Why the study?
Up to 15% of AF occurs without related risk factors, and the prevalence of pathogenic variants and structural cardiac abnormalities in early-onset AF without known risk factors needed to be determined.
What is the prevalence of pathogenic genetic variants and structural cardiac abnormalities in patients with early-onset atrial fibrillation without traditional risk factors?
What is the prevalence of pathogenic genetic variants and structural cardiac abnormalities in patients with early-onset atrial fibrillation without traditional risk factors?
Early-onset atrial fibrillation without traditional risk factors is frequently associated with pathogenic variants in cardiomyopathy-related genes, particularly TTN, which may predispose patients to ventricular dilation.
Answers are grounded in this paper and cite their sources.
May support cardiomyopathy-gene testing plus imaging follow-up in unexplained early-onset AF; leaves open routine use given this small observational series.
BACKGROUND: Atrial fibrillation (AF) is the most common arrhythmia and typically occurs in elderly patients with other cardiovascular and extracardiac diseases. However, up to 15% of AF develops without any related risk factors. Recently, the role of genetic factors has been highlighted in this particular form of AF. AIMS: The aims of this study were to determine the prevalence of pathogenic variants in early-onset AF in patients without known disease-related risk factors and to identify any structural cardiac abnormalities in these patients. MATERIALS AND METHODS: We conducted exome sequencing and interpretation in 54 risk factor-free early-onset AF patients and further validated our findings in a similar AF patient cohort from the UK Biobank. RESULTS: Pathogenic/likely pathogenic variants were found in 13/54 (24%) patients. The variants were identified in cardiomyopathy-related and not arrhythmia-related genes. The majority of the identified variants were TTN gene truncating variants (TTNtvs) (9/13 (69%) patients). We also observed two TTNtvs founder variants in the analysed population-c.13696C>T p.(Gln4566Ter) and c.82240C>T p.(Arg27414Ter). Pathogenic/likely pathogenic variants were found in 9/107 (8%) individuals from an independent similar AF patient cohort from the UK Biobank. In correspondence with our Latvian patients, only variants in cardiomyopathy-associated genes were identified. In five (38%) of the thirteen Latvian patients with pathogenic/likely pathogenic variants, dilation of one or both ventricles was identified on a follow-up cardiac magnetic resonance scan. CONCLUSIONS: We observed a high prevalence of pathogenic/likely pathogenic variants in cardiomyopathy-associated genes in patients with risk factor-free early-onset AF. Moreover, our follow-up imaging data indicate that these types of patients are at risk of developing ventricular dilation. Furthermore, we identified two TTNtvs founder variants in our Latvian study population.
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Rudaka et al. (2023) studied this question.
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