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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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Recently, semi-synthetic cannabinoids have entered the illegal market and are produced to mimic the psychoactive effects of tetrahydrocannabinol (THC). This is a case report of a 19-year-old man, who was hospitalized due to severe sedation, hypotension and tachy- and bradycardia after ingestion of the semi-synthetic cannabinoids hexahydrocannabinol (HHC) and hexahydrocannabiphorol (HHC-P) mixed in food. HHC-P, HHC and metabolites were identified in blood samples.

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Implantable cardiac devices are a vital treatment option in the management of tachy/brady-arrhythmias and heart failure with conduction disease. In the recent years, these devices have become increasingly sophisticated, with high implantation success rates and longevity. However, these devices are not without risks and complications, which need to be carefully considered before implantation.

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The intricate relationship between sports participation and cardiac arrhythmias is a key focus of cardiovascular research. Physical activity, integral to preventing atherosclerotic cardiovascular disease, induces structural, functional, and electrical changes in the heart, potentially triggering arrhythmias, particularly atrial fibrillation (AF). Despite the cardiovascular benefits, the optimal exercise amount remains unclear, revealing a J-shaped association between AF and exercise.

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Article Synopsis
  • * The patient's laryngeal cancer caused lymphedema that compressed the carotid arteries, leading to symptoms like hypotension and bradycardia, which were managed with atropine and a pacemaker, although he eventually continued treatment with non-invasive theophylline.
  • * The report highlights the rarity of CSS in head and neck malignancies and stresses the need for further research to improve understanding and treatment options for managing CSS in these patients, as current evidence is limited.
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