Key result
Primary and secondary atrial cardiomyopathy, driven by genetic mutations such as NPPA and SCN1B, lead to significant structural remodeling and electromechanical dysfunction of the atria.
Why the study?
Atrial cardiomyopathy is a relatively common but clinically underestimated disorder whose concept was revised in 2016 by the EHRA working group to include secondary atrial remodeling.
Case Report (n=2)
The review highlights the clinical importance of the atrial cardiomyopathy concept, emphasizing its role as an independent risk factor for thromboembolic complications regardless of atrial fibrillation.
Answers are grounded in this paper and cite their sources.
May guide genetic testing in early-onset AF; hypothesis-generating and requires validation before clinical use.
Atrial cardiomyopathy (ACM) is a relatively common but clinically underestimated disorder, which is characterized by an increased atrial size and dysfunction. Previously, ACM was considered a primary disorder, but in 2016 this concept was revised by European Heart Rhythm Association (EHRA) working group with inclusion of secondary atrial remodeling. The EHRA document details aspects of atrial anatomy and pathophysiology, proposes definitions of ACM, histological classification, outlines the molecular mechanisms of atrial arrhythmia and the problems of personalized treatment and optimization of indications for catheter ablation.Practical application of the proposed ACM classification system, the clinical significance of novel ACM concept and the potential role of this information for a practitioner are presented in this article. Two clinical cases of ACM with “primary” (familial form of ACM due to NPPA gene mutation with primary defect in atrial structure and function) and “secondary” atrial remodeling (ACM caused by a longterm supraventricular tachyarrhythmias due to SCN1B gene mutation).
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Vaikhanskaya et al. (2020) conducted a case report in Atrial cardiomyopathy (n=2). Genetic mutations (NPPA and SCN1B) was evaluated. Primary and secondary atrial cardiomyopathy, driven by genetic mutations such as NPPA and SCN1B, lead to significant structural remodeling and electromechanical dysfunction of the atria.
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