Associazione Nazionale Medici Cardiologi Ospedalieri

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CONGRESS ABSTRACT

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LONG-TERM CLINICAL OUTCOME OF NEONATAL ONSET HYPERTROPHIC CARDIOMYOPATHY ASSOCIATED TO NOONAN SYNDROME AND MITOCHONDRIAL DISEASES
Anno:
2025
BACKGROUND: Hypertrophic Cardiomyopathy (HCM) presenting during the neonatal period has different etiologies, risk factors, and clinical course comparing to HCM with onset during childhood, adolescence or adult age. Noonan and mitochondrial disease have the worst prognosis among all neonatal HCM. PURPOSE: We aim to describe the clinical course and…
Steroid-Induced Transient Obstructive Hypertrophic Cardiomyopathy in a six months old boy
Anno:
2025
  Background Steroid-induced hypertrophic cardiomyopathy is a rare complication in children undergoing prolonged corticosteroid therapy. Although typically reported in preterm neonates, this case in a six-month-old infant without pre-existing cardiac conditions broadens current understanding of this condition, highlighting the importance of thorough diagnostic and therapeutic approaches.    Case Description…
Beyond cardiomyopathy: a case of coronary artery disease in Becker Muscular Dystrophy
Anno:
2025
INTRODUCTION Becker Muscular Dystrophy (BMD) is a genetic disorder, caused by mutations in the dystrophin gene. Cardiac involvement is a common feature in this condition, often manifesting as dilated cardiomyopathy (DCM) or arrhythmias. In patients with BMD, ventricular dysfunction is frequently attributed to non-ischemic mechanisms, given the known pathophysiology…
Incidental cardiac amyloidosis with an arrhythmic presentation in an asymptomatic athlete
Anno:
2025
Background: Cardiac amyloidosis (CA) is a progressive infiltrative cardiomyopathy commonly linked to heart failure (HF) symptoms. Ventricular arrhythmias (VA) may occur in a small subset of patients, typically in advanced stages when amyloid deposits compromise myocardial and conduction system integrity. Managing VA in asymptomatic patients with CA remains complex…
IMPACT OF BASELINE HEART FAILURE SEVERITY ON EFFICACY OF VUTRISIRAN IN PATIENTS WITH TRANSTHYRETIN AMYLOIDOSIS WITH CARDIOMYOPATHY IN THE HELIOS-B TRIAL: A SUBGROUP ANALYSIS
Anno:
2025
Background: In the phase 3 HELIOS-B trial (NCT04153149), vutrisiran reduced the risk of all-cause mortality (ACM) and recurrent cardiovascular (CV) events, and improved functional capacity and quality of life (QoL) vs placebo in patients with transthyretin amyloidosis with cardiomyopathy (ATTR-CM). Methods: This subgroup analysis assessed the effect of vutrisiran…
Compassionate use program with mavacamten in patients with hypertrophic obstructive cardiomyopathy: first insights from a single-centre real world experience
Anno:
2025
Introduction: Mavacamten, a first-in-class cardiac myosin ATPase inhibitor used as second-line therapy for hypertrophic obstructive cardiomyopathy (HOCM),  became available in Italy in early 2024 under a compassionate use program.  Aim: To describe early experience of compassionate use of Mavacamten in a single centre HOCM population.  Methods: All patients had…
Impact of genetic test results on clinical and instrumental characteristics in patients with hypertrophic cardiomyopathy: a single-centre retrospective analysis
Anno:
2025
Introduction Genetic testing identifies a pathogenic variant, typically involving sarcomeric genes, in about 40-60% of patients with Hypertrophic Cardiomyopathy (HCM). When no pathogenic variant is found, clinical presentation may differ from that of genetic-positive individuals. The aim of this study was to investigate clinical and demographic characteristics of HCM patients…
TRANSIENT MOTOR APHASIA AND LEFT ENDOVENTRICULAR THROMBOSIS IN DUCHENNE MUSCULAR DYSTROPHY – DILATED CARDIOMYOPATHY
Anno:
2025
Duchenne muscular dystrophy (DMD) is a progressive myopathic disorder caused by a recessive mutation in the dystrophin gene on the X chromosome, that result in progressive skeletal muscle weakness as the main clinical manifestation. However, cardiac muscle is also affected. Dilated cardiomyopathy (DCM), arrhythmias, and congestive heart failure (HF)…
Cardiac Amyloidosis Awareness among General Practitioners
Anno:
2025
Background: The incidence of cardiac amyloidosis (CA) is constantly rising. Studies on CA awareness among cardiologists, internal medicine specialists, and geriatricians have identified various gaps in physicians' knowledge. General practitioners (GPs) are often gatekeepers who may generate a suspicion and influence time to diagnosis. However, little is known about…
KAWASAKI DISEASE AND OVERLAP WITH MACROPHAGE ACTIVATION SYNDROME PRESENTING AS MYOCARDITIS IN A TEENAGER
Anno:
2025
This report presents a case of a teenager with Kawasaki disease (KD) complicated by macrophage activation syndrome (MAS), an overlap condition presenting initially as myocarditis. The patient arrived with persistent fever, rash, and conjunctivitis, along with cardiac dysfunction marked by reduced ejection fraction on echocardiography and elevated cardiac laboratory markers…