Purpose: Pulmonary hypertension (PH) is a complex disease classified into five groups (I-V) by the European Society of Cardiology/European Respiratory Society (ESC/ERS) guidelines. Chest contrast-enhanced computed tomography (CECT) is crucial in the non-invasive PH assessment. This study aimed to develop a machine learning (ML)-based educational resource for classifying PH cases via CECT according to ESC/ERS groups.
View Article and Find Full Text PDFAims: Some studies about myocarditis and pericarditis following COVID-19 vaccination raised concerns worldwide. However, the heterogeneous diagnostic criteria for postvaccination inflammatory heart diseases may result in overestimating incidence rates. The aim of this multicentre Italian registry is to evaluate the impact of COVID-19 vaccines on the incidence of myocarditis and pericarditis in the Italian population.
View Article and Find Full Text PDFLast year, the European Society of Cardiology (ESC) published the first guidelines to comprehensively address the management of cardiomyopathies. This document by the Working Group on Myocardial and Pericardial Diseases of the Italian Society of Cardiology aims at highlighting the most relevant messages and novelties introduced by these guidelines for the management of patients affected by cardiomyopathies. Five main messages are summarized: the key role of the phenotype, the new classification of cardiomyopathies provided in the ESC guidelines, the importance given to new techniques such as cardiac magnetic resonance (CMR) and genetic testing, the newly provided recommendations given on sport activities and finally how the importance of follow-up evaluations is highlighted.
View Article and Find Full Text PDFJAMA Cardiol
February 2025
Importance: Filamin C truncating variants (FLNCtv) are a rare cause of cardiomyopathy with heterogeneous phenotypic presentations. Despite a high incidence of life-threatening ventricular arrhythmias and sudden cardiac death (SCD), reliable risk predictors to stratify carriers of FLNCtv are lacking.
Objective: To determine factors predictive of SCD/major ventricular arrhythmias (MVA) in carriers of FLNCtv.
Eur Heart J Cardiovasc Imaging
February 2025
Anderson-Fabry disease (AFD) is a rare genetic disease with X-linked transmission characterized by a defect in the enzyme alpha-galactosidase A (alpha-GAL), which impairs glycosphingolipid metabolism and leads to an excessive storage of globotriaosylceramide (Gb3) within lysosomes. AFD involves renal, cardiac, vascular, and nervous systems and is mainly observed in male patients with onset in childhood, although cardiac manifestation is often shown in adults. AFD cardiomyopathy is caused by the accumulation of Gb3 within myocytes first showed by left ventricular (LV) hypertrophy and diastolic dysfunction, leading to restrictive cardiomyopathy and systolic heart failure with biventricular involvement.
View Article and Find Full Text PDFBackground: Ventricular arrhythmias (VAs) frequently occur in the acute phase of myocarditis. Possible arrhythmic recurrences and the risk of sudden cardiac death (SCD) in this setting are reasons for concern, and limited data have been published to guide clinical management of these patients. The aim of the present paper is to report the incidence of major arrhythmic events, defined as sustained VA, SCD and appropriate implantable cardiac-defibrillator (ICD) treatment, in patients with acute myocarditis and ventricular arrhythmic phenotype.
View Article and Find Full Text PDFObjective: We aimed to investigate the remission rate and disease duration in idiopathic or post-cardiac injury pericarditis and risk factors for disease duration and anti-interleukin-1 (IL-1) agent discontinuation.
Methods: This was a multicenter, longitudinal, observational study including 370 patients (51.4% female).
Background: Patients with pericarditis may show elevation of C-reactive protein (CRP) and pericardial effusion at presentation. There are limited data on the prognostic implications of this inflammatory phenotype.
Objectives: Aim of the present study is to evaluate the outcome of the inflammatory phenotype in a cohort of patients with acute pericarditis.
The knowledge of pericardial diseases has now improved, including prospective and retrospective cohort studies focusing on the pathogenesis, diagnosis, treatment, and outcomes. The complex interplay between genetic predisposition (especially for autoinflammatory conditions), inflammation, and autoimmunity is now known to trigger recurrences of pericarditis. Moreover, diagnostic capabilities have improved with the implementation of multimodality imaging, particularly cardiac magnetic resonance (CMR), to detect and monitor pericardial inflammation, to allow diagnosis in more complicated cases, and tailor the duration of therapy based on objective parameters.
View Article and Find Full Text PDFJ Cardiovasc Med (Hagerstown)
December 2024
: the role of the R202Q (c.605G>A, p.Arg202Gln) missense variant of the MEFV gene has been debated as either a benign polymorphism or a potentially pathogenic mutation.
View Article and Find Full Text PDFJ Cardiovasc Med (Hagerstown)
November 2024
Aims: Presence of family cases and multiple recurrences of pericarditis suggest the existence of a possible genetic background in at least 10% of cases. The aim of the present study is to describe the genetic landscape of a cohort of patients with multiple recurrences (at least two recurrences).
Methods: Retrospective cohort study of consecutive adult patients referred for at least two episodes of recurrences in a tertiary referral centre.
Background: Current guidelines for the diagnosis and treatment of pericarditis refer to the general adult population. Few and fragmentary data regarding recurrent pericarditis in older adults exist.
Objective Of The Study: Given the absence of specific data in scientific literature, we hypothesized that there might be clinical, laboratory and outcome differences between young adults and older adults affected by idiopathic recurrent pericarditis.
Importance: Pericarditis accounts for up to 5% of emergency department visits for nonischemic chest pain in North America and Western Europe. With appropriate treatment, 70% to 85% of these patients have a benign course. In acute pericarditis, the development of constrictive pericarditis (<0.
View Article and Find Full Text PDFThe authors present a case report of a 68-year-old man evaluated at the emergency department for repeated syncope, asthenia, and general malaise, suggesting heart failure in a patient with several comorbidities. At presentation, the patient was afebrile, but he had reported a low-grade fever in the previous six months. At first glance, transthoracic echocardiography was not clear, while transesophageal echocardiography revealed an echo-free image at the level of the non-coronary sinus of the aortic root, suggestive of a pseudoaneurysm, communicating with the right atrium with continuous systo-diastolic flow, compatible with the aorto-cavitary fistula between the aortic root and the RA.
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