Ebstein's anomaly (EA) is a rare congenital heart disease characterized by "atrialization" of the right ventricle, due to apical displacement of the tricuspid leaflets into the right ventricle. Patients with EA may develop all kinds of supraventricular arrhythmias requiring radiofrequency ablation. Atrial fibrillation (Afib) is a common arrhythmia in EA patients, and results in debilitating symptoms that often require surgical treatment. This is a follow-up report of 2 patients with EA undergoing radiofrequency ablation for Afib. The first patient underwent pulmonary vein isolation (PVI) and the ablation of a concomitant atrioventricular nodal reentrant tachycardia. The second patient was also treated with a PVI and a redo PVI 8 months later. Both patients remain in sinus rhythm 8 months on. Radiofrequency ablation is the therapy of choice for patients with pharmacological refractory Afib, but it is not common in patients with EA.

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http://dx.doi.org/10.1159/000484039DOI Listing

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