Atrial fibrillation as a clinical characteristic of arrhythmogenic right ventricular cardiomyopathy: Experience from the Nordic ARVC Registry

Abstract

Background: Recent studies in arrhythmogenic right ventricular cardiomyopathy (ARVC) patients have drawn attention to atrial fibrillation (AF) as an arrhythmic manifestation of ARVC and as an indicator of atrial involvement in the disease progression. We aimed to assess the prevalence of AF in the Scandinavian cohort of ARVC patients and to evaluate its association with disease clinical manifestations. Methods: Study sample comprised of 293 definite ARVC patients by 2010 Task Force criteria (TFC2010) and 141 genotype-positive family members (total n = 434, 43% females, median age at ARVC diagnosis 41 years [interquartile range (IQR) 28-52 years]). ARVC diagnostic score was calculated as the sum of major (2 points) and minor (1 point) criteria in all categories of the TFC2010. Results: AF was diagnosed in 42 patients (10%): in 41 patients with definite ARVC diagnosis (14%) vs in one genotype-positive family member (1%), p b 0.001. The median age at AF onset was 51 (IQR 38-58) years. The prevalence of AF was related to the ARVC diagnostic score: it significantly increased starting with the diagnostic score 4 (2% in those with score 3 vs 13% in those with score 4, p = 0.023) and increased further with increased diagnostic score (Somer's d value is 0.074, p b 0.001). Conclusion: AF is seen in 14% of definite ARVC patients and is related to the severity of disease phenotype thus suggesting AF being an arrhythmic manifestation of this cardiomyopathy indicating atrial myocardial involvement in the disease progression.

Spectrum of desmosomal gene variations in patients with arrhythmogenic right ventricular cardiomyopathy

“…Но только у 2 (2,5% обследованной когорты) пробандов с вероятным диагнозом АКПЖ в результате ДНК-диагностики нами были выявлены варианты IV-V класса патогенности, и удалось поднять статус диагноза до достоверного. Данные нашего исследования показывают, что наибольшая выявляемость мутаций наблюдается у пробандов, имеющих развёрнутую клиническую [24]. В нашей группе больных мутации были обнаружены в генах PKP2, DSG2 и DSP.…”

Efficacy of Catheter Ablation for Atrial Arrhythmias in Patients with Arrhythmogenic Right Ventricular Cardiomyopathy—A Multicenter Study

“…Up to 15-20% of ARVC patients enrolled in large and well-characterized cohorts were reported to suffer from episodes of atrial arrhythmias, with AF being the most common one [3,7,8]. Atrial arrhythmias in these patients may develop as part of a primary pathogenic process (atrial cardiomyopathy) due to desmosomal/connexome impairment in the atria or as a consequence of atrial dilation following depressed ventricular function and dilation.…”

“…The atria of patients with ARVC have been shown to be dilated and their function impaired, with the right atrium (RA) being predominantly affected by this process [3][4][5]. Considering electrocardiographic features, Platonov PG et al first described an altered electrical conduction within the atria of patients with ARVC [6], and subsequent cohort studies reported a high rate of atrial arrhythmias in ARVC [3,7,8]. Particularly, atrial fibrillation (AF) and atrial flutter (AFl) were shown to be more frequent and presented at a younger age in patients with ARVC as compared to the general population [7,9].…”

A meta-analysis of ECG abnormalities (arrhythmia) in cardiomyopathies

“…AF is an arrhythmic manifestation of ARVC and an indicator of atrial involvement in disease progression. The prevalence correlates with ARVC diagnostic score -considerably increasing with a diagnostic score of 4 and increases further with increased diagnostic score [61].…”

“…The involvement of the LV leading to systolic dysfunction may also be associated with complications such as AF and thromboembolic events [62]. The development of atrial myocardial substrate in ARVC may be influenced by genetically determined desmosomal dysfunction [61]. AF may also result from the same mechanisms responsible for fibrotic replacement of the myocardium in the RV walls and occurs in parallel with the involvement of the ventricular myocardium [61,62].…”