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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 PDFDiagnostic Utility of Relative Apical Sparing Index in Cardiac Amyloidosis Subtypes: A Comparative Study of Immunoglobulin Light Chain and Transthyretin Amyloid Cardiomyopathy.
Echocardiography
February 2025
Cardiovascular Department, Tokushima University Hospital, Tokushima, Japan.
Background: Speckles tracking echocardiography imaging enables clinicians to detect subtle systolic dysfunction. The aim of the present study was to elucidate the differences in speckle tracking echocardiographic findings between immunoglobulin light chain amyloid cardiomyopathy (AL-CM) and transthyretin amyloid cardiomyopathy (TTR-CM).
Methods: The patients with a confirmed diagnosis of cardiac amyloidosis through cardiac biopsy from March 2013 to October 2022 were included.
Multisociety endorsement of the 2024 European guideline recommendations on coronary revascularization.
Braz J Cardiovasc Surg
January 2025
Coronary Center, Department of Thoracic and Cardiovascular Surgery, Miller Family Heart, Vascular, & Thoracic Institute, Cleveland Clinic, Cleveland, Ohio, United States.
Patient Preferences in Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) Screening and ICD Implantation: Canadian ARVC Registry Substudy.
CJC Open
January 2025
Population Health Research Institute, Hamilton, Ontario, Canada.
Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is typically diagnosed following an arrhythmic event or during screening after a family member experiences sudden cardiac death. Implantation of a defibrillator (ICD) improves survival but can be associated with morbidity and risks, an important consideration within a shared decision-making context. This study examined patient decisional needs and preferences surrounding ARVC screening and prophylactic ICD implantation.
View Article and Find Full Text PDFPioneering Use of Extracorporeal Life Support in Intraoperative Cardiac Arrest in Dubai: A Case Report.
Cureus
December 2024
Department of Anaesthesia and Intensive Care, Latifa Hospital, Dubai Health, Dubai, ARE.
We describe, to our knowledge, the first use in Dubai of extracorporeal life support (ECLS) in a patient who suffered intraoperative cardiac arrest due to presumed cardiac channelopathy. A 40-year-old patient presented for open myomectomy surgery. She had no other medical problems apart from obesity.
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