""Ricerca corrente"", Fondazione Sviluppo Ca' Granda ""Liver-BIBLE"" (PR-0391), Fondazione IRCCS Ca' Granda ""5permille"" ""COVID-19 Biobank"" (RC100017A). Andrea Biondi was supported by the grant from Fondazione Cariplo to Fondazione Tettamanti: "Biobanking of Covid-19 patient samples to support national and international research (Covid-Bank). This research was partly funded by a MIUR grant to the Department of Medical Sciences, under the program "Dipartimenti di Eccellenza 2018–2022". This study makes use of data generated by the GCAT-Genomes for Life. Cohort study of the Genomes of Catalonia, Fundació IGTP. IGTP is part of the CERCA Program / Generalitat de Catalunya. GCAT is supported by Acción de Dinamización del ISCIIIMINECO and the Ministry of Health of the Generalitat of Catalunya (ADE 10/00026); the Agència de Gestió d’Ajuts Universitaris i de Recerca (AGAUR) (2017-SGR 529). Marta Marquié received research funding from ant PI19/00335 Acción Estratégica en Salud, integrated in the Spanish National RDI Plan and financed by ISCIIISubdirección General de Evaluación and the Fondo Europeo de Desarrollo Regional (FEDER-Una manera de hacer Europa")., Beatriz Cortes is supported by national grants PI18/01512. Xavier Farre is supported by VEIS project (001-P-001647) (cofunded by European Regional Development Fund (ERDF), “A way to build Europe”). Additional data included in this study was obtained in part by the COVICAT Study Group (Cohort Covid de Catalunya) supported by IsGlobal and IGTP, EIT COVID-19 Rapid Response activity 73A and SR20-01024 La Caixa Foundation. Antonio Julià and Sara Marsal were supported by the Spanish Ministry of Economy and Competitiveness (grant numbers: PSE-010000-2006-6 and IPT-010000-2010-36). Antonio Julià was also supported the by national grant PI17/00019 from the Acción Estratégica en Salud (ISCIII) and the FEDER. The Basque Biobank is a hospitalrelated platform that also involves all Osakidetza health centres, the Basque government's Department of Health and Onkologikoa, is operated by the Basque Foundation for Health Innovation and Research-BIOEF. Mario Cáceres received Grants BFU2016-77244-R and PID2019-107836RB-I00 funded by the Agencia Estatal de Investigación (AEI, Spain) and the European Regional Development Fund (FEDER, EU). Manuel Romero Gómez, Javier Ampuero Herrojo, Rocío Gallego Durán and Douglas Maya Miles are supported by the “Spanish Ministry of Economy, Innovation and Competition, the Instituto de Salud Carlos III” (PI19/01404, PI16/01842, PI19/00589, PI17/00535 and GLD19/00100), and by the Andalussian government (Proyectos Estratégicos-Fondos Feder PE-0451-2018, COVID-Premed, COVID GWAs). The position held by Itziar de Rojas Salarich is funded by grant FI20/00215, PFIS Contratos Predoctorales de Formación en Investigación en Salud. Enrique Calderón's team is supported by CIBER of Epidemiology and Public Health (CIBERESP), "Instituto de Salud Carlos III". Jan Cato Holter reports grants from Research Council of Norway grant no 312780 during the conduct of the study. Dr. Solligård: reports grants from Research Council of Norway grant no 312769. The BioMaterialBank Nord is supported by the German Center for Lung Research (DZL), Airway Research Center North (ARCN). The BioMaterialBank Nord is member of popgen 2.0 network (P2N). Philipp Koehler has received non-financial scientific grants from Miltenyi Biotec GmbH, Bergisch Gladbach, Germany, and the Cologne Excellence Cluster on Cellular Stress Responses in Aging-Associated Diseases, University of Cologne, Cologne, Germany. He is supported by the German Federal Ministry of Education and Research (BMBF)., Oliver A. Cornely is supported by the German Federal Ministry of Research and Education and is funded by the Deutsche Forschungsgemeinschaft (DFG, German Research Foundation) under Germany's Excellence Strategy – CECAD, EXC 2030 – 390661388. The COMRI cohort is funded by Technical University of Munich, Munich, Germany. Genotyping was performed by the Genotyping laboratory of Institute for Molecular Medicine Finland FIMM Technology Centre, University of Helsinki. This work was supported by grants of the Rolf M. Schwiete Stiftung, the Saarland University, BMBF and The States of Saarland and Lower Saxony. Kerstin U. Ludwig is supported by the German Research Foundation (DFG, LU-1944/3-1). Genotyping for the BoSCO study is funded by the Institute of Human Genetics, University Hospital Bonn. Frank Hanses was supported by the Bavarian State Ministry for Science and Arts. Part of the genotyping was supported by a grant to Alfredo Ramirez from the German Federal Ministry of Education and Research (BMBF, grant: 01ED1619A, European Alzheimer DNA BioBank, EADB) within the context of the EU Joint Programme – Neurodegenerative Disease Research (JPND). Additional funding was derived from the German Research Foundation (DFG) grant: RA 1971/6-1 to Alfredo Ramirez. Philip Rosenstiel is supported by the DFG (CCGA Sequencing Centre and DFG ExC2167 PMI and by SH state funds for COVID19 research). Florian Tran is supported by the Clinician Scientist Program of the Deutsche Forschungsgemeinschaft Cluster of Excellence “Precision Medicine in Chronic Inflammation” (EXC2167). Christoph Lange and Jan Heyckendorf are supported by the German Center for Infection Research (DZIF). Thorsen Brenner, Marc M Berger, Oliver Witzke und Anke Hinney are supported by the Stiftung Universitätsmedizin Essen. Marialbert Acosta-Herrera was supported by Juan de la Cierva Incorporacion program, grant IJC2018-035131-I funded by MCIN/AEI/10.13039/501100011033. Eva C Schulte is supported by the Deutsche Forschungsgemeinschaft (DFG; SCHU 2419/2-1).

Aims Mitral annulus disjunction (MAD) has been associated with sudden cardiac death in selected patients with arrhythmic presentation, while its clinical significance in unselected cohorts remains unknown. Our purpose was to assess the prevalence and clinical significance of MAD in consecutive patients referred to cardiovascular-magnetic-resonance (CMR). Methods and results Our population included 103 consecutive patients undergoing CMR at our Institution, between August and September 2021. MAD was defined as a ≥ 1 mm atrial displacement of the mitral leaflet hinge point in standard long-axis cine images during end-systole. MAD analysis was performed in 97 patients (feasibility = 94%) and resulted positive in 49 (51%). MAD—patients were more often males (75% vs. 57%; P = 0.045) and affected by ischaemic (35% vs. 12%, P = 0.01) and non-ischaemic cardiomyopathy (38% vs. 16%, P = 0.026) compared to MAD+ patients. No significant differences were found in terms of age, history of ventricular arrhythmias, bi-ventricular and bi-atrial volumes, bi-ventricular ejection fraction, native T1 and T2 mapping values, extracellular volume, and prevalence of late gadolinium enhancement (P > 0.05 for all) between MAD + vs. MAD—patients. MAD extent was higher in patients with mitral valve prolapse (MVP; n = 7), (3.5 ± 1.5 mm in MVP+ vs. 2.0 ± 1.0 mm in MVP– patients; P = 0.004). No significant differences were conversely found in MAD extent between patients with and without ventricular arrhythmias (2.5 ± 1.1 mm vs. 2.3 ± 1.1 mm; P = 0.815). Conclusions Our findings suggest a high prevalence of MAD in unselected cohorts of patients, with no clinical significance. Prospective studies are needed to further elucidate the interplay between MAD and malignant ventricular arrhythmias in unselected cohorts of patients.

Aims ST segment elevation is an electrocardiogram (ECG) finding first of all suggestive of acute coronary syndrome (ACS). However, there are other causes of ST segment elevation that seem to not have any relationship with coronary artery disease (CAD). One of these is the so-called ‘Spiked Helmet Sign’ (SHS), an ECG pattern characterized by upslope of ST segment before R wave onset that is reported to be found in critical illness and is associated with negative outcomes. Methods and results A 14-years-old boy came comatose (Glasgow Coma Scale = 4) to the Emergency Room after being run over by a car. He was immediately intubated and mechanically ventilated. Baseline ECG was reported to be normal, showing an incomplete right bundle branch block and a slightly prolonged QTc interval. CT-scan showed subarachnoid haemorrhage and multiple skull fractures requiring decompressive craniectomy. During hospitalization the patient developed marked hypotension complicated by rise of inflammation indexes and 12 lead ECG revealed new-onset of diffuse ST-segment elevation with spike-and-dome appearance mainly in lateral precordial leads, while lead II and V2 didn’t show any ST-segment alteration. Transthoracic echocardiogram was reported to be normal. Due to critical conditions and low likelihood of CAD, angiography wasn’t performed. The clinical course was worsened by occurrence of hyperkalaemia, acute kidney injury, and multiple episodes of ventricular tachycardia evolving in pulselessness electrical activity which required advanced life support. The patient subsequently died due to multi-organ failure, without the possibility of escalation therapy due to his status. SHS is an emerging ECG sign that is reported to be associated with critical conditions (mechanical ventilation, sepsis, bowel perforation) and in the majority of cases it is not related to CAD. It usually occurs in inferior leads (especially in case of abdominal disease) and in precordial leads (pneumothorax, aortic dissection, mechanical ventilation). Our patient had features consistent with previous cases reported in literature and, even if he experienced several episodes of cardiac arrest and blood exams revealed elevated values of cardiac troponin, the typical ECG pattern and the normal echocardiogram suggest SHS instead of ACS, avoiding unnecessary percutaneous coronary intervention. As reported in literature, even if in our case the appearance of this sign was associated with poor prognosis. Conclusions SHS mainly occurs in critically ill patients and is associated with death and poor outcomes. The potential pathophysiological mechanisms are still unclear. It is important to promptly recognize this pattern and differentiate between other causes of ST-segment elevation to select the appropriate therapy according to the setting. This is the first case-report among Italian hospitals of SHS.

Hypertrophic cardiomyopathy (HCM) is a genetic cardiac disease that presents with cardiac hypertrophy. HCM phenocopies are clinical conditions that are phenotypically undistinguishable from HCM, but with a different underlying etiology. Cardiac tumors are rare entities that can sometimes mimic HCM in their echocardiographic appearance, thus representing an example of HCM phenocopy. At present, only case reports of tumoral HCM phenocopies can be found in literature. In this systematic review, we analyzed all the published cases in which a cardiac tumor mimicked HCM to the point of misleading the diagnosis, providing a structured overview of the currently available evidence on this topic.