ArticleCirculation. Arrhythmia and electrophysiology2025
Early Atrial Remodeling Drives Arrhythmia in Fabry Disease.
Article in Circulation. Arrhythmia and electrophysiology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 7 papers, 1 of them a synthesis that pooled it.
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Who cites it
7 citing papers in PubMed, 1 synthesis or guideline pooled it.
- Fabry disease cardiomyopathy: A state-of-the-art review.Progress in cardiovascular diseasesPooled it
- Progressive conduction system disease preceding hypertrophy in IVS4+919G>A Fabry variant: a population-based electrocardiographic study.Europace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology · 2026Article
- A titin truncating variant linked to atrial fibrillation increases atrial profibrotic signalling and cholinergic sensitivity.Cardiovascular research · 2026Article
- Visinin-like protein 1 disrupts calcium homeostasis and promotes atrial fibrillation in human and rodent models.Signal transduction and targeted therapy · 2026Article
- The Predictive Value of the Pan-Immune-Inflammation Value for Atrial Fibrillation Risk in Patients with Coronary Artery Disease: A Multicenter Machine Learning Study.International journal of general medicine · 2026Article
- Arrhythmias and clinical outcomes in Fabry disease with cardiac and renal involvement.Orphanet journal of rare diseases · 2025Article
- Anderson-Fabry Disease: An Overview of Current Diagnosis, Arrhythmic Risk Stratification, and Therapeutic Strategies.Diagnostics (Basel, Switzerland) · 2025Review
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Authors and funding
21 authors.
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Abstract
backgroundFabry disease (FD) is an X-linked lysosomal storage disorder caused by α-Gal A (α-galactosidase A) deficiency, resulting in multiorgan accumulation of sphingolipid, namely globotriaosylceramide. This triggers ventricular myocardial hypertrophy, fibrosis, and inflammation, driving arrhythmia and sudden death. Atrial fibrillation is common, yet the cellular mechanisms accounting for this are unknown.
methodsTo address this, we conducted ECG analysis from a large cohort of 115 adults with FD at varying cardiomyopathy stages. ECG P-wave characteristics were compared with non-FD controls. Cellular contractile and electrophysiological function were examined in a novel atrial cellular FD model developed and imputed into in silico atrial models to provide insight into mechanisms of arrhythmia. Induced pluripotent stem cells were genome-edited using Clustered Regularly Interspaced Short Palindromic Repeats-Cas9 to introduce the
resultsECG analysis demonstrated P-wave duration and PQ interval shortening in FD adults before the onset of cardiomyopathy. Patients with FD exhibited a higher incidence of premature atrial contractions and increased risk of atrial fibrillation compared with healthy controls.
conclusionsThese findings provide novel insights into underpinning mechanisms for atrial arrhythmia and a rationale for early P-wave changes in FD. These may be targeted to develop therapeutic strategies to reduce the arrhythmic burden in FD.
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