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Background: Arrhythmogenic cardiomyopathy (ACM) is an inherited cardiomyopathy characterized by high risks of sustained ventricular tachycardia (sVT) and sudden cardiac death. Identifying patients with high risk of sVT is crucial for the management of ACM.

Methods: A total of 147 ACM patients were retrospectively enrolled in the observational study and divided into training and validation groups.

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  • - Diagnosing cardiac sarcoidosis (CS) is difficult, often leading to heart transplantation (HT) as the last option, especially when immunosuppressive therapies fail in advanced cases.
  • - The case study presents four patients, each with different symptoms and backgrounds, who ultimately required HT for their CS, revealing the condition's complexity and urgency.
  • - There are significant mid- and long-term challenges following HT for CS, notably the need for careful management of immunosuppression to prevent rejection and recurrence of sarcoidosis.
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  • Autoantibodies against Desmoglein-2 (anti-DSG2-ab) were found in patients with Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) and myocarditis, indicating a potential link to immune responses against desmosomal proteins.
  • The study aimed to evaluate the specificity of anti-DSG2-ab in ARVC, compare detection methods (ELISA vs. IFL), and identify clinical correlates related to these antibodies among various patient groups.
  • In a cohort of patients, 56% of those with ARVC tested positive for anti-DSG2-ab, showing a higher rate of positivity in those also positive for anti-intercalated disk autoantibodies (A
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Epilepsy and Cardiac Arrhythmias: A State-of-the-Art Review.

JACC Clin Electrophysiol

November 2024

Department of Cardiology, St Vincent's Hospital Melbourne, Fitzroy, VIC, Australia; Faculty of Medicine, Dentistry & Health Sciences, The University of Melbourne, Fitzroy, VIC, Australia; HEART Lab, St Vincent's Institute of Medical Research, Fitzroy, VIC, Australia; HEART Lab, Victor Chang Cardiac Research Institute, Darlinghurst, NSW, Australia. Electronic address: https://twitter.com/pretzeldr.

Epilepsy is an important cause of disability and mortality worldwide. It can be frequently misdiagnosed, and detailed history and relevant investigations are needed to differentiate epilepsy from syncope. Electroencephalogram is a key noninvasive assessment of neurological function, and the diagnostic yield is increased when performed for an extended period in the ambulatory setting with concurrent electrocardiogram and video monitoring.

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Long-term prognosis of patients with an SCN5A loss-of-function variant and progressive cardiac conduction disorder or Brugada syndrome.

Heart Rhythm

November 2024

Department of Clinical Cardiology, Heart Center, Amsterdam University Medical Centers, University of Amsterdam, Amsterdam, The Netherlands; European Reference Network for rare, low prevalence, and complex diseases of the heart (ERN GUARD-Heart), Amsterdam, The Netherlands. Electronic address:

Background: The long-term prognosis of patients with a loss-of-function variant in the cardiac sodium channel gene SCN5A is unknown.

Objective: This study aimed to evaluate the long-term arrhythmic risk in patients with an SCN5A loss-of-function variant to identify predictors of arrhythmic events.

Methods: Probands and family members with (likely) pathogenic SCN5A loss-of-function variants were retrospectively included.

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