Cardiac amyloidosis (CA) manifests as infiltrative cardiomyopathy with a hypertrophic pattern, usually presenting with heart failure with a preserved ejection fraction. In addition, degenerative valvular heart disease, particularly severe aortic stenosis, is commonly seen in patients with CA. However, amyloid fibril deposition might also infiltrate the conduction system and promote the development of electrical disorders, including ventricular tachyarrhythmias, atrio-ventricular block or acute electromechanical dissociation. These manifestations can increase the risk of sudden cardiac death. This review summarises the pathophysiological mechanisms and risk factors for sudden cardiac death in CA and focuses on the major current concerns regarding medical and device management in this challenging scenario.
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http://dx.doi.org/10.31083/j.rcm2310345 | DOI Listing |
Eur J Prev Cardiol
December 2024
Cardiovascular and Genomics Institute, City St George's University of London, Cranmer Terrace, London SW17 0RE, UK.
Aims: Sudden arrhythmic death syndrome (SADS) refers to a sudden death, which remains unexplained despite comprehensive post-mortem examination and a toxicological screen. We aimed to investigate the impact of age and sex on the overall diagnostic yield and underlying aetiology in decedents with SADS using a combined approach of familial evaluation (FE) and molecular autopsy (MA).
Methods And Results: Consecutive referrals to a single centre for FE only, MA only or both, following a SADS death were included.
Int J Cardiol Congenit Heart Dis
March 2024
Department of Cardiovascular Diseases, Mayo Clinic, Jacksonville, FL, USA.
Aims: Patients with D-transposition of the great arteries (D-TGA) and atrial switch experience late morbidity and mortality related to atrial arrhythmias and systemic right ventricular (SRV) failure. We sought to analyze the influence of atrial arrhythmias on long-term outcomes in this group.
Methods: A retrospective review of all patients with D-TGA and atrial switch followed at a tertiary care center was performed.
Glob Heart
December 2024
Instituto do Coração, Faculdade de Medicina Universidade de São Paulo, São Paulo, Brazil.
Background: Sudden cardiac death (SCD) is a major concern in patients with hypertrophic cardiomyopathy (HCM). The American College of Cardiology/American Heart Association (ACC/AHA) and the European Society of Cardiology (ESC) have different guidelines for SCD risk stratification. Their comparative performance in diverse populations remains uncertain.
View Article and Find Full Text PDFCureus
November 2024
Department of Cardiovascular Surgery, Tokyo Metropolitan Bokutoh Hospital, Tokyo, JPN.
Trauma to the ascending aorta may progress to a cardiac tamponade, which is often life-threatening. Here, we report on a case of traumatic dissection of the ascending aorta, complicated by multiple injuries. A 24-hour follow-up period was provided to evaluate the traumatic bleeding, and a large tear that extended over three-quarters of the circumference of the aortic intima was identified.
View Article and Find Full Text PDFInt J Cardiol Congenit Heart Dis
September 2024
First Cardiology Department, AHEPA University Hospital, School of Medicine, Faculty of Health Sciences, Aristotle University of Thessaloniki, Thessaloniki, Greece.
Pulmonary arterial Hypertension (PAH) is a progressive disease marked by significant morbidity and mortality due to pulmonary vasculopathy and right ventricular (RV) dysfunction. Despite advances in PAH medical therapies which have improved clinical outcomes and survival, patients continue to face severe complications, including a notable incidence of sudden cardiac death (SCD). The high arrhythmic burden, coupled with mechanical complications such as left main compression syndrome, pulmonary artery dissection, rupture, and severe hemoptysis, significantly contribute to the risk of SCD.
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