We report a case of arrhythmogenic right ventricular cardiomyopathy revealed by sudden death during exercise in a 13-year-old patient. Postmortem diagnosis was made on multiple tissue samples taken from right ventricular free wall, showing light adipous infiltration of the myocardium at gross examination. Arrhythmogenic right ventricular cardiomyopathy is histologically characterized by fibro-fatty replacement of right ventricular myocardium. Left ventricular involvement may be observed. Diagnosis at an early stage is often difficult. Etiology remains unknown. Since familial occurrence has been documented, postmortem identification is useful for the other members of the family.
Download full-text PDF |
Source |
|---|
Publication Analysis
Top Keywords
Similar Publications
Atypical Left Ventricular Remodeling in Fabry Cardiomyopathy: The Key Diagnostic Role of T1 Mapping.
Circ Cardiovasc Imaging
January 2025
Multimodality Cardiac Imaging Section, IRCCS Policlinico San Donato, Milan, Italy (L.T., G.D., M.L., A.C.).
Naxos disease is a rare autosomal recessive condition combining arrhythmogenic right ventricular cardiomyopathy, woolly hair, and palmoplantar keratoderma. The first identified causative variant was in the gene encoding the desmosomal protein plakoglobin. Naxos disease exhibits fibro-fatty myocardial replacement with immunohistological abnormalities in cardiac protein and signaling pathways, highlighting the role of inflammation and potential anti-inflammatory treatments.
View Article and Find Full Text PDFArrhythmogenic right ventricular cardiomyopathy (ARVC) is a heritable myocardial condition that mostly affects the right ventricle (RV). Atrial involvement is poorly understood and the evidence for atrial involvement remains limited. In this case report, we describe an 18-year-old woman who had ARVC with the atrial tachyarrhythmia and initially presented with palpitations.
View Article and Find Full Text PDFLeft Atrial Markers in Diagnosing and Prognosticating Non-Ischemic Cardiomyopathies: Ready for Prime Time?
Echocardiography
February 2025
Department of Cardiology, Loyola University Medical Center, Maywood, Illinois, USA.
The left atrium (LA) is pivotal in cardiac hemodynamics, serving as a dynamic indicator of left ventricular (LV) compliance and diastolic function. The LA undergoes structural and functional adaptations in response to hemodynamic stress, infiltrative processes, myocardial injury, and arrhythmic triggers. Remodeling of the LA in response to these stressors directly impacts pulmonary circulation, eventually leading to pulmonary capillary involvement, pulmonary artery hypertension, and eventually right ventricular failure.
View Article and Find Full Text PDFAnthracycline-induced cardiomyopathy: risk prediction, prevention and treatment.
Nat Rev Cardiol
January 2025
Cardioncology Unit, Cardioncology and Second Opinion Division, European Institute of Oncology, Istituto di Ricovero e Cura a Carattere Scientifico, Milan, Italy.
Anthracyclines are the cornerstone of treatment for many malignancies. However, anthracycline cardiotoxicity is a considerable concern given that it can compromise the clinical effectiveness of the treatment and patient survival despite early discontinuation of therapy or dose reduction. Patients with cancer receiving anthracycline treatment can have a reduction in their quality of life and likelihood of survival due to cardiotoxicity, irrespective of their oncological prognosis.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!