Information on atrial arrhythmia associated with right ventricular cardiomyopathy/dysplasia (ARVC/D) is limited. In 36 patients with task force criteria for ARVC/D and history of ventricular tachycardia (VT), we confirmed the incidence and type of atrial arrhythmia, onset related to referral for VT ablation, fastest documented ventricular rate, management, and clinical and hemodynamic factors associated with their development. Thirty-six patients (28 men) had a mean age of 47 years (range 17 to 80) and mean follow-up of 56 +/- 44 months. Thirty-five patients (97%) had implantable cardioverter-defibrillator (ICD) devices, 15 with atrial leads. Fifteen of 36 patients (42%) had documented atrial arrhythmias, with atrial flutter (aFL) in 11, atrial fibrillation (AF) in 11 patients, and aFL and AF in 7 patients. Maximum heart rate noted with atrial arrhythmia was 62 to 150 beats/min. In 9 patients, initial atrial arrhythmia preceded or was concurrent with presentation for VT ablation. In the remaining 6 patients, atrial arrhythmia (symptomatic in 4 patients) followed VT presentation. Three of these patients received ICD shock therapy for atrial arrhythmias. Seven of 11 patients with recurrent aFL required aFL ablation, 1 patient underwent His-bundle ablation for AF with rapid rate, and 8 patients required long-term drug therapy for AF control. Atrial arrhythmias were more common in patients with RV enlargement and moderate/severe tricuspid regurgitation. In conclusion, in patients with ARVC/D and VT, atrial arrhythmias are common, frequently necessitate ablative or pharmacologic treatment, and are more common in patients with moderate/severe tricuspid regurgitation and markedly enlarged right ventricle.
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